You are on page 1of 8

J Am Acad Audiol 6 : 39-46 (1995)

Down Syndrome :
A Multidisciplinary Perspective
Allan O. Diefendorf*
Marilyn J. Bull*
Debbie Casey-Harvey*
Richard T. Miyamoto*
Molly L. Pope*
Julia J. Renshaw*
Richard L. Schreiner*
Michelle Wagner-Escobar*

Abstract
Trisomy 21, a genetic disorder resulting from a chromosomal abnormality, is one of the most
common forms of mental disability in the United States . Individuals with Down syndrome fre-
quently present with a constellation of medical problems including conductive hearing loss
and, to a lesser degree, sensorineural hearing loss . As part of a health care team, audiolo-
gists must be sensitive to and aware of medical conditions prior to establishing intervention
strategies . Medical conditions, by necessity, precede audiologic interventions and, therefore,
a close working relationship among team members is critical . Yet, audiologic and communi-
cation interventions should be established at the earliest possible time for maximizing an
individual's development potential . This article stresses the importance of a multidisciplinary
team in the provision of services so that prevention of further disabilities, improved outcomes
of medical interventions, and appropriate habilitative and educational planning may ensue.

Key Words: Amplification, audiologic intervention, Down syndrome, hearing impairment

risomy 21, a genetic disorder resulting drome. Skilled pediatric audiologic and oto-
from a chromosomal abnormality, is one laryngologic management in association with
T of the common forms of mental dis- appropriate communication intervention can
ability in the United States, with an incidence greatly enhance the social and cognitive function
of approximately 1/700 live births . In 1866, of children with Down syndrome .
Langdon Down prepared the first adequate
description of this syndrome - hence, the GENETIC CONSIDERATIONS
name, Down syndrome . The correct diagnosis
of Down syndrome can often be made at birth, he genetic imbalance leading to Down syn-
fostering early identification and immediate T drome can be represented by either Full
habilitative planning . 21 trisomy (nondisjunction), Partial 21 trisomy
Otolaryngologic and audiologic problems (translocation), or mosaicism (postfertilization
are common in Down syndrome and constitute nondisjunction) . When nondisjunction occurs
potential additional disability. These topics are during the first meiotic division of reproductive
the focus of this paper and emphasize the impor- cells, both #21 chromosomes end up in one cell.
tance of early and regular re-evaluation of the Instead of both cells having 23 chromosomes, one
audiologic status of every child with Down syn- cell has 24 chromosomes, and the other has 22 .
The loss of a #21 chromosome makes it highly
unlikely for an egg containing 22 chromosomes
'Indiana University School of Medicine, James Whit- to survive. However, the egg with 24 chromo-
comb Riley Hospital for Children, Indianapolis, Indiana
Reprint requests : Allan O . Diefendorf, Indiana Uni- somes can survive. Union with a cell containing
versity School of Medicine, James Whitcomb Riley Hospi- 23 chromosomes results in a child with 47 chro-
tal for Children, Indianapolis, IN 46202 mosomes who has Down syndrome or trisomy 21 .
Journal of the American Academy of Audiology/ Volume 6, Number 1, January 1995

Paternal origin of the extra chromosome is esti- OUTER AND MIDDLE EAR.
mated to occur in less than 5 percent of cases of ANOMALIES
trisomy 21, with the remaining 95 percent to 97

T
percent being maternal in origin . In over 94 he communication handicap of Down syn-
percent of the cases, Down syndrome is due to drome children is well documented . How-
nondisjunction (Cooley and Graham, 1991). ever, the causative factors (see Table 1) under-
Translocation (approximately 4%) involves lying that problem are not well understood . It
the transfer of one portion of a chromosome to is known that these children demonstrate a sub-
a completely different chromosome . During stantially higher prevalence of hearing impair-
meiosis, the chromosomes are close together ment than the general population and that hear-
for extended periods of time . They may touch, ing loss is usually present in early infancy. Oto-
stick to each other, and then separate . When logic findings and coexisting hearing losses are,
they separate, a segment of a chromosome might therefore, suspect in hindering their acquisi-
be pulled off and lost (deletion) or attach itself tion of language skills .
to another chromosome (translocation). As an The craniofacial configuration of children
example, a part of the #21 chromosome might with Down syndrome is a contributory factor to
attach itself to the #14 chromosome . If this many of the otolaryngologic problems that occur.
occurs during meiosis, one cell will then have Anatomically, diminished dimensions of the
23 chromosomes, with one 21 and one #14/21 external ear structures are characteristic of this
chromosome . Fertilization of the egg or sperm population . Aase et al (1973) have documented
containing the #14/21 translocated chromosome that the longitudinal dimension of the pinna of
will result in a child with 46 chromosomes, newborns with Down syndrome is more than two
including two #21 chromosomes and one #14/21 standard deviations below that of normal new-
chromosome . This child, too, will have Down borns. Moreover, the pinna is usually low set. The
syndrome because of the partial trisomy 21 external auditory meatus is frequently narrow
caused by the translocation . If nondisjunction or stenotic (1/z to ~/s the size of age-matched con-
happens after fertilization, mosaicism results. trols), predisposing to occlusion by even minimal
For example, nondisjunction may take place quantities of cerumen. Anomalies of the middle
just after fertilization when four cells are divid- ear that have been noted (Harada and Sando,
ing to form eight cells. If one of the four cells
divides unevenly, this leads to 47 chromosomes
Table 1 Factors that Impact Language and
on one daughter cell, 45 in another, and 46 in
Speech Development
the remaining six cells. The cell containing 45
chromosomes will die. If the 47-chromosome Medical Complications
Heart problems
cell contains a third #21 chromosome, all sub-
Failure to thrive
sequent daughter cells of this cell will also have Seizures
47 chromosomes, or trisomy 21 . The end result Frequent URls
will be a child who will have about 80 percent
normal cells and 20 percent trisomic cells. The Cognitive/Language-Speech Deficits
Language deficits
child may look as though he has Down syn-
Speech production deficits
drome. However, the physical abnormalities Memory and attention deficits
can be less obvious, and the mental disability
may be less severe . Mosaicism is rare and Hypotonia
accounts for fewer than 3 percent of all children Delayed acquisition of coordination movement (sitting,
crawling, standing, ambulating)
with Down syndrome .
Poor oral motor skill development (feeding,
Although the clinical diagnosis of Down swallowing, tongue coordination, jaw stability)
syndrome can often be made readily, diagnosis Reduced speech production
in each child should be confirmed by chromosome
analysis . Chromosomal studies are useful in Sensory Deficits
Hearing loss/auditory perceptual dysfunction
the prenatal detection of the syndrome and for
Visual deficits
recognizing women who may have an increased Sensory integration deficits
risk of having a child with Down syndrome (i .e .,
those over 35 years or having previously borne Social Communication Difficulties
a child with Down syndrome). However, this Complicated nurturing/lack of bonding
Stressed relationships
"at-risk" group accounts for only about one-third
Reduced interaction patterns
of Down syndrome births .

40
Down Syndrome/Diefendorf et al

1981) include hypoplasia of the epitympanum, al (1977) and Harada and Sando (1981) reported
varying amounts of mesenchymal tissue in the that the length of the cochlear spirals are shorter
middle ear including the round window niche, than that of normal controls . In a series of 12
a poorly developed and narrow round window temporal bones studied by Harada and Sando
niche, ossicular abnormalities, and wide angle (1981), the most frequently observed abnor-
of the facial genu . Congenital eustachian tube mality of the inner ear was endolymphatic
abnormalities have been documented by Sando hydrops confined to the apical turn of the cochlea.
and Haruo (1990) in a study of temporal bone Although there are no reports of specific
histopathology. Structural anomalies of the abnormalities of the VIIIth nerve in Down
eustachian tube exist, including a far more acute syndrome, central nervous system abnormal-
angle of entry into the nasopharynx. This, cou- ities are likely to be seen. Crome et al (1966)
pled with diminished tube size, predisposes and Crome and Stern (1972) reported that
these children to stasis of secretions, ascending the weight of the cerebellum and brain stem
infection, and recurrent otitis media. is reduced when compared with normal sub-
It has been suggested that the child with jects and may be consistent with the lack of
Down syndrome may be more susceptible to development in these structures . In addition,
upper respiratory tract infection than the nor- Palo and Savolainen (1973) suggested that
mal child because of peculiar nasopharynx devel- there is a deficiency of the specific myelin
opment . In addition to an increase in the preva- basic protein in Down syndrome . Banik et al
lence of otitis media with effusion, anatomic (1975) reported evidence of abnormal or incom-
considerations in nasopharyngeal development plete myelination, which may reflect general
are likely responsible for nasal obstructions and structural changes affecting neuronal growth
subsequent rhinorrhea . Obstructive apnea is a and synaptogenesis .
problem recently recognized to occur with sig-
nificant frequency in persons with Down syn- HEARING LOSS
drome. Failure to treat in severe conditions may
result in failure to thrive, hypoxemia, and sub-
optimal mental function and progress to heart T he reported prevalence of hearing loss in
Down syndrome varies, depending on the
study population, age of the study group, crite-
disease secondary to disease of the lung .
Generalized muscular hypotonia is also ria used to specify hearing loss, and procedural
characteristic of Down syndrome . It is postulated variables, including the type of test administered.
that this hypotonia might extend to the tensor Strome (1981) noted a prevalence of 42 percent
veli palatini muscle causing eustachian tube to 78 percent, verifying that the occurrence of
dysfunction. This would result in poor middle ear hearing loss is much more frequent in the Down
aeration and subsequent middle ear effusions syndrome population than in the population at
and infections . Moreover, the epithelium of the large. Gerber (1977) has indicated that deaf-
middle ear may be roughened due to vitamin A ness is rare in this group. On the other hand,
deficiency, increasing the chance of effusion studies utilizing developmentally appropriate
(Coleman et al, 1979). test techniques report that the majority of hear-
Ossicular abnormalities include congenital ing impairments are mild to moderate in sever-
malformation and bony erosion from inflam- ity, varying 15 to 40 dB above normal threshold
mation . It is difficult, if not impossible, to deter- (Greenberg et al, 1978 ; Balkany et al, 1979).
mine whether deformed ossicles represent con- Glovski (1966), describing audiometric eval-
genital malformation or osseous remodeling by uation by air conduction of 38 children with
chronic infection. Ossicular fixation also occurs Down syndrome, found decreased hearing
in Down syndrome and may result in a poten- sensitivity in over 70 percent. The type of hear-
tially correctable conductive hearing loss . ing loss was not reported since sensori-
neural integrity was not evaluated via bone-
INNER EAR AND conduction audiometry. Brooks et al (1972)
RETROCOCHLEAR ANOMALIES found 77 percent of their subjects with Down
syndrome to have decreased hearing. Middle ear
nner ear abnormalities are reported for both pathologic conditions were reported in 60 per-
cochlear and vestibular structures . Cochlear cent of the patients, despite a mean age of 26
abnormalities are more prevalent than vestibu- years for men and 24 years for women. Schwartz
lar abnormalities. When present, inner ear and Schwartz (1978) reported that 67 percent
abnormalities are generally mild . Igarashi et of their sample (39 children with a mean age of
Journal of the American Academy of Audiology/Volume 6, Number 1, January 1995

3 years) demonstrated otoscopic evidence of conducted auditory evoked response testing as


pathologic ear conditions . Balkany et al (1979) well . Conversely, successful resolution and hear-
examined 107 patients with Down syndrome ing improvement following medical manage-
(mean age of 12 years). Hearing loss was iden- ment of a conductive hearing loss can camou-
tified in 78 percent of their population . Addi- flage an underlying mild sensorineural hearing
tionally, among the 64 percent of ears identified loss for years. Although hearing sensitivity and
with binaural losses, 54 percent were of a con- responsiveness may be somewhat or even
ductive nature, 16 percent had sensorineural greatly improved following medical manage-
loss, and 8 percent had mixed-type hearing ment, a significant hearing loss may still exist.
loss . According to Balkany et al (1979), about 40 Clearly, accurate post-treatment hearing assess-
percent of the children with conductive hearing ment is critical in the early identification of
loss could not be explained by middle ear effu- the child with Down syndrome with permanent
sion or chronic otitis media. These patients had hearing impairment .
normal-appearing otoscopic examinations, sug-
gesting the presence of middle ear anomalies. AUDIOLOGIC CONSIDERATIONS
Surgical procedures on selected Down syndrome
patients revealed ossicular malformations and
destruction caused by inflammation due to A udiometric evaluation of the Down syn-
drome population is an essential part of
their habilitative management . While conduc-
chronic infection.
These studies, coupled with surgical and tive pathology is high, underlying sensorineural
histopathologic findings, confirm that the major- hearing loss may be present. Therefore, the first
ity of hearing losses appear to be conductive in objective of audiologic testing is to obtain reli-
nature . Furthermore, in contrast to the natural able auditory responses. Visual reinforcement
history of middle ear effusion in the normal audiometry (VRA), conditioned play audiometry,
pediatric population, the prevalence of this con- or conventional test procedures must be selected
dition tends to remain high in the Down syn- based on the child's developmental age.
drome population, regardless of age. It is rec- Greenberg et al (1978) reported the use of
ommended that individuals with Down syn- VRA(Wilson et a1,1976; Wilson and Moore, 1978 ;
drome and persistent conductive hearing loss be Thompson and Wilson, 1984; Wilson and Thomp-
treated aggressively from five perspectives : son, 1984) on 41 subjects with Down syndrome
to normalize hearing, to break the cycle of recur- between the ages of 6 months and 6 years. Thresh-
rent suppurative otitis media, to prevent chronic olds, or minimum response levels, were obtained
ear disease, for early identification of long- on 81 percent of these subjects . Consistent with
standing hearing loss, and for provision of appro- other reports, there was higher than normal inci-
priate amplification. dence of hearing loss among these children .
Once the child who is at risk for long- Thompson et al (1979) also found that subjects
standing hearing loss is identified, the nature of with Down syndrome needed to be functioning
the long-standing loss must be assessed . For above a 10- to 12-month developmental age level
the Down syndrome population with stenotic to be successfully tested using the VRAprocedure.
ear canals, wax accumulation, otitis media, If clinicians are interested in predicting potential
ossicular malformation, or adhesions, the audi- success with the VRAprocedure for children with
ology-otology team is critical . Even for the child Down syndrome, the Bayley Scales of Infant
identified with a moderate sensorineural hear- Development (BSID) (Bayley, 1969) mental age
ing loss, the potential of a conductive overlay equivalent score provides the most distinct dis-
complicating the habilitation process is great. A tribution between successful and unsuccessful
close working relationship with the otologist is tests with the dividing point being a BSID men-
essential for the audiologist to know when the tal age equivalent of at least 10 months .
timing is right to plan appropriate intervention In early studies, reliable hearing tests were
with amplification. felt to be difficult to obtain due to the mental dis-
For many children, medical and surgical ability associated with Down syndrome . How-
management of a conductive hearing loss may ever, if clinicians consider test procedures suited
continue for months or years without complete to the patient's mental age and developmental
resolution of the hearing loss . This delay in level, audiometric data can be more easily
identification of a potentially unresolvable con- acquired . Play audiometry is usually very suc-
ductive hearing loss can be reduced by routine cessful with older individuals with Down syn-
postoperative audiograms and/or air- and bone- drome, even at full adult chronological age. The

42
Down Syndrome/Diefendorf et al

"eager to please" nature of children with Down tonicity may reduce the tension of the stapedius
syndrome can lead to frequent false responses muscle . Subsequently, greater sound intensity
when testing this population . Therefore, regard- may be necessary to achieve sufficient muscu-
less of behavioral approach, stringent criteria lar contraction . In addition, it is also possible that
must be established and met to ensure condi- there may be certain subtle changes in the neu-
tioned responses to sound. Additionally, the use ronal organization of the reflex arc in these chil-
of control trials must be implemented to moni- dren ; thus, these changes might preclude con-
tor false responding behavior . traction of the stapedius muscle to acoustic stim-
Speech audiometry is a very important tool ulation. Each of these findings is potentially
for the audiologist . Speech is familiar to young relevant to understanding how children with
children and will hold their attention. Beginning Down syndrome process auditory information.
measures may need to be completed in the Interpretation of patent pressure-equaliza-
sound field due to typically poor headphone tion tubes can be difficult due to the unusually
acceptance and potential for collapsed ear small volume of the ear canal and middle ear
canals . Obtaining a soundfield speech recep- space in Down syndrome children . It is helpful
tion threshold (SRT) followed by a bone-con- and recommended to compare pre- and postop-
ducted SRT as an indicator of conductive erative volume readings to aid in the interpre-
involvement may initially be of most diagnos- tation of tube patency.
tic value. Individual ear SRTs can also be To encourage early identification of hearing
obtained with insert phones to minimize the loss, the utilization of the auditory brainstem
complications of collapsed ear canals . response (ABR) in the newborn nursery or in
Audiologic testing of a child with Down early infancy for children with Down syndrome
syndrome can be influenced by many vari- is an accepted practice. Squires et al (1980) pub-
ables . Use of familiar items, toys, or spondees lished one of the first comparative studies of
may often yield more reliable results. Estab- the ABR in individuals with Down syndrome . As
lishing accurate audiometric results on a young a group, the subjects showed shorter central
child with Down syndrome frequently requires conduction times than a normal comparison
multiple test sessions . Therefore, keeping group. Additionally, absolute wave V latencies
things the same, or routine, will often help . for the subjects with Down syndrome tended to
Consistent use of the same examiner, sound be shorter, despite the fact that the investiga-
suite, and toys and effective and consistent tors did not control for hearing loss . Folsom et
praise and reinforcement can often facilitate al (1983) also reported shorter wave V latencies
testing. Once children are comfortable and in for their study group of children (12 months
a "routine," progress can be made toward and younger) with Down syndrome . The infants
obtaining reliable measures . with Down syndrome also showed a steeper
The high incidence of conductive hearing latency-intensity slope. Taken together, these
loss makes acoustic immittance an essential studies point out the necessity of establishing
part of each hearing evaluation . For younger chil- latency curves across intensity that are specific
dren, the effectiveness of tympanometry may be to children with Down syndrome . The reported
compromised by narrow and stenotic ear canals, abnormalities in the cochlear and/or neural
wax accumulation, and patient cooperation. structures in Down syndrome appear to be
However, Schwartz and Schwartz (1978) demon- reflected in their ABR latencies and, as such,
strated the differential diagnostic value of tym- compromise the use of normal latency curves,
panometry for children ages 2 weeks to 11 years across intensity, as indices of hearing level.
with Down syndrome by reporting: (1) the abil- Hearing levels in infants with Down syndrome,
ity to complete tympanograms on 100 percent of particularly older than 6 months, that are deter-
their sample ; and (2) the ability to differentiate mined solely by using the expected latency curves
normal ears from ears with middle ear effusion for normal infants, rather than completing a
or suspected ossicular fixation . Of additional threshold search at decreasing intensity levels,
significance was the high percentage of absent may underestimate the degree of hearing loss
or elevated acoustic stapedial reflex thresholds and result in false negative findings .
found in their sample of children with Down Children with Down syndrome should be
syndrome . Schwartz and Schwartz (1978) spec- given every opportunity to improve their hear-
ulated that the absent acoustic reflexes may be ing by appropriate amplification strategies . The
related to the generalized hypotonia that is com- presence of any hearing loss may further com-
mon to children with Down syndrome . Loss of plicate the known speech and language delays
Journal of the American Academy of Audiology/ Volume 6, Number 1, January 1995

and cognitive limitations that characterize these aid turned off. It may be necessary to allow peri-
children . Therefore, children with even mini- ods of time at home when the child doesn't wear
mal hearing loss should be considered candi- his hearing aids, in order to keep them on at
dates for amplification in an attempt to maxi- school. There are no easy answers in dealing with
mize opportunities for advancement. these behavioral issues either for the parents or
Although an aggressive approach to fitting the audiologist . Although reduced wearing time
amplification is stressed, the timing of this inter- may not be the best answer audiologically, it
vention must be viewed from a wide perspective . may be the only solution to keep the family
Frequently, the recommendation for amplifica- working toward success.
tion will be delayed due to a history of fluctuating The use of loaner amplification will not only
conductive hearing loss . Further, many infants facilitate rapid delivery after a long-standing
and children with Down syndrome will experi- hearing impairment is identified but will also
ence prolonged hospitalizations, multiple surg- allow the audiologist to meet the demands of
eries, and other medical complications so that changing circumstances such as fluctuant and/or
hearing loss may not become a priority until progressive hearing loss . For children with small
they become stable medically. It is appropriate or stenotic ear canals, use of a bone-conduction
in these circumstances for the audiologist to hearing aid with a stretch headband to hold the
serve as a resource for the child and his family, bone oscillator in place may be the most effec-
communicating with otologists, pediatricians, tive amplification option until the child's ear
and speech/language pathologists to ensure canals grow or can be surgically altered. If the
eventual intervention with amplification as soon size of the child's ears are adequate to allow the
as it is feasible . use of effective earmolds, conventional air-con-
As with all populations, it is essential to duction hearing aids would be the instruments
consider the child's family agenda in terms of of choice . Due to the power limitations of the bone
their readiness and commitment to proceed with oscillator, effective amplification with bone-
amplification. In general, the earlier amplifica- conduction instruments is limited to those
tion can be introduced, the more easily it may patients with mild to moderate conductive
be incorporated into the child's daily routine impairment. Although it is common practice to
and the better the prognosis for long-term accep- fit children with bilateral hearing loss with bin-
tance. Adjustment to and successful use of ampli- aural amplification, initially fitting one hearing
fication is often more challenging when intro- aid and later adding the second may often
duced to the older child with Down syndrome increase the potential for eventual long-term
because the child may resist the implementation acceptance in this population . Given the high
of a new routine. Regardless of the child's age, incidence of chronic middle ear effusions, an
ongoing and collaborative consultation with the earmold for each ear is provided so the hearing
family is essential in determining the most aid can be alternated between ears if necessary
appropriate timing for intervention with ampli- during periods when one ear is draining .
fication . Once a collaborative relationship with This strategy promotes gradual and eventual
the family is established, the audiologist may adjustment to amplification and reduces
also comfortably address important issues such the possibility of the child having to readjust
as anticipated benefits and limitations, con- to amplification after long periods of time
cerns of family members and others working without it .
with the amplification system, and special man-
agement considerations . For example, most chil- LANGUAGE AND SPEECH PLANNING
dren are initially amused and entertained by the
fact that they can make the hearing aid squeal
by putting their hand over the microphone . In
most cases, this behavior will last a few days and
C hildren with Down syndrome are predis-
posed for language learning deficits . The
profile of language learning is quite different
then lose its charm and disappear. In the child when compared to the "normal developmental
with Down syndrome, the ability to make the profile" and when compared to the profiles of
hearing aid squeal may not extinguish quickly other children with mental disability. The child
and may become a behavior that continues long with Down syndrome demonstrates a decrease
after the fitting. "Feedback is fun" may become in the rate of acquiring speech and language as
a behavioral issue that is annoying to parents. other cognitive skills increase with age (Stoel-
Another common behavioral complaint by par- Gammon, 1990). Additionally, research has indi-
ents is that they frequently find the hearing cated that speech and language development for

44
Down Syndrome/Diefendorf et al

children with Down syndrome progresses in an The complexity of language development


uneven pattern characterized by rapid spurts patterns described demonstrates that children
and changes with long periods of plateau (Miller, with Down syndrome represent a heterogeneous
1987). Moreover, Miller and his colleagues (1989, population . As such, factors that influence lan-
1990) have described various language learning guage development must be viewed carefully
profiles that have been noted with Down syn- for each child. From this analysis, goals (see
drome children . Three profiles have been Table 2) to enhance language learning can be
described: (1) production and comprehension developed.
skills that are commensurate with mental age; Given that Down syndrome will most likely
(2) production skills that lag substantially behind continue to be a leading cause of mental dis-
comprehension skills and mental age; and (3) pro- ability, the challenge is to continue to improve
duction and comprehension skills that are behind. early intervention strategies . Goals for the future
should include close otologic management,
Table 2 Goals for Enhancing Speech and improved identification of the long-standing
Language Learning hearing loss, earlier intervention with amplifi-
Medical Complications cation, aggressive speech/language program-
It is important to have a pediatrician that leads a ming, and continued empowerment of the fam-
multidisciplinary team and supports early intervention ily striving to meet the needs of a child with
for communication . Down syndrome .

Cognitive/Language-Speech Deficits
Developing joint attention
Developing cognitive language skills (object REFERENCES
permanence, cause-effect, object concept)
Increasing initiated communication Aase JM, Wilson AC, Smith DW (1973). Small ears in
Improving imitation skills Down's syndrome : a helpful diagnostic aid. J Pediatr
Expanding turn taking 82 :845-847 .
Increasing functional interaction with objects
Balkany T, Downs MP, Jafek BW, Krajicek MJ. (1979) .
Developing vocabulary/concepts
Hearing loss in Down's syndrome . Clin Pediatr
Increasing mean length of utterance 18 :116-188 .
Enhancing processing of language
Increasing flexible use of vocabulary Balkany TJ, Mischke RE, Downs MP, Jafek BW. (1979) .
Developing pragmatic skills Ossicular abnormalities in Down's syndrome . Otolaryngol
Utilizing augmentative communication Head Neck Surg 87 :372-384 .
(i .e ., sign language)
Expanding consonant/vowel variety Banik NL, Davison AN, Palo J, Savolainen H. (1975) .
Increasing sequencing of syllable structures Biochemical studies of myeline isolated from the brains
of patients with Down's syndrome . Brain 98 :213-218 .
(CV, VC CVC)
Improving phonologic skills Bayley N. (1969) . Bayley Scales of Infant Development.
Decreasing rate of speech New York : Psychological Corp .
Monitoring fluency
Brooks DN, Wolley H, Kanjilal GC . (1972). Hearing loss
Motor Skill Deficits and middle ear disorders in patients with Down's syn-
Cotreatment with occupational and physical therapy drome (mongolism) . J Ment Defic Res 16:21-29 .
Movement and exploration of environment
Oral motor stimulation and feeding Coleman M, Schwartz RH, Schwartz DM . (1979) . Otologic
manifestations in Down's syndrome . Down's Syndrome
Papers and Abstracts for Professionals 2:1 .
Sensory Deficits
Otologic intervention Cooley WC, Graham JM . (1991) . Down syndrome - an
Audiologic intervention update and review for the primary pediatrician . Clin
Ophthalmologic intervention Pediatr 30 :233-253 .
Sensory integration programming
Enhancing visual/auditory attention Crome L, Cowie W, Slater E . (1966) . A statistical note on
cerebellar and brainstem weight in mongolism . J Ment
Social Communication Limitations Defic Res 10 :69-72 .
Parent education
Crome L, Stern J. (1972) . Pathology ofMental Retardation .
Increasing initiated communication attempts Baltimore : Williams and Wilkins .
Increasing parent identification of communication
attempts Down JL. (1866) . Observations on ethnic classifications.
Following child's lead London Hospital Reports 3 :259-262 .
Turn taking
Developing conversational formats Folsom RC, Widen JE, Wilson WR. (1983) . Auditory brain-
Developing repair strategies stem responses in Down's syndrome infants . Arch
Otolaryngol 109:607-610 .

45
Journal of the American Academy of Audiology/ Volume 6, Number 1, January 1995

Gerber SE . (1977) . High risk conditions . In : Gerber SE, Sando I, Haruo T. (1990) . Otitis media in association with
ed . Audiometry in Infancy. New York: Grime & Stratton. various congenital diseases. Ann Otol Rhinol Laryngol
Suppl 148 :13-16 .
Glovski L . (1966) . Audiological assessment of a mongoloid
population . D-aining School Bull 63 :27-36 . Schwartz DM, Schwartz RH . (1978) . Acoustic impedance
and otoscopic findings in your children with Down's syn-
Greenberg DB, Wilson WR, Moore JM, Thompson G. drome. Arch Otolaryngol 104:652-656 .
(1978) . Visual reinforcement audiometry (VRA) with
young Down's syndrome children . J Speech Hear Disord Squires N, Aine C, Buchwald J, Norman R, Galbraith G.
43 :448-458. (1980). Auditory brain stem response abnormalities in
severely and profoundly retarded adults . Electro-
Harada T, Sando I. (1981). Temporal bone histopatho- encephalogr Clin Neurophysiol 50:172-185 .
logic findings in Down's syndrome . Arch Otolaryngol
107 :96-103. Stoel-Gammon C. (1990) . Down syndrome : effects of lan-
guage development . ASHA 42-44.
Igarashi M, Takahashi M, Alford BR, Johnson PE . (1977).
Inner ear morphology in Down's syndrome . Acta Strome M. (1981) . Down's syndrome : a modern otorhi-
Otolaryngol 83 :175-181 . nolaryngological perspective. Laryngoscope 91 :1581-1594.

Miller J. (1987). Language and communication charac- Thompson G, Wilson WR . (1984) . Clinical application of
teristics of children with Down syndrome . In : Pueschel visual reinforcement audiometry. Semin Hear 5:88-99 .
S, Tingey C, Rynders J, Crocker A, Crutcher D, eds. New
Perspectives on Down Syndrome . Baltimore : Paul H. Thompson G, Wilson WR, Moore JM . (1979). Application
Brookes, 233-261. of visual reinforcement audiometry (VRA) to low-func-
tioning children . J Speech Hear Disord 44 :80-90.
Miller J, Miolo G, Sedey A, Rosin M . (1990, April) .
Wilson WR, Moore JM, Thompson G. (1976) . Sound-Field
Productive Language Deficits in Children with Down
Auditory Thresholds of Infants Utilizing Visual
Syndrome . Paper presented at the 23rd Annual
Reinforcement Audiometry (VRA) . Paper presented at
Gatlinburg Conference on Research and Theory in Mental
the American Speech and Hearing Association
Retardation, Brainerd, MN .
Convention, Houston, TX .

Miller J, Rosin M, Pierce K, Miolo G, Sedey A. (1989,


Wilson WR, Moore JM. (1978). Pure Tone Earphone Thresh-
Nov.) . Language Profile Stability in Children with Down
olds ofInfants Utilizing Visual Reinforcement Audiometry
Syndrome . Presented at the American Speech-Language-
(VRA). Paper presented at the American Speech and
Hearing Association Convention, St . Louis, MI .
Hearing Association Convention, San Francisco, CA.

Palo J, Savolainen H. (1973) . The proteins of human Wilson WR, Thompson G. (1984) . Behavioral audiome-
myelin in inborn errors of metabolism and in chromoso- try. In : Jerger J, ed . Pediatric Audiology. San Diego:
mal anomalies . Acta Neuropathol 24 :56-61 . College-Hill Press, 1-44 .

You might also like