You are on page 1of 46

4

Special Considerations for Special Populations

CHAPTER OBJECTIVES
Readers of this chapter will be able to: 4. Highlight the language and communication impairments
1. Describe the similarities and differences between the associated with psychiatric disorders of complex genetic
speech, language, and communication profiles of chil- origin.
dren with disorders of known genetic origin and more 5. Explain the role of extreme environmental disadvantage
primary developmental language disorders (DLDs). in language and communication impairment.
2. Discuss language disorders and differences associated 6. Consider the particular challenges that arise when assess-
with sensory impairments. ing language and communication in the nonverbal child.
3. Describe the ways in which acquired language disorders 7. Discuss the relationship between social, cognitive, and
in children differ from congenital DLDs. emotional factors in language development and disorder.

Our perspective in this book is that it is more important in but is likely to contribute to multidisciplinary team assessments
clinical practice to describe the nature of a child’s language that gather evidence of a child’s speech, language, communica-
disorder than to get to the root cause of the problem. We dis- tion, and literacy needs. In the case of a child with ASD, for ex-
cussed earlier how the diagnostic category in which a child is ample, the SLP may be required to document the social commu-
placed may not always either explain or predict language and nication difficulties that the child displays, which are part of the
communication behavior. We know there is considerable vari- core symptoms of this condition. To fulfill this role, it is impor-
ability within a single diagnostic category; sometimes the differ- tant to know the standard definitions of each disorder. That’s one
ences between children with the same “diagnosis” are as striking reason we discuss diagnostic criteria in this chapter.
as the similarities. We’ve also talked about the fact that many Second, although the etiological classification associated
children don’t fit very neatly into one diagnostic classification. with developmental language disorder (DLD) does not dictate
The causal model we outlined in Chapter 1 (see Fig. 1.2) gives the assessment and intervention strategies appropriate for each
us a clue as to why this is so: several genetic, environmental, and child, knowing the classification often provides hints about
cognitive risk factors are common across disorders, increasing what areas to look at in the assessment or what areas might
the chances that children may have symptoms of different dis- receive priority in intervention. For example, if we know that a
orders at the same time. For example, many children with intel- child has ASD, we can make an informed guess that pragmatic
lectual disability (ID) have characteristics of autism spectrum aspects of language will be impaired, among other things. This
disorder (ASD), and many children with ASD have additional could suggest that we include a detailed pragmatic evaluation
intellectual impairments. Finally, we’ve said that knowing a in our assessment plan. In addition to the key aspects of lan-
child’s diagnostic label often doesn’t precisely indicate a specific guage likely to be compromised in a given disorder, many dis-
child’s assessment or intervention needs. Knowing that a non- orders have associated cognitive or perceptual impairments
verbal child has ASD, for example, does not automatically pre- that influence the course of language development. Knowing
scribe the program. Should he or she be given intervention in what these are and how they impact children’s language can
the speech modality, or should an alternative modality such as help us devise interventions, or modify the child’s environ-
sign language be introduced? This decision is not very different ment in a way that minimizes the negative effects of associated
from the decision that must be made in the case of a nonspeak- deficits. Remember, however, that although characteristics may
ing child who has a hearing impairment or a severe motor be typical of a particular diagnostic group, they are by no
speech disorder. means inevitable or universal. Not all children with ASD echo
Is there any reason then, to use diagnostic labels in clinical language, for example, although echolalia is a typical symptom
practice? Although diagnostic category may not be the primary of this disorder. Diagnostic categories provide signposts for
determiner of clinical decisions in language disorders, knowledge assessment and intervention; these must be followed through
of different diagnostic groups can be useful in a number of ways. with a detailed description of the individual’s actual needs and
First, a diagnostic label may be necessary to secure access to abilities.
speech-language pathologist (SLP) services and educational sup- Third, the clinical reports and medical histories of clients
port. The SLP alone is not usually responsible for the diagnosis often contain information about the diagnostic categories. To
102
CHAPTER 4  Special Considerations for Special Populations 103

decisions. Let’s look first at the standard definition of ID


and caveats associated with that diagnosis. We’ll then con-
sider some of the typical (but, of course, by no means univer-
sal) relationships between language and communication
skills and level of nonverbal cognitive ability. Finally, we’ll
consider the language profiles that accompany ID in the con-
text of known genetic etiology. The most important thing
to remember is that, even if we know a child’s intelligence
quotient (IQ) score and the genes that play a causal role in ID,
we cannot accurately predict what the child’s language profile
will be or how it will change over time. Careful assessment
of form, content, use, and literacy development is therefore
needed.

Definition and Classification


Language disorders can be associated with a variety of The American Association on Intellectual and Developmen-
congenital conditions. tal Disabilities (AAIDD, www.aaidd.org) provides the follow-
ing definition of ID (Schalock et al., 2010):
“Intellectual disability is a disability characterized by
understand these documents fully, we need to understand
significant limitations both in intellectual functioning
what the labels mean. In this chapter, we look at some of the
and in adaptive behavior, which covers many everyday
major developmental disorders that are often associated with
social and practical skills. This disability originates before
language impairment. We outline standard definitions of
the age of 18.”
each of these conditions, then talk about the typical cognitive,
linguistic, and literacy characteristics of each disorder, and In addition, the Diagnostic and Statistical Manual Mental
the implications of these learning challenges for clinical prac- Disorders, Fifth Edition (DSM-5) (American Psychiatric As-
tice in speech-language pathology. sociation, 2013) diagnosis requires that:
1. Deficits in intellectual functions, such as reasoning,
INTELLECTUAL DISABILITY problem-solving, planning, abstract thinking, judg-
ment, academic learning and learning from experience,
CASE STUDY and practical understanding confirmed by both clinical
assessment and individualized, standardized intelli-
Meredith had been a placid baby. She sat up late, not until
gence testing.
9 months old. She didn’t walk until she was 25 months
2. Deficits in adaptive functioning that result in failure to
old, and she hardly talked at all before her third birthday. Her
parents expressed some concern to their family doctor,
meet developmental and sociocultural standards for per-
who referred Meredith for an evaluation when she was 31⁄2. sonal independence and social responsibility. Without
Meredith’s cognitive skills were found to be quite delayed. ongoing support, the adaptive deficits limit functioning in
Her parents were interviewed about her self-help skills, and one or more activities of daily life, such as communica-
they reported that she did not have independent feeding and tion, social participation, and independent living, and
wasn’t yet toilet trained. Her parents said Meredith acted across multiple environments, such as home, school, work,
more “like a 2-year-old” than a preschooler. After some obser- and recreation.
vations of Meredith’s play and further discussion with her 3. The onset of intellectual and adaptive deficits is during the
family, Meredith’s parents were told that she had global devel- developmental period.
opmental delay and recommended that she be enrolled in a
Notably, specifiers of severity may be noted, but these
preschool program for children with special educational
should be based on the severity of adaptive functioning defi-
needs. Her parents were distressed to learn that her prob-
lems were serious, but they were relieved that their concerns
cits, not level of performance on an IQ test (American Psy-
were justified and that help was available. chiatric Association, 2013). The major purpose of describing
adaptive limitations is to develop a profile of individualized
supports needed to help ensure that, if appropriate, supports
Meredith is just one example of the kind of child who can are provided over a sustained period, and that these supports
receive the diagnosis of global developmental delay or ID and will improve the person’s level of life functioning.
has just one of the several possible types of communicative Both definitions stress intellectual and adaptive function.
disorders associated with her diagnosis. In Meredith’s case, as Intellectual functioning, or intelligence, refers to general men-
in a significant proportion of cases with ID, the cause of dis- tal capacity, such as learning, reasoning, and problem solving.
order is unknown. Diagnosis of ID in these cases is based on This may be operationalized on the basis of IQ test scores;
behavioral rather than medical characteristics, and the cause generally, an IQ test score of around 70 to 75 or below indi-
of disorder is not necessary for diagnosis or intervention cates a limitation in intellectual functioning. This range of
104 SECTION I  Topics in Childhood Language Disorders

scores translates to a criterion of at least 2 standard devia- such as Down syndrome. However, it is important to remem-
tions (SDs) below the normative mean of 100 on a standard ber that this is not always the case. A population study of
IQ test. You’ll remember from Chapter 2 that fewer than 3% school-aged children in an urban area of England revealed
of a normally distributed population will score farther from that 5% to 10% scored below 70 on the Wechsler Intelligence
the mean than 22 SD (see Fig. 2.11). You’ll also note that this Scale for Children (WISC), but only 15% of those low scorers
is not an absolute score; the inclusion of adaptive skills in the were receiving special educational support (Simonoff et al.,
definition leaves open the option of diagnosing an ID in an 2006). Provision of special educational services was more
individual who has a borderline IQ score but significant likely to occur if children had overt emotional or behavioral
limitations in adaptive behavior. problems in addition to low IQ. Nevertheless, children with
Adaptive behavior comprises skills of daily living, for ex- low IQ may be struggling academically, and it is worthwhile
ample: conceptual skills—language and literacy; money, considering the cognitive, linguistic, and literacy abilities of
time, and number concepts; and self-direction; social skills— any child not meeting age expectations at school.
interpersonal skills, social responsibility, self-esteem, gullibil-
ity, naïveté, social problem solving, and the ability to follow Cognitive Characteristics
rules/obey laws and to avoid being victimized; and practical Early reports of the cognitive profile of individuals with
skills—activities of daily living (personal care), occupational nonspecific ID reported a similar pattern of cognitive devel-
skills, health care, travel/transportation, schedules/routines, opment to typically developing children but a slower develop-
safety, use of money, and use of the telephone. Note that defi- mental trajectory (Owens, 2009). Other researchers have
cits in adaptive behavior must be relative to the individual’s reported a more uneven profile of cognitive development with
cultural group. This criterion is necessary because people more pronounced deficits in executive functioning (Willner,
from diverse cultural backgrounds may have different expec- Bailey, Parry, & Dymond, 2010; Hessl et al., 2016) and work-
tations of individuals at different ages. In mainstream Amer- ing memory (Henry & Winfield, 2010; Carretti, Belacchi, &
ican culture, we expect children to be able to separate from Cornoldi, 2010) than would be expected given overall level of
their mothers easily at age 5 for example; other cultures ex- intellectual ability. Willner and colleagues (2010) noted that,
pect much longer-term dependency on mothers. In the past, in a cohort of individuals with ID attending day center ser-
too many children were classified as having an ID because vices, executive functioning skills were not strongly correlated
their experiences were different from those of middle-class with IQ scores, but that impairments in executive functioning
children, which resulted in their scoring low on IQ tests that may be more closely associated with impairments in adaptive
contained culturally-biased items. As we have already dis- behavior. Henry and Winfield (2010) considered the relation-
cussed, it is also critically important to rule out language ship between different components of working memory and
differences as a source of “failure to adapt.” scholastic attainment in 11- to 12-year-old children with ID.
Although adaptive behavior is often evaluated subjec- They found that measures of phonological working memory
tively, standardized measures exist for various aspects of (word and digit repetition) accounted for a large degree of
adaptive performance. The most widely used measure is the variance in literacy skill, whereas measures of the “central ex-
Vineland Adaptive Behavior Scales–II (Sparrow, Cichetti, & ecutive” (a listening span task, in which children make true/
Balla, 2005). Ecological inventories and environmental as- false judgements about statements while simultaneously re-
sessments (see Chapter 8) also can be used to assess various membering the final word of each statement) were more
aspects of adaptive performance. predictive of numeracy skill. Carretti and colleagues (2010)
The definition of ID also includes the requirement that onset indicated that working memory performance in ID was par-
occurs before 18 years old, or during the developmental period. ticularly influenced by attentional control and “updating”
This criterion is used to differentiate ID, which is considered a information held in temporary store. In real terms, cognitive
developmental disorder, from acquired deficits and forms of abilities do generally improve throughout childhood and into
dementia that result in intellectual impairment and deteriora- adulthood. Standard scores, which you will remember from
tion during adulthood. Of course, there can be cases that are Chapter 2 take account of age, can provide an indication of
difficult to judge. For example, suppose a typically developing stability if repeated over time. Stability refers to the extent that
child has a serious accident at age 10 that results in brain damage an individual’s rank order in the population remains the same
and subsequent IQ scores of more than 2 SD below the norma- (Bornstein, Hahn, Putnick, & Suwalsky, 2014). Although ID
tive mean. By definition, this child would be considered to have during childhood is a relatively stable phenomenon, individ-
an ID (assuming his or her adaptive skills were also compro- ual stability of IQ is only moderate, with a reported intra-class
mised) even though the origins of impairment are traumatic correlation coefficient of 0.58 in a relatively large sample of
rather than developmental. Nevertheless, this child would likely children with mild/moderate ID (Jenni et al., 2015). This fluc-
benefit from the types of interventions or supports that other tuation is likely caused by test/retest reliability, which may be
children with ID require. This is an important reminder that affected by level of child’s cooperation, motivation, or atten-
clinicians need to determine the child’s individual needs rather tion. Therefore, clinical decisions and predictions should
than focusing too much on diagnostic labels. not rely on single IQ assessments, but should also consider
For many children, ID occurs in the context of a recognized repeated measurement, adaptive functioning, and previous
disorder of known or unknown (complex) genetic origin, developmental history.
CHAPTER 4  Special Considerations for Special Populations 105

Language Characteristics may be slow to develop intentional communication in the pre-


Delayed language acquisition is often one of the first signs of verbal stages of development. Once some language is acquired,
ID. One question that the clinician is likely to face is whether children with ID are able to engage in socially meaningful con-
language skills are in line with nonverbal mental age expecta- versations, with adequate turn-taking and topic maintenance
tions, or whether language is impaired relative to other cog- skills. However, they may be less able to clarify meaning and
nitive achievements. Both patterns of language acquisition request clarification when they have not understood utterances.
have been observed; Miller and Chapman (1984) estimated In addition, using language forms for different social purposes
that approximately 50% of children with nonspecific ID had may also be challenging. Recent research suggests that individu-
language skills commensurate with nonverbal abilities. The als with ID have considerable difficulties constructing coherent
remainder have more uneven profiles; 25% had expressive narratives (Murfett, Powell, & Snow, 2008), but they are able to
language deficits relative to comprehension skill (which was make use of nonverbal cues, such as gesture to support under-
on par with nonverbal mental age), whereas the remainder standing, for instance in the context of understanding verbal
had deficits in both comprehension and expression. This humor (Degabriele & Walsh, 2010).
variation may be related to differences in cognitive abilities, Literacy. Like other aspects of language development, liter-
for instance the differences in working memory and attention acy is slower to progress for children with ID. However, just as
control we discussed earlier (Abbeduto & Boudreau, 2004). we see in typical development, phonological processing skills
Form. In general, the acquisition of specific grammatical predict word and non-word reading in this population (Barker,
devices follows a typical developmental sequence, albeit at a Sevcik, Morris, & Romski, 2013b), whereas word reading and
slower developmental pace. However, once the mean length oral language skills predict reading comprehension abilities
of utterance (MLU) is above 3, children with ID tend to use (van Wingerden, Segers, van Balkom, & Verhoeven, 2017).
shorter, less complex sentences with fewer elaborations and However, an atypical route to reading has also been suggested,
relative clauses than do typical peers at the same MLU level because nonverbal IQ and rhythmic skills are much more pre-
(Abbeduto & Boudreau, 2004). dictive of word reading in children with ID, than is the case for
Content. It has generally been thought that vocabulary is typically developing children (van Tilborg, Segers, van Balkom,
easier for children to learn than syntax; however, recent re- & Verhoeven, 2014). In addition, children with ID may be par-
search suggests this may be artifact of test selection (Chapman, ticularly challenged by “higher-level” or implicit comprehen-
2006). Specifically, the Peabody Picture Vocabulary Test–IV sion processes that require children to go beyond what is ex-
(PPVT-IV) (Dunn & Dunn, 2007) exaggerated differences plicitly stated in the text and are not predicted by oral language
between PPVT-IV vocabulary and syntax in adolescents with skills (van Wingerden, Segers, van Balkom, & Verhoeven,
nonspecific ID relative to the vocabulary subtest of the Test of 2014). Differences in literacy achievement are not caused by
Auditory Comprehension for Language–3 (Carrow-Woolfolk, lack of reading opportunity in the home literacy environment;
1999). On this measure, age-equivalent scores did not differ van der Schuit, Peeters, Segers, van Balkom, and Verhoeven
significantly from syntactic measures. Chapman (2006) con- (2009) demonstrated that parents of children with ID pro-
cluded that, although vocabulary size may be an advantage in vided similar literacy opportunities as other families, although
ID, conceptual knowledge is more in keeping with develop- the children with ID initiated these activities less often. Home
mental expectations (see also Norbury, Griffiths, & Nation, literacy experiences were associated with the child’s verbal and
2010, for a similar pattern of results in ASD). nonverbal abilities, indicating that parents adapt their level of
Children with ID may also show atypical patterns of vo- engagement to the child’s linguistic abilities.
cabulary knowledge. Young typically developing children and
children with Down syndrome show a similar pattern of Summary
lexical knowledge, performing better on nouns than both Nonspecific, or idiopathic, ID is relatively common, with
verbs and attributes (Loveall, Channell, Phillips, Abbeduto, & prevalence estimates ranging from approximately 1% to 5%
Conners, 2016). In contrast, children with ID performed of the school-aged population, depending on the severity of
similarly on nouns and verbs, and in fact demonstrated supe- the intellectual impairment. In all cases, ID can have pro-
rior verb knowledge relative to peers matched for overall found implications for language development and academic
mental age, but had significantly poorer knowledge of attri- success. In general, children with nonspecific ID follow a
butes (Loveall et al., 2016). typical trajectory at a much slower developmental pace.
Use. The ability to use language meaningfully in social con- However, at times language and communication skills may be
texts is an important component of adaptive behavior, yet the out of step with nonverbal abilities. The acquisition of par-
pragmatic skills of children with idiopathic ID have attracted ticular language forms does not guarantee that children will
relatively little research attention. Pragmatic competence in automatically use these forms in socially appropriate ways,
everyday situations requires the integration of cognitive, lin- which may further interfere with the development of adap-
guistic, and social/emotional cues, making it particularly vul- tive behaviors. These are key aspects of development to con-
nerable in ID. Not surprisingly then, the evidence that exists sider, and each individual requires a thorough assessment of
suggests that pragmatic development often lags behind cogni- language abilities in different environmental contexts, sup-
tive development, although it may not be qualitatively different plemented by detailed discussion with families about suc-
(Abedutto & Boudreau, 2004). Specifically, individuals with ID cesses and challenges in daily communication.
106 SECTION I  Topics in Childhood Language Disorders

of ID, are at greatly increased risk of experiencing early onset


DEVELOPMENTAL LANGUAGE DISORDER Alzheimer disease (Castro, Zaman, & Holland, 2016). In
ASSOCIATED WITH DISORDERS OF KNOWN Down syndrome, the earliest symptoms of Alzheimer disease
GENETIC ORIGIN are marked changes in behavior, rather than cognitive de-
cline, and efforts are underway to identify earlier signatures
Down Syndrome of risk for dementia in order to implement effective interven-
Down syndrome is the most common genetic cause of ID, tions aimed at minimizing cognitive decline (Hithersay,
occurring in approximately 1 in 700 live births (Canfield Hamburg, Knight, & Strydom, 2017).
et al., 2006). Down syndrome is named for John Langdon
Down, the nineteenth-century English physician who first Cognitive Characteristics
published a description of a group of clients with the syn- Children with Down syndrome experience global develop-
drome. In the majority of cases, Down syndrome results from mental delays in fine and gross motor skills. These motor
an extra (third) copy of chromosome 21 (which is why it is delays are accompanied by mild to moderate ID, with the
sometimes referred to as trisomy 21); increasing maternal age majority of IQ scores between 40 and 70 (Grieco et al., 2015).
significantly increases risk of Down syndrome (see Grieco, Individuals with Down syndrome generally have an uneven
Pulsifer, Seligsohn, Skotko, & Schwartz, 2015, for a compre- profile of cognitive development that may impact on lan-
hensive review). Down syndrome is characterized by mild to guage development and language processing. For instance,
moderate ID, hypotonia (low muscle tone), distinctive facial they have marked deficits on measures of working memory,
features such as microgenia (an abnormally small chin), but these are more pronounced with verbal material relative
round face, macroglossia (protruding or oversized tongue), to visuospatial working memory (Lanfranchi, Baddeley,
epicanthal fold (fold of skin on the eyelid), short stature and Gathercole, & Vianello, 2012), a pattern that appears to be
shorter limbs, and hyperflexibility of joints (Fig. 4.1). Down unique to Down syndrome and not evident in other syn-
syndrome is also associated with a number of health con- dromes of ID (Edgin, Pennington, & Mervis, 2010). Visuo-
cerns, including a higher risk for congenital heart defects, spatial working memory skills appear to be more variable—a
gastroesophageal reflux disease, recurrent ear infections, ob- recent meta-analysis cautioned that visuospatial skills were at
structive sleep apnea, and thyroid disfunction. Comorbid best commensurate with overall levels of nonverbal cognitive
autism is diagnosed in 10% of children with Down syn- functioning and often lagged behind nonverbal mental age
drome, although there is debate concerning the degree to equivalents (Yang, Conners, & Merrill, 2014), even though
which severe cognitive impairments increase the likelihood visual memory may be superior to verbal memory. Executive
of a dual diagnosis. Because individuals with Down syn- functions, the cognitive processes integral to adaptive, goal-
drome are now living longer, it has become apparent that directed actions, are vulnerable in Down syndrome (Will,
adults with Down syndrome, unlike adults with other forms Fidler, Daunhauer, & Gerlach-McDonald, 2016). These in-
clude problems with response inhibition (impulse control),
cognitive flexibility, and planning. Limitations in response
inhibition are greater than those with idiopathic ID (Hessl
et al., 2016) and have been linked to reduced generation of
strategies for delaying gratification, difficulties persisting
with learning tasks, and engaging in more off-task behavior
(Kopp et al., 1983; Vlachou & Ferrell, 2000). Executive func-
tioning skills are critical to developing academic success, and
here again children with Down syndrome are vulnerable (Will
et al., 2016). Individuals with Down syndrome have greater
difficulty than mental-age–matched comparison groups with
learning new rules and applying them (Lanfranchi, Jerman,
Dal Pont, Alberti, & Vianello, 2010). They also take longer to
solve problems and are more likely to abandon efforts at
problem solving, reflecting difficulties with planning and
persistence (Fidler et al., 2005; Lanfranchi et al., 2010). How-
ever, recent work suggests that relative to other individuals
with ID (e.g., Williams syndrome), individuals with Down
syndrome may engage in more compensatory strategies to
assist with problem solving, such as asking others for help
(Camp, Karmiloff-Smith, Thomas, & Farran, 2016). Clearly,
FIGURE 4.1  Children with Down syndrome have characteris- deficits in executive functioning can impact academic perfor-
tic features. (Reprinted with permission from Zitelli, B. J., & mance because children with Down syndrome struggle to
Davis, H. W. [2002]. Atlas of pediatric physical diagnosis stay on task and monitor and adapt their own behavior to
[ed 4]. St Louis: Mosby.) achieve learning goals. Thus identifying potential avenues for
CHAPTER 4  Special Considerations for Special Populations 107

compensation is an important aim for the SLP when working receptive/expressive vocabulary as there are in grammatical
with this population. development. Some investigators have reported receptive
vocabulary scores in line with cognitive expectations (Laws &
Language Characteristics Bishop, 2004), whereas other have reported that expressive
The most consistently reported language profile in Down vocabulary is impaired relative to peers matched on nonver-
syndrome is one in which expressive language is more severely bal IQ (Caselli et al., 2008; Price et al., 2007). Differences
impaired than receptive language abilities (Laws & Bishop, between studies may be due, in part, to differences in the
2004; Martin, Klusek, Estigarribia, & Roberts, 2009). Here we vocabulary measures used (cf. Chapman, 2006), although
consider language development and disorder in Down syn- differences in participants (hearing status or parental educa-
drome in relation to form, content, use, and literacy. tion levels) cannot be ruled out. Results of receptive vocabu-
Form. Speech intelligibility in Down syndrome is poor rela- lary tests such as the PPVT-IV alone may be misleading; Laws
tive to cognitive ability and is particularly pronounced in con- and colleagues (2015) found that receptive vocabulary scores
nected speech (Barnes et al., 2009; Kent & Vorperian, 2013). were a strength relative to nonverbal cognitive abilities but
Most speech sound errors are developmental in nature (e.g., that depth of semantic knowledge was much weaker than
cluster reduction and final consonant deletion), although expected. Thus, it is important to probe not only how many
some atypical errors are also evident, such as vowel distortions words a child knows, but also how robust conceptual knowl-
and inconsistent pronunciations (Kent & Vorperian, 2013). edge of those words is.
Reduced intelligibility may be attributed in part to anomalies There is some evidence that gesture is preferentially used
of the articulatory structures or complications arising from by young children with Down syndrome and supports vo-
frequent bouts of middle ear infection (Martin et al., 2009). cabulary comprehension, and it may be predictive of later
Apraxia of speech has also been reported in Down syndrome vocabulary development (Zampini & D’Odorico, 2009).
(Rupela & Manjula, 2007; Rupela, Velleman, & Andrianopou- Pointing gestures may be particularly important to early lan-
los, 2016), suggesting assessment of oral-motor structure and guage acquisition, because they elicit verbal labels from care-
function and hearing is warranted. givers (Dimitrova, Özçalışkan, & Adamson, 2016). However,
Like children with primary DLD, children with Down syn- use of baby sign by toddlers with Down syndrome is a better
drome have disproportionate difficulties acquiring and using predictor of later expressive language than spontaneous use
syntax (Chapman, 2006; Martin, Losh, Estigarribia, Sideris, & of gestures (Özçalişkan, Adamson, Dimitrova, Bailey, &
Roberts, 2013). Syntactic comprehension is characterized by Schmuck, 2016). Individuals with Down syndrome are profi-
slowed growth and even decline in late adolescence (Laws & cient in using referential cues to learn new words (McDuffie,
Gunn, 2004) and is more impaired than overall cognitive abil- Sindberg, Hesketh, & Chapman, 2007; Zampini, Salvi, &
ity and vocabulary size (Martin et al., 2013). Expressive syntax D’Odorico, 2015). Vocabulary growth may be hampered by
presents even greater challenges and can be an earlier indicator limitations in phonological short-term memory (Jarrold,
of language difficulties. Children with Down syndrome pro- Thorn, & Stephens, 2009), although there is some evidence
duce shorter and less complex sentences and fewer question/ for better than expected word learning, implicating involve-
negation forms than typically developing peers matched for ment of other memory systems and/or learning strategies
nonverbal mental age (Caselli, Monaco, Trasciani, & Vicari, (Mosse & Jarrold, 2011).
2008; Price et al., 2008). Similarities and differences have also Use. Pragmatics is more variable in Down syndrome, with
been noted between the grammatical profiles of individuals with many strengths and some challenges. Early joint communica-
Down syndrome and individuals with other DLDs (Ypsilanti & tive behaviors, such as mutual eye contact, vocalizations, and
Grouios, 2008; Finestack, Sterling, & Abbeduto, 2013). For in- dyadic interactions with caregivers, may be delayed or less
stance, numerous similarities between Down syndrome and coordinated than those observed in typically developing in-
more specific language impairments have been noted with fants (Berger & Cunningham, 1983; Jasnow et al., 1988). By
particular limitations in tense marking (past tense –ed; third the age of 2, however, many children with Down syndrome
person singular –s) (Caselli et al., 2008; Laws & Bishop, 2004). show more social-interactive behaviors than typically devel-
On the other hand, individuals with Down syndrome appear oping peers (Mundy et al., 1988). Indeed, relative to children
to have more pronounced grammatical deficits relative to with Williams syndrome who have more advanced vocabu-
other groups with ID of known genetic origin. For instance, laries and higher nonverbal IQs, young children with Down
Finestack and colleagues (2013) reported that grammar was syndrome show advanced socio-communicative competen-
more severely impaired in Down syndrome than in fragile cies in skills that underpin successful social referencing, such
X syndrome (FXS) and that differences persist into adolescence as initiating eye contact, following eye gaze, and emotional
and early adulthood (Finestack & Abedduto, 2010). responsivity (Thurman & Mervis, 2013). Children with
Content. Acquisition of first words in Down syndrome is Down syndrome also use the same variety of communicative
significantly delayed and subsequent growth of expressive functions (comment, answer, protest) as language- or non-
vocabulary is slower than expected (Berglund, Eriksson, & verbal ability-matched younger children, although they dem-
Johansson, 2001). Once words are acquired, there is some onstrate fewer requesting behaviors (Beeghly et al., 1990).
debate as to whether vocabulary keeps pace with nonverbal Conversational development may also represent an area of
cognitive abilities, and whether there are asymmetries in strength, because children with Down syndrome demonstrate
108 SECTION I  Topics in Childhood Language Disorders

high levels of contingent responding and topic maintenance longitudinal stability in reading, indicating that early reading
(Beeghly et al., 1991; Tager-Flusberg & Andersen, 1991). Nar- competence is highly predictive of later reading competence.
rative skills of children with Down syndrome reflect a good Hulme and colleagues (2012) also emphasized that oral lan-
conceptual understanding of the story. When narrating a guage competence strongly constrains initial reading levels, a
wordless picture book, children with Down syndrome pro- finding confirmed by a meta-analysis that identified vocabu-
duce more plot lines and thematic elements relative to MLU- lary, as opposed to phonological awareness, as key longitudi-
matched peers (Miles & Chapman, 2002). This narrative nal predictor of word reading and decoding in children with
strength may depend in part on the level of support provided Down syndrome (Næss, Melby-Lervåg, Hulme, & Lyster, 2012).
and expressive language competence. For instance, when Such findings suggest that oral language may be an important
asked to narrate stories without picture support, individuals target of early intervention for reading. Indeed, Burgoyne and
with Down syndrome may recall fewer important story ele- colleagues (2012) demonstrated modest but clinically significant
ments (Kay-Raining Bird, Chapman, & Schwartz, 2004; effects of an oral language intervention on word reading and
Murfett, Powell, & Snow, 2008), although differences in the oral language skills in children with Down syndrome. Outcomes
ability to convey story elements may be attributable to limita- were variable, but age, number of therapy sessions completed,
tions in expressive language, rather than pragmatic deficits and initial letter knowledge significantly predicted growth in
(Channell, McDuffie, Bullard, & Abbeduto, 2015). reading following intervention. Vocabulary and letter knowledge
Other aspects of language use may be more vulnerable. were highly correlated, and vocabulary was also strongly corre-
Martin and colleagues (2013) administered the pragmatics sub- lated with growth in reading, underscoring the importance of
test of the Comprehensive Assessment of Spoken Language developing vocabulary for literacy.
(CASL; Woolfolk, 1999) to children with Down syndrome, FXS Comparison of word reading and comprehension skills
with and without additional autism diagnosis, and young typi- suggests that individuals with Down syndrome are more
cally developing children. The children with Down syndrome likely to have a profile similar to that of “poor compre-
obtained worse scores on the pragmatics sub-test than both the henders” in which reading comprehension skills are poorer
typically developing and the FXS-only groups, and they did not than expected given word reading abilities (Roch & Levorato,
differ from children with FXS and additional ASD diagnoses. 2009; Laws, Brown, & Main, 2016). Poor reading comprehen-
Individuals with Down syndrome are less likely to signal non- sion is also associated with levels of oral language competence
comprehension of language or request clarifications in referen- in both typical development and Down syndrome, suggesting
tial communication tasks (Abbeduto et al., 2008). Abbeduto that oral language comprehension should form the founda-
and colleagues (2008) reported that the ability to request clari- tions of educational interventions aimed at improving literacy
fication was associated with vocabulary and syntactic skills, skill for this population (cf. Clarke, Snowling, Truelove, &
highlighting the strong links between core language skills and Hulme, 2010).
use of language in social contexts. These pragmatic behaviors
may also be associated with executive skill, and particularly the Implications for Clinical Practice
ability to monitor comprehension, although further research is In summary, children with Down syndrome have a pro-
needed in this area. tracted rate of language and literacy development. Striking
Literacy. Reading skills of children with Down syndrome similarities between Down syndrome and more specific
are extremely variable, and there is some debate about whether DLDs have been noted: relative strengths in vocabulary and
reading development in Down syndrome follows a qualita- pragmatic skill in the context of pronounced difficulties in
tively different course. Children with Down syndrome are of- syntax, morphosyntax, and phonological/verbal memory.
ten characterized as having poor phonological awareness in Literacy is also particularly vulnerable, with increased risk of
the context of good visual skills, which gives rise to a reading poor reading comprehension. Special considerations for this
pattern in which word recognition is much better than word population include the need to monitor hearing, because of
decoding (cf. Burgoyne et al., 2012). As in the case of typical recurrent ear infections and their association with language
development, deficient word and non-word reading in Down development in this population (Laws & Hall, 2014), and the
syndrome is associated with phonological awareness skills, need to give detailed consideration to oral-motor structure
leading some to advocate phonologically based intervention and function, because anomalies in oral-motor development
approaches (Lemons & Fuchs, 2010). However, a recent large- may affect speech production and intelligibility.
scale longitudinal study suggests potentially different develop- Detailed assessment of language attainment is necessary,
mental routes to reading (Hulme et al., 2012). In this study, and we cannot assume that level of nonverbal ability will be
phonological awareness and reading skills were assessed at predictive of language skill. We should also be cautious in
two time points in children with Down syndrome and typical assuming that acquisition of a particular skill results in ap-
peers matched for initial word reading level. Both phonologi- propriate use of that skill for learning or social exchanges.
cal awareness and oral vocabulary were strong concurrent Observation and analysis of language in less structured con-
predictors of initial reading levels in both groups. Over time, texts is warranted. Finally, children with Down syndrome
phonological awareness was a longitudinal predictor of read- demonstrate relative strengths in visual-spatial memory, and
ing, but only for the typically developing children. The chil- using gesture and other social cues may support comprehen-
dren with Down syndrome experienced a very high degree of sion and learning of new information. Any assessment profile
CHAPTER 4  Special Considerations for Special Populations 109

should therefore detail the child’s communication strengths


and use of compensatory strategies, as well as his or her
needs, because these strengths may be usefully exploited in
intervention contexts.
With regard to intervention, the ultimate goal should be to
improve functioning in communication, academic, social,
and vocational areas. Decisions about what to prioritize in
intervention should be made in collaboration with families
and clients themselves, and should focus not just on develop-
ing skills, but on the functional use of those skills in aca-
demic, vocational, and social contexts. With this in mind,
Martin and colleagues (2009) suggest that general priorities
for working with infants and toddlers with Down syndrome
will be to target early communication using milieu commu-
nication techniques (see Chapter 3) with families to support
development of early vocalizations, gesture, and eye gaze to
initiate and respond to “conversational” exchanges (cf. Fey
et al., 2006). For school-aged children, interventions that
target vocabulary and complex language structures, may ben-
efit both oral language and early literacy skills (Burgoyne
et al., 2012). Although reading development may be seen as
an outcome of early intervention strategies, there is also evi-
dence that using written language in intervention programs
FIGURE 4.2  Children with Williams syndrome have an up-
may, because of its visual modality, support oral language, turned nose and small chin. (Reprinted with permission from
speech, and memory development in Down syndrome (Roberts, Collins, T. [2013]. Cardiovascular Disease in Williams Syn-
Torgesen, Boardman, & Scammacca, 2008; Laws, 2010). drome. Circulation, 127:21, 2125-2134.)
In addition to improving language skills, it is worth re-
membering that children with Down syndrome may need
support in attending to relevant information, staying on task, with Williams syndrome generally have mild to moderate ID
and recognizing/signaling when they have not understood and deficits in adaptive behavior, although there is consider-
something. Providing strategies for these behaviors is critical able variability (Mervis & John, 2010). Longitudinal studies
to academic achievement. Finally, it may be prudent to con- have documented strong stability in nonverbal IQ scores, with
sider using augmentative or alternative communication to minimal change in standard scores particularly for children
improve the communicative competence of children with over the age of 7 (Fisher, Lense, & Dykens, 2016; Mervis & Pitts,
Down syndrome. Many children with Down syndrome use 2015). In contrast, adaptive behavior scores show a tendency to
sign language, and there is evidence that the use of sign lan- decrease over time, indicating that individuals with Williams
guage may support oral language development (see Brady, syndrome do not make the same degree of progress in every-
2008; Özçalişkan et al., 2016). day functioning that their typically developing peers do (Fisher
et al., 2016; Mervis & Pitts, 2015). Williams syndrome is also
Williams Syndrome associated with a unique profile of cognitive strengths and
Definition and Classification weaknesses. Most notably, children with Williams syndrome
Williams syndrome is a complex neurodevelopmental disor- have profound difficulties with visual-spatial construction,
der that results from the deletion of approximately 25 genes with scores on the Spatial Cluster of the Differential Ability
on one copy of chromosome 7q11.23 (Osborn, 2006). It is a Scales (Elliot, 2007) some 20 points lower than scores on other
relatively rare disorder with a prevalence rate of 1 in 7,500 intelligence scales (Mervis & John, 2010).
live births (Stromme et al., 2002). Williams syndrome is as-
sociated with multiple physical, cognitive, and behavioral Language Characteristics
features. Physical features include characteristic facial dys- Traditionally, Williams syndrome has been put forward as the
morphology (Fig. 4.2), cardiovascular heart disease, growth archetypal evidence for dissociations between cognitive and
deficiency, and connective tissue abnormalities. The striking linguistic skill, with some suggesting “exquisite mastery” of
behavioral phenotype is one of overfriendliness, social gre- syntax and vocabulary in the context of pronounced nonver-
gariousness, and marked anxiety (see Mervis & John, 2010, bal cognitive deficits (cf. Piattelli-Palmarini, 2001). Recent
and Pober, 2010, for reviews). investigations provide a more nuanced view of the relation-
ship between language and cognition. For a start, the onset of
Cognitive Characteristics first words and phrases is almost always delayed in Williams
Infants and toddlers with Williams syndrome experience syndrome (Levy & Eilam, 2013). Once words have appeared,
global developmental delays, and older children and adults the pattern of linguistic strengths and weaknesses closely
110 SECTION I  Topics in Childhood Language Disorders

mimics those observed in nonverbal cognition. Let’s look at highlighted overlaps between the two disorders. For example,
this in a little more detail. although children with Williams syndrome are more likely to
Form. Canonical babbling is significantly delayed in in- look at faces than children with ASD, their ability to integrate
fants with Williams syndrome relative to age-matched infants gaze cues for communication purposes is impaired (Lincoln,
(Mervis & Becerra, 2007). Onset of babbling is predictive of Searcy, Jones, & Lord, 2007; John & Mervis, 2010). Even when
onset of word production. There are no reports of significant children do not meet criteria for ASD, a significant proportion
speech sound disorders or reduced intelligibility in older, of children with Williams syndrome have marked pragmatic
verbal children with Williams syndrome, despite evidence for difficulties on parent-report measures, such as the Children’s
deficits in oral-motor praxis, which may contribute to early Communication Checklist–2 (CCC-2; Bishop, 2003). Laws and
language delays (Krishnan, Bergström, Alcock, Dick, & Bishop (2004) reported that 79% of children with Williams
Karmiloff-Smith, 2015). syndrome studied were rated as having pragmatic difficulties
Initial reports of grammatical development suggested that on the CCC-2. The prevalence of pragmatic impairment de-
grammar was “intact” and much better than expected for pends in part on the measure used; Hoffman, Martens, Fox,
overall level of nonverbal cognitive ability. Indeed, when Rabidoux, and Andridge (2013) reported that considerably
compared with ability-matched peers with Down syndrome, more children with Williams syndrome evidence pragmatic
the grammatical skills of children with Williams syndrome deficits on the Test of Pragmatic Language, Second Edition
are superior (Joffe & Varlokosta, 2007; Mervis & Velleman, (TOPL-2) relative to the CCC-2. Regardless of measure, prag-
2011). However, Mervis and John (2010) point out that these matic difficulties are especially evident in conversational be-
findings may reflect the more pronounced grammatical limi- havior, in which individuals with Williams syndrome are less
tations that characterize children with Down syndrome likely to provide contingent and informative responses than
rather than demonstrating superior grammatical skills in peers with more specific DLDs (Stojanovik, 2006) and many
Williams syndrome. When compared to younger typically more off-topic, irrelevant remarks compared to children with
developing children with equivalent cognitive levels or to other IDs (van den Heuvel, Manders, Swillen, & Zink, 2016).
other participants with ID, grammatical skills are more in Pre-school pragmatic skills are strongly predictive of later con-
line with, or sometimes below, developmental expectations versational competencies. For example, John, Dobson, Thomas,
(Mervis & Becerra, 2007). Deficits in grammatical under- and Mervis (2012) found that the ability to verbally contribute
standing are evident, but these are strongly related to verbal new information to a social interaction beyond what was re-
working memory abilities and general levels of cognitive abil- quired when answering a question and the ability to pair such
ity (Mervis & John, 2010). verbalizations with eye contact at 4-years-old predicted the
Content. Understanding and production of concrete vo- ability to verbally contribute new information beyond what
cabulary are relative strengths for individuals with Williams was required to answer a question at 9 to 12 years old. Qualita-
syndrome, resulting in consistently higher scores on mea- tive differences in narrative skill have also been reported;
sures of vocabulary such as the PPVT-IV (Dunn & Dunn, relative to other populations with ID, children with Williams
2007) and the Expressive Vocabulary Test (Williams, 2006), syndrome make considerably more social evaluative statements
relative to other language measures (cf. Brock, 2007). How- and fewer cognitive inferences (Reilly, Losh, Bellugi, & Wulfeck,
ever, as we’ve seen in our earlier discussions, this profile is not 2004). Like other children with ID, children with Williams syn-
unique to Williams syndrome and characterizes many DLDs. drome have more difficulty monitoring their own comprehen-
What is less common across disorders is the profound diffi- sion and signaling when their conversational partners provide
culty with relational or conceptual vocabulary experienced ambiguous or inadequate messages (John et al., 2009).
by individuals with Williams syndrome. This vocabulary is Literacy. The reading skills of children with Williams syn-
important for marking spatial, temporal, and dimensional drome are variable, with some achieving word recognition
concepts, as well as for devices such as conjunction and dis- and non-word reading skills that are broadly in line with
junction. Deficits with these terms mimic deficits in spatial their nonverbal abilities, whereas other are unable to read at
abilities (Mervis & John, 2008). Longitudinal studies also in- all (see Mervis, 2009, for review). Even when functional levels
dicate a slight but significant decline in vocabulary standard of reading are achieved, the developmental path may be
scores over the school years (Mervis & Pitts, 2015). atypical; vocabulary is strongly associated with reading prog-
Use. Children with Williams syndrome have pronounced ress, whereas letter knowledge and phonological awareness
pragmatic difficulties, despite the superficial air of social skill do not predict growth in reading for children with Williams
and a clear desire for social relationships. The emergence of syndrome as they do in typical development (Steele, Scerif,
joint attention is delayed, and there is an atypical temporal Cornish, & Karmiloff-Smith, 2013). Consistent with reading
relationship between gesture and word production. In typical profiles seen in other populations with ID, reading compre-
development (as well as in Down syndrome), referential ges- hension scores are generally significantly lower than word
tures such as pointing precede referential word production reading abilities (Laing et al., 2001).
(Mervis & Becerra, 2007).
Although Williams syndrome is often conceptualized as the Implications for Clinical Practice
“opposite” of ASD because of the increased interest in social Mervis and John (2010) suggest that intervention approaches
interaction in Williams syndrome, systematic investigation has developed for other populations with ID and social impairments
CHAPTER 4  Special Considerations for Special Populations 111

can also be useful for children with Williams syndrome. In


particular, working with families to develop language and
communication is a priority for young children with Williams
syndrome. Language intervention is likely to be necessary
throughout the school years; the focus of intervention may
change and should emphasize use of language targets in aca-
demic and socially meaningful contexts. Social skills training for
older children is also advocated; these not only aim to pro-
mote socially appropriate communication behaviors, but
could help children with Williams syndrome to be more dis-
cerning in approaching others and in reading more subtle
social-communication cues. To date, only one study has ex-
plored literacy intervention in this population. Mervis (2009)
suggests that a systematic phonics based approach in the context
of direct reading instruction is preferable to a whole word ap-
proach for these children. Oral language instruction aimed at FIGURE 4.3  Boys with fragile X syndrome (FXS) typically
improving reading comprehension is also likely to be important have long, narrow faces and large ears. (By Peter Saxon (Own
(cf. Clarke et al., 2010) and will need to be complemented by work) [CC BY-SA 4.0 (http://creativecommons.org/licenses/
explicit strategies for comprehension monitoring and linking by-sa/4.0)], via Wikimedia Commons.)
text information to general knowledge.

Fragile X Syndrome associated with FXS therefore include occasional joint disloca-
Definition and Classification tions, recurrent otitis media (OM), strabismus, mitral valve
FXS is a single gene disorder, caused by an expansion of the tri- prolapse, and/or dilation at the base of the aorta and gastrointes-
nucleotide (CGG), which repeats too often on the fragile X men- tinal reflux, which is seen in the majority of male infants with
tal retardation gene (FMR1), located on the bottom end of the FXS (Hagerman & Hagerman, 2002).
X chromosome (see Lozano, Rosero, & Hagerman, 2014, for Comorbid conditions are extremely common in FXS and
extensive review). Typical individuals have 5 to 44 repeats on affect language development and disorder. Most striking are
FMR1; premutation carriers of FXS have 55 to 200 repeats, the high rates of ASD identified in males with the full FXS
whereas individuals with the full mutation have in excess of mutation; approximately 53% of infants with FXS show
200 CGG repeats (Schneider, Hagerman, & Hessl, 2009). This symptoms of ASD (Roberts, Tonnsen, McCary, Caravella, &
expansion leads to eventual silencing of the FMR1 gene, re- Shinkareva, 2016), and these symptoms appear to become
ducing or completely eliminating production of its associated more severe over time (Lee, Martin, Berry-Kravis, & Losh,
gene protein, FMRP. FMRP is critically important for experi- 2016). Approximately 50% to 74% of boys with FXS meet
ence-dependent neural development, particularly for the criteria for ASD (see McCary & Roberts, 2013, for review).
maturation of synapses and synaptic pruning in the develop- This makes FXS the single largest known genetic cause of
ing brain; as such, there is a direct positive correlation be- ASD, although only 2% to 6% of ASD cases can be attributed
tween the amount of FMRP expressed and level of cognitive to FXS (Reddy, 2005). Despite these behavioral similarities,
functioning (Lozano, Rosero, & Hagerman, 2014). there is considerable evidence that the developmental course,
Unlike Down syndrome, which is not passed down from one psychiatric comorbidities and underlying neurobiological
generation to another, FXS is an inherited disorder, and is the mechanisms may distinguish the two disorders, suggesting dif-
most common inherited form of ID. FXS occurs in approxi- ferent approaches to addressing social communicative chal-
mately 1 in 4000 males and 1 in 8000 females; it is more com- lenges may be necessary (Abbeduto, McDuffie, & Thurman,
mon in males because males have only one X chromosome. The 2014). Although we’ve mentioned that level of FMRP expres-
prevalence of the premutation is much more common, with ap- sion is predictive of cognitive ability, it does not appear to be
proximately 1 in 250 females and 1 in 600 to 800 males having associated with severity of ASD symptoms (Loesch et al.,
the premutation (Beckett, Yu, & Long, 2005). The full mutation 2007; Harris, Graham, Mason, & Friedlander, 2008). It is
is associated with a characteristic, although variable, physical clearly important to distinguish the cognitive and language
and behavioral phenotype. Boys with FXS do not have clearly characteristics of individuals with FXS who also have ASD
dysmorphic features and are often difficult to identify before from those who do not. Other comorbidities include atten-
3 years old, unlike children with Down syndrome, whose physi- tion deficit hyperactivity disorder (ADHD), which is re-
cal features are noticeable from birth. With increasing age, how- ported to affect 44% to 93% of children with FXS, is evident
ever, characteristic physical features emerge (Fig. 4.3). These in- in preschool, and remains remarkably stable during the
clude elongated face, long and prominent ears, highly arched school years (Grefer, Flory, Cornish, Hatton, & Roberts,
palate, enlarged head, hypotonia, flat feet, hyperextensible finger 2016); seizures, which affect approximately 20% of males;
joints, and large testicles (macroorchidism). FMRP is also associ- and high rates of anxiety, reported to affect 86.2% of males
ated with the formation of connective tissue; medical difficulties and 76.9% of females, with social phobia the most commonly
112 SECTION I  Topics in Childhood Language Disorders

diagnosed anxiety disorder (Cordeiro, Ballinger, Hagerman, peers (Estigarribia, Martin, & Roberts, 2012). Phonological
& Hessl, 2011). awareness, in contrast, is commensurate with nonverbal ability
(Adlof, Klusek, Shinkareva, Robinson, & Roberts, 2015).
Cognitive Characteristics Compared to younger typically developing children
ID is the predominant cognitive characteristic; nearly all matched for nonverbal ability, boys with FXS are delayed in
males have a degree of ID that is similar to that seen in Down both their comprehension (Oakes, Kover, & Abbeduto, 2013)
syndrome. IQ is significantly correlated with FMR protein and production of grammar and morphosyntax. Impairments
expression; for that reason, females tend to have less severe ID are noted on both standardized measures and analyses of
with most obtaining standard scores of 70 to 90 (Huddleston, more spontaneous language samples, and individuals with
Visootsak, & Sherman, 2014). The rate of intellectual growth FXS demonstrate a slower rate of language growth relative to
is reported to be about half that of typically developing chil- peers (Martin et al., 2013). In conversation samples, boys with
dren, the gap between individuals with FXS and their peer FXS have shorter MLUs relative to matched comparison
group increases with time, causing an age-dependent gradual groups even when nonverbal mental age and level of maternal
decline in IQ. In addition to cognitive impairment, a core education has been taken into account (Roberts et al., 2007). In
deficit in executive function has also been proposed, with addition, less complex noun and verb phrases are evident in
significant deficits in sequential processing, working memory conversational language, although production of questions/
deficits, cognitive flexibility, planning, selective attention, in- negation may be more in line with nonverbal skills. Where
hibitory control problems, and fine and gross motor delay direct comparisons have been made, the expressive and recep-
(see Hessl et al., 2016). tive grammatical skills of boys with FXS are somewhat better
Of course, there are pockets of relative cognitive strength, than boys with Down syndrome (Martin et al. 2014) and are
which include simultaneous processing and long-term memory comparable in individuals with and without comorbid ASD,
(see Finestack, Richmond, & Abbeduto, 2009, for review). In- although significant, negative correlations between language
triguingly, a longitudinal investigation of academic achieve- scores and autistic symptomatology have been reported
ment in FXS found that nonverbal IQ and FMR protein expres- (McDuffie, Kover, Abbeduto, Lewis, & Brown, 2012).
sion were not associated with academic level or rate of change Content. Investigations of receptive vocabulary knowledge
in academic performance; however, autistic behavior and level in FXS have yielded mixed results, with some investigators
of maternal education were significantly related to academic reporting weaker vocabulary scores and others suggesting
achievement scores (Roberts et al., 2005). that vocabulary is commensurate with nonverbal mental age
expectations (Finestack et al., 2009). A more consistent find-
Language Characteristics ing is that expressive vocabulary, as measured by number of
Gender differences in language attainment are particularly different words used in connected discourse, is impaired and
pronounced in FXS, with girls invariably demonstrating higher rates of vocabulary growth are slower than those seen for
levels of linguistic competence relative to males with FXS. It younger typically developing children (Roberts, Mirrett,
will be absolutely essential for the clinician to establish whether Anderson, Burchinall, & Neebe, 2002; Martin et al., 2013). In
comorbid ASD is present, because this will have significant general, the presence of comorbid ASD does not result in
implications for language development and particularly the more severe vocabulary deficit (Kover & Abbeduto, 2010;
social use of language. It is also important to realize that the Martin et al., 2013), although the small number of children
bulk of research in FXS has been directed at understanding with comorbid diagnoses means we should be cautious in
the genetic pathways to behavior; as a result very little is known assuming this is always the case.
about environmental influences on language development in Studies to date have focused on lexical diversity in dis-
FXS, or whether modifying the language-learning environ- course; there is a dearth of research evidence about what
ment can positively alter developmental trajectories (Finestack children with FXS understand about the words they use. We
et al., 2009). Recent work has suggested that the pragmatic also know very little about the integrity of semantic networks
skills of mothers with the permutation were significantly as- in FXS or about how flexibly children with FXS use their se-
sociated with the receptive and expressive language skills of mantic knowledge, for example in understanding figurative
their sons (Klusek, McGrath, Abbeduto, & Roberts, 2016). Al- expressive or verbal humor.
though this does not imply that maternal speech is causing Use. Pragmatic language impairments (PLIs) in FXS are
child language difficulties, it does suggest that a family-­centered associated with FMR1 molecular variation and are evident to
approach to language intervention is warranted. some degree in pre-mutation carriers of the disorder (Losh
Form. In general, the speech sound production of boys with et al., 2012). In addition, the presence of comorbid ASD con-
FXS is commensurate with nonverbal mental age expectations. tributes significantly to variation in pragmatic skill, although
Although boys with FXS tend not differ from younger, language- core cognitive and linguistic competencies account for a
ability matched typically developing peers on phoneme accuracy whopping 78% of variation in pragmatic language scores of
or the number of developmental phonological processes, intel- boys with FXS (Klusek, Martin, & Losh, 2014).
ligibility is significantly reduced in connected speech, and sig- Pragmatic deficits are more evident in naturalistic tasks that
nificant impairments in phonological short-term memory are include narrative or spontaneous conversation, as opposed to
also evident, relative to ability matched typically developing standardized tests that rely on metacognitive appraisals of social
CHAPTER 4  Special Considerations for Special Populations 113

communication (Klusek, Martin & Losh, 2014). Qualitative dif- reading skills of children with FXS are commensurate with
ferences in social communication have included increased use nonverbal age expectations (Buckley & Johnson-Glenberg,
of tangential language, perseverative and repetitive speech, de- 2008; Klusek et al., 2015). Finestack and colleagues (2009)
layed echolalia, and use of stereotyped phrases (Cornish et al., point out that relative strengths in word reading in FXS may
2004). These qualitative differences disrupt conversational ex- be confounded by large age differences between individuals
changes; relative to developmental expectations, boys with FXS with FXS and their ability-matched typical peers, which af-
have difficulty maintaining coherent, semantically rich conver- fords the FXS group considerably more print exposure than
sational exchanges (Roberts et al., 2007). For example, boys the typically developing children. This may also explain the
with FXS are more likely to provide conversational turns that relative advantage in reading comprehension when children
are tangential or unrelated, and provide fewer turns in which with FXS are compared with younger, nonverbal IQ matched
they add or request new, on-topic information. These anoma- typically developing peers (Klusek et al. 2015). Nevertheless,
lies are particularly pronounced in those who also meet criteria only 2% of boys with FXS achieve age-appropriate reading
for ASD, but are not limited to this subgroup (Roberts et al., scores, and the average standard score reported by Klusek and
2007; Klusek, Martin, & Losh, 2014). This raises interesting colleagues was less than 50. Furthermore, growth in reading,
questions about the source of these conversational errors; in and the phonological skills that underpin reading, appear to
ASD pragmatic errors are largely attributed to deficits in social- plateau at around 10 years old (Adlof, Klusek, Shinkareva,
cognitive understanding. Children with FXS also show evidence Robinson, & Roberts, 2015). Given oral language weaknesses
of poor understanding of other people’s minds, as indexed by and pervasive pragmatic difficulties, it is not surprising that
false belief tasks (Grant, Apperly, & Oliver, 2007), and prag- reading comprehension presents significant challenges to in-
matic deficits are closely aligned with increasing social-affective dividuals with FXS. However, the impact of additional ASD
symptoms over time (Lee, Martin, Berry-Kravis, & Losh, 2016). does not appear to influence reading development, once the
However, these deficits appear to be associated with deficits in effects of phonological awareness, nonverbal IQ, and care-
working memory and executive control (inhibition) rather giver education are taken into account (Klusek et al., 2015).
than social understanding per se. This suggests that conversa-
tional anomalies may also reflect problems with inhibition and Implications for Clinical Practice
working memory. Children with FXS have complex cognitive and behavioral
Further support for this assertion comes from studies of challenges that impact language development and language
narrative production; in narrative recall tasks, when narra- processing. However, as we’ve seen with other disorders of
tives are elicited in the context of a wordless picture book, known genetic origin, there is a paucity of evidence regarding
few differences have been found between individuals with the best course of clinical action or how therapeutic and edu-
FXS and ability-matched comparison groups (Keller-Bell & cational practices may influence developmental trajectories.
Abbeduto, 2007; Estigarribia et al., 2011). Estigarribia and Children with FXS are often referred to SLP services when
colleagues (2011) further found that verbal short-term mem- they are very young (Brady et al., 2006); a top priority for the
ory and nonverbal IQ were significant predictors of narrative SLP is to work closely with families and other professionals to
macrostructure, whereas expressive grammar and caregiver ascertain ASD status and other comorbid conditions that can
education were not. Importantly, presence of co-occurring negatively impact language development. Cognitive and lin-
ASD was detrimental to narrative scores, underlining the guistic strengths should be documented alongside weaknesses,
importance of considering ASD symptomatology in assess- because these may be used to support language and commu-
ment and intervention planning. nication. Throughout development, interventions to increase
Measures of referential communication also reveal prag- linguistic competencies should be embedded in socially mean-
matic weaknesses in FXS. For instance, relative to ability- ingful contexts, with the goal of improving social interaction
matched peers, boys with FXS are less able to provide consis- and pragmatic language skills. Literacy development also re-
tent, unambiguous language to describe a target shape to quires attention; early oral language programs may have a
listeners (Abbeduto et al., 2006) and are less likely to indicate positive effect on later reading comprehension. It also likely
that the verbal messages of others are inadequate to meet task that there will be a need to work with families to decrease in-
demands (Abbeduto et al., 2008). In the latter case, signaling appropriate communication and challenging behaviors.
non-comprehension was positively correlated with vocabu-
lary and receptive grammar, and associated with gender; girls LANGUAGE DISORDERS ASSOCIATED
with FXS were more likely to signal non-comprehension than WITH SENSORY IMPAIRMENTS
male counterparts. Again, these findings appear to indicate a
reduced appreciation of listener need and/or deficits in ex- Visual Impairment
ecutive skill and comparisons of those with/without comor- Children with congenital visual impairments (VIs) may ex-
bid ASD further illuminate the extent to which pragmatic perience some early delays in the acquisition of language, but
deficits are characteristic of FXS generally, or more symp- by school age these problems are largely resolved (Mulford,
tomatic of co-occurring ASD. 1988; Tadic, Pring, & Dale, 2010). Children with VI may also
Literacy. Few investigations of literacy development in learn to read with the help of specially adapted writing sys-
FXS are available. Preliminary evidence suggests that the tems (such as Braille) and computer programs that convert
114 SECTION I  Topics in Childhood Language Disorders

text to speech. However, pragmatic skills are vulnerable in parents to recognize and explicitly comment on and reinforce
children with VI, and there is increasing evidence that many nonverbal communication behaviors they themselves emit or
social-communication behaviors in VI resemble those seen in observe in their children. It is also necessary to help families
sighted children with ASD (Tadic, Pring, & Dale, 2010). This find alternative ways of establishing joint attention and use
section focuses on the nature of these pragmatic deficits and these opportunities to provide rich linguistic stimulation.
implications for clinical treatment. Some tried and true methods of facilitating language and
Early differences in language acquisition may be attribut- social communication in this population include:
able in part to disruptions in early visual experiences, for • Provide labels and descriptions of the objects the child
example triadic joint attention. As a result, toddlers with VI handles and what he or she can do with these objects
are delayed in their acquisition of first words and phrases • Ask both open-ended and more directive questions
(Lahey, 1988). Despite these early delays, research has consis- • Provide more qualitative information not only about the
tently demonstrated that children with VI develop age- child’s actions but also other things going on in the envi-
appropriate vocabularies and MLUs by their third birthday ronment
(Andersen, Dunlea, & Kekelis, 1984; Landau & Gleitman, • Model and encourage the child to engage in pretend play
1985). However, social use of language may be disrupted; for • Engage in shared book reading activities
example, children with VI and their conversational partners Such activities foster strong links between the child’s lan-
may have difficulty understanding each other’s referents guage and the surrounding environment.
(Landau, 1997). Other pragmatic impairments include the
extensive, and sometimes inappropriate, use of questions; a Hearing Impairment
paucity of communicative gestures; and extensive use of
imitative speech, repetitions, and verbal routines (Norgate,
CASE STUDY
Collis, & Lewis, 1998; Preisler, 1991). Tadic and colleagues Helen was a very bright toddler. At age 2, she was already say-
(2010) compared children with VI to sighted peers on mea- ing sentences, chatting away to anyone who would listen. She
sures of “structural” language (as measured by the Clinical liked to draw and had great fun playing “family” with her dolls.
When she was 21⁄2, she suffered a serious bout of meningitis,
Evaluation of Language Fundamentals [CELF]) and parent
resulting in hospitalization. Her hearing was tested during her
report of pragmatic impairments. On the whole, the groups
hospital stay, and she was found to have a severe loss in both
did not differ on structural language measures, with the chil- ears. She was fitted with hearing aids before she returned
dren with VI outscoring their peers on the Recalling Sen- home. Her parents were distraught that she had permanent
tences subtest, demonstrating good verbal memory. However, hearing damage as the result of her illness, but they were de-
on the CCC-2, children with VI received consistently poorer termined to minimize any adverse effects. They made sure she
scores on the semantics scale, the social interaction scale, and wore her hearing aids at all times and were careful to speak
all scales of pragmatic functioning (nonverbal communica- clearly and directly to her only when she was fully attending to
tion, inappropriate initiation, coherence, stereotyped lan- them. When she turned 3, they enrolled her in a preschool
guage, and use of context). Scores on the CCC-2 were sig- program that combined hearing-impaired and mainstream chil-
nificantly correlated with a checklist screening for ASD but dren in an intensive language stimulation program. When their
physician saw Helen for a follow-up, she discussed the possibil-
were not related to structural language scores.
ity of a cochlear implant to improve Helen’s hearing. Her par-
Children with VI are less likely to capitalize on nonverbal
ents spent a great deal of time carefully considering the risks
communicative cues to understanding the internal states of versus the benefits of this treatment option.
their conversational partners, leading to the hypothesis that
deficits in theory of mind development may underpin social
communicative challenges. However, in structured tasks, no Helen has one kind of hearing impairment that can pro-
differences have been found between children with VI and foundly affect oral communication. Her story is also a power-
typically developing peers of similar age and verbal ability on ful reminder that even today, common childhood illnesses
measures of either theory of mind and comprehension of can have devastating consequences. Her case also illustrates
non-literal language (Pijnacker, Vervloed, & Steenbergen, the crucial role that families can have in influencing the out-
2012). Nevertheless, such deficits may be evident in more come of disorder. Finally, Helen’s experiences show us the
naturalistic, dyadic exchanges. Mothers of children with VI tough choices families and clinicians often face in selecting
were found to provide more elaborative verbal descriptions the best intervention strategies for a particular child with a
in a shared book reading task and more references to charac- hearing impairment. Let’s look at some of these issues in a
ter mental states relative to mothers of typically developing little more detail.
children (Tadic, Pring, & Dale, 2013). Maternal verbal behav- Hearing impairments may be characterized by both de-
ior was significantly correlated with children’s social com- gree and type. The degree of hearing loss is defined by the
municative competence, suggesting a key role for caregivers audiometric classification of Bess and McConnell (1981).
in scaffolding social communication. Their system is provided in Table 4.1. It is based on the pure
Given these findings, the role of the SLP likely involves tone average, or the average threshold a client displays in pure
facilitating early social-communicative exchanges between tone testing at the “speech” frequencies of 500, 1000, and
parents and their children with VI. This may involve helping 2000 Hz. Helen’s audiogram appears in Figure 4.4.
CHAPTER 4  Special Considerations for Special Populations 115

TABLE 4.1  Categories of Hearing Loss interference in the transmission of sound from the auditory
canal to the inner ear, while the inner ear itself functions nor-
Degree of Hearing Loss Hearing Range (dB SPL)
mally (Northern & Downs, 2002). Conductive losses are usually
Normal 210 to 15 treatable and transient. The most common conductive losses in
Slight 16 to 25 children are associated with otitis media (OM), the inflamma-
Mild 26 to 40
tion or infection of the middle ear. Hearing loss in relation to
Moderate 41 to 55
OM is usually fluctuating and intermittent. Sensorineural losses
Moderately severe 56 to 70
Severe 71 to 90 result from damage to the inner ear. They can be congenital or
Profound 911 result from injury, infection, ototoxicity, or the degenerative
effects of aging. They are not usually directly treatable or revers-
dB SPL, Decibel sound pressure level.
ible, although cochlear implants are used to provide one form
of surgical intervention. Mixed losses are caused by problems in
Frequency in Hertz both the conductive and sensorineural mechanisms. Later, we
125 250 500 750 1000 2000 4000 8000 consider language and literacy development in children with
1500 3000 6000
–10 –10 sensorineural and mixed hearing impairments. Then, we briefly
0 0 consider the impact of OM and conductive losses on language
10 10 and literacy development. Finally, we provide an overview of
auditory processing disorder (APD). This disorder does not
Hearing level in decibels (ANSI, 1969)

20 20
involve perceptual hearing loss but is thought to result from
30 30 atypicalities in neural responses to auditory information
40 40 (American Speech-Language-Hearing Association, 2005a).
50 50
60 60 Sensorineural Hearing Loss
70
Cognitive Characteristics
70
Sensorineural hearing loss is associated with a large number
80 80
of etiologies, although in the majority of cases, the cause is
90 90 unknown (Petersen, Jørgensen, & Ovesen, 2015). Approxi-
100 100 mately 30% of children with moderate to profound losses
110 110 have additional medical conditions that may adversely affect
120 120 cognitive development (Fortnum, Marshall, & Summerfield,
2002). However, intellectual outcomes vary and many chil-
125 125
KHz10 11 12 13 14 15 16 17 18 19 20 dren score within the normal range on appropriate tests of
nonverbal reasoning. Testing individuals with hearing im-
Weber (M, R or L) Lateralization
pairment using appropriate measures can be a challenge.
Frequency in Hertz
Tests such as the Universal Nonverbal Intelligence Test (UNIT;
FIGURE 4.4  Helen’s audiogram.
Bracken & McCullum, 1998) or the Leiter International Per-
formance Scale–Revised (Roid & Miller, 1997) do not involve
Of course an audiogram alone tells us very little about a oral language instructions or responses, correlate with mea-
child’s language competence. Moeller and Tomblin (2015) ar- sures of adaptive behavior functioning, and so may be ideal
gue that hearing loss results in reduced access to linguistic for assessing this population (Meinzen-Derr, Wiley, Phillips,
input, affecting both what is learned and how rapidly it can be Altaye, & Choo, 2017).
learned. They emphasize the need to identify sources of incon-
sistent access to linguistic input. In addition to severity of loss, Language Characteristics
factors may influence consistency of access to input, and thus Unlike the other disorders covered in this chapter, a major
language outcome, include: aided audibility (the degree to consideration for families of children with profound hearing
which hearing aids improve hearing level); how hearing ampli- losses is what language the child will learn and when he or
fication devices are used, including the age at which devices are she will learn it. Historically, this has been a contentious is-
fitted, the duration and consistency of device use; and the quan- sue. Deaf children born to deaf parents are likely to learn
tity and quality of caregiver linguistic input (Moeller & Tomblin, American sign language (ASL) (or British sign language in
2015). Population studies have also emphasized the importance the United Kingdom) and will have exposure to this language
of maternal education, child sex, and the presence of additional from the earliest opportunity. These parents may not view
medical conditions in predicting language outcome (Cleary, their child’s hearing impairment as a disorder at all, but
2009; Ching et al., 2013). Thus, individualized assessment of rather see themselves and their child as culturally different.
language skills and careful consideration of individual needs are Members of deaf culture do not see the need for hearing aids
necessary to plan the intervention program. or cochlear implants, because they have developed a rich
Three types of hearing loss are usually described: conduc- culture and fulfilling social world for members of the com-
tive, sensorineural, and mixed. Conductive losses result from munity, with a fully developed language, set of beliefs, and
116 SECTION I  Topics in Childhood Language Disorders

social mores. As clinicians, we must be sensitive to and children with cochlear implants also appear to be delayed,
respect these cultural views, as we would those of hearing but group means mask considerable within-group variation
clients from culturally diverse backgrounds. (Szagun, 2001; Koehlinger, Van Horne, & Moeller, 2013;
Four issues require further consideration. First, approxi- Tavakoli, Jalilevand, Kamali, Modarresi, & Zarandy, 2015). It
mately 90% of deaf children are born to hearing parents who appears that age of implantation or level of residual hearing
do not know ASL and may not even be immediately aware that can dramatically influence growth; in this case, earlier and
their child has a hearing impairment (Mitchell & Karchmer, more are definitely better, with implantation before 12 months
2004). For these children, there is a real possibility that early old yielding minimal language differences for those with and
language and communication opportunities will be disrupted, without cochlear implants (Pimperton et al., 2016). In terms
with consequences for later language proficiency and social- of morphological structure, the use of signed systems of
cognitive reasoning (Woolfe et al., 2010). A second issue, how- English can be useful in highlighting the sound, spelling, and
ever, is that very limited, and therefore insufficient, high-quality morphological conventions of English (Cleary, 2009), many
evidence exists to determine whether sign language in combi- of which are perceptually non-salient. However, as we’ve
nation with oral language is more effective than oral language seen, outcomes are decidedly mixed and in general, the pic-
therapy alone for young children with sensorineural hearing ture is one of qualitatively similar but often delayed acquisi-
loss (Fitzpatrick et al., 2016). A third issue is that even within tion of grammar relative to hearing peers. Nevertheless, early
ASL communities, there are individual differences in language audiological intervention and, in particular, cochlear im-
competence. Until recently, we have lacked linguistically and plants use in the first year of life yields marked improvement
culturally appropriate assessment instruments, but that is in language growth approaching 100% of gains seen in the
changing (visit www.dcal.ucl.ac.uk for an overview). It is now normal hearing population (Niparko et al., 2010).
possible to assess at least some aspects of language using ASL Content. Like language forms, language content can vary
tests normed on deaf populations (Mason et al., 2010; Enns & dramatically depending on the situation. Overall, there is
Herman, 2011). Finally, improvements in universal screening of some evidence that vocabulary levels may be delayed in hear-
hearing of newborn infants mean that hearing impairment is ing impairment, but that early cochlear implants use can alter
now identified at birth. In 2000, the U.S. Food and Drug Ad- the developmental trajectory such that children achieve typi-
ministration (FDA) approved cochlear implantation for chil- cal levels of receptive vocabulary (Hayes, Geers, Treiman, &
dren as young as 12 months old. These two advances mean that Moog, 2009). A study of deaf children exposed from birth to
many more SLPs will be working with infants and their families British sign language revealed similar growth curves and pat-
to prepare them for cochlear implants. Therefore, the implica- terns of vocabulary development, and indicated that predic-
tions of early implantation and the outcomes for children with tors of language growth in signing hearing impairment chil-
cochlear implants will be considered. dren are similar to those seen in normal hearing populations,
Form. Not surprisingly, the early speech sound inventories namely maternal education and maternal language input
and patterns of canonical babbling observed in children with (Woolfe et al., 2010). Much less is known about the detailed
hearing impairment are different from those of hearing chil- semantic knowledge of children with hearing impairment
dren (Cleary, 2009). However there is enormous variability that and the integrity of their semantic networks.
is contingent on use of amplification or cochlear implants. Children with hearing impairment are reportedly less suc-
Once words are acquired, the sequence of phonemes learned is cessful on experimental tasks of word learning (Davidson,
roughly similar to that of hearing children, although protracted Geers, & Nicholas, 2014). Children with hearing impairment
in development. There is also some evidence that, in addition to make the typical inference that a novel label refers to a novel
typical phonological processes, children with hearing impair- object rather than a familiar one (Lederberg, Prezbindowski, &
ment are more likely to produce voicing errors, extra nasality, Spencer, 2000) but have more difficulty labelling new referents
and initial syllable omission. Many children with severe and and recalling the label after training (Houston et al., 2005).
profound hearing losses will have lower levels of speech intelli- However, rapid word learning is facilitated by good audibility,
gibility, but again rates vary depending on aids/implantation and good phonological memory skills in this population,
and their impact on audibility, listener experience, and topic though direction instruction is likely necessary to enhance vo-
content. In one study that directly compared intelligibility rat- cabulary development (Davidson, Geers, & Nicholas, 2014).
ings of speech in cochlear implants, hearing aid, and normal Use. There is substantial evidence that learning about men-
hearing groups, children with cochlear implants had intelligi- tal states requires rich conversational experience with others; as
bility scores that were indistinguishable from normal hearing such, deaf children born to deaf parents tend to outperform
peers and significantly higher than ratings for the hearing aid deaf children born to hearing parents on measures that tap
group (Baudonck, Dhooge, & Van Lierde, 2010). social-cognitive understanding (Woolfe, Want, & Seigal, 2002).
Spoken language morphology and syntax have long been What is less clear is whether these inefficiencies in social under-
recognized as particularly challenging for children with hear- standing result in difficulties with social interaction or the social
ing impairment, because hearing loss impedes access to the use of language. Falkman and Hjelmquist (2006) reported that
acoustic-phonetic properties in speech that signal grammati- non-native signers were less successful than hearing peers on
cal contrasts (Moeller & Tomblin, 2015). In the early stages, referential communication tasks but that performance was as-
rate of MLU growth is slower than that seen in normal sociated with working memory, rather than social cognition.
hearing children (Geffner, 1987). Rates of MLU growth in These studies highlight that, in terms of language use, early
CHAPTER 4  Special Considerations for Special Populations 117

exposure to language and communication in socially meaning-


ful contexts is more important than hearing status per se. Formal
assessment of pragmatic judgement indicates wide variation in
the skills of children with cochlear implants. Here, implantation
prior to age 21⁄2 makes a big difference to pragmatic competence
(Tobey et al., 2013). Pragmatic skills in everyday contexts have
not been extensively researched and clearly more work is needed
to characterize the pragmatic abilities of children with different
language and amplification experiences.
Literacy. As with spoken language outcomes, literacy out-
comes for children with hearing impairment have changed
dramatically with the introduction of cochlear implants. In the
past, it was not uncommon to find children with hearing im-
pairment leaving school with little functional literacy and a
reading age equivalent to a normal hearing 9-year-old (Conrad, Sign language is often used as a communication modality for
1979). It has also been the case that the gap between hearing children with hearing impairment.
impairment and normal hearing readers has increased over
time; a delay of 1 year at age 8 can become a 4-year delay at age
14 (Harris & Moreno, 2004). This is not surprising given that Implications for Clinical Practice
successful reading depends on establishing grapheme-phoneme There is little doubt that cochlear implants have radically
correspondences, signing children with hearing impairment changed the expected outcomes for children with severe and
have weak to non-existent spoken phonological representa- profound hearing impairments. There is also little doubt that
tions, and manual (sign) phonemes that do not correspond to earlier cochlear implants use is associated with better long-
written graphemes (Lederberg, Schick, & Spencer, 2013). For term outcomes. For example, Niparko et al. (2010) reported
these children, combining reading instruction with fingerspell- that for each year the history of hearing deficit was shortened,
ing and/or explicit instruction in morphological consistencies there was a significantly steeper rate of increase in language
in print may be advantageous (see Lederberg et al., 2013, for comprehension and production scores. As we’ve seen, early
discussion). There is evidence that cochlear implant use can implantation also yields better literacy outcomes, although
result in near normal levels of reading comprehension (Spencer, literacy remains a challenging area of development for chil-
Barker, & Tomblin, 2003), and outcomes are particularly good dren with hearing impairment (Geers, Tobey, Mogg, &
for when cochlear implants are fitted in the first year of life Brenner, 2008). In addition, there is some evidence that
(Pimperton et al., 2016). Interestingly, Harris and Terlektsi delayed exposure to language can alter neurocognitive mech-
(2010) reported that adolescents using hearing aids were better anisms in ways that affect learning. For instance, Conway,
than peers with cochlear implants on measures of literacy at- Pisoni, Anaya, Karpicke, and Henning (2011) reported that
tainment, although they were still below age expectations. This children with hearing impairment who were cochlear im-
could be due to the age at which children received cochlear plants users were less adept than normal hearing peers at
implants (average age of 4) but may also reflect differences in implicitly learning visual sequences. In contrast, early expo-
educational placement; hearing aid users were more likely to be sure to visual language appears to enhance early reading ex-
placed in specialist educational provision, whereas cochlear periences by facilitating visual attention and development of
implants users were more likely to be in mainstream class- letter knowledge (Allen, Letteri, Choi, & Dang, 2014).
rooms. Importantly, measures of oral language significantly The net result is that children with hearing impairment
predict reading outcome, as do measures of family education will be referred to hearing and SLP services at even earlier
and engagement (Lederberg et al., 2013; Nittrouer, Caldwell, ages. Prior to implantation, many infants will be required to
Lowenstein, Tarr, & Holloman, 2012). undergo a trial period of hearing aid use. The clinician needs
Another factor that may contribute to literacy development to work closely with the family and the audiologist during
in this population is early exposure to print. The focus on am- this sensitive period. Spencer (2009) outlined key issues fac-
plification issues and establishment of oral language skills can ing families and professionals. These include maximizing
sometimes mean that less attention is paid to preliteracy skills. device compliance, exploring communication philosophies
Kretschmer and Kretschmer (2001) reported that children with and options (i.e., decisions regarding cochlear implants and
hearing impairment exhibit more emergent literacy behaviors sign language use), and the need for genetic testing. All of this
when they are provided with engaging, print-rich environ- will be occurring within the first year of life, which is a chal-
ments at home and at school, and when their early attempts at lenging and emotional time for any new parent! Working
writing in these environments are similar in form and content with families and infants will involve helping families to rec-
to normal hearing peers. Clearly, exposing children with hear- ognize and respond to their child’s communication attempts,
ing impairment to books and stories, demonstrating the uses of as well as helping them to recognize how their own behavior
writing in everyday activities, and providing attractive writing facilitates language and communication in their child. Once
materials and opportunities will be useful in this population, as the cochlear implant is in place, the clinician works with
in others, for fostering literacy development. families and other professionals to establish treatment goals
118 SECTION I  Topics in Childhood Language Disorders

focused on improving listening skills and responses to new Auditory Processing Disorder
sounds, as well as early speech and language goals to foster in APD is a controversial diagnosis that is not currently part of
a socially meaningful context. Intervention at this early age conventional diagnostic systems (e.g., Diagnostic and Statis-
should pay off in the longer term, although monitoring of tical Manual of the American Psychiatric Association, Fourth
children throughout the school years is recommended, with Edition [DSM-5]) but is increasingly identified in the United
particular focus on developing literacy skills. States, Australia, and the United Kingdom. Increasing interest
in the disorder led American Speech-Language-Hearing
Otitis Media Association (ASHA) to issue a position statement in 1996,
According to the U.S. Department of Health and Human which was updated in 2005. According to ASHA (2005a),
Services, OM is one of the most common diseases of young APD may be defined as difficulties in the perceptual process-
children. Three quarters of all children experience at least one ing of auditory information in the central nervous system as
episode of OM during the preschool years. Children who experi- demonstrated by poor performance in one or more of the
ence OM, particularly with effusion, often suffer some degree of following areas:
conductive hearing loss during the OM episode. It has long been • Auditory discrimination
thought that such mild, fluctuating hearing losses, when experi- • Auditory pattern recognition
enced repeatedly during the sensitive period of language devel- • Temporal aspects of audition
opment, can have a lasting and negative impact on language • Auditory performance in competing acoustic signals
learning. Shriberg and Kwiatkowski (1982b), for example, found • Auditory performance with degraded acoustic signals
that one-third of children enrolled in speech-language interven- Making clear recommendations about the assessment, diag-
tions had a history of recurrent middle ear disease. nosis and treatment of APD is challenging in part due to the
Many have questioned this long held assumption. Results dearth of high-quality research investigations. A recent system-
such as those reported by Shriberg and Kwiatkowski (1982b) atic review (de Wit et al., 2016) rated methodological quality
may be influenced by ascertainment bias; children who attract using ASHA’s Levels of Evidence scheme (Mullen, 2007), which
clinical attention are more likely to experience multiple devel- evaluates reporting of study design, participants, outcomes,
opmental concerns, of which OM may be one. Of the entire and so on. Of 48 eligible papers, only one had received a rating
population of children who experience OM though, it may be of strong methodological quality. The authors report that the
that (all other things being equal) very few have lasting prob- majority of studies used case series designs, and it was often
lems with language development. Population studies are re- not clear if the measures of interest were reliable and valid.
quired to determine the true risk of OM on language out- Inconsistencies in terminology and variable inclusion and ex-
comes. A very large population study of more than 6000 clusion criteria also hampered comparability of findings. None
preschool children reported weak and largely non-significant of the studies included longitudinal follow-up, so the longer
correlations between number or duration of OM episodes and term impact of apparent differences between cases and con-
later language scores, with sociodemographic variables prov- trols on key measures is simply unknown. What was clear was
ing the best predictors of outcome (Paradise et al., 2003). The that children identified as having APD differed from children
same study also used a randomized controlled trial in which in the comparison group on a range of developmental vari-
children with persistent OM were randomly allocated to re- ables and that their difficulties were rarely confined to listening
ceive tympanostomy tubes immediately or after a delay. At skills. The authors therefore conclude that (1) the problems of
follow-up, there were no differences between the two groups in children identified as having possible APD are multimodal and
general cognitive outcomes or any measure of speech or lan- may be caused by cognitive, memory, attention, and language
guage. Importantly, no differences emerged over time; at ages deficits; and (2) the interventions that these children may re-
9 to 11, the two treatment groups performed similarly on an quire might more usefully focus on cognitive or language skills
extensive battery of cognitive, language, and literacy measures rather than only auditory functioning (de Wit et al., 2016).
(Paradise et al., 2007). Even when early delays are detected in Part of the controversy surrounding this disorder appears to
clinically referred samples, these early differences appear to stem from the methods of assessment and the degree to which
wash out over time, with typical language status apparent by they involve speech stimuli (Dawes & Bishop, 2009; DeBonis,
school age (Zumach, Gerrits, Chenault, & Anteunis, 2010). 2015). DeBonis (2015) argued that the most commonly used
Thus, in otherwise healthy children, OM does not confer are substantially influenced by nonauditory factors such as at-
increased risk for long-term language or literacy impairment. tention, memory, executive function, and language. When such
However, it is important to remember that many develop- tasks are included, it is difficult to ascertain the causal connec-
mental disorders, such as Down syndrome, are particularly tion: Is language impaired because of APD, or is performance
susceptible to OM, which may exacerbate language-learning on the task compromised because of limitations in linguistic
difficulties. In these populations, hearing should be moni- ability? Clearly though, many language-based tasks will require
tored closely. In addition, chronic OM, which may be a the abilities listed earlier. ASHA (2005a) clarifies the situation
marker for chronic illness in the preschool years, does appear to some extent by stating:
to increase risk for reduced school readiness (Bell, Bayliss,
Glauert, Harrison, & Ohan, 2016). Thus, clinicians should be Although abilities such as phonological awareness, atten-
aware of a child’s history of OM and monitor language and tion to and memory for auditory information, auditory
other developmental milestones carefully. synthesis, comprehension and interpretation of auditorily
CHAPTER 4  Special Considerations for Special Populations 119

presented information, and similar skills may be reliant that attempt to target auditory processing directly appear to
on or associated with intact central auditory function, have little impact on children’s language and literacy outcomes
they are considered higher order cognitive-communicative (Fey et al., 2011; Strong, Torgerson, Torgerson, & Hulme, 2011).
and/or language-related functions and, thus, are not in-
cluded in the definition of APD. (p. 4) Deaf-Blind
Children with significant deficits in both hearing and vision are
High rates of co-occurring disorder, such as ADHD, fur- considered deaf-blind even though some may have useful re-
ther complicate interpretation of task performance (Bishop & sidual vision and/or hearing. There are two major causes of
Dawes, 2009; DeBonis, 2015; de Wit et al., 2016). This rein- deaf-blindness. One is rubella syndrome, a congenital condition
forces the need for specific measures that are unconfounded that arises when the mother contracts rubella, or German mea-
by language and/or attentional demands. This also raises is- sles, during the first months of pregnancy. Thanks to wide-
sues about APD as a coherent diagnostic entity, or whether the spread immunization for rubella, deaf-blindness attributable to
label reflects the conceptualization of the problem by the pro- this syndrome has been greatly reduced. The second major cause
fessional assessing the child (Bishop & Dawes, 2009). In other is Usher syndrome (Shprintzen, 1997), a rare genetic disorder.
words, a child with poor attention and language delay may be Because of the multisensory deprivation that children with
diagnosed with APD by an audiologist, DLD by a SLP, or deaf-blindness experience, Nelson (1998) recommended us-
ADHD by a clinical psychologist. DeBonis (2015) concludes ing contextualized and dynamic assessment techniques to
that there is no evidence that APD is a coherent diagnostic evaluate skills and identify communicative needs in this pop-
entity and that although some children with a range of devel- ulation. If children have object permanence, it may, in the first
opmental diagnoses experience problems with auditory pro- instance, be possible to develop a system of object referents,
cessing, the contribution of auditory processing (at least as where particular objects may signify people or events (Muller
assessed by current measures) to listening, language, literacy, & Hodges, 2005). Such object referents could be used to de-
and academic attainments is not significant when other devel- velop daily timetables, to help children anticipate changes and
opmental factors are taken into account. Clearly more re- reduce anxiety. It is worth remembering, too, that although
search is needed to resolve these issues. In the meantime, these children have complex and severe disabilities, they may
improving methods of assessment and consensus on diagno- have normal cognition. When accurate cognitive assessment is
sis is a top priority. Critically, measures should be selected that not available or feasible, it is best to set aside questions of basic
do not involve speech or language stimuli and so do not con- intelligence and work to expand conceptual, social, and com-
found poor performance with poor attention. municative skill as far as possible. Some form of augmentative
Even when a problem is diagnosed, there remains no clear and alternative communication (AAC) is almost always useful
consensus about how this should be managed. Bamiou et al. in these cases. Communication devices that emulate the re-
(2006) highlights the dearth of studies investigating treatment ceiving and transmitting modes of tactile finger spelling have
efficacy for APD. These authors further indicate that current been shown to be useful with this population and activate
clinical practices do not aim to treat the auditory deficit directly, neural circuitry involved with language processing (Obre-
but rather aim to reduce the impact of auditory processing tenova, Halko, Plow, Pascual-Leone, & Merabet, 2010). Some
deficits through environmental modification or signal enhance- examples of AAC interventions that may be used with deaf-
ment, such as the use of auditory trainers in classrooms. Studies blind children are outlined in Box 4.1.

BOX 4.1  Augmentative and Alternative Forms of Communication Intervention Techniques


for Children with Deaf-Blindness
Unaided Techniques Print/Braille: Children with significant residual vision can be in-
Signaling: Simple body signals, such as coordinated rocking troduced to print when level of functioning appears appropri-
with reciprocal cues to start and stop ate. Braille may be appropriate for those who can make fine
Gestures: Conventional gestures, such as hi, bye-bye, or head nods tactile discriminations.
Anticipatory cues: Cues used to signal an upcoming action so
that the child may anticipate events, such as rubbing the Aided Techniques
child’s cheek with a washcloth to signal bath time Opticon: This device changes print to a tactile representation
Adapted signs: The child’s hand can be shaped to produce signs, and may assist higher functioning deaf-blind students who
and the child can be encouraged to feel the clinician’s hand shape rely on Braille for academic instruction.
to perceive signs. At first, gross approximations can be accepted Teletouch: This device allows sighted people to type messages on
and then gradually shaped to more conventional signing. a standard keyboard so that each letter is reproduced as Braille.
Finger spelling: Finger spelling can be introduced by first ma- Communication boards: Pictures of symbols can be labeled
nipulating the fingers in playful, interactive games. Eventu- with Braille or more concrete tactile cues and used for both
ally, familiar objects and actions within routines can be la- receptive and expressive communication.
belled with finger-spelled words. Typing and writing: Computers and dedicated electronic aug-
Speech: Children with residual hearing may be taught speech, mentation devices can be used and coupled with speech
but other modes of communication can coexist with speech synthesis software to allow an individual’s message to be
instruction. written out and spoken.
120 SECTION I  Topics in Childhood Language Disorders

DEVELOPMENTAL LANGUAGE DISORDER great deal of spontaneous recovery. Recent research suggests
that age of injury significantly predicts language and literacy
ASSOCIATED WITH ACQUIRED outcome, with younger children showing more rapid initial
NEUROLOGICAL DISORDER recovery followed by poorer outcomes overall (Hanten et al.,
2009; Dennis, 2010). Poor prognosis is also indicated by pre-
CASE STUDY morbid cognitive and adaptive status (Anderson, Godfrey,
Freddie had been a precocious preschooler. In fact, he was so Rosenfeld, & Catroppa, 2012), the location, type, and severity of
bright that his parents had him tested to determine whether injury (as measured by the Glasgow Coma Index at hospital
he could enter school a year early. The results of the assess- admission; Ewing-Cobbs et al., 2006) and by family factors, such
ment, copies of which his parents kept and showed to every as socioeconomic status and family functioning (Anderson
clinician who saw Freddie later, indicated an IQ in the superior et al., 2012; Hanten et al., 2009). There is considerable debate
range and very advanced verbal skills. When Freddie was 7, he as to whether age at injury predicts outcome, with some
experienced a series of seizures for no apparent reason. After
researchers suggesting that earlier injury results in poorer
Freddie was hospitalized several times, including extensive
prognosis, and others arguing that injuries that occur later in
experimentation with drug treatments in order to determine
appropriate dosage, the seizures were partially, but not fully, development may be more debilitating. Comparisons across
controlled. His parents began to notice that Freddie’s speech studies may be difficult due to differences in population ages
was beginning to deteriorate; his sentences got shorter, and and injuries, and the measures used to establish outcome. For
he couldn’t think of the words he wanted to use. His concen- example, Hanten and colleagues (2009) used standardized
tration was poor, and he became increasingly impulsive, so measures of reading comprehension and expressive language
much so that his parents had to lock cabinets and keep dan- to measure outcome in children experiencing TBI between
gerous substances well out of reach. Even when the seizures the ages of 4 and 15 years old. On these measures, children
were fairly well controlled, the language and cognitive prob- injured at earlier ages had poorer overall outcomes. On the
lems did not go away. His parents struggled for years to find other hand, Ewing-Cobbs and colleagues (2006) studied a
a way to release the real, bright Freddie they had known be-
smaller, more homogeneous group of children injured be-
fore the seizures started. They firmly believed that Freddie
tween the ages of 4 and 71 months old and measured out-
was just as clever as he had been, but was locked inside his
own body. Indeed, his nonverbal IQ scores were age appropri- come using standardized tests of academic attainment some
ate, but his expressive language was telegraphic, and he had 5 years later. At this age, using these measures, age of injury
severe comprehension deficits. He had a terrible time at did not significantly predict outcome, whereas severity of
school and, eventually, his parents agreed to place him in a initial injury did. In terms of adaptive outcomes, severe in-
special educational setting so that his language and learning jury earlier in childhood results in poorer quality of life and
needs could be met. community reintegration outcomes (Chevignard, Brooks, &
Truelle, 2010).

As Freddie’s case illustrates, acquired brain damage can have Cognitive Characteristics
severe and long-lasting effects on language, communication, Cognitive outcomes after TBI are variable but are important
and academic success. This section reviews three types of predictors of language ability and adaptive behavior. For ex-
acquired neurological insult that can result in a DLD, before ample, Ewing-Cobbs and colleagues (2006) reported that 48%
considering implications for clinical practice. of children with TBI had nonverbal IQ scores in the bottom
10th percentile. Cognitive deficits frequently co-occur with
Traumatic Brain Injury deficits in attention, executive function, and working memory,
Traumatic brain injuries (TBIs) can be focal in nature. When all of which may affect later skill acquisition (Anderson et al.,
they are, they are usually open-head injuries (such as gunshot 2012), with cascading negative impacts on academic, social,
wounds), and their impact on language development is simi- and behavioral functions. Indeed, children with TBI are more
lar to that described for other focal lesions (see the Focal likely to require increasing levels of school support services
Brain Lesions section). Closed-head injuries, such as those and have lower ratings of academic competence relative to
resulting from blows or collisions, tend to involve diffuse peers with orthopedic injuries 6 years post-injury (Prasad,
damage, affecting large areas of the brain and are the more Swank, & Ewing-Cobbs, 2017).
common type of TBI in childhood. Road accidents and falls
account for the largest proportion of cases, although child Language Characteristics
abuse is an important consideration, accounting for approxi- Gerring and Carney (1992) detailed the language recovery
mately 16 to 33 cases per 100,000 children per year in the first process in the immediate aftermath of trauma. At first, chil-
2 years of life (Narang & Clarke, 2014). Boys are more likely dren tend to be mute and may only follow simple commands.
to engage in risk-prone behavior, less likely to use protective Early language productions often reflect the confused state
devices (e.g., cycle helmets), and more likely to be injured that the child is in and are often dysarthric or nonfluent.
deliberately, resulting in higher incidence of TBI and higher Speech may be slow, and prosody may be affected so that
mortality rates compared with girls (Collins et al., 2013). Im- speech sounds monotonic and “flat.” Swallowing disorders
mediately following injury, children with TBI experience a are also common during this phase of recovery. During this
CHAPTER 4  Special Considerations for Special Populations 121

period, two types of language patterns may emerge. The first or unmotivated, scattered and inattentive, and to have a
is “sparse language production” in which the child does not different personality entirely. Freddie’s family’s response ex-
initiate communication and will only answer questions with emplifies this problem. They keep trying to find the “old”
single words or short phrases. The second is “excess speech Freddie, and think that they could “unlock” him if only they
production” in which the child talks too much and makes could get the right kind of help. One challenge for clinicians,
tangential statements that are off-topic, irrelevant, and some- therefore, is to work with families to deal with the Freddies as
times inappropriate. we find them today, enabling them to establish the maximum
From this point, language function can show rapid im- levels of functional skill and independence.
provement, followed by relatively normal developmental Assessment needs. Identifying a child’s stage of recovery
progression, as evidenced by stable age-standardized scores from brain injury can be important for assessing needs and
over a 10-year period (Anderson et al., 2012). This means planning programs. Blosser and De Pompei (2001) suggested
that narrowing the gap with peers is unlikely, and full recov- that there are three stages to the assessment process in this
ery of language function is rare (Ewing-Cobbs et al., 2006). population:
Factors that further contribute to language and literacy dif- • Phase I: The child is recovering medically, usually in an
ficulties are marked impairments in attention and other as- acute-care facility
pects of executive control, such as working memory, and • Phase II: The child is medically stable and ready to begin
broader cognitive deficits. In general, sentence repetition and rehabilitation
tactile naming are not impaired in individuals with TBI, al- • Phase III: Ongoing assessment is needed in the child’s
though mild deficits in naming, word fluency, and expressive/ educational and daily living settings
receptive grammar are probable (Sullivan & Riccio, 2010). During phase I, assessment will focus on the physical care
Use. Pragmatic language skills are particularly vulnerable in needs that affect treatment, such as respiratory, swallowing,
TBI. Marked deficits in discourse processing are common and or motor control problems. This also is a time to collect case
may include problems with turn-taking, topic maintenance, history data from the family about premorbid functioning—
generating verbal responses, and understanding the intentions the child’s communication and academic strengths and
of others (Ewing-Cobbs & Barnes, 2002). More formal assess- weaknesses prior to the accident—and to help families un-
ment of pragmatic language may also reveal difficulties under- derstand the child’s current condition. In phase II, assess-
standing non-literal language, generating inferences, resolving ment focuses on determining the child’s functional strengths
ambiguous messages, and a heavy reliance on verbatim mem- and needs in behavioral, cognitive, and communicative do-
ory, rather than interpretation, in narrative tasks (see Sullivan mains. Phase III entails using formal and informal methods,
& Riccio, 2010, for review). Pragmatic deficits may be further as we discussed in Chapter 2, to establish baseline functions,
complicated by deficits in mental state reasoning and poor identify goals for intervention, and evaluate change in the
understanding and use of deceptive emotion (e.g., looking therapy program. The Pediatric Test of Brain Injury (Hotz,
happy when feeling disappointed) (Dennis, 2010). 2010) has ten subscales and is designed to assess neurocogni-
Literacy. The extent of literacy impairment may depend tive, language, and literacy abilities that are relevant to the
crucially on the age of injury and the extent to which the child school curriculum of children and adolescents recovering
was already literate prior to injury. As with most other disor- from brain injury. As the child continues to recover, it is also
ders, reading comprehension is more likely to be impaired important to include assessment of higher level language
relative to word reading and decoding skills (Hanten et al., functions known to be vulnerable in children with TBI.
2009). This is perhaps not surprising given that skilled reading During phase III, an assessment of the child’s environment
comprehension requires many of the pragmatic language is also necessary in order to identify the demands and expecta-
abilities known to be impaired in TBI. Oral language deficits tion of the child’s daily living situations (Blosser & De Pompei,
may not be the only factor contributing to literacy outcome, 2001). This assessment can be used to develop a profile of the
however. Although word reading accuracy may be at the ex- most important environmental requirements that should serve
pected level, many children demonstrate reduced reading flu- as the focus for treatment. For example, it may involve helping
ency following TBI. Slowed word recognition in connected to sensitize communication partners to the child’s needs and
text may tax already limited memory capacity, further inter- eliminating barriers in the environment to successful commu-
fering with reading comprehension (Sullivan & Riccio, 2010; nication. Apparently good performance on a standardized test
Krause, Byom, Meulenbroek, Richards, & O’Brien, 2015). may not translate into effective communication in everyday
environments, because standardized test procedures provide
Implications for Clinical Practice support that helps children compensate for the impairments
As Freddie’s story demonstrates, often the hardest thing for in executive functions that frequently disrupt performance in
families and teachers to accept after an acquired brain injury less-structured settings. Looking at language competence in
is that, in many ways, they are dealing with a different person both structured and more unpredictable settings gives us a
than the one they knew before the neurological damage broader picture of the client’s abilities (Sullivan & Riccio, 2010;
took place. Both the child and the adults may feel confused Anderson et al., 2012).
and frustrated that things that came easily before seem im- Finally, clinicians need to take account of the child’s pre-
possible now. The child may seem to be less compliant, “lazy” morbid levels of functioning in developing assessment and
122 SECTION I  Topics in Childhood Language Disorders

BOX 4.2  Assessment Strategies for Traumatic Brain Injury Using World Health Organization
Framework
Impairments in Body Structure and Function • Obtain informant data, such as interviews and rating scales
• Use standardized tests to examine all major areas of cogni- (e.g., Vineland Adaptive Behavior Scales–II [Sparrow, Cichetti,
tive and communicative functioning: & Balla, 2005])
• Intelligence • Identify successful/unsuccessful participation in real-world
• Executive function activities
• Judgment and reasoning • Systematically explore factors that influence performance
• Problem solving on everyday activities, including possible compensatory
• Attention and concentration strategies
• Memory
• Perceptual motor skill Contexts and Environments
• Academic achievement • Document the cognitive and communicative demands of ev-
• Speech eryday environments (e.g., “curriculum based assessments”)
• Language form and content • Evaluate the communication and support abilities of relevant
• Language use (pragmatics) people in the child’s environment
• Systematically manipulate test variables to identify factors • Systematically manipulate environmental factors, includ-
that influence success or failure on standardized tests ing supports/behaviors of key communication partners, to
identify context-specific features that support successful
Activities and Participation participation
• Use structured assessment/observations of individual per-
forming functional activities
Adapted from Turkstra et al. (2005). Practice guidelines for standardized assessment for persons with traumatic brain injury. Journal of Medical
Speech-Language Pathology, 13(2), ix-xxxviii.
World Health Organization. (2001). International classification of functioning, disability and health. Geneva: Author.

treatment plans, which is something that is unique to this can be introduced. These may involve physical response
population. Assessment in the rehabilitation setting should (nodding or other nonverbal gesture) to questions aimed at
include obtaining school records and discussing the child’s orienting to the child to his or her current circumstances (i.e.,
academic status with teachers and parents. Skills in which the date, time, place), basic self-care, and simple visual motor
child was very proficient before the injury could provide activities.
good targets for retraining, because over-learned skills may Once the child has moved into phase II, intervention
be better preserved. However, inconsistent performance is a should emphasize structured tasks. Because of frequent dif-
hallmark of TBI; we can’t assume that just because the child ficulties with attention, concentration, and impulsivity in
was able to do long division before the injury that he or she TBI, the context of these activities should be free of distrac-
will be able to do simpler arithmetic problems post injury. tions, repetitive, predictable, and intrinsically rewarding.
Assessment principles and specially designed tools for chil- Their goal is to develop functional and adaptive behaviors,
dren with TBI are outlined in Box 4.2. and it may include work on language comprehension, simple
Intervention issues. As we’ve seen with most of the disor- verbal problem solving, and the use of self-monitoring to
ders reviewed in this chapter, developing an intervention detect and self-correct errors or request clarification from
program with TBI involves close collaboration with both others when needed.
families and the multidisciplinary team managing the child’s care. In phase III, the child returns to home and school, and the
In TBI, interventions can be divided into two classes: (1) those clinician needs to work more closely with parents and school
that seek to retrain or develop cognitive skills, and (2) those that staff to facilitate the child’s transition back to the learning en-
teach compensatory strategies (Semrud-Clikeman, 2010). Appli- vironment (Mealings, Douglas, & Olver, 2016). Here, the clini-
cation of these approaches is likely to depend on the phase of cian may serve in a consulting role, helping the classroom
recovery the child is in. Unlike other disorders, in the early teacher reintegrate the student into the mainstream program,
stages of recovery, the setting for intervention is likely to be as well as providing collaborative lessons that focus on inte-
in a hospital or rehabilitation setting, and the medical needs grating communication skills within class lessons. For children
of the child at this stage obviously places constraints on the with more severe DLD following a TBI, the clinician may need
nature of therapy offered. At this time, the child is also in a to provide an individualized language or literacy programs
period of spontaneous recovery, and the goal of intervention (Krause et al., 2015), as well as consulting with education staff
is to improve levels of residual function maximally. Sessions to ensure generalization of skills learned in therapy sessions to
should be short and aimed at stimulating one modality at a the classroom environment. Mealings and colleagues (2016)
time, with tactile and motor stimulation preceding visual and interviewed young people with TBI and found their interven-
auditory stimulation. Once a response to stimuli has been tion priorities extended beyond academic goals to increase
established and is more reliable, more functional activities community awareness of TBI, strategies for adapting daily
CHAPTER 4  Special Considerations for Special Populations 123

BOX 4.3  Reintegrating Students with Traumatic Brain Injury into the Classroom
• Plan small group activities to help develop interaction skills. • Decrease distractions in the classroom; if mobility problems
• Clarify verbal and written instructions by reading written instruc- are present, carefully arrange classroom furniture to allow
tions out loud and accompanying verbal instructions with writ- freedom of movement.
ten ones. Repeat and paraphrase often; define unknown terms. • Modify assignments by reducing the number of questions to
• Explain core vocabulary and concepts; pre-teach this infor- be answered or material to be read; let student record lec-
mation in individual sessions. tures, give test answers verbally to a scribe; go over tests
• Pause when giving instructions to allow extra processing time. and explain answers.
• Give the student extra time to respond, because processing • Augment textbooks with pictures and vocabulary lists, high-
speed may be slow. light key information; provide a “podcast” with a summary of
• Avoid figurative language, or explain it when used. textbook information; assign review questions and use recip-
• Give the student a classroom “buddy” to help him or her keep on rocal teaching techniques.
top of instructions, assignments, and classroom transition times. • Teach compensatory strategies.
• Let the student use assistive devices, such as a computer or iPad. • Announce and clarify conversational/lesson topics.
• Help the student “get organized” by having him or her keep • Support communication with gesture, pictures, print, and so on.
a written (or computer based) log of classes, assignments, • Require and expect communication, reinforce all communi-
due dates, and so on; monitor the log regularly. cative attempts; construct opportunities to communicate
• Set aside time for the student to talk to a trusted adult about (e.g., lunch buddies, paired classroom activities).
feelings and frustrations. • Practice higher level reasoning skills in small groups with
• Plan extracurricular activities based on interests before the peers engaged in problem-solving activities.
injury, as well as on current abilities. • Encourage memory skills by teaching strategies, such as cat-
• Avoid direct, confrontational questions in class; ask leading or indi- egorizing, association, rehearsing, visualizing, and chunking.
rect questions (“Tell me about . . .”) to encourage responsiveness.
Adapted from Semrud-Clikeman, M. (2010). Pediatric traumatic brain injury: Rehabilitation and transition to home and school. Applied Neuro-
psychology, 17(2), 116-122.

routines, and planning how to manage the school routine. due to trauma-induced sluggishness or medication, reduced
Adjusting to long-term changes and peer acceptance were also insight into his or her own learning problems, and labile
priorities, thus working with peers may be another avenue the and sometimes unpredictable emotions not always linked
SLP can facilitate in the transition back to education. Semrud- to immediate context (Blosser & DePompei, 2002; Semrud-
Clikeman (2010) has provided some suggestions for helping Clikeman, 2010).
students with TBI reintegrate into the school setting, which are
summarized in Box 4.3. Focal Brain Lesions
Intervention may focus on developing metacognitive Lesions that are focal, or localized to a specific area of the brain,
strategies or retraining clients to use executive control to are usually caused by cerebrovascular accidents (CVAs), such as
monitor their own cognitive processes and regulate learning strokes, and are relatively rare in children; however, children with
behavior (Treble-Barna, Sohlberg, Harn, & Wade, 2016); congenital heart defects are particularly vulnerable to CVAs, and
many strategies for this population are similar to those we premature babies may suffer focal damage as a result of intracra-
use for children with language-learning disorders. Table 4.2 nial bleeding during their first weeks of life outside the womb. A
summarizes some similarities and differences between these body of work by researchers in San Diego has prospectively
two conditions. Later chapters cover more about developing followed the developmental trajectories of language and cogni-
metacognitive and other learning strategies; many of the tion in children with focal lesions, considering outcomes in
techniques we use to develop these skills with children who relation to side and site of lesion and developmental timing of
have more specific DLDs, children with ASD or ADHD, are lesion (Bates, 2004; Dick, Wulfeck, Krupa-Kwiatkowski, & Bates,
appropriate to use with children who have experienced TBI. 2004; Reilly et al., 2004; Wulfeck, Bates, Krupa-Kwiatkowski, &
In addition, language use is most likely to be disrupted in Saltzman, 2004). These studies revealed remarkable language
individuals with TBI, and therapies that target pragmatics plasticity in the developing brain and suggested altered develop-
and social language use may also be appropriate to use with mental trajectories. These were characterized by early delays in
this population. However, it is always important to bear in word comprehension and gesture (although deficits are more
mind that children with TBI have particular learning needs likely following right hemisphere lesions than following left
that are not always present in other disorders and that these hemisphere lesions) and delays in word and sentence produc-
need to be taken into account when adapting therapy ap- tion (although these deficits were more pronounced if lesions
proaches. These may include: more marked memory deficits occurred in left temporal brain regions, as opposed to more
for recent events, potential for physical impairments (paresis frontal areas). These delays were followed by rapid acceleration
or weakness), cognitive impairment, poor retention of new of language function such that, by school-age, children with fo-
information, visual deficits, rapidly changing behavior, inter- cal lesions were largely indistinguishable from typical peers on
nal as well as external distractions, adverse effects on learning measures of vocabulary, grammar, tense-marking, and narrative
124 SECTION I  Topics in Childhood Language Disorders

TABLE 4.2  Differences between Developmental and Acquired Language Disorders


Developmental Language Disorder Acquired Language Disorder
Variable memory problems Severe, recent memory problems, with difficulty carrying over
new learning
Early onset Later onset
Probabilistic differences in brain structure and function Direct evidence of neurological impairment
No pre/post contrast Marked pre/post contrast of abilities, self-perception, and perception
of self by others
Skills and knowledge may show uneven development Some old skills and knowledge remain, but there are inconsistencies
of performance
Physical problems usually include mild motor coordination Physical disabilities may include paresis (weakness) or spasticity
deficits
Basic cognitive skills may be intact Basic cognition is commonly disrupted
Acquisition of new skills may be slow, but what is learned is What is learned may not be retained; much repetition and practice
usually retained using compensatory strategies are needed
Status changes slowly Status may change rapidly during recovery
Visual perceptual problems rarely by visual impairment (VI) Visual problems often include double vision, poor depth perception,
inability to adjust from near (book) to far (black board) vision,
partial loss of vision
Client is distracted by external events Client is distracted by both external and internal events, with
internal events related to the brain damage
Normal or high activity level Recovery from coma may include slowness or lethargy
Seizure medication, which can cause dulling of cognitive Seizure medication may be used to prevent seizures, even if they
function, used only if frank seizures are present have never occurred, and their cognitive dulling effects may
influence learning
Usually aware of own learning problems Injury may cause lack of awareness of learning problems in some
cases
Behavior modification strategies are often effective Organic dysfunction and memory losses may decrease the success
of behavior modification
New learning can often be linked to past learning, although Loss of some long term memory may make linking new learning
memory problems are present to old more difficult
Emotional reactions connected with present situation Emotions can be labile and unpredictable and may not be linked to
immediate situation

Adapted from Blosser, J., & DePompei, R. (1992). Serving youth with TBI: Circumventing the obstacles to school integration. Mini-seminar
presented at the annual convention of the American Speech-Language-Hearing Association, San Antonio, TX; Blosser, J., & DePompei, R. (2002).
Pediatric traumatic brain injury (ed 2). San Diego, CA: Singular Publishing Group.

production (Bates, 2004; Reilly et al., 2004; Wulfeck et al., 2004). lesions did not differ from the comparison group on standard-
More recent studies have suggested that although language per- ized measures of grammar and vocabulary (see also Demir et al.,
formance on standardized tasks may be within normal limits, 2015). Thus, most children with focal lesions make more or less
children with early left hemisphere lesions may have subtle defi- complete recoveries in terms of speech, language, and commu-
cits in language processing relative to peers (Raja et al., 2010) and nication, although the clinician should be alert to subtle deficits
that measures of narrative may be particularly sensitive to subtle in higher-level language tasks that may interfere with academic
language differences (Demir, Levine, & Goldin-Meadow, 2010; achievement. Although most academic research has focused on
Demir, Rowe, Heller, Goldin-Meadow, & Levine, 2015). At older the impact of lesion size, type (cerebrovascular versus periven-
ages, laterality of lesion site may be more influential in complex tricular), and location (left versus right hemisphere) as explain-
narrative tasks; children with left hemisphere lesions were found ing individual differences in language outcome, recent work has
to make more morphological errors, use few syntactic construc- highlighted the role of caregiver input in supporting language
tions and less complex syntax, as well as impoverished story growth (Rowe, Levine, Fisher, & Goldin-Meadow, 2009). A series
settings (Reilly, Wasserman, & Appelbaum, 2013). In contrast, of studies have shown that amount and complexity of parental
children with right hemisphere lesions were indistinguishable decontextualized talk (talk about things removed from the here
from typically developing peers, apart from reduced use of com- and now) at 30 months old significantly predicts child language
plex syntax. Demir and colleagues (2010) also report narrative outcomes at 5 years old (Demir et al., 2015; Goldin-Meadow
deficits that extend beyond syntax; in this research, children with et al., 2014). Furthermore, exposure to speech-gesture combina-
early focal lesions produced shorter stories that were structurally tions in narrative enable children to produce more structured
less complex, used less diverse vocabulary, and made fewer infer- narratives (Demir, Fisher, Goldin-Meadow, & Levine, 2014); the
ences regarding the cognitive states of the story characters. These impact of parental input is particularly enhanced for children
deficits occurred despite the fact that the children with focal with focal lesions and is most evident in complex language tasks,
CHAPTER 4  Special Considerations for Special Populations 125

such as narrative. Therefore, intervention programs could alternative means of communication, such as sign language,
usefully focus on developing caregiver input and encouraging which can be used in conjunction with verbal language (Deonna,
use of nonverbal cues (such as gesture) to enhance language Prelaz-Girod, Mayor-Dubois, & Roulet-Perez, 2009). Some
learning and language processing in this population (Goldin- children with Landau-Kleffner syndrome may develop prob-
Meadow et al., 2014). lems with behavior and social interaction that are similar to
autism spectrum behaviors (Deonna & Roulet-Perez, 2010;
Seizure Disorders (Landau-Kleffner Syndrome) Riccio et al., 2016). For these children, an emphasis on devel-
Some children, like Freddie, go through a period of normal oping social communication skills, imaginative play, and
development, then suddenly or gradually lose language skills emotional understanding of self and others may be high
in association with a seizure disorder. Landau-Kleffner syn- priorities. Developing language and conversational skills in
drome, also known as acquired epileptic aphasia, is a rare everyday social settings is also recommended.
seizure disorder that causes severe language disorder. Onset is In Landau-Kleffner syndrome, visual processing is still
usually between 3 and 6 years old, although it can occur any relatively normal and can be used to support oral language.
time in childhood. It is often misdiagnosed because overt In addition to sign language, symbol systems may be used for
epileptic seizures are uncommon. The typical clinical picture communication or to provide visual cues to help structure
is of a child who loses language skills rapidly after a period of the learning environment (i.e., classroom and therapy time-
normal development, and comprehension is usually most tables). Reading may also be possible, although children ac-
severely affected. The difficulties in language understanding quiring literacy after Landau-Kleffner syndrome may benefit
are variable; a recent review of fourteen case studied reported more from whole word strategies as opposed to more typical
that 86% of cases demonstrated continued expressive lan- phonics based approaches (Great Ormond Street Hospital for
guage deficits, whereas 50% had receptive language impair- Children, 2010).
ments (Riccio, Vidrine, Cohen, Acosta-Cotte, & Park, 2016).
A further 50% to 57% had deficits in auditory working DEVELOPMENTAL LANGUAGE DISORDER
memory and verbal memory, whereas the majority had poor ASSOCIATED WITH OTHER
reading fluency and comprehension and 50% exhibited aca-
demic challenges in mathematics. Deafness may be suspected
NEURODEVELOPMENTAL DISORDERS
but ruled out after a hearing test is conducted; the problem is One reason for the recent debate about terminology in this
not with hearing, but with making sense of the auditory in- field (Reilly et al., 2014) has been the growing recognition that
put. Difficulties with comprehension often occur along with “specific” language impairment is anything but specific! Chil-
difficulties speaking. In the most severe cases, children may dren presenting with DLD as a group are very likely to demon-
lose speech altogether and may resort to gesture to convey strate deficits in other aspects of development, including mo-
meaning. Therefore, selective mutism may also be considered tor development (Flapper & Schoemaker, 2013), executive
given the child’s history of verbal communication. However, function (Henry, Messer, & Nash, 2012), peer relations (Mok,
in the case of Landau-Kleffner syndrome, there is a genuine Pickles, Durkin, & Conti-Ramsden, 2014), and social, emo-
loss of language. Although the language impairment may be tional, and behavioral difficulties (Yew & O’Kearney, 2013).
relatively circumscribed with nonverbal cognitive abilities Although there is little doubt that rates of comorbidity are
intact, Landau-Kleffner syndrome may be associated with higher than would be expected in the general population, there
behavioral difficulties and stereotypes that resemble autism is much debate surrounding the causal relationships between
and may further confuse the clinical picture (Deonna & DLD and other neurodevelopmental disorders, such as ASD
Roulet-Perez, 2005; Deonna & Roulet-Perez, 2010). Thus, and ADHD (Bishop, Snowling, Thompson, Greenhalgh, &
when a child presents with severe comprehension deficits and CATALISE consortium, 2016). There is now substantial evi-
language regression, referral to a pediatric neurologist is war- dence that at least some of the genetic factors that confer risk
ranted so that a sleep electroencephalogram (EEG) may be for language impairment are shared across developmental dis-
carried out in order to demonstrate EEG abnormalities. orders (Rodenas-Cuadrado, Ho, & Vernes, 2014); therefore, a
All children with Landau-Kleffner syndrome have lan- prudent approach would be to assess language functioning in
guage disorders and require assessment and support from the any child referred for developmental evaluation, even if behav-
SLP. Prognosis is more optimistic for children in whom onset ior is the primary presenting complaint.
occurs after the age of 6, after language has been established.
However, outcomes for children with onset in the preschool Autism Spectrum Disorders
years are particularly poor, and significant language deficits ASD is an umbrella term that encompasses a range of disor-
may persist into adulthood. Pharmacological treatments may ders that are characterized by core impairments in social
be effective, and there is some evidence that early diagnosis communication and a restricted repertoire of interests and
and prompt medical intervention is important for improved behaviors (American Psychiatric Association, 2013). In the
prognosis, but outcome is variable and controlled clinical tri- past, terms such as Asperger syndrome, pervasive developmen-
als are lacking (Mikati & Shamseddine, 2005). When lan- tal disorder–not otherwise specified, autism, and autistic disor-
guage has regressed and comprehension deficits persist for der all came under this umbrella. However, the most recent
more than a few weeks, it is essential to provide children with revision to the DSM-5 (American Psychiatric Association,
126 SECTION I  Topics in Childhood Language Disorders

BOX 4.4  Diagnostic Criteria for Autism Spectrum Disorders


A. Persistent deficits in social communication and social inter- 2. Excessive adherence to routines, ritualized patterns of
action across contexts, currently or by history, not ac- verbal or nonverbal behavior, or excessive resistance to
counted for by general developmental delays, and mani- change (such as motoric rituals, insistence on same route
fested by the following: or food, repetitive questioning, or extreme distress at
1. Deficits in social/emotional reciprocity; ranging from ab- small changes)
normal social approach and failure of normal back and 3. Highly restricted, fixated interests that are abnormal in
forth conversation through reduced sharing of interests, intensity or focus (such as strong attachment to or preoc-
emotions, or affect to total lack of initiation of or response cupation with unusual objects, excessively circumscribed
to social interaction or persevering interests)
2. Deficits in nonverbal communicative behaviors used for 4. Hyper- or hypo-reactivity to sensory input or unusual in-
social interaction; ranging from poorly integrated verbal terest in sensory aspects of environment (such as appar-
and nonverbal communication, through abnormalities in ent indifference to pain/heat/cold, adverse response to
eye contact and body-language, or deficits in understand- specific sounds or textures, excessive smelling or touch-
ing and use of nonverbal communication, to total lack of ing of objects, fascination with lights or spinning objects)
facial expression or gestures C. Symptoms must be present in early childhood (but may not
3. Deficits in developing and maintaining relationships; rang- become fully manifest until social demands exceed limited
ing from difficulties adjusting behavior to suit different capacities or be masked by learned strategies in later life)
social contexts through difficulties in sharing imaginative D. Symptoms cause clinically significant impairment in social,
play and in making friends to an apparent absence of in- occupation or other impairment areas of current functioning
terest in people E. These disturbances are not better explained by intellectual
B. Restricted, repetitive patterns of behavior, interests, or ac- disability (intellectual developmental disorder) or global de-
tivities as manifested by at least two of the following, cur- velopmental delay. Intellectual disability and autism spec-
rently or by history: trum disorders frequently co-occur; to make comorbid diag-
1. Stereotyped or repetitive speech, motor movements, or noses of autism spectrum disorder and intellectual disability,
use of objects (such as simple motor stereotypes, echo- social communication should be below that expected for
lalia, repetitive use of objects, or idiosyncratic phrases) general developmental level
From American Psychiatric Association. (2013). Diagnostic and statistical manual of mental disorders (DSM-5), ed 5, Washington, DC: Author.

2013) abolished these labels in favor of one diagnostic term: Kanner suggested a biological basis for the disorder, but he
ASD. Changes to the core symptom structure and the intro- also remarked that the most of the mothers of these autistic
duction of severity criteria, to help families and practitioners children had university educations (unusual at the time) and
make sense of where a particular child sits on this very broad that the disorder might at least partially result from the care
spectrum, are outlined in Box 4.4 and Table 4.3. received by these “refrigerator” mothers. It took years of grass-
Leo Kanner (1943) first described 11 case studies of children roots efforts on the part of devoted parents and research that
with this disorder, highlighting profound social disturbances, demonstrated that parents of children with ASD do not cause
qualitative differences in language development and language the patterns of behavior that characterize ASD to dispel this
use, and remarkably good memory for details and rote learning myth (Anderson & Hoshino, 2005). Today we know that ASDs
(see Donvan & Zucker, 2010, for a fascinating account of the life are strongly influenced by complex genetic risk factors that
of Donald Triplett, the first case study). alter neurobiological development (Berg & Geschwind, 2012).
The influence of epigenetics, the interaction between genes
and the environment, is also increasingly recognized (Loke,
Hannan, & Craig, 2015). Parents are important people, be-
cause they can positively influence the language and commu-
nication development of their children (Bang & Nadig, 2015),
but are in no way responsible for causing the disorder.
Of all the developmental disorders we’ve talked about,
ASD is probably the most intensively researched and the
most variable in terms of cognitive profile, language ability,
comorbid diagnoses, and eventual outcomes. This variability
makes it extremely difficult to identify proximal causes of
disorder and especially challenging to develop and evaluate
treatment approaches for this population. The descriptions
that follow will give you a flavor of this heterogeneity and
highlight some key cognitive and language characteristics of
Children with ASD have difficulty developing communication children with ASD. However, it is important to remember
skills. that, for this population, as with many others we’ve talked
CHAPTER 4  Special Considerations for Special Populations 127

TABLE 4.3  Severity Levels of Autism Spectrum Disorder


Severity Level for Autism Restricted Interests and Repetitive
Spectrum Disorder Social Communication Behaviors
Level 3 Severe deficits in verbal and nonverbal social Inflexibility of behavior, extreme difficulty
“Requiring very substantial communication skills cause severe impairments in coping with change, or other restricted/
support” functioning; very limited initiation of social interac- repetitive behaviors markedly interfere
tions and minimal response to social overtures with functioning in all spheres. Great
from others. distress/difficulty changing focus or action.
Level 2 Marked deficits in verbal and nonverbal social Inflexibility of behavior, difficulty coping
“Requiring substantial communication skills; social impairments apparent with change, or other restricted/repetitive
support” even with supports in place; limited initiation of behaviors appear frequently enough to be
social interactions and reduced or abnormal obvious to the casual observer and interfere
response to social overtures from others. with functioning in a variety of contexts.
Distress and/or difficulty changing focus or
action.
Level 1 Without supports in place, deficits in social commu- Inflexibility of behavior causes significant
“Requiring support” nication cause noticeable impairments; difficulty interference with functioning in one or
initiating social interactions and demonstrates clear more contexts. Difficulty switching be-
examples of atypical or unsuccessful responses tween activities. Problems of organization
to social overtures of others; may appear to have and planning hamper independence.
decreased interest in social interactions.
From American Psychiatric Association. (2013). Diagnostic and statistical manual of mental disorders (DSM-5), ed 5, Washington, DC: Author.

about so far, the differences between children are as striking Esler, Kanne, and Hus (2014) found that early language re-
as the similarities and an assessment or intervention ap- gression (whether full or sub-threshold) predicted poorer
proach that works well with one child may be completely functional outcomes in middle childhood. Studies suggest
inappropriate for another child with the same ASD diagnosis. that by the first year, infants at high risk for autism are devel-
oping in a way that limits opportunity for language develop-
Early Communication ment in social interaction contexts. A failure to develop
There is increasing interest in identifying the earliest signs of joint attention may be particularly detrimental to language
autism in infancy. A research strategy that has really taken off acquisition (Mundy, Sullivan, & Mastergeorge, 2009; Yoder,
in the last few years is to recruit babies who are at genetic risk Watson, & Lambert, 2014).
of developing ASD by virtue of having an older sibling with
the disorder (see Jones, Gliga, Bedford, Charman, & Johnson, Cognitive Characteristics
2014, for a review). These studies have yielded some surpris- The popular media often depicts individuals with ASD as
ing findings; for the most part, within the first year of life possessing super abilities in skills such as music, math, or
infants who later receive a diagnosis of ASD are indistin- drawing. Unfortunately, individuals with these “splinter
guishable from low-risk peers in terms of social behavior skills” form a minority of the ASD population, and, as many
(Elsabbagh & Johnson, 2016). Instead, subtle differences in parents will tell you, these amazing abilities rarely contribute
motor development (Leonard et al., 2014), visual attention to better academic or adaptive outcomes. The more typical
(Sacrey, Bryson, & Zwaigenbaum, 2013), and interest in picture is that many children with ASD also have ID, with
objects may be the earliest signs of atypical development, al- 50% to 70% of children with ASD obtaining scores on non-
though these behaviors may be markers of other develop- verbal IQ measures of less than 70 (Matson & Shoemaker,
mental disorders as well. However, sometime between the 2009). Increasingly, ASD is diagnosed in children with IQ
end of the first year and the child’s second birthday, differ- ranges in the average (28%) or above average range (3%)
ences in social interaction behaviors become more apparent, (Chita-Tegmark, 2016; Charman et al., 2011). Although IQ is a
and some children show signs of regression (Ozonoff et al., good indicator of prognosis, in that those with lower overall
2014). These aberrant social behaviors include reduced eye cognitive abilities tend to have less favorable outcomes (Howlin,
contact, social smiling, social interest, and social imitation, 2005), two issues should be borne in mind. First, very low IQ
reduced response to their own name, fewer responses to bids often occurs in the context of a comorbid disorder that may
for joint attention, and atypical regulation of emotion (see also adversely affect outcome. Second, high IQ scores do
Zwaigenbaum, Bryson, & Garon, 2013, for review). This not always predict outcome; recent studies have found poor
combination of social behaviors often co-occurs with delays correlations between IQ and academic achievement (Estes,
in gestural communication and language, although some Rivera, Bryan, Cali, & Dawson, 2010) or adaptive behavior
children appear to develop language typically and then expe- (Charman et al., 2011).
rience regression of those language skills (Barger, Campbell, Numerous cognitive theories have been put forward in an
& McDonough, 2012; Pickles et al., 2009). Goin-Kochel, attempt to explain the core behavioral features of ASD. None
128 SECTION I  Topics in Childhood Language Disorders

have directly attempted to explain variation in language skill, 2015; Pickles et al., 2014). Several studies report on a small
although cognitive deficits in these areas would likely have a minority of children who make rapid gains in the early years
negative impact on language development and language pro- and attain “Optimal Outcomes” (e.g., Fountain, Winter, &
cessing. As in many other disorders we’ve seen, executive func- Bearman, 2012). Another important finding from longitudi-
tions are very often impaired in children with ASD, although nal research suggests that a significant percentage of children
working memory may be an area of strength. Intriguingly, with minimal language skills in early life develop at least some
measures of executive functioning do not always neatly map spoken language skills by the age of nine with only 9% to 15%
on to the symptom profile we’d expect; for example, problems of children remaining nonverbal in later childhood (Hus,
with cognitive flexibility are clearly an issue for many individu- Pickles, Cook, Risi, & Lord, 2007; Norrelgen et al., 2015).
als with ASD and should be related to restricted interests and The degree of language variability within the autism spec-
rigid behaviors. However, correlations between symptom pro- trum has led some to question whether there are distinct
files and standardized measures of executive functioning are subgroups of children with ASD, who may be distinguished
disappointingly low (Geurts, 2010). Weak central coherence by different “neurocognitive phenotypes” (Jansen et al., 2013;
has been put forward as an explanation of cognitive differences Tager-Flusberg & Joseph, 2003). Remember that a phenotype
seen in ASD (Happe & Frith, 2006). For example, success on is the set of observed characteristics that are associated with
measures of visual processing that require attention to detail in a particular genetic profile; “neurocognitive” suggests that
the context of poor integrative functioning are often seen. particular patterns of brain development and cognition may
Problems integrating information in context could lead to also be associated with a particular genetic profile. Tager-
many problems with discourse processing, although other Flusberg (2006) has argued that there are at least two distinct
explanations have also been put forward (cf. Norbury, 2004; phenotypes within ASD: (1) an autism language normal
Eberhardt & Nadig, 2016). Atypical attention allocation (Chita- (ALN) phenotype, in which language form is unimpaired
Tegmark, 2016), as well as delayed attentional learning (Field, and typical patterns of neuroanatomical asymmetry are ob-
Allen, & Lewis, 2016), may contribute to impaired processing served, and (2) autism language impaired (ALI), in which
of linguistic and social information. Finally, deficits in social language form impairment is seen in association with anom-
cognition, or in understanding other people’s minds, are the alies in left hemisphere brain structure and function (De
most well-known and well supported cognitive deficits that Fossé et al., 2004; Verhoeven et al., 2012). The degree to which
distinguish children with ASD from children with other devel- ALI and more specific DLDs overlap at behavioral, cognitive,
opmental disorders. Problems understanding the intentions of neurological, and genetic levels is a matter of intense debate
speakers have also been explicitly linked to problems learning (Taylor, Maybery, Grayndler, & Whitehouse, 2015; Tomblin,
new words (Parish-Morris et al., 2007) and understanding 2011). For our purposes, the underlying source of this vari-
non-literal language (Martin & McDonald, 2004), although a ability matters less than our knowing that in addition to the
recent body of work suggests that difficulties interpreting some social, cognitive, and behavioral challenges that a child with
figurative language may also be due to underlying syntactic ASD may face, a large percentage of children with ASD (ap-
deficits (Whyte, Nelson, & Scherf, 2014) or an inability to sup- proximately 47%; Loucas et al., 2008) will have additional
press literal meanings (Chouinard & Cummine, 2016). impairments in phonological processing and grammar that
Evidence from twin studies has suggested that there is un- resemble non-autistic children with more specific DLDs.
likely to be a single cognitive impairment that can explain the Form. One consistent finding in the autism literature is
range of strengths and deficits observed in ASD (Brunsdon & that, once some verbal language is acquired, articulation of
Happé, 2014; Happe, Ronald, & Plomin, 2006), although few speech sounds is relatively unimpaired across language phe-
studies have taken a developmental approach to exploring the notypes (Kjelgaard & Tager-Flusberg, 2001; Shriberg, Paul,
relationships between areas of cognitive development, and Black, & van Santen, 2011). However, performance on more
how they influence one another, over time (Pellicano, 2009). complex tests of phonological processing is less clear-cut.
Therefore, the clinician should be aware of the different cogni- Numerous investigators have reported that a significant
tive challenges that may be present when making assessment proportion of children with ASD perform poorly on mea-
and intervention plans for children with ASD, because these sures of nonsense word repetition, which taps phonological
additional impairments may influence task performance. short-term memory (Bishop et al., 2004; Harper-Hill,
Copland, & Arnott, 2013; Kjelgaard & Tager-Flusberg, 2001;
Language Characteristics Tager-Flusberg, 2006; Tager-Flusberg & Joseph, 2003; White-
Structural language skills are extremely variable in ASD. The house, Barry, & Bishop, 2008; Williams, Payne, & Marshall,
range of abilities across all age groups extends from nonverbal 2013). Other aspects of phonological processing appear to be
to verbose; it is not unusual to find standardized scores on more universally challenging for individuals with ASD. For
verbal measures spanning 50 to 70 points even within the example, performance on more meta-linguistic tasks of pho-
same study (cf. Toichi & Kamio, 2003). Early language trajec- nological awareness, such as rhyme awareness, is very poor
tories are particularly variable prior to age 6, whereby they (Nation et al., 2006). In addition, atypical patterns in process-
become more stable (Pickles, Anderson, & Lord, 2014). Ex- ing and producing speech prosody are seen across the range
pressive and receptive language largely develop in tandem of speakers with ASD from childhood to adulthood (Chan &
(Hudry et al., 2014; Kwok, Brown, Smyth, & Oram Cardy, To, 2016; Gebauer, Skewes, Hørlyck, & Vuust, 2014; Peppé,
CHAPTER 4  Special Considerations for Special Populations 129

Cleland, Gibbon, O’Hare, & Castilla, 2011), although these scoring highly on the Colorado Meaningfulness Scale, which is
may be more prominent at the sentence level than at the level an index of how many different contexts a word can be used in.
of an individual word (Järvinen-Pasley, Peppé, King-Smith, However, the findings of many of these studies are somewhat
& Heaton, 2008). Finally, although sound substitutions are hampered by large within group variation and have failed to
rare, distortions of speech sounds and voicing patterns have distinguish semantic profiles within ASD that might align with
been noted in adult speakers with ASD (Shriberg et al., 2001). specific neurocognitive phenotypes.
Relative to phonology and lexical knowledge, deficits in mor- Use. Pragmatic deficits are universal within ASD (Baird &
phosyntax and grammar are more pronounced for children with Norbury, 2015) and may be particularly evident in higher level
ASD in general (Boucher, 2012; Eigsti, de Marchena, Schuh, & discourse processing and narrative tasks. Individuals with ASD
Kelley, 2011) and for those with the ALI phenotype in particular have significant deficits in conversational skill (Adams, Baxendale,
(Tek, Mesite, Fein, & Naigles, 2014). Children with ASD use fewer Lloyd, & Aldren, 2005; Hale & Tager-Flusberg, 2005; Nadig et al.,
grammatical morphemes than non-ASD peers to mark verb 2010; Paul et al., 2009), demonstrating either too many or too few
tense and agreement (Roberts et al., 2004), although errors of initiations, poor topic maintenance, fewer contingent conversa-
commission are rare (Eigsti, Bennetto, & Dadlani, 2007; Roberts tional responses, and non-contextual or socially inappropriate
et al., 2004). Analyses of spontaneous language samples indicate utterances. Such deficits are also evident in narrative tasks, with
that many children with ASD produce short and grammatically ASD individuals producing higher proportions of contextually
simple sentences relative to non-ASD peers, despite producing irrelevant propositions (Norbury, Gemmell, & Paul, 2014), poor
equivalent numbers of utterances (Eigsti, Bennetto, & Dadlani, referencing throughout the narrative (Diehl et al., 2006; Losh &
2007). Furthermore, many are characterized by uneven syntactic Capps, 2003; Losh & Gordon, 2014), and ignoring the motivations
and morphological development, where some sub-skills are in- of characters or events (Tager-Flusberg, 1995). Understanding of
tact and others are delayed or atypical (Park et al., 2012). More language in context is regarded as particularly problematic for
structured tasks involving sentence repetition also reveal poorer individuals with ASD as evidenced by poor understanding of
performance for individuals with ALI (Norbury et al., 2009; figurative and metaphorical language (Happe, 1997; Norbury,
Riches et al., 2009), highlighting the utility of this task as a marker 2004; 2005), poor inferencing skills (Jolliffe & Baron-Cohen, 2000;
for language impairment in ASD. Impaired sentence comprehen- Norbury & Bishop, 2003), and reduced ability to resolve ambigu-
sion is particularly striking in ALI (Loucas et al., 2008), although ous language (Happe, 1997; Norbury, 2005). Few studies have
there are very few studies exploring comprehension of particular explored the extent to which language-based pragmatic deficits
syntactic structures (Durrleman, Hippolyte, Zufferey, Iglesias, & align with core language profile; those that do so report that chil-
Hadjikhani, 2015; Perovic, Modyanova, & Wexler, 2013) dren with ALI are more likely to have difficulties with higher level
Content. At the broadest level, vocabulary scores are consis- pragmatic language skills than ALN peers (Norbury, 2005; Whyte
tently depressed in a large proportion of children with ASD & Nelson, 2015).
across a number of studies, relative to typically developing peers Literacy. Given the pronounced difficulties with social-
(Kjelgaard & Tager-Flusberg, 2001; Norbury, 2005; Loucas et al., interaction and oral language development experienced by
2008; Lindgren, Folstein, Tomblin, & Tager-Flusberg, 2009). On many children with ASD, it is perhaps not surprising that
the other hand, for a substantial minority of individuals with much less attention has been paid to the reading abilities of
ASD, receptive vocabulary is considered to be a “peak of ability” children with this diagnosis. Early reports centered on the
(Mottron, 2004). However, what these children know about the impressive abilities of some young children with ASD to read
words in their vocabularies and the processes by which they words given limited verbal and cognitive abilities. Such chil-
acquire them may be qualitatively different relative to typical dren were given the label “hyperlexia,” and there is continued
peers (Arunachalam & Luyster, 2016; Henderson, Powell, Gareth debate over the definitions of hyperlexia and the extent to
Gaskell, & Norbury, 2014). For example, Norbury, Griffiths, and which hyperlexic reading profiles are specific to ASD or may
Nation (2010) found that children with ASD, matched to a occur in other developmental disorders (Grigorenko, Klin, &
comparison group on both raw scores and standard scores of Volkmar, 2003; Nation, 1999). More recent investigations have
the British Picture Vocabulary Scales (BPVS), scored more revealed much more varied reading patterns in larger cohorts
than 1 SD below the comparison group on a measure of verbal of children with ASD; for example, Nation and colleagues
definitions. Other investigators have suggested that, in general, (2006) found that approximately 30% of the children with
the underlying organization of the semantic system in ASD is ASD that they studied were impaired on both word and non-
atypical and impoverished. For instance, individuals with ASD word reading measures, while most of the children had defi-
show reduced priming effects for semantically related words cits in reading comprehension. Asberg and Sandberg (2012)
(Kamio et al., 2007) and do not use semantic information found similar patterns of individual differences in word
to facilitate encoding and recall (Bowler et al., 1997; Tager- reading skill as those seen in children without ASD. Norbury
Flusberg, 1991). Henderson, Clarke, and Snowling (2011) and Nation (2011) found that, although younger children
propose that semantic representations are activated during with ASD and good oral language skills had age-appropriate
priming, but a difficulty with top-down processing prevents word reading abilities, standard scores reduced over time such
appropriate meaning selection for ambiguous words. Interest- that significant differences between individuals with ASD
ingly, Schafer, Williams, and Smith (2013) found that vocabu- and their typically developing peers were evident by adoles-
laries of children with ASD contained relatively fewer words cence. One possible reason is that many of these children had
130 SECTION I  Topics in Childhood Language Disorders

reading comprehension difficulties and so, as they grow older, genetic risk of ASD and/or displaying early signs of autism
they may not have been able to use written text to learn new (Green et al., 2015; Rogers et al., 2014). Wallace and Rogers
words to the extent that their peers could (Lucas & Norbury, (2010) reviewed evidence-based practices for intervening with
2014). Early language skills are an important predictor of infant populations in other disorders and have highlighted
later reading ability in ASD (Davidson & Ellis Weismer, 2014; essential components of treatment programs for infants and
Ricketts, Jones, Happé, & Charman, 2013). toddlers that could be applied to intervention programs for
In most of the disorders we’ve talked about, you’ll have infants with ASD. These are (1) parent involvement in inter-
noticed strong links between oral language skills and aspects vention, including parent-child interaction therapies that in-
of reading; those with poor phonological skills tend to have volve coaching parents to alter their own communication and
problems with decoding text (i.e., non-word reading and responses to the child in order to maximize language and
spelling), whereas those with poor semantics and grammar communication opportunities (cf. Green et al., 2010; Pickles
tend to have greater difficulty with reading comprehension. In et al., 2016); (2) individualization to each infant’s develop-
ASD, those links are not quite so strong. Norbury and Nation mental profile; (3) focusing on a broad rather than a narrow
(2011) divided an ASD cohort into different language pheno- range of learning targets; and (4) temporal characteristics in-
types (ALI and ALN); though the ALN children as a group volving beginning as soon as the risk is detected and providing
had better literacy skills than the ALI group, there were chil- greater intensity and duration of the intervention. These
dren in each group who had difficulties with word/non-word developments require clinicians to be alert to the early warn-
reading and children in each group with above average perfor- ing signs of disorder and develop skills in working with and
mance. In addition, despite age-appropriate language scores, through parents as children are diagnosed at ever earlier ages.
children in the ALN group were not as skilled as typically de-
veloping peers in making inferences and monitoring their Conditions Associated with Autism Spectrum Disorder
reading comprehension. It would seem that, in addition to Social communication disorder (formally pragmatic
language, aspects of autistic cognition may also influence lit- language impairment or semantic-pragmatic disorder). Rapin and
eracy development (Brown, Oram-Cardy, & Johnson, 2013). Allen (1983) were the first to describe a communication profile
For example, most stories require the reader to draw infer- in which children with DLD did not have a primary deficit with
ences about characters’ mental and emotional states in order language form, but substantial impairments in language con-
to understand why they do the things they do. Skilled readers tent and use; these children were labeled with “semantic prag-
are also required to maintain this narrative thread over hun- matic disorder” (see also Bishop & Rosenbloom, 1987). Bishop
dreds of pages, integrating information across text and with (1998) later proposed the term pragmatic language impair-
their own experiences and world knowledge. Finally, skilled ment (PLI) because semantic and pragmatic deficits did not
readers recognize when a passage does not make sense or co- always co-occur. Children with PLI were described as having
here with what they’ve been reading and have strategies for intermediate symptom profiles that are not severe enough to
recovering uncertain meaning. All of these are skills most warrant a diagnosis of autism, but whose language difficulties
people employ effortlessly when reading and yet are likely to affect communication, social interaction, and use of language
pose significant challenges for readers with ASD. Lucas and in context. From the beginning, there has been significant
Norbury (2014) extended this work to look at comprehension clinical and theoretical debate as to whether these children
at the sentence and passage level in children with ASD with constitute a unique diagnostic entity or whether they have a
and without language impairment. They found that, just as social-cognitive deficit that is more consistent with a diagnosis
for typical populations, a combination of decoding skill and of ASD. Part of the difficulty in resolving this debate is that
oral language ability predict comprehension success. diagnostic criteria for ASDs are constantly evolving, and many
would argue are more inclusive than they once were (Bishop,
Implications for Clinical Practice Whitehouse, Watt, & Line, 2008). DSM-5 introduced a
Special considerations for assessment and intervention with new diagnostic category “Social (Pragmatic) Communication
ASD are considered throughout this text. Here, we only stress Disorder” in which the diagnostic criteria overlap to a consid-
that, as with hearing impairment and cochlear implants, there erable degree with PLI (Baird & Norbury, 2015). Four key
is considerable effort now to identify children with ASD at criteria are that children have deficits in (1) using language for
younger ages so that appropriate interventions and family social purposes; (2) changing communication to match the
support can be put in place. There is some evidence that early context and/or needs of the listener; (3) following the rules of
intervention attenuates symptom severity (Oono, Honey, & narrative and/or conversational discourse; and (4) under-
McConachie, 2013; Peters-Scheffer, Didden, Korzilius, & Sturmey, standing what is not explicitly stated. These deficits should
2011), but evidence for interventions in infancy are currently occur in the absence of ASD, ID, or significant impairment in
lacking, perhaps in part due to the challenges involved in ac- language structure (American Psychiatric Association, 2013).
curately diagnosing children with ASD at this age and the These exclusion criteria are particularly controversial, because
rapidly changing social and communication profiles of chil- restricted interests and behaviors, as well as core linguistic
dren younger than 3 years old (Siller et al., 2013; Vivanti, Prior, deficits, are often associated with social communication disor-
Williams, & Dissanayake, 2014). However, recently published ders (Norbury, 2014; Swineford, Thurm, Baird, Wetherby, &
findings show promise when targeting infants who are at high Swedo, 2014).
CHAPTER 4  Special Considerations for Special Populations 131

It may be most helpful to think of social (pragmatic) com- syndrome (Klin, Volkmar, Sparrow, Cicchetti, & Rourke,
munication as a descriptive term rather than a diagnostic 1995; Williams, Goldstein, Kojkowski, & Minshew, 2008).
category, which can be applied to children with DLD and There is an assumption that the pragmatic deficits of both
ASD alike (cf. Rapin & Allen, 1983). Indeed, Norbury, Nash, groups stem from deficits in right hemisphere brain func-
Baird, and Bishop (2004) found that children with DLD who tions, but neurobiological evidence for this view in children
were not thought to have significant pragmatic deficits nev- is lacking. There is considerable controversy about whether
ertheless achieved lower scores on pragmatic sub-tests of the nonverbal learning disabilities is a distinct diagnostic entity,
CCC-2 (Bishop, 2003) than typical peers. However, these low with many unanswered questions about defining criteria, the
pragmatic scores were entirely in keeping with their low pervasiveness of the academic, social and psychopathological
scores on structural language scales. In contrast, children difficulties, the source of the nonverbal learning disabilities
with SCD profiles who do not have ASD will have problems syndrome, the degree to which it overlaps with other condi-
with conversation, using language context to resolve ambigu- tions, and the long-term stability of this profile of verbal/
ity, and difficulties with narrative that are out of keeping nonverbal abilities (Volden, 2013). When verbal IQ (VIQ) is
with their structural language deficits. Individuals with ASD greater than non-verbal or performance IQ, we need to ensure
who did not have structural language impairment may also that children with this profile are properly assessed for prag-
show disproportionate weaknesses on pragmatic measures, matic function so that if pragmatic skills are compromised
particularly in more naturalistic tasks that are less structured and result in social or academic limitations, they may qualify
and where interlocutor behavior may be more difficult to for SLP services. To this end, measures such as the CCC-2
predict. Finally, most children with ASD will have social com- (Bishop, 2003) may be most effective in highlight pragmatic
munication deficits that are far worse than would be pre- and social communication deficits in children with good
dicted given language ability. However, it is important to structural language skills (Volden & Phillips, 2010).
remember that many children with ASD have additional lan-
guage difficulties and that these children also have rigid inter- Attention Deficit Hyperactivity Disorder
ests and behaviors that will interfere with pragmatic/social In recent years, the number of children receiving services for
communication development. ADHD and the amount of research relating to this disorder
Nonverbal learning disability. Rourke (1995) and Rourke has grown exponentially (Bishop, 2010). This is not surpris-
and colleagues (2002) advanced the idea that there is a distinct ing; ADHD is a debilitating and chronic condition that af-
clinical syndrome in which children show a profile of skills that fects the child’s ability to control attention and behavior in an
is opposite of the one seen in DLD. Children with nonverbal optimal and adaptive manner (Redmond, 2016a). DSM-5
learning disabilities have normal verbal IQs, but nonverbal IQs diagnostic criteria (American Psychiatric Association, 2013)
that are significantly below verbal scores. You will probably think focus on two components of the disorder: (1) symptoms of
that this is quite an unusual cognitive profile; of all the disorders inattention and (2) symptoms of hyperactivity/impulsivity.
we reviewed so far, apart from Williams syndrome, if verbal and Children can have predominantly Inattentive or Hyperactive
nonverbal abilities are discrepant, it is usually verbal abilities that subtypes, or they can have a combined subtype, in which
are more impaired. In fact, Rice (2016) reported that 11.6% of criteria are met in both domains (Box 4.5). The symptoms of
Tomblin’s epidemiological sample (Tomblin et al., 1997) had ADHD must be present for at least 6 months, with an onset
profiles in which language skills were better than expected for before the age of 12, be present in two or more contexts (i.e.,
level of nonverbal IQ. Thus the profile is fairly common, but the both at home and at school), and significantly interfere with
extent to which children with this profile experience functional social, academic, or vocational functioning before a diagnosis
impacts, or form a distinct clinical entity is a matter of much can be made. According to DSM-5, the expected prevalence
debate. Investigations have been hampered by lack of consis- rate is approximately 5% to 7% of school-aged children with
tency in diagnostic definitions. In general, children with nonver- boys outnumbering girls 3:1. When ADHD is primarily of the
bal learning disabilities are reported to have particular difficulties inattentive type, problems with poor attention and concen-
with visual-spatial, visual-motor, and fluid reasoning measures tration, distractibility, poor organizational skills, and diffi-
compared to children with other developmental disorders culty completing tasks without close supervision occur. Chil-
(Semrud-Clikeman, Walkowiak, Wilkinson, & Christopher, dren with the hyperactivity/impulsivity type may be described
2010). Other reported deficits include bilateral tactile-perceptual as fidgety, always on the go, interrupting and talking inces-
and coordination deficits, deficits in novel problem solving and santly, and acting without thinking.
concept formation, and poor mechanical arithmetic skills in the
context of well-developed rote verbal capacities, proficient single Cognition
word reading, and fluent speech. Behavioral descriptions have The majority of children with ADHD will have nonverbal
highlighted deficient social perception and judgment, verbosity, IQ scores within the normal range, although ADHD has
repetitive speech, and problems adapting to novel situations also been observed in children with ID and children with
(Rourke & Tsatsanis, 2000). exceptional IQs (above 120; Katusic et al., 2011). Interest-
These descriptions highlight difficulties with pragmatic ingly, Katuisic and colleagues (2011) did not find any differ-
aspects of language and have invited comparisons with chil- ences between children with high, low, and average IQ scores
dren diagnosed with “high-functioning” autism or Asperger with respect to severity of ADHD symptomatology, rates of
132 SECTION I  Topics in Childhood Language Disorders

BOX 4.5  Diagnostic Criteria for Attention Deficit Hyperactivity Disorder*


A. A persistent pattern of inattention and/or hyperactivity-­ c. Often runs about or climbs in situations where it is
impulsivity that interferes with functioning or development, inappropriate (e.g., in adolescents or adults, may be
as characterized by (1) and/or (2) limited to feeling restless).
1. Inattention: Six (or more) of the following symptoms have d. Often unable to play or engage in leisure activities quietly.
persisted for at least 6 months to a degree that is incon- e. Often “on the go” acting as if “driven by a motor” (e.g.,
sistent with developmental level and that directly affects is unable to be or uncomfortable being still for extended
social and academic/occupational activities. time, as in restaurants, meetings; may be experienced
a. Often fails to give close attention to details or makes by others as being restless or difficult to keep up with).
careless mistakes in schoolwork, at work, or during f. Often talks excessively.
other activities. g. Often blurts out answers before questions have been
b. Often has difficulty sustaining attention in tasks or play completed (e.g., completes people’s sentences; cannot
activities. wait for turn in conversation).
c. Often does not seem to listen when spoken to directly. h. Often has difficulty awaiting turn (e.g., while waiting
d. Frequently does not follow through on instructions. in line).
e. Often has difficulty organizing tasks and activities. i. Often interrupts or intrudes on others (e.g., butts into
f. Characteristically avoids, seems to dislike, and is reluc- conversations, games, or activities). May start using
tant to engage in tasks that require sustained mental other people’s things without asking or receiving per-
effort. mission; for adolescents and adults, may intrude into
g. Frequently loses objects necessary for tasks or activities. or take over what others are doing).
h. Is often easily distracted by extraneous stimuli. B. Several inattentive or hyperactive-impulsive symptoms were
i. Is often forgetful in daily activities, chores, and running present prior to age 12 years
errands. C. Several inattentive or hyperactive-impulsive symptoms are
2. Hyperactivity and impulsivity: Six (or more) of the following present in two or more settings (e.g. at home, school, work;
symptoms have persisted for at least 6 months to a degree with friends or relatives; in other activities)
that is inconsistent with developmental level and that di- D. There is clear evidence that the symptoms interfere with, or re-
rectly affects social and academic/occupational activities. duce the quality of, social, academic, or occupational functioning
a. Often fidgets with or taps hands or squirms in seat. E. The symptoms do not occur exclusively during the course of
b. Often leaves seat in situations when remaining seated schizophrenia or another psychotic disorder, and are not
is expected (e.g., leaves his or her place in the class- better explained by another mental disorder (e.g. mood dis-
room, in the office or other workplace, or in other situ- order, anxiety disorder, dissociative disorder, personality
ations that require remaining in place). disorder, substance intoxication or withdrawal).
*Note, only five symptoms required for adolescents and adults. Onset of symptoms must occur before age 12.
From American Psychiatric Association. (2013). Diagnostic and statistical manual of mental disorders (DSM-5), ed 5, Washington, DC: Author.

stimulant medication, or rates of comorbid disorder. Chil- other words, children with ADHD find it difficult to wait for
dren with high IQs were more likely to have highly educated something desirable, even if the payoff for waiting brings
parents and, as a group, had significantly higher scores on greater reward. For example, if given the choice between $2 now
measures of literacy. It is also important to remember that, or $20 tomorrow, children with ADHD are far more likely than
across developmental disorders, rates of comorbidity with peers to take the money and run (Aase & Sagvolden, 2006)! The
ADHD are high and the interactions between ID and ADHD ability to defer gratification underlies our ability to sustain at-
in these populations have not been fully explored. tention and work consistently; without this ability, it is very
Despite normal range IQ, there are differences in the cogni- difficult to learn to control impulses, and distracting behavior
tive profiles of children with ADHD that may affect language results. These problems may be exacerbated with lower levels of
learning and language processing. For a long time, disruptions arousal, which may lead to “sluggish” performance and a lack of
in the development and deployment of executive functions effort (Sergeant, 2005). In reality, it is likely that the complex
were thought to be the core cognitive deficit in ADHD (Barkley, behavioral profile that characterizes ADHD can only be ex-
1997). You’ll remember that executive functions are cognitive plained by multiple cognitive deficits; from our point of view,
processes associated with the frontal lobes of the brain that en- we need to know how deficits in executive functioning, motiva-
able us to remember, plan, organize, and inhibit irrelevant tion, and arousal may affect language development, and how
information/responses in order to achieve our goals. However, these cognitive deficits will influence service delivery.
not all children with ADHD demonstrate impairments on mea-
sures of executive functioning (Wilcutt et al., 2005). Executive Language
functions that are most likely to be impaired in ADHD include Although many children with ADHD do not present with
inhibition, working memory, and planning. Cognitive theories any additional language impairments, language profiles are
of ADHD are being refined, and researchers and clinicians are variable and rates of comorbidity are higher than would be
recognizing the role of reward processing and motivation, as expected in the general population (Redmond, 2016a), al-
well as delay aversion in understanding ADHD behavior. In though others have reported that it is only when language
CHAPTER 4  Special Considerations for Special Populations 133

impairments and speech sound disorders occur together that speech sound production. Redmond (2011) reported that
there is an increased rate of comorbid ADHD (McGrath clinical markers of specific language impairments, such as
et al., 2008). The nature of the relationship between DLD and tense marking, sentence repetition, and narrative, reliably
ADHD is a matter of debate. One possibility is that the two distinguished children with ADHD from those with primary
disorders may share at least some biological risk factors. For DLDs. However, the ADHD group did not differ from typical
example, neural circuits in the frontal lobe of the brain have peers on overall levels of verbal ability; one study of children
been implicated in ADHD, and at least partially overlap with with comorbid ADHD and language impairment indicate
neural circuitry involved in language production (Nicolson & minimal differences on grammatical markers, such as tense
Fawcett, 2007). Another possibility is that the cognitive and marking, and better performance on sentence repetition rela-
behavioral deficits associated with ADHD can disrupt lan- tive to peers with DLD only (Redmond, 2015). Why ADHD
guage learning and/or language processing. Imagine the tod- would have a protective effect on sentence processing is un-
dler who is irritable, disruptive, and constantly on the go— clear; it could reflect greater likelihood of service provision,
opportunities for engaging and interacting with this child in but this is speculative at the moment and requires replication
a way that supports language growth may be limited (not to and further investigation. Using a similar participant design,
mention the exhausted and frustrated parent may struggle to Cardy, Tannock, Johnson, and Johnson (2010) found that
communicate optimally)! However, poor language skills may both ADHD and DLD groups had difficulties with rapid
lead to behaviors that are characteristic of ADHD; longitudi- temporal processing of auditory stimuli, but that the children
nal studies have consistently demonstrated that early lan- with additional ADHD had greater difficulty on nonverbal
guage deficits increase risk for later attention and behavior tests of processing speed than DLD counterparts.
difficulties (Peyre et al., 2016), and that this direction of ef- Content. Studies involving in-depth assessment of the vo-
fects is much stronger than prediction from early attention cabulary and semantic skills of children with ADHD are lack-
and behavior to later language (Petersen et al., 2013; Petersen, ing. In contrast, studies of adults with reported histories of
Bates, & Staples, 2015). Think of it like this: If you were asked attention difficulties has revealed that those with ADHD were
to sit through a lecture on particle physics, it wouldn’t be long less accurate at mapping semantic features and slower to re-
before your mind began to wander and you started fidgeting. spond to lexical labels than both typical adults and adults
Direct, behavioral assessment of ADHD is not recom- with a history of language impairment only (Alt & Gutmann,
mended because candidate measures have not demonstrated 2009). These results suggest that it is likely that receptive vo-
adequate psychometric properties of validity, reliability, or cabulary scores will be in the average range, but that children
sensitivity and specificity. Instead, ADHD symptoms are with ADHD may have subtle difficulties rapidly accessing the
typically documented using parent- and teacher-reported lexicon or making connections between words. Such difficul-
checklists (see Redmond, 2016a, for review). Teachers consis- ties may be most evident when flexible word knowledge is
tently rate children with DLD as having increased ADHD needed, for example, in understanding humor and nonliteral
symptom profiles relative to parents; Redmond (2016b) ar- language, making inferences, and understanding metaphor.
gues that this highlights the inability of many checklists to Use. The ability to use language in socially appropriate
distinguish DLD and ADHD. One reason is endorsement of ways is most likely to be a problem for children with ADHD
items, such as “has trouble listening” or “fails to complete as- (Hawkins, Gathercole, Astle, The CALM Team, & Holmes,
signments on time,” which may reflect language limitations 2016; Staikova, Gomes, Tartter, McCabe, & Halperin, 2013).
rather than ADHD per se (Redmond, 2016b). Removal of Using the Children’s Communication Checklist (Bishop 1998;
these items improved diagnostic discrimination between 2003), researchers have found that children with ADHD are
DLD and ADHD, without reducing diagnostic accuracy of reported to have significant pragmatic language difficulties,
ADHD relative to typically developing children. Nevertheless, sometimes indistinguishable from peers with ASD (Bishop &
removing these items may hamper identification of truly co- Baird, 2001), although unique profiles have also been reported
morbid cases. In addition, such items may usefully signal to (Geurts et al., 2008). A broad spectrum of deficits, including
parents and practitioners the functional impact of language narrative, inferencing, and understanding ambiguous lan-
disorder within the classroom. Thus, such information is vi- guage, has been identified, even after controlling for structural
tal, but how it is used to inform treatment decisions, and in language abilities (Staikova et al., 2013). Problems with inap-
particular pharmaceutical intervention for ADHD, requires propriate initiation, interruption, difficulty maintaining a
careful consideration. topic, and responding with appropriate amounts of informa-
Regardless of the causal relationship between the two, the tion are also likely to be evident in the conversations of chil-
clinician needs to be alert to the fact that many, though not dren with ADHD relative to comparison groups (Bishop &
all, children referred because of problematic behavior may Baird, 2001; Mikami, Jack, Emeh, & Stephens, 2010). Difficul-
have additional language impairments. It is also important to ties with pragmatic aspects of language have been found to
consider whether and how additional ADHD symptoms im- mediate social skills deficits in this population (Leonard,
pact child language. Let’s consider what the pattern of im- Milich, & Lorch, 2011; Staikova et al., 2013).
pairment is likely to be. Literacy. Rates of comorbidity between ADHD and read-
Form. There is no evidence that children with ADHD as a ing disorders are extremely high; the prevalence rates for each
group have disproportionate difficulties with phonology or disorder alone in the general population is approximately
134 SECTION I  Topics in Childhood Language Disorders

5%, whereas the rates of comorbid disorder are 25% to 40% interventions; drug treatments of choice include methylphe-
(Wadsworth, DeFries, Willcutt, Pennington, & Olson, 2015). nidate (Ritalin), dextroamphetamine (Dexedrine), amphet-
Numerous studies have attempted to identify the genetic and amine and dextroamphetamine (Adderall), and pemoline
cognitive risk factors that are specific to each disorder and (Cylert). There is considerable evidence that drug treatments
those that are potentially shared. McGrath and colleagues are successful in reducing the adverse behaviors associated
(2011) reported that processing speed was the only cognitive with ADHD, at least in the shorter term (MTA Cooperative
variable with significant unique relationships to both reading Group, National Institute of Mental Health Multimodal Treat-
disorders and ADHD dimensions, particularly inattentive ment, 2004). Their success over the longer term appears to be
aspects of ADHD. This finding highlights the multiple cogni- influenced by drug compliance; therefore, the SLP should be
tive skills that are necessary for skilled fluent reading; limita- prepared to work closely with the child’s family, physician, and
tions in processing speed may result in slow and labored school nurse about the medication regime and any changes in
reading of single words in connected text. prescription or behavior. The effectiveness of behavioral in-
As in all other populations, reading comprehension is in- terventions is decidedly more mixed (Young & Amarasinghe,
fluenced by both word recognition skills and oral language 2010), although most guidelines for best practice advocate
skills; however, aspects of ADHD behavior also seem to pre- treatment protocols that combine medication with behavioral
dict differences in reading comprehension skills (Cutting, interventions. Overall, treatment tends to result in more fa-
Materek, Cole, Levine, & Mahone, 2009). In particular, defi- vorable long-term outcome relative to no treatment, although
cits in executive functioning can adversely affect how the rarely to typical levels (Shaw et al., 2012). Behavioral treat-
child approaches the reading task itself, particularly in the ments include family therapy and support to deal with chal-
child’s ability to effectively plan and organize reading tasks lenging behavior and to foster good language and communi-
(reading headings, linking pictures with text, reading intro- cation experiences, interventions aimed at modifying the
ductions and summary paragraphs, predicting what might environment at home and at school to maximize attention
happen), as well as monitoring comprehension (realizing and minimize distraction, and direct behavioral interventions
when a word or passage doesn’t make sense and taking steps with the child to help him or her internalize rules, develop
to improve comprehension through utilizing surrounding strategies for planning and organizing work, and managing
context, use of a dictionary, and so on) (Locascio, Mahone, his or her own behavior. Specific strategies for the school-aged
Eason, & Cutting, 2010). Miller and colleagues (2013) found child with ADHD are outlined in Box 4.6.
that after controlling for word reading abilities, children with
ADHD were poorer than typically developing children at re- Selective Mutism
calling central elements of a story, with negative conse- The SLP is usually the first professional consulted when a
quences for comprehension. Working memory completely child is not speaking at school. Selective mutism may be diag-
mediated the relationship between ADHD symptoms and nosed in a child who consistently does not speak in certain
recall of central ideas; it seems likely that inattention prohib- situations, such as school, where there is an expectation for
its children with ADHD from making all necessary text con- speech, but does speak normally in other situations, like at
nections, and working memory compounds this problem by home (American Psychiatric Association, 2013). DSM-5 con-
making it more challenging to hold previous story informa- siders selective mutism under Anxiety Disorder (as opposed
tion in memory while updating the mental representation to Language Disorder) and further stipulates that selective
when new information comes on line. mutism must persist for more than 1 month (not including
Finally, many studies exploring the relationship between the first month of school) and cannot be accounted for by a
ADHD and reading disorders have focused on the overlap- DLD or by unfamiliarity with the language environment. It is
ping genetic contributions to disorder, but such studies tell also recommended that bilingual children are not diagnosed
us important things about environmental influences as well with selective mutism unless the mutism persists beyond
(Hart et al., 2010). For instance, the child’s disruptive behav- 6 months or is evident in both languages (Toppleberg, Tabors,
ior can have implications for the learning environment; sit- Coggins, Lum, & Burger, 2005). It is a relatively rare disorder,
ting quietly with a book is something the child with ADHD with prevalence rates of 0.3 to 0.8 per 1000 (American Psychi-
may find particularly challenging, reducing opportunities atric Association, 2013) and, unlike other DLDs, it is much
for exposure to text. Remember, too, that level of maternal more common in girls, with a gender ratio of 2:1 (McInnes,
education has been associated with reading outcomes for Fung, Manassis, Fiksenbaum, & Tannock, 2004).
children with ADHD (Katusic et al., 2011), suggesting that Social anxiety is rarely the only problem for children with
early and consistent exposure to books and literacy as a plea- selective mutism, and language impairments are frequently
surable experience may help to increase motivation to read present; 60% to 75% of children with selective mutism have
in these children. experienced some form of language deficit (Sharkey &
McNicholas, 2008). This strongly suggests that self-
Implications for Clinical Practice consciousness about communicative abilities plays a part in
You may have noticed that ADHD diagnosis is on the rise maintaining the disorder. Assessment of language in a child
and almost every classroom will include children with ADHD. with selective mutism is likely to be challenging, because
Most of these children will be receiving pharmacological most children are reticent to speak when they feel they are
CHAPTER 4  Special Considerations for Special Populations 135

BOX 4.6  Classroom Strategies for Children with Attention Deficit Hyperactivity Disorder
Universal Level of Intervention • Parent involvement: Provide frequent, brief home-school
• Model and practice explicit strategies for memorization, test- communication focused on progress toward goals and on
taking, study, and active reading solving problems before they grow
• Verbal and written practice focused on reading comprehension
instruction Tertiary (Individualized) Level of Intervention
• Monitoring of how to organize notebooks and binders and • Computer-assisted instruction provides students with ADHD im-
written work mediate feedback, one-to-one attention, and content presented
• Use planners and calendars in an interesting way
• Develop and teach clear school-wide or class-wide expectations • Well-constructed computerized instruction uses color and design
• Teachers use prompts and signals to remind students to follow to help the student focus on critical information, breaks material
rules down into smaller chunks to promote mastery, provides immedi-
• Staff practice active supervision, scanning for problem areas ate feedback, and addresses specific instructional objectives
or interactions, and interacting frequently with students to re- • Functional behavioral assessment to identify behavior(s) of
duce problem behaviors greatest concern and the triggers for those behaviors
• Implement plan to reinforce positive or pro-social behavior
Secondary (Targeted) Level of Intervention and reduce problems
• Peer tutoring: Benefits children with ADHD by providing individ- • Encourage self-monitoring by teaching children to observe
ual attention, a self-determined pace, and frequent prompting and record their own behaviors (such as on-task behavior)
and feedback • Use a reminder system, such as the Motiv-Aider (www.
• Providing choices: Can increase engagement and work com- habitchange.com), an electronic pager-type device that sends
pletion for children with ADHD a silent pulsing signal and does not require verbal teacher
• Note taking: Teach students to create an outline based on reminders
lecture materials, including main idea and supporting details, • Provide checklists of important tasks or steps for students to
and to teach the effective use of self-questioning complete tasks
ADHD, Attention deficit hyperactivity disorder.

under the spotlight. Therefore, initial investigations may cen- family therapy, and self-modeling techniques, in which the
ter on taking a detailed case history from the parents, detail- child listens to him or herself speaking in situations in which
ing where, when, and with whom the child does speak and he or she is usually mute (these and other therapeutic tech-
obtaining examples of the child’s communicative efforts in niques are outlined in Johnson & Wintgens, 2016). McInnes
different contexts for transcription of spontaneous speech and Manassis (2005) suggested that intervention should take
(McInnes et al., 2004). Unobtrusive observation of the child into account the child’s social anxiety and begin by encourag-
playing alone, or with parents and siblings, may also give an ing the child to articulate rote language (numbers, days of the
indication of the child’s language abilities. week) or answer simple, factual questions (What color is
The most successful treatments are reportedly those that this?) rather than asking questions that involve self-disclosure
combine behavioral and pharmacological interventions, al- (What is your favorite color?). In addition, public speaking
though there is limited research on the efficacy of this ap- should progress in stages, at first involving a parent or one
proach (Sharkey & McNicholas, 2008). Cognitive behavioral person that the child does talk to and increasing confidence
therapy (CBT) is one preferred behavioral intervention that with speaking to a familiar person (teacher), then group of
has yielded some promising results, although outcomes may familiar people (classmates) before tackling unfamiliar peo-
be variable (Lang et al., 2016). For instance, Oerbeck, Stein, ple (restaurant or shop). Techniques that have been used
Pripp, and Kristensen (2015) followed up 24 children who with this population are summarized in Box 4.7. Longitudi-
had completed a 6-month program of CBT for 1 year post- nal studies report improvements in the core symptoms of
treatment. The good news was that as a group, immediate selective mutism over time; although rates of psychiatric dis-
posttreatment gains had been maintained and 50% of the order, especially social phobia, remain high and prognosis is
group no longer had diagnoses of selective mutism. The less particularly poor when there is a family history of selective
encouraging news was that one-third of the children still met mutism (Steinhausen, Wachter, Laimbock, & Winkler Metzke,
criteria for selective mutism, with minimal change in school 2006; Keeton & Crosby Budinger, 2012).
speaking behavior. The age at which treatment was started
and severity of selective mutism symptoms were significant DEVELOPMENTAL LANGUAGE DISORDER
predictors of response to treatment. Behavioral interven- ASSOCIATED WITH EXTREME
tions, including CBT, should always be considered with input
from a multidisciplinary team (psychologist, psychiatrist,
ENVIRONMENTAL DISADVANTAGE
SLP, teacher) in close collaboration with families. Strategies DLDs that result from maternal substance abuse (such as
may include language therapy, positive reinforcement for alcohol) or from parental maltreatment (such as abuse
speaking, desensitization to anxiety-provoking situations, and neglect) are some of the most tragic aspects of our
136 SECTION I  Topics in Childhood Language Disorders

BOX 4.7  Strategies for Intervening with Selective Mutism


. Stimulus fading: Audience or setting changes, rather than the child
1 c. Create communication games to elicit speech from child;
a. Child talks alone with trusted adult (parent) and anxiety is intro- for example, barrier games or Simon Says.
duced (e.g., clinician stands outside leaving the door ajar so that d. Positively reinforce all verbal communication.
the child may be heard). Child is encouraged to keep talking. 3. Desensitization: Child gets used to thought of doing some-
i. Gradually increase proximity to child. thing that he or she would not previously have considered.
ii. Avoid direct eye gaze. a. Child allows others to hear recording of voice.
iii. For older children, make a specific, non-threatening b. Child speaks to friends/family on telephone, before face-
speech target, such as counting, or days of the week, to-face meeting.
until comfortable with other person present. c. Child speaks to class teacher in person in whispered voice.
2. Shaping: Child’s behavior starts to change d. Ensure child is aware of targets and can monitor suc-
a. Start with nonverbal communication with clinician (or key- cesses.
worker) in minimal anxiety situation. i. Use motivators, such as stickers.
b. Gradually increase child participation. e. When progressing, change only one thing at a time; if child
i. Increase eye contact. is comfortable speaking with a friend at home, try speak-
ii. Increase voicing and volume. ing with teacher at home or speaking to friend in class, but
iii. Increase number/length of words and messages. not speaking to teacher in class.
Adapted from Johnson, M., & Witgens, A. (2016). The selective mutism resource manual, ed 2, London: Speechmark Publishing.

clinical practice, because these kinds of disorders could Recent research using brain imaging techniques reveals that
have been prevented. As Joseph’s story indicates, these fac- environmental disadvantage can have a profound effect on
tors often operate in concert to produce a range of long- structural and functional brain development, especially in the
term developmental problems. This section outlines the domains of language and prefrontal executive control (Tomalski
communication patterns seen in children exposed to these & Johnson, 2010). However, the causal pathways ways from
hazards to understand how they might influence clinical socio-economic disadvantage to aberrant neural development
decision making. Two major types of environmental dis- to language dysfunction are multiple and complex (Ellwood-
advantage are discussed: maternal substance abuse and Lowe, Sacchet, & Gotlib, 2016). It is important to remember that
maltreatment. it is not necessarily the substance itself or the lack of parental
warmth and contact that can lead to these changes, but the envi-
ronmental circumstances that are not conducive to child devel-
opment. Other factors include poor diet, increased exposure to
CASE STUDY accidents and other risks, and a lack of stimulating opportunities
Joseph was born to a mother who had been severely alco- and experiences. Individuals from deprived backgrounds are
holic during her pregnancy. During his stay in the hospital, it also less likely to seek help from professional services, or to com-
was noted that he had some dysmorphic facial features, in- ply with clinical or educational recommendations, making this a
cluding microcephaly (small head size), micrognathia (small particularly challenging client group. Finally, disadvantaged so-
jaw), a thin upper lip with an indistinct philtrum, and a flat cial backgrounds may reflect genetic risk for DLD, because of the
midface. He was extremely irritable as an infant. During his negative consequences of DLD for later employment and social
preschool years, Joseph’s mother continued to drink, and she relations (Law, Reilly, & Snow, 2013).
also began using cocaine. She was often absent, leaving
Joseph with whatever neighbor would take him, while she Abuse/Neglect
earned money by prostitution to buy drugs. Joseph grew
The World Health Organization (WHO) estimates that 40 mil-
slowly in size. He experienced many developmental delays,
including late motor milestones, slow language development,
lion children younger than 15 years old experience abuse and
and eventually poor reading and spelling. When his mother neglect requiring health and social care. Types of maltreatment
enrolled herself in a drug treatment program when he was 3, may include physical abuse, sexual abuse, emotional abuse (ex-
he was assessed and diagnosed with fetal alcohol syndrome cessive belittling, verbal attack, or overt verbal rejection), and
(FAS). His IQ was in the low average/borderline range, and he neglect (abandonment, inadequate supervision, and/or failure
was enrolled in an intervention program while his mother to provide necessary items, such as adequate nutrition or cloth-
completed the drug rehabilitation program. Both made sig- ing). Children experiencing abuse and neglect are likely to be on
nificant progress; and by age 5, Joseph was enrolled in a clinical caseloads, because children with developmental disor-
mainstream classroom. He was very personable and chatty, ders and language impairments are more likely to be abused
but he still required special educational support. He had diffi-
or treated harshly than typically developing children (Sullivan
culty understanding classroom instructions and struggled
& Knutson, 2000; Hendricks, Lansford, Deater-Deckard, &
with reading lessons and arithmetic. In high school, his poor
judgment and impulsive behavior often got him in trouble. At
Bornstein, 2014), and because, as we have seen, abuse and
16, he dropped out. neglect may disrupt development, especially for language and
executive control processes (Bick & Nelson, 2016).
CHAPTER 4  Special Considerations for Special Populations 137

Maltreatment interferes with normal social-interaction anomalies in the premaxillary zone (e.g., flat upper lip,
processes and thus reduces the opportunities for language flattened philtrum, and flat midface)
learning in socially meaningful exchanges. As a result, young • Growth retardation: At least one of the following:
maltreated children may have reduced rates of vocabulary • Low birth weight for gestational age
growth and shorter MLUs than non-abused peers (Coster, • Decelerating weight over time not due to nutrition
Gersten, Beeghly, & Cicchetti, 1989; Sylvestre, Bussieres, & • Disproportional low weight to height
Bouchard, 2016). Deficits in expressive syntax persist into • Central nervous system abnormalities: At least one of the
school age (Eigsti & Cicchetti, 2004) and adolescence following:
(McFayden & Kitson, 1996), although vocabulary scores may • Decreased cranial size at birth
improve to near normal levels in the oldest groups. Less has been • Structural brain anomalies
reported about the social communication skills of children ex- • Neurological hard or soft signs (age appropriate)
periencing maltreatment, but such children are reported to FASD is a lifelong disorder, and there is a predictable
have difficulties using language to articulate their feelings and progression of maladaptive behaviors and communication
needs as necessary for self-regulation; to convey abstraction, disorders, which are outlined in Box 4.8. DLDs are nearly uni-
which is necessary for advanced literacy and reading compre- versal in this population and may be related to overall cognitive
hension; and to sustain coherent narrative dialogue, which is
key to social exchange (Coster & Cicchetti, 1993). In addition,
maltreated children are more likely than peers to engage in BOX 4.8  Characteristics of Fetal Alcohol
challenging behaviors that are likely to elicit further negative Spectrum Disorder
reactions from teachers and peers (Westby, 2007). Infancy and Early Childhood
• Sleep disturbances
Fetal Alcohol Spectrum Disorder • Poor sucking response
Maternal substance abuse can affect a child’s development in • Failure to thrive
at least two ways: • Prone to middle ear disease
• Substances (such as alcohol and cocaine) can have negative ef- • Poor habituation
• Delays in walking and talking
fects during prenatal development. These substances can cross
• Delays in toilet training
the placental barrier and affect the intrauterine environment. In • Difficulty following directions
the case of alcohol, the fetus is unable to metabolize the alcohol • Temper tantrums
as an adult can. Alcohol acts as a teratogenic agent and interferes
with chemical processes in fetal cells. Abuse of other drugs, such School Years
as cocaine, can increase the probability of a premature birth, • Hyperactivity, distractibility
which carries its own set of developmental risks. • Poor attention
• Language and communication development is also influenced • Delayed motor, cognitive, and speech development
by the effects of substance abuse on the caregiving environment. • Difficulties understanding consequences of actions
• Temper tantrums and conduct problems
A mother (or father) who is frequently drunk, high on drugs, or
• Fine motor difficulties
driven to get drugs by any means necessary is not a person who • Learning and memory problems
can devote much energy to childrearing. These parents often • Lack of inhibition
have difficulty understanding their children’s communication • Interest in social engagement but poor social skills
attempts and may not respond appropriately to them, often • Indiscriminate attachment to adults
rejecting or criticizing their efforts (Sparks, 2001). • Withdrawal, depression
In fact, Coggins, Timler, and Olswang (2007) refer to this • Poor judgment, difficulty matching aspirations to ability,
as a “double jeopardy,” pointing out that it is often challeng- failure to learn from past experience
ing to separate the effects of the substance itself from the • Good verbal facility, giving appearance of strong verbal skills,
chaotic environments that are prevalent in parental substance but poor language comprehension
abuse. Understanding cognitive and language outcomes in • Better performance in reading and writing than in mathematics
• Good performance on concrete tasks, poor abstract reasoning
these children requires exploration of both.
Fetal alcohol spectrum disorder (FASD) is a syndrome of Adolescence
birth anomalies associated with excessive alcohol intake during • Reach academic ceiling
pregnancy. Originally referred to as fetal alcohol syndrome • Depression, social isolation
(FAS), the change in diagnostic label reflects the differing de- • Naive, childlike manner
grees of impairment and outcome associated with the disorder. • Sexual difficulties (inappropriate behavior, easily exploited)
Different diagnostic criteria are employed in different coun- • Poor impulse control
tries, but they all include the following four criteria (Institute • Difficulty seeing cause-effect relationships
of Medicine, 1996): • Memory, learning, attention, activity, and judgment prob-
lems persist
• Confirmed or unconfirmed maternal alcohol exposure
• Pragmatic language difficulties
• Facial features: Evidence of a characteristic pattern of fa- • Truancy and school dropout problems
cial anomalies that includes short palpebral fissures and
138 SECTION I  Topics in Childhood Language Disorders

achievements (Cone-Wesson, 2005). Systematic reviews have


highlighted enormous rates of comorbidity in FASD, due to
THE NONSPEAKING CHILD
the permanent effects of alcohol exposure on the developing Some of the children SLPs treat have limited, if any, spoken
fetus. One such review and meta-analysis identified 428 co- language. Traditionally, AAC systems were developed to re-
morbid conditions reported in the literature, spanning 18 of place speech, but AAC devices are now seen primarily as tools
the 22 chapters of the ICD-10 (Popova et al., 2016)! Five co- to supplement the child’s communication skills and to facili-
morbid conditions had the highest pooled prevalence esti- tate spoken language development (Barker, Akaba, Brady, &
mates (50% to 91%), including peripheral nervous system Thiemann-Bourque, 2013a). One of the challenges facing
dysfunction, conduct disorder, chronic OM, and receptive and SLPs is the heterogeneity of AAC users, who may experience
expressive language disorders (Popova et al., 2016). Smaller motoric challenges, cognitive deficits, and/or social interac-
cohort studies have emphasized deficits in social communica- tion issues, all of which may affect decisions about AAC sup-
tion, particularly difficulties in producing narratives that have port (Lund, Quach, Weissling, McKelvey, & Dietz, 2017). For
sufficient semantic elaboration and referencing (Coggins et al., these clients, we have two immediate assessment priorities;
2007; Thorn & Coggins, 2008). Deficits in executive control are first, to establish the child’s level of comprehension and sec-
also likely to impact on pragmatic language skills. ond, to establish whether any intentional communication is
taking place and if so how and for what purposes. Knowing
Clinical Implications how much a child understands helps both to structure our
Speech-language clinicians have a number of responsibilities own input and to select among language goals in production.
and challenges when working with children experiencing Criterion-referenced assessment methods may be useful here.
extreme environmental disadvantage. First and foremost, In Chapters 6 and 7, some assessment techniques that can be
clinicians have a legal duty to report maltreatment and pre- used to establish nonverbal, intentional communication are
vent the child coming to harm. This can be tricky in cultur- discussed in detail. Knowing that a child has a desire to com-
ally diverse communities where standards of discipline and municate can help us distinguish between language problems
parent-child interaction may differ from our own. Westby that arise from impairments to oral-motor structures and
(2007) addresses these issues and stresses that any practice functions or sensory impairments from those that are associ-
that causes a real and present danger to the child requires im- ated with psychiatric conditions, such as selective mutism or
mediate action. ASDs. Here, knowing what is causing the problem is very
A second consideration is that the majority of children important, because we need to tailor our interventions to the
with FASD experience chronic OM (Popova et al., 2016). particular needs of the child. For many nonspeaking chil-
Thus, careful monitoring of hearing status is advocated. Mal- dren, AAC should be considered, even if only as a temporary
treated children and children with FASD are also very likely bridge to other communication systems, in order to provide
to have challenging behaviors and may meet criteria for the child with a viable communication system of some kind.
ADHD and/or conduct disorder. Working as part of a multi- Let’s take a look at some of the issues that arise when assess-
disciplinary team is necessary to reduce undesirable behav- ing and recommending AAC for the nonspeaking child.
iors and encourage language for the purposes of reflection,
negotiation, and behavioral control. Severe Speech-Motor Disorders
Despite an estimated annual cost in 1:1 SLP therapy Many disorders can affect the orofacial structures or neuro-
hours for treating children with FASD of between $72.5 to motor functions that serve speech production. Some of these
$144.1 million Canadian dollars (Popova, Lange, Burd, can leave the understanding and formulation of language, as
Shield, & Rehm, 2014), there remains a dearth of high- well as general cognitive skill, more or less intact, resulting in
quality intervention studies to test the long-term effects of more circumscribed speech impairments. Cerebral palsy,
intervention efforts. Numerous treatment approaches have certain congenital facial anomalies, and brain injuries spe-
been proposed; some advocate focus on general skills such as cifically affecting neuromotor tracts are some examples. In
executive function and self-regulation to increase the child’s Chapter 3, some principles to use in making decisions about
capacity to learn from environmental inputs (Kodituwakku augmentative communication for children with severe speech
& Kodituwakku, 2011). Until there is sound evidence that and physical impairment (SSPI) were discussed. We’ll want to
this approach yields treatment gains in language processing, apply those principles when choosing an augmentative or
and social communication skills, we suggest direct focus on alternative system for these clients. Many such disorders also
these skills because there is preliminary evidence for treat- affect feeding and swallowing. These problems require an
ment effectiveness, at least in the short to medium term intervention program beyond the scope of this text, but clini-
(Jirikowic, Gelo, & Astley, 2010; Paley & O’Connor, 2009; cians should be aware of the need for assessing and planning
Peadon, Rhys-Jones, Bower, & Elliott, 2009). Intervention treatment (perhaps in collaboration with physical and occu-
studies differ considerably in quality, in treatment content, pational therapists) for these aspects of the disability in chil-
context, and in dosage (i.e., amount of treatment offered). dren with SSPI.
All of these factors are likely to influence outcome, and clini- Sturm and Clendon (2004) discussed some of the reasons
cians should take these factors into account when planning why children with SSPI may have trouble learning language.
intervention services. Some have to do with the external barriers they face. They
CHAPTER 4  Special Considerations for Special Populations 139

cannot learn through the usual sensorimotor interactions with also comment, ask questions, express emotions, and build
people and objects because of their physical disabilities. They relationships. A third role of AAC is to reduce challenging
don’t have constant access to their mode of communication as behaviors by providing an alternative means of rejecting un-
speakers do; if they use a board or device, someone has to get it wanted approaches and requesting help. Finally, AAC can
and set it up for them before they can communicate. Their provide a bridge into symbolic reasoning and later language
limited mobility gives them fewer opportunities to interact with development.
other people. They aren’t able to develop from babble to speech When introducing vocabulary, it is important to offer not
by playing with sound and using sound as interaction tool; de- just single nouns, but “chunks” of language that the child can
vices and outputs are chosen for them and may not be the best use as speech acts (e.g., “Don’t do that,” “Lemme see”). This
match for their abilities and intentions. In designing language- diversity allows AAC users to choose from the range of
learning systems for individuals who use AAC systems, we need language-learning styles seen in normal development. When
to modify our usual approach. Instead of focusing on the next introducing word combinations and sentences, it may be more
developmental stage of language output, we need to focus more important to stress the communicative functions of these ut-
on the child’s functional communication needs. terances, rather than focusing strictly on the grammatical
Lund and colleagues (2017) detail the decision-making forms needed to express ideas. Still, since speech generation
process of professionals in response to two case histories: one devices can be programmed with whole sentences, children
describing an AAC user with cerebral palsy and one with ASD. using these devices might be given ways to produce sentences,
In both cases, the broad areas of assessment were the child’s such as “I drank all my milk.” These sentences might be possi-
communication needs, device features, and partner skills. ble through the device before the point at which irregular past
Within these broad categories, assessment was more tailored forms would normally be acquired in developmental sequence.
to individual priorities. Child level characteristics will start Paul (1997b) has suggested that programming the device with
with observation of how the child currently communicates, some “giant phrases” (e.g., See you later alligator), often used as
with whom, and in what settings. Establishing comprehension gestalt forms by young children, can help the child using AAC
levels and literacy skills is critical in determining scope and to develop the analytical skills these forms facilitate in typical
format of language content. It is also important to assess prag- speakers. Wilkinson and Henning (2007) also point out that a
matic and cognitive skills in this population. We can then combination of preprogrammed phrases alongside single
follow the child’s developmental profile by targeting words words can also speed up message formulation and reduce cog-
and word combinations that would be expected given the nitive demands for the speaker.
child’s cognitive, comprehension, and communicative profile. The focus on an AAC device may bias the intervention
Emerging technologies are making AAC systems much toward talk about the “here and now” for a longer period
more accessible, less expensive, and more acceptable to chil- than would be typical in normal development. Interactive
dren and their peers, but they increase the number of variables book reading may be a particularly naturalistic context in
that the clinician needs to consider when making decisions which such talk could take place (Kent-Walsh, Binger, &
about optimal functionality (Lund et al., 2017). As just one Hasham, 2010). It is important for clinicians to begin to in-
example, Proloquo2go is a speech generating system that can troduce some talk about past time, predictions about future
be used on an iPhone, iTouch, or iPad. These devices are much events, discussions of pretend, and so on. Following some of
more inclusive and less stigmatizing than traditional AAC de- the guidelines given in Chapter 9 for incorporating play
vices (Dada, Horn, Samuels, & Schlosser, 2016). contexts in language intervention can be helpful in achieving
Device features are key considerations. Lund and col- this goal. Finally, Barker and colleagues (2013) highlight the
leagues (2017) detail factors such as portability, array size and important role that communication partners may play in
layout, navigation (number of pages/levels of the system that modeling the use of the AAC device, in much the same way
can be managed), vocabulary and how it is organized (cate- that adults model language for typically developing children.
gorical, situational), and speech generation characteristics are This requires significant others to use the device themselves,
all critical to device uptake and functional use. Families, which has the added benefits of enabling communication
teachers, and the AAC users should be fully involved in the partners to become familiar with the words and messages
decision making process to ensure that the system is indi- available to the child, and reduces the stigma of using the
vidualized, adaptable to different environments and commu- device. This may require detailed and sustained training in
nication partners, and supports full participation in society how to optimally use the AAC device both to enhance com-
(Reichle, Drager, Caron, & Parker-McGowan, 2016). munication and to facilitate language growth. Sadly, many
Although AAC devices are often aimed at increasing teachers feel such training is currently lacking (Barker et al.,
language output, Wilkinson and Henning (2007) remind us 2013). As children typically learn language partially through
that, for some children, these devices will also open doors the desire to emulate others, it is important that both adults
to language understanding, as well as nonverbal communica- and peers use AAC as a means of interacting with the child.
tive exchanges. Second, they highlight the importance of en- Use of AAC devices by children’s peers to provide aug-
suring that the vocabulary targeted can be used for a range of mented input has been associated with stronger language
communicative functions; users of AAC must be able to do growth, as well as enhancing social and personal well-being
more than request, protest, and answer questions, they must (Barker et al., 2013a).
140 SECTION I  Topics in Childhood Language Disorders

speech are impaired in the absence of neuromuscular defi-


cits. . . . The core impairment in planning and/or program-
ming spatiotemporal parameters of movement sequences re-
sults in errors in speech sound production and prosody” (p. 1).
Unlike SSPI, the difficulty here is not the result of muscle
weakness, paralysis, or obvious neurological impairment. In-
stead, in CAS there is a problem with motor planning; the child
knows what he or she wants to say, but there is a deficit in the
motor planning/coordination of the articulators necessary to say
it. Alternative terms for this problem include developmental
verbal apraxia or dyspraxia. Although there is no agreed-upon
gold-standard assessment for CAS, consensus criteria include
the following: (1) inconsistent errors on repeated productions of
syllables and words; (2) lengthened or disrupted co-articulation
transitions between sounds/syllables; and (3) inappropriate
prosody, especially realization of lexical and phrasal stress
(ASHA, 2007; Murray, McCabe, Heard, & Ballard, 2015).
Assessment of volitional movement patterns for the pur-
pose of performing an action (i.e., puckering lips for a kiss,
blowing a bubble) has also traditionally been a common
clinical approach to identifying CAS. However, Shriberg and
colleagues (2003) compared children with suspected CAS to
children with speech sound disorders to find out whether the
two groups could be differentiated on the basis of behaviors
directly related to speech praxis. They concluded that only
two linguistic behaviors differentiated the groups: (1) incon-
sistent production of stress in tasks involving the naming of
Augmentative and alternative forms of communication (AAC) two-syllable words, and (2) the degree of variation in the tim-
systems increase communicative opportunities for children ing of speech. These results led Shriberg and colleagues to
with severe speech production impairments.
develop automated speech recognition methods for distin-
guishing speech samples of children with and without CAS
Although for many years children with SSPI were given little (Hosom, Shriberg, & Green, 2004), leading to more accurate
access to literacy, much has changed in the last 25 years. Consid- diagnoses. Without these automated, instrumental tech-
erable research and clinical effort has been devoted to develop- niques, however, it can be very difficult for a clinician to be
ing literacy skills and using AAC devices to transmit written definitive about whether a child is experiencing CAS or de-
messages. This change has literally revolutionized the commu- velopmental speech sound disorder. Murray and colleagues
nicative capacity of many children with SSPI. Written output, (2015) contrasted the ASHA criteria with the Shriberg crite-
which is understandable by most adults and older children in ria to establish optimal differential diagnosis of CAS from
our culture, allows the child with SSPI to express the full range other speech sound disorders. They reported that a combina-
of meanings available in language to the broadest possible audi- tion of polysyllabic production accuracy and an oral motor
ence. Some children with SSPI will continue to perform below assessment, including diadochokinesis, resulted in 91% diag-
developmental expectations on literacy measures despite in- nostic accuracy and was more clinically feasible.
struction, but there are methods that can be used to improve There are numerous controversies surrounding CAS. Like
access to the written word. Improving basic language develop- “developmental dysphasia” CAS was originally defined as an
ment is an important part of this picture, as is the provision of analogue to an adult-acquired neurological disorder, apraxia
early, intensive exposure to storybook reading (Wood & Hood, of speech, or a neurologically-based difficulty in program-
2004), opportunities for carefully scaffolded phonemic aware- ming speech movements, thought to take place at a pre-
ness, and letter-sound association. Light, McNaughton, Weyer, articulatory motor planning level. Intensive investigation,
and Karg (2008) and Light and McNaughton (2009; 2015) have however, has not been able to document any consistent neu-
developed an evidence-based literacy intervention program for ropathology in children who show this speech pattern, even
users of AAC. Some of their recommended techniques are using the neuroimaging techniques outlined in Chapter 1.
outlined in Table 4.4 (see also http://aacliteracy.psu.edu/). The fact that the behavioral symptomatology identified with
CAS overlaps so much with other conditions, such as speech
Childhood Apraxia of Speech sound disorder and expressive language delays, contributes to
According to ASHA (2007), childhood apraxia of speech this view and makes differential diagnosis problematic. Shriberg
(CAS) is “a neurological childhood speech sound disorder in and colleagues (2011) reported that the population prevalence
which the precision and consistency of movement underlying of CAS is estimated at 0.1%, and that false positive diagnostic
CHAPTER 4  Special Considerations for Special Populations 141

TABLE 4.4  Strategies for Developing Literacy Skills in Children Using Augmentative
and Alternative Forms of Communication
Literacy Skill Example Target Example Activity Example Materials
Sound blending Child will listen to sounds and blend Clinician produces sounds in isola- Selection of four symbols
them together tion, slowly: “mmmooommm” including:
Child will say word out loud, sign it, Child indicates target Mom
or select the correct picture or AAC Mop
symbol from a group of 4, with 80% Pot
accuracy Man
Phoneme Child will listen to a phoneme presented Clinician says “m” Selection of four symbols
segmentation orally Child indicates target word begin- including:
Child will indicate a word that begins with ning with that phoneme Mom
that phoneme by saying it out loud, Up
signing it, or selecting the appropriate Bat
picture or AAC symbol from a choice of Pot
four with at least 80% accuracy
Letter-sound Child will listen to a target sound Clinician says “m” Computer keyboard with
correspondences presented orally Child indicates target letter repre- various letters highlighted
Child will select the appropriate letter senting that phoneme (depending on number of
from a group of letter cards, an alpha- letter-sound correspon-
bet board, or a keyboard with at least dences child knows)
80% accuracy
Decoding When presented with a simple three-letter Child is presented with written Selection of four symbols
word in print, the child will indicate the word “big” including:
word by saying it out loud, signing it, or Child must select the matching Big
selecting the appropriate picture or AAC picture/symbol from choice of Pig
symbol with at least 80% accuracy four Bug
Bib
Shared book When the instructor reads a sentence in While reading a short book, clinician Commercially available books
reading a book out loud, pauses, and points to points to word so child can see or personalized books
a regular three-letter word in print, the Child points to picture/symbol from Symbols representing key
child will indicate the word by saying it display of symbols relevant to the characters, events, emo-
out loud, signing it, or selecting the story tions in the story
appropriate picture or AAC symbol with
at least 80% accuracy
Sight word When a word is spoken aloud, the Clinician says word “big” Four printed words:
recognition child will select the matching printed Child points to matching printed Big
word from a choice of four with 80% word Bib
accuracy Bug
Pig
Reading simple When presented with simple written Child reads sentence: “The boy Four pictures;
sentences and sentence, child will (a) read the sen- has a dog.” Boy with dog
stories tence, sign the sentence, or match Child selects picture corresponding Girl with dog
picture to sentence and (b) answer to “Who is it about?” Boy with cat
“Who is it about?” “What happened?” Girl with bird
questions with 80% accuracy Can tailor story/pictures to
child’s interest
Reading Targets may include: The clinician models or demonstrates Choose books of interest or
comprehension • Summarizing the strategy curriculum related materials
• Generating questions The clinician provides scaffolding Adapt response options to
• Answering questions support child’s skills and abilities
• Semantic/graphic organizers The clinician gradually fades this These may include commu-
• Predict next words/sentences in text support nication boards, symbol
• Activate prior knowledge The child has repeated opportuni- book, signing, computer
ties for independent practice keyboard, computer, or
The clinician, child, and family make other consumer electronic
a plan to ensure generalization device with speech output
and continued use of the strategy
AAC, Augmentative and alternative forms of communication.
Adapted from: Light, J., & McNaughton, D. (2009). Accessible literacy learning: Evidence-based reading instruction for learners with autism, cerebral
palsy, Down syndrome, and other disabilities. San Diego, CA: Mayer Johnson. See also: http://aacliteracy.psu.edu/index.php/page/show/id/1
142 SECTION I  Topics in Childhood Language Disorders

rates run at 80% to 90%. In other words, CAS is a rare disor-


der, and children with other kinds of speech problems are very
often misdiagnosed as having CAS. One important fact to
note is that CAS affects not only speech sounds, but prosody,
particularly stress and timing, as well. And since prosody oc-
curs only in connected speech, it does not make sense to make
a diagnosis of CAS unless there is enough continuous speech
to judge whether prosody is affected. For this reason, our ap-
proach is to counsel caution in diagnosing this disorder. For
pre-verbal children, it is simply too early to know whether
speech has failed to emerge because of CAS, some other motor
speech problem, or a more pervasive communication deficit.
In these cases, work on developing receptive language, en-
couraging vocal production and working toward a first pro-
ductive lexicon (see Chapter 7) is appropriate, regardless of
what the diagnosis turns out to be. A diagnosis should be de-
ferred until there is enough connected speech to judge accu-
rately whether criteria for CAS are met. For children who use
connected speech but have poor intelligibility, inconsistent
speech errors, and prosodic deficits, CAS can be considered as
a diagnosis, but only after other conditions, such as hearing Aided augmentative and alternative forms of communication
impairment, dysarthria, and more common speech delays (AAC) devices are sometimes used with children with severe
have been ruled out; and it is important to remember how childhood apraxia of speech (CAS).
rare CAS is. For those few children who do meet criteria for
CAS, intervention should focus on developing motor patterns
that automatize speech production, primarily through re- Ballard, 2014). Nevertheless, two motor speech programs
peated practice of words and phrases, rather than isolated (Integral Stimulation/DTTC and Rapid Syllable Transition
sounds. Sample activities for addressing CAS are presented in [ReST]) and one linguistic program (Integrated Phonological
Box 4.9. A recent systematic review included 42 papers report- Awareness Training) demonstrated preponderant evidence in
ing intervention for CAS, although none of these were high- methodolocially sound, experimental single case series. All
quality randomized controlled trials (Murray, McCabe, & three programs yielded positive treatment and generalization

BOX 4.9  Intervention Approaches for Children with Childhood Apraxia of Speech
Motor Approaches that go beyond basic sit-and-drill to maintain interest and
• Massed practice: Schedule frequent, short sessions; use a motivation.
small set of stimuli (five to seven words or phrases) practiced
over and over before moving on to another small set. Prosodic Approaches
• Use block practice schedules early on: Practice each utter- • Practice analyzing words into syllables: Have clients clap out
ance or stimulus many times in a row in the early stages of the syllables in a word, or have them use large blocks to
learning, because these facilitate retention. represent stressed syllables in a word and small blocks to
• Use random practice schedules later: When production is represent unstressed syllables. Be careful not to produce
stabilized, use random practice, in which items are inter- unnatural stress in word productions.
spersed in random order, to facilitate generalization. • Identify stressed syllables in words (Which part of rhiNOSceros
• Provide feedback: Provide feedback after a small number, but is the loudest?) and imitate multisyllabic words with appropri-
not every, response. Provide the feedback quickly, within less ate stress. If necessary, use backward chaining to achieve this
than a second of the production. Fade the amount of feed- (e.g., have the child say y, city, tricity, lectricity, electricity).
back as the intervention proceeds, and encourage client self- • Match phrases with meaning according to stress patterns:
monitoring. Have clients match BLACKboard to a picture of a chalkboard,
• Provide slowed-down models: Provide extra time for the cli- and BLACKBOARD to a picture of a painted board, for example.
ent to process and program the target movement. As accu- • Have children identify stressed words in sentences: Initially
racy of movement increases, increase rate of presentation of use exaggerated stress, then gradually fade the exaggeration.
stimuli gradually. • Use “wh-” questions: Have children use stress to contrast
• Practice, practice, practice: The fundamental tenet of a motor between answers to “wh-” questions, such as: Who ate the
approach is that learning takes place as a result of repeated cheese? The MOUSE ate the cheese. What did the mouse
successful trials that lead to habituation and automatization eat? The mouse ate the CHEESE. What did the mouse do?
of processing. Develop strategies for imitation and practice The mouse ATE the cheese.
CHAPTER 4  Special Considerations for Special Populations 143

effects across several different children, provided intervention The Nonverbal Child with Autism Spectrum
was delivered at least twice per week with a minimum of Disorder
60 speech trials per session (Murray et al., 2014). Murray went As we saw earlier, a small but significant proportion of chil-
on to directly compare two motor interventions (ReST and dren with ASD fail to acquire any verbal language. It is also
the Nuffield Dyspraxia Programme) in a small scale RCT the case that in the early school years many children with
(Murray et al., 2015). Both groups received 12 hours of treat- ASD may have limited expressive language, although this may
ment over a 4-week period, and both demonstrated large improve with time and intensive intervention. Plesa Skwerer,
treatment gains. Those in the ReST group, however, were Jordan, Brukilacchio, & Tager-Flusberg (2016) compared
more likely to maintain those gains at 4 months posttreat- methods for assessing receptive language skills in this popula-
ment. Thus, although there is a need for further intervention tion and found significant heterogeneity across the sample
research in this area, there is promising evidence that may and across assessment measures, highlighting a need to care-
guide the clinician in choice of program, dosage, and mean- fully assess receptive abilities in nonverbal individuals. What
ingful outcome measures. causes some children with ASD to be nonverbal is still a
For children with severe CAS, it may be necessary to sup- matter of debate, but it is likely that reduced motivation to
plement speech and vocalizations with AAC in order to fa- communicate with others contributes to this problem. Our
cilitate communication for some period of time. But it will primary goals, therefore, are to establish intentional and
also be crucial for these children to receive appropriate, fo- functional communication for a variety of purposes.
cused, intensive speech-language therapy, following guide- As with other disorders, a likely finding for this popula-
lines like those in Box 4.10. Research suggests that speech tion will be that some requests and protests are expressed,
sound disorders tend to improve in these children as they but joint attention or social interactions are not. When this
reach school age, whereas language and literacy problems is the case, we’ll want to do two things. First, we’ll want to
may persist (Lewis et al., 2004). The danger of CAS as a diag- provide some conventional means—gestures, signs, vocal-
nostic category lies in the tendency to lead clinicians to ig- izations, words, or some form of augmentative communi-
nore the language needs of these children to focus on speech cation, such as a picture board—for expressing the intents
production or AAC exclusively. Thus, the clinician needs to the child is already producing. Second, we’ll want to pro-
ensure that adequate assessment of language content and use, vide extensive support for eliciting joint attentional and
as well as literacy is made, even if speech is the most obvious social interactive behaviors. When these emerge in pre-
presenting complaint. symbolic form, we will need to find more conventional

BOX 4.10  Intervention Approaches for the Child with Autism Spectrum Disorder
Who Is Nonverbal
Establish receptive joint attention: Use loud, exaggerated cues and require the child to say or do something and look at the
and intense reinforcement to encourage child to look at what adult before continuing with the game.
the clinician points out or looks at. Use sounds the child is already producing to encourage first
Establish initiation of joint attention: Follow the child’s lead as if words: At first, associate the sound the child makes with a
the child were attempting to establish shared attention. Look meaningful object or outcome. For instance, link “ooh”
at or touch object child is engaged with. Intrude so that sound with a train, saying “choo-choo; yes it’s a choo-choo.”
child’s attention shifts toward adult, then provide exagger- Reinforce and encourage approximations of adult input.
ated praise for looking at and sharing with the adult. Replace unconventional communication: Replace maladaptive
Work with parents to increase synchronous responses to behaviors with gestures, vocalizations, or actions. Try to es-
child’s behavior/communication attempts: Use video to help tablish communicative function of challenging behaviors.
parents identify child’s communicative signals and encourage Expand range of communicative functions: Use communication
immediate response. “temptations” to provide opportunities for child initiation in
Focus on language input: Help parents to adapt their language socially meaningful contexts.
and communication to the child’s developmental level. En- Develop strategies to maintain and repair breakdowns: Use
courage parents to talk about the child’s current focus of highly motivating activities to keep the client focused. Create
interest. opportunities for repair by delaying responses or feigning
Encourage imitation: Play “copy-cat” games in which reinforce- misunderstanding.
ment is provided for vocal or gestural imitation. Start by imitating Provide environmental supports to enhance social communication:
the child; then reward the child’s imitation of the adult. Use visually cued instruction (PECS, visual schedules and calen-
Encourage development of social interactive routines: Use en- dars, sign) and modified linguistic input (exaggerated intonation
joyable routines (e.g., tickling routines, favorite songs, etc.) and facial expression, simple, routine, and repetitive language).

PECS, Picture Exchange Communication System.


Adapted from Aldred, C., Byford, S., Charman, T., Le Couteur, A., Howlin, P., et al. (2010). Preschool Autism Communication Trial (PACT)
Intervention Procedure. Retrieved from: http://research.bmh.manchester.ac.uk/pact/about/PACTtherapydescription.pdf; Paul, R., & Sutherland,
D. (2005). Enhancing early language in children with autism spectrum disorders. In F. R. Volkmar, R. Paul, A. Klin, & D. J. Cohen (Editors):
Handbook of autism and pervasive developmental disorders (pp. 946-976). New York: Wiley.
144 SECTION I  Topics in Childhood Language Disorders

means of expression for them. Use of AAC may be helpful, preschool period. Direct speech instruction, or methods fo-
and may lead to small increases spontaneous speech pro- cused on verbal imitation and speech production (cf. Koegel
duction (Schlosser & Wendt, 2008). Some techniques for et al., 2006) can be combined with AAC, as well as other
working with the child with ASD who is not producing therapy approaches designed to increase joint attention and
speech are outlined in Box 4.10. social interaction behaviors. However, we know very little
about the relative efficiency of these approaches, and more
Augmentative and Alternative Forms of Communication research is needed to guide clinicians as to what mix of ap-
in Autism Spectrum Disorder proaches will yield the most direct route to spoken language.
The issue of alternative modes of communication is often For individuals with ASD, it would seem important to in-
raised for nonverbal children with ASD. Although these chil- clude direct attempts to elicit and develop spoken language
dren have no known motoric impediments to speech, advo- so that they may have more opportunity to reach their poten-
cates of AAC, using a “communication needs” model, recom- tial (Helt et al., 2008).
mend providing AAC to any nonspeaking child, regardless of
the reason, because everyone needs some way to communi-
cate (Beukeleman & Mirenda, 2005). Signed language is one
CONCLUSION
alternative often used, although evidence for its functional This chapter has included a very long discussion of a range
use in children with ASD is limited (Prelock, Paul, & Allen, of developmental disorders that frequently involve impair-
2011). In current practice, AAC systems for people with ASD ments to speech, language, communication, and literacy. Is
often begin with object or picture exchange systems (Bondy it necessary to know the ins and outs of all these disorders
& Frost, 1998). Here the child is given an object (such as a in order to assess and treat them? Yes and no. Yes, it is help-
spoon to represent a bowl of cereal to eat) or picture that ful to know about these disorders and their key features
represents the desired goal, and the child is taught to give it disorder, because these will give us hints about assessment
to the clinician (or parent or education personnel) to obtain and treatment. We also need information about these dis-
the goal. One popular example of this approach is the Picture orders so that we can interpret the medical records in case
Exchange Communication System (PECS; Bondy & Frost, histories, and to facilitate professional dialogues with our
1998). Numerous studies have demonstrated positive effects colleagues who will form the multidisciplinary teams pro-
in language and communication development for PECS us- viding care for these children and their families. Knowing
ers; direct comparison with more direct speech elicitation has more about the disorder will also help us to prepare for as-
revealed that outcomes vary according to child characteris- sociated difficulties in behavior, perception, or cognition
tics. Yoder and Stone (2006) compared PECS with an inter- that may adversely affect language development and that
vention focused on eliciting spoken language and found that will need to be taken into account when planning treat-
children with high object exploration responded better to ment and educational programs. However, we could an-
PECS, whereas those with low object exploration responded swer “No,” in the sense that knowing all of these things will
better to the spoken language intervention. Importantly, the not help us to know about an individual child’s profile of
child’s developmental level may predict the degree to which a communication strengths and weaknesses. Thus every as-
child with ASD adapts to the PECS system; those with devel- sessment will need to start with the child and his or her
opmental levels of 16 months or higher are likely to benefit family, and we must ensure that we do not miss anything
the most from PECS (Pasco & Tohill, 2011). Other forms of important that may not fit our stereotypical profile of a
AAC, such as voice output communication aids (VOCAs; child with a given disorder. “No” is also apt in the sense that
Schlosser & Koul, 2015), have been studied and appear to be there is a great deal of overlap in the cognitive and lan-
helpful, but studies are few and small. Gevarter and col- guage characteristics associated with different diagnostic
leagues (2013) state the importance of considering individual groups (Table 4.5) and that these categories are not mutu-
skills and preferences when selecting AAC methods. Kasari ally exclusive; as we’ve seen comorbidity is the norm, not
and colleagues (2014) highlight the need for AAC interven- the exception, in developmental disorders. Many of the
tions to be trialed in an adaptive fashion, rather than assum- treatment approaches we use will be applicable across dif-
ing a one-size-fits-all approach. Furthermore, a possible ferent diagnostic categories. So, knowing a child’s diagnosis
barrier to their widespread use in the community is that is only a signpost; we need to work closely with families to
other AAC types do not come with an established instruc- discover their primary concerns and priorities, and to con-
tional package for caregivers and teachers (cf. PECs; Tager- duct a thorough assessment of a broad range of language
Flusberg & Kasari, 2013). Overall, we can say that AAC meth- and related functions, using the guidelines presented in
ods have been shown to be compatible with the development Chapter 2. Then we need to develop intervention goals and
of speech, although benefit beyond that offered by direct methods based on the assessment data, choosing among a
speech-language therapy has yet to be established. Because we repertoire of procedures and contexts that we discussed in
know that acquisition of meaningful speech by school entry Chapter 3. That’s the real work of designing a language
is a powerful predictor of later outcome (Howlin, 2005), it is program. Although it is influenced by the child’s diagnosis,
important to make every effort to elicit speech during the it cannot be fully determined by it.
CHAPTER 4  Special Considerations for Special Populations 145

TABLE 4.5  Cognitive and Language Characteristics across Diagnostic Categories


Nonverbal Cognition Executive Functions Working Memory
Primary developmental language Not usually impaired Variable Impaired
disorder (DLD)
Down syndrome Impaired Impaired Impaired
Williams syndrome Impaired Impaired Impaired
Fragile X syndrome (FXS; males) Impaired Impaired Impaired
Visual impairment (VI) Not usually impaired Not usually impaired Not usually impaired
Hearing impairment Not usually impaired Not usually impaired Not usually impaired
Traumatic brain injury (TBI) Variable Impaired Impaired
Focal brain lesions Not usually impaired Not usually impaired Variable
Landau-Kleffner syndrome Not usually impaired ? Impaired
Autism spectrum disorder (ASD) Variable Impaired Variable
Attention deficit hyperactivity disorder Not usually impaired Impaired Impaired
(ADHD)
Fetal alcohol syndrome (FAS) Mildly impaired Impaired Impaired
Language Form Language Content Language Use
Primary DLD Impaired Relative strength Relative strength
Down syndrome Impaired Relative strength Relative strength
Williams syndrome Not usually impaired (relative Relative strength Impaired
to nonverbal mental age
FXS (males) Impaired Impaired Impaired
VI Not usually impaired Relative strength Vulnerable
Hearing impairment Variable Not usually impaired Relative strength
TBI Variable Relative strength Vulnerable
Focal brain lesions Complex syntax/ narrative Relative strength Relative strength
impaired
Landau-Kleffner syndrome Impaired Impaired Vulnerable
ASD Variable Relative strength Impaired
ADHD Variable Relative strength Vulnerable
FAS Variable Impaired Vulnerable
Decoding
Delayed Language Onset (Non-Word Reading) Reading Comprehension
Primary DLD Yes Vulnerable Impaired
Down syndrome Yes Vulnerable Impaired
Williams syndrome Yes Impaired Impaired
FXS (males) Yes Impaired Impaired
VI Yes Alternative method Alternative method
Hearing impairment No, if deaf parents Vulnerable Variable
TBI No Vulnerable Impaired
Focal brain lesions Yes ? ?
Landau-Kleffner syndrome No May be strength May be strength
ASD Yes Variable Impaired
ADHD Maybe Variable Variable
FAS Maybe Vulnerable ?

STUDY GUIDE
I. Intellectual Disability II. Developmental Language Disorder associated with Disor-
A. Define intellectual disability (ID), and describe the diag- ders of Known Genetic Origin
nostic criteria discussed by the American Association of A. Down syndrome
Intellectual and Developmental Disabilities (AAIDD). 1. What causes Down syndrome?
B. Describe the cognitive and linguistic characteristics of 2. Describe language form, content, and use in Down
children with ID. syndrome.
C. What are “adaptive behaviors,” and why are they 3. What are the cognitive characteristics of children
important to assess when working with individuals with Down syndrome, and how do they influence
with ID? language development?
146 SECTION I  Topics in Childhood Language Disorders

B. Williams syndrome B. Focal brain lesions


1. Describe the social skills of children with Williams 1. What is the pattern of language development and
syndrome. disorder in children with early acquired focal brain
2. How are language and cognition related in Williams lesions?
syndrome? 2. What language skills are most vulnerable in chil-
3. What aspects of language content are particularly dren with focal lesions?
challenging for children with Williams syndrome? C. Seizure disorders (Landau-Kleffner syndrome)
Why? 1. What are the developmental characteristics of
C. Fragile X Landau-Kleffner syndrome that should alert the
1. How significant are gender differences in the clinician to consider this diagnosis?
language and cognitive profiles of children with 2. Discuss language form, content, and use in children
fragile X? with Landau-Kleffner syndrome.
2. High rates of comorbidity are seen with what 3. What alternative methods of communication have
other developmental disorders? been suggested for use in Landau-Kleffner syndrome?
3. Describe the language and cognitive characteris- V. Developmental Language Disorder Associated with
tics of children with fragile X. What are the impli- other Neurodevelopmental Disorders
cations for assessment and treatment? A. Autism spectrum disorder (ASD)
III. Developmental Language Disorder Associated with Sen- 1. What are the key diagnostic features of ASD? How
sory Impairments do severity criteria help clinicians?
A. Visual impairment (VI) 2. Describe the cognitive profiles of children with ASD.
1. How does blindness affect language development 3. How might problems with social interaction and
in terms of language form, content, and use? social understanding affect language development
2. How can clinicians support literacy development and language processing?
in children with VI? 4. Discuss the literacy skills of children with ASD.
B. Hearing impairment 5. How are language form, content, and use related
1. Otitis media (OM) in ASD?
i. Discuss the effects of OM on communication B. Attention deficit hyperactivity disorder (ADHD)
development. 1. What are the cognitive features of ADHD? How
ii. What clinical implications can be drawn from might they influence language development?
the research on OM and language disorders? 2. What additional factors should the clinician con-
iii. What other developmental disorders are as- sider when developing intervention programs for
sociated with high rates of OM? children with ADHD?
2. Sensorineural hearing impairment C. Selective mutism
i. Define the types and degrees of hearing loss. 1. What are the diagnostic criteria for children with
ii. How have cochlear implants changed the role selective mutism?
of speech-language pathologists (SLPs)? 2. Discuss some strategies for working with children
iii. Describe the differences in language form, who have selective mutism?
content, use, and literacy for children with VI. Developmental Language Disorder Associated with Envi-
sensorineural hearing impairment. ronmental Disadvantage
iv. What are the effects of cochlear implants on A. Maltreatment
language and literacy outcomes for children 1. Why are children with language disorders at in-
with hearing impairment? creased risk for maltreatment?
C. Auditory processing disorder (APD) 2. How does maltreatment affect communication
1. Is APD a valid diagnostic entity? Explain your answer. development?
2. Discuss the guidelines for assessment of APD. 3. What are some of the implications for interven-
IV. Developmental Language Disorder Associated with Ac- tion with children who have been maltreated?
quired Neurological Disorder B. Fetal alcohol spectrum disorder (FASD)
A. Traumatic brain injury (TBI) 1. Describe the physical and cognitive characteristics
1. Describe the three phases of recovery from TBI of children with FASD.
and the assessment/intervention approaches most 2. How does FASD interact with other environmental
appropriate for each phase. risks for DLD?
2. Discuss the cognitive impairments associated with 3. Describe the language profiles of children with
TBI and their implications for learning and lan- FASD. Which aspects of communication are most
guage processing. vulnerable?
3. What issues do clinicians need to consider when 4. What other agencies will the SLP need to work with
reintegrating children with TBI into the main- in effectively managing provision for children with
stream classroom? FASD?
CHAPTER 4  Special Considerations for Special Populations 147

III. General Questions


V
VII. The Nonverbal Child A. What other professionals might the SLP being
A. Describe the assessment process in augmentative working with when assessing and treating children
and alternative forms of communication (AAC). with developmental disorders?
What factors do SLPs need to take into account? B. How can language interventions influence the de-
B. Will the use of AAC help or hinder spoken lan- velopment of literacy skills in these populations?
guage development? Explain your answer. C. Executive function deficits are reported in a num-
C. Describe a literacy program for children using ber of developmental disorders. What are they, and
AAC. What special considerations need to be made how might they influence language development
in developing the program? and language processing?
D. Explain the developmental sequence for social- D. What is the role of parents in developing and im-
communicative goals of intervention programs for plementing intervention? Is it different for different
nonverbal children with ASD. disorders?

You might also like