Acta Oto-Laryngologica, 2013; 133: 5969

ORIGINAL ARTICLE

The auditory and speech performance of children with intellectual

disability after cochlear implantation

HYE-YOUN YOUM*, IL JOON MOON*, EUN YEON KIM, BO YOUNG KIM,

YANG-SUN CHO, WON-HO CHUNG & SUNG HWA HONG

Department of OtorhinolaryngologyHead and Neck Surgery, Sungkyunkwan University School of Medicine,

Samsung Medical Center, Seoul, Republic of Korea

Abstract
Conclusion: The results revealed that children with intellectual disability (ID) who underwent cochlear implantation (CI)
showed gradual progress in their auditory perception and speech development. ID in children should not be considered
a contraindication for CI, because they are able to obtain a chance to develop oral communication skills following CI.
Objective: The purpose of this study was to assess the auditory and speech performance of 14 young deaf children with ID
after CI. Methods: Fourteen children with ID who underwent CI between December 2002 and February 2010 were included.
Improvement in auditory perception and speech production over time was evaluated longitudinally with the Categories of
Auditory Performance (CAP) score and Korean version of Lings stages (K-Ling). The results were compared with those of
age- and gender-matched implanted controls without additional disabilities. All tests were performed four times in each
patient: before implantation and at 3, 6, and 12 months after implantation. Preoperative and postoperative communication
modes were also assessed and compared between the two groups. Results: Auditory perception and speech production of deaf
children with an ID improved consistently after CI. In addition, the communication mode also took a favorable turn from
nonverbal to vocalizations or oral communication or from vocalizations to oral communication.

Keywords: Speech development, auditory perception, communication mode, language acquisition

Introduction candidacy criteria have broadened with advancement

Deaf children who cannot obtain sufcient benet with
hearing aids are candidates for cochlear implants [1].
Cochlear implantation (CI) plays an important role
in both audiological development and linguistic
acquisition for children with a profound hearing
impairment. It is well known that the rst few
years from birth are very critical for developing the
functional network of the central auditory nervous
system [2]. Candidacy is based on a number of factors
including verication of limited benet from conven-
tional amplication and no medical contraindication
as well as the degree of hearing loss. Recently,
of technology and reports of increasingly positive
changes in speech and language development of
cochlear implant recipients. Therefore, CI in children
with profound hearing loss with various other physical
and mental disabilities has been popularized.
Hearing loss often coexists with other impairments,
whether presenting as a syndrome or some other
constellation of disabilities [3,4]. Intellectual disabi-
lity (ID), which was formerly called mental retarda-
tion, is a term used to describe general mental
disability develops due to abnormalities in brain struc-
ture or function. ID is characterized by signicant
limitations in both intellectual functioning and

Correspondence: Sung Hwa Hong MD PhD, Department of OtorhinolaryngologyHead and Neck Surgery, Sungkyunkwan University School of Medicine,
Samsung Medical Center, 50 Irwon-dong, Gangnam-gu, Seoul, 135-710 Republic of Korea. Tel: +82 2 3410 3579. Fax: +82 2 3410 3879.
E-mail: hongsh@skku.edu
*These authors contributed equally to this work.

(Received 12 June 2012; accepted 29 July 2012)

ISSN 0001-6489 print/ISSN 1651-2251 online
2013 Informa Healthcare
DOI: 10.3109/00016489.2012.720031
60 H-Y. Youm et al.

adaptive behavior as expressed in conceptual, social, Material and methods

and practical skills, which are apparent before the
age of 18 years [5]. ID is relatively common and Participants
causes a lifelong condition of deteriorated cognitive
In this retrospective study, 14 deaf children (8 girls
and adaptive function. It impacts many aspects of
and 6 boys) with IDs undergoing CI at a single tertiary
the childrens lives and gives rise to functional limita-
hospital between December 2002 and February
tions that reect inability in both personal functioning
2010 were included. Another 14 children without
and performing roles and tasks expected of an
additional disabilities, who received cochlear implants
individual within a social environment. Therefore,
at a similar time, were allocated as age- and gender-
patients with intellectual impairment are thought to
matched controls. All 28 patients were prelingually
have limited benet from cochlear implants secondary
deafened children and they had severe-to-profound
to difculties in postoperative rehabilitation, and if
hearing loss. Individual demographic information for
children have moderate to severe ID, realistic expec-
all the children is provided in Tables I and II, inclu-
tations of what they will be able to accomplish with a
ding gender, cause of deafness, age at implantation,
cochlear implant would be minimal.
associated disabilities, preoperative communication
However, this limitation has not been studied
mode, cochlear implant model, device insertion
thoroughly, and little is known about the effects of
depth, and speech processing strategy. All of the
accompanying ID on improvements in auditory and
participants were born with profound hearing loss.
speech performance after CI. Currently, there is no
Two patients with intellectual impairment were
consensus in the literature on whether to implant
diagnosed as having attention decit and hyperactivity
children with ID, but many authors support the
disorder (ADHD) after implantation and two patients
effectiveness of CI in these children [610].
with moderate ID had CHARGE syndrome or Di
Hamzavi et al. studied auditory responses and
George syndrome, respectively, with hearing loss
speech perception abilities in 10 deaf children with
etiology. We designated the patients with ID to the
a wide range of additional disabilities who underwent
ID group for convenience. Each group included six
CI [6]. They concluded that multi-handicapped
males and eight females. The CI models used in the
children are not contraindicated for CI, but children
ID group were the Nucleus 24 Contour (Cochlear) in
with auto aggression, severe IDs, or malignant
six children, the Nucleus 24 Contour Advance
diseases are considered to be poor candidates.
(Cochlear) in three children, the Nucleus 24 Straight
Holt and Kirk showed that children with mild
(Cochlear) in two children, and the Clarion Hi Res
cognitive delay had signicantly lower scores on
(Advanced Bionics) in three children. In the control
receptive and expressive language measures and
group, the Nucleus 24 Contour (Cochlear) was used
slower rates of speech recognition development;
in ve children, the Nucleus 24 Contour Advance
however, these patients could achieve measurable
(Cochlear) in ve children, the Nucleus 24 Straight
benets in daily life [8]. In 2008, Berrettini et al.
(Cochlear) in one child, and the Clarion HiRes
analysed post implant outcomes of 23 pediatric
(Advanced Bionics) in three children. None of
cochlear implant recipients with additional neuro-
the patients had inner ear malformations, and all
psychiatric disabilities [9]. The results showed over-
the participants received a complete insertion of the
all improvement but were quite variable, reecting
electrode array.
the heterogeneity of the impairments associated
Twelve children in the ID group underwent
with deafness. When they further evaluated 10
mental function assessments in the Department of
children with ID, they obtained satisfactory results
Psychiatry before implantation and the other two
overall, although they could not demonstrate clear
children received these assessments just after implan-
correlation between degree of ID and outcome.
tation. The mental function tests administered were
In this regard, previous studies in the literature
the Bayley Scale of Infants Development-II
were often limited to one or a few heterogeneous
(BSID-II), the Social Maturity Scale, a temperamen-
cases with a wide range of accompanying disabilities,
tal assessment, and the ParentChild Relationship
making it problematic to determine which aspects
and Associated Parents questionnaire [11]. Accord-
of each disability impacts post implant outcome.
ing to the battery of tests, nine children were diag-
Therefore, the goals of this study were to assess
nosed with mild ID and the other ve with moderate
the auditory performance and speech development
ID by clinical psychologists and psychiatrists. Chil-
in young CI recipients with ID compared to those of
dren in the control group also received the same
age- and gender-equivalent implanted children with-
mental function test battery, and all of them showed
out such disability. We also determined the effect of
normal results. All 28 children participated in the
the severity of ID on postoperative outcome.
Table I. Demographic data for the 14 children with intellectual disability (ID).
Subject Gender Etiology of Age at Associated Preoperative Cochlear implant Device insertion Speech processing
no. deafness implantation (years) disabilities communication mode device depth strategy

1 F Congenital unknown 1.8 Mild ID, ADHD Non-verbal Nucleus 24 contour Full insertion ACE
2 F Congenital unknown 3.0 Mild ID Vocalization Nucleus 24 contour Full insertion ACE
3 F Congenital unknown 3.9 Mild ID Non-verbal Nucleus 24 contour, Full insertion ACE
advance
4 F Congenital unknown 4.0 Mild ID Non-verbal Nucleus 24 contour Full insertion ACE
5 F Congenital unknown 5.0 Mild ID Non-verbal Nucleus 24 contour, Full insertion ACE
advance
6 F Congenital unknown 7.7 Mild ID Oral Nucleus 24 contour Full insertion ACE
7 M Congenital unknown 7.7 Mild ID Oral Nucleus 24 contour Full insertion ACE
8 M Congenital unknown 7.9 Mild ID Oral Clarion HiRes Full insertion HiRes/P
9 M Congenital unknown 9.5 Mild ID Oral Clarion HiRes Full insertion HiRes/P
10 M Congenital unknown 2.3 Moderate ID Non-verbal Clarion HiRes Full insertion HiRes/P
11 M CHARGE syndrome 5.2 Moderate ID Non-verbal Nucleus 24 straight Full insertion ACE
12 F Congenital unknown 5.8 Moderate ID Non-verbal Nucleus 24 straight Full insertion ACE
13 M Congenital unknown 10.6 Moderate ID, ADHD Oral Nucleus 24 contour Full insertion ACE
14 F Di George syndrome 11.1 Moderate ID Non-verbal Nucleus 24 contour, Full insertion ACE
advance
ADHD, attention decit and hyperactivity disorder.
Auditory performance of children with intellectual disability after CI
61
 62
H-Y. Youm et al.

Table II. Demographic data for the 14 children in the control group.

Subject no. Gender Etiology of Age at implantation Associated Preoperative Cochlear implant Device insertion Speech processing
deafness (years) disabilities communication mode device depth strategy

1 F Congenital unknown 1.9 None Non-verbal Clarion HiRes Full insertion HiRes/P
2 F Congenital unknown 2.6 None Non-verbal Nucleus 24 contour Full insertion ACE
3 F Congenital unknown 3.9 None Oral Nucleus 24 contour, advance Full insertion ACE
4 F Congenital unknown 4.0 None Oral Nucleus 24 contour Full insertion ACE
5 F Congenital unknown 4.2 None Oral Nucleus 24 contour, advance Full insertion ACE
6 F Congenital unknown 8.2 None Oral Nucleus 24 contour, advance Full insertion ACE
7 M Congenital unknown 7.1 None Vocalization Nucleus 24 contour, advance Full insertion ACE
8 M Congenital unknown 8.1 None Oral Nucleus 24 contour Full insertion ACE
9 M Congenital unknown 8.8 None Oral Nucleus 24 contour Full insertion ACE
10 M Congenital unknown 2.0 None Vocalization Nucleus 24 contour Full insertion ACE
11 M Congenital unknown 5.0 None Oral Clarion HiRes Full insertion HiRes/P
12 F Congenital unknown 5.8 None Oral Nucleus 24 contour, advance Full insertion ACE
13 M Congenital unknown 10.9 None Oral Clarion HiRes Full insertion HiRes/P
14 F Congenital unknown 9.0 None Oral Nucleus 24 straight Full insertion ACE
 Auditory performance of children with intellectual disability after CI
auditory and oral rehabilitation program at least twice
per week before and after CI. This study was
approved by the Institutional Review Board of
Samsung Medical Center (IRB le no. 2012-04-010).
Evaluation
Changes in auditory perception and speech produc-
tion performance after CI were evaluated with two
standardized assessment tools, the Categories of
Auditory Performance (CAP) score and the Korean
version of Lings stages (K-Ling). CAP comprises
eight hierarchical divisions of auditory perceptive
ability: 0, the lowest level, describes no awareness
of environmental sounds, and 7, the highest level,
describes the use of a telephone with a familiar talker.
K-Ling was developed by modifying Lings seven-
speech teaching model with regard to the normal data
of Korean phoneme development to assess speech
development in young children after CI [12,13]. It
includes the phonetic (articulation) and phonologic
(meaningful speech) levels of acquisition at each stage
(Figure 1). Phonetic development reects the orosen-
sory motor act of producing vowels and consonants,
the basis of speech, whereas phonologic development
refers to the higher-level cognitive processing that
organizes speech sounds into patterns of sound
Phonologic development
Speech intelligible with good voice pattern
Some clear sentences with good voice pattern
Some clear phrase with good voice pattern
Some clear words with good voice pattern
Uses vowels to approximate words
3
Uses different voice patterns
2
meaningfully
Vocalization as means of
1 Vocalizes freely and on demand
communication
Figure 1. The Korean version of Lings model (K-Ling) of the seven speech acquisition stages.
63
contrasts. Therefore, K-Ling represents the serial
and parallel order in which speech patterns develop.
We identied K-Ling as a valid evaluation tool for
speech development in young children who have
undergone CI and who are in the early stages of
language development in our previous study [14].
In addition, we assessed the main communication
mode before and after CI and categorized the parti-
cipants as having nonverbal communication, vocali-
zation, and oral communication based on the K-Ling.
The nonverbal communication level was dened as
no vocalization output depending on communicative
intents, with phonation at the reexive level such as
vegetative sounds, grunt-like sounds with mufed
resonance, or vowel-like sounds that could not be
transcribed as an adult vowel. When children could
control their phonation and when they could also keep
the consonant-vowel syllable structure including
vowel nuclei, we judged that their communication
mode had reached the vocalization level. The use of a
word approximated product as a means of commu-
nication was considered vocalization; that is to say,
K-Ling stage 1 or 2. Children using this communi-
cation mode are able to deliver what they think and
want to tell through vocalization. Lastly, the oral
communication level corresponds to K-Ling stage
37; children at this level not only use some clear
7 Consonant: liquid
6 Consonant: fricatives
5 Consonant: affricatives
Most vowels & some consonants
4
(velar stops)
Most vowels & some consonants
(nasal, bilabial/alveolar stops)
Base of supra-segmental patterns & some vowel
with voice pattern
Phonetic development
64 H-Y. Youm et al.
words, phrases, or sentences with a good voice pattern
but use most vowels with good articulation.
One experienced speech language pathologist
working at our CI center assessed the participants
according to CAP score and K-Ling stage to evaluate
their longitudinal progress preoperatively and 3, 6,
and 12 months postoperatively. The main communi-
cation mode was evaluated before and 12 months
after implantation. When all patients were perio-
dically followed up and monitored, data relevant to
communication mode were gathered and the assess-
ments largely coincided with the parents comments.
We compared the results in the ID group and
control group.
Statistical analysis
We used the two sample t test to identify differences
in the baseline characteristics of the two groups,
which were continuous variables. The generalized
estimating equation (GEE) was used to investigate
longitudinal changes in auditory perception and
speech production performance between the ID
group and control group. All analyses were performed
with the Statistical Package for Social Science package
version 19 (SPSS, Chicago, IL, USA). The indepen-
dent variables were accompanying ID and duration of
cochlear implant use, and the dependent variables
were changes in auditory and speech performance
after the surgery. A p value 0.05 was considered
statistically signicant.
Results
Demographics and hearing results
The ID group consisted of 14 children. Among them,
mild ID was identied in nine and moderate ID was
found in ve. Age at CI ranged from 1.8 to 11.1 years
(mean SD, 5.8 2.9 years). Fourteen age- and
gender-matched cochlear implant recipients without
ID were allocated to the control group and their mean
( SD) age at time of implantation was 6.1 3.0 years
(range 110 years). Age at CI was not different
between the two groups (p = 0.776, two sample
t test). Fourteen children in the experimental group
were classied as the mild vs moderate group: nine
patients were allocated to the mild group and another
ve patients to the moderate group. Although the
number of children in the mild and moderate groups
was not the same, their mean age was 5.6 and 7.0,
respectively, and this difference was statistically
insignicant (p = 0.435).
Post implant hearing evaluation was done at
regular intervals; 3 months, 6 months, and 1 year
p = 0.030*
50
Warble tone threshold (dB HL)
40
30
20
10
0
Control group ID group
Figure 2. Mean warble tone threshold in the intellectual disability
(ID) group and control group after cochlear implantation. The
average of warble tone threshold after implantation in the ID group
was 30.98 dB HL (SD, 5.42), which was signicantly worse than
that in the control group, 27.11 dB HL (SD, 3.28) (*p = 0.030).
Warble tone threshold: average air conduction threshold at 500,
1000, 2000, and 4000 Hz, 6 months postoperatively.
*Statistical signicance.
postoperatively, and then annually. The authors
calculated averages of warble tone threshold at 500,
1000, 2000, and 4000 Hz, at 6 months post implan-
tation. The average aided air-conduction threshold
of the children with intellectual impairment was
30.98 dB HL (SD, 5.42), while that in the control
group was 27.11 dB HL (SD, 3.28). Although the
mean hearing impairment in the ID group was sig-
nicantly worse than that in their counterparts
(Figure 2, p = 0.030, two sample t test), we found
that the children with ID and profound hearing loss
could also achieve a favorable threshold in the sound
eld after CI. Individual audiometric data including
the frequencies tested and individual scores of all
28 subjects are demonstrated in detail in Tables III
and IV.
Categories of Auditory Performance (CAP)
Children with accompanying ID had lower scores on
the CAP measures. The mean CAP score in the ID
group was initially 0, which improved gradually to 4 at
12 months post implantation. The average CAP score
before CI in the control group was 3, and it increased
to 6 at 1 year after implantation. The CAP score in the
ID group was signicantly lower than that in the
control group at all times and this difference was
signicant (Figure 3, p = 0.038, GEE). However,
both groups of children demonstrated obvious
improvements in CAP score during the rst year
postoperatively (p < 0.001, GEE). The interaction
between time after CI and degree of accompanying
ID showed signicant difference in the performance
of auditory perception (p = 0.006, GEE).
 Auditory performance of children with intellectual disability after CI 65

Table III. Individual subject data for the warble tone threshold after cochlear implantation (CI) in the group of children with intellectual
disability (ID).

Patient no. Gender Mean warble tone threshold 250 Hz 500 Hz 1 kHz 2 kHz 4 kHz 8 kHz

1 F 27.5 30 20 30 30 30 25
2 F 28.75 35 35 20 35 25 35
3 F 28.75 35 35 35 20 25 35
4 F 23.75 20 25 25 20 25 25
5 F 45 50 45 45 45 45 45
6 F 31.25 30 30 30 30 35 35
7 M 32.5 35 25 35 35 35 30
8 M 25 25 30 20 25 25 25
9 M 31.25 25 30 30 30 35 40
10 M 25 30 25 25 25 25 25
11 M 32.5 45 35 30 30 35 35
12 F 35 30 35 35 35 35 40
13 M 32.5 30 25 30 35 40 50
14 F 35 35 35 35 30 40 35

Korean version of Lings stages (K-Ling)
Phonetic development was not different between
the two groups (Figure 4, p = 0.199). However,
phonetic development was apparent in both groups
as time passed (p < 0.001). We identied an inter-
action effect of accompanying ID on phonetic deve-
lopment with the use of CI (p = 0.049, GEE).
No difference between the two groups was observed
for phonological development (Figure 4, p = 0.308),
as both groups showed improvements over time
(p < 0.001). The interaction between time after
implantation and mental status showed no signicant
difference (p = 0.292).
We performed a post hoc analysis of the group of
patients with ID according to the severity of accom-
panying intellectual impairment. Nine patients with
mild ID and hearing loss revealed signicant improve-
ment in auditory perception and language acquisition
performance after the surgery: median CAP score was
1 before CI and 4 at the last follow-up; phonetic
development went from 1 to 3, phonologic develop-
ment went from 2 to 4 (p < 0.001, p < 0.001, and
p = 0.001, respectively). In contrast, ve patients with

Table IV. Individual subject data for the warble tone threshold after cochlear implantation (CI) in the control group.

Patient no. Gender Mean warble tone threshold 250 Hz 500 Hz 1 kHz 2 kHz 4 kHz 8 kHz

1 F 31.25 35 30 30 30 35 40
2 F 31.25 20 30 30 30 35 35
3 F 23.75 25 20 25 25 25 30
4 F 23.75 25 25 25 25 20 25
5 F 21.25 20 25 20 15 25 25
6 F 25 30 25 25 25 25 30
7 M 27.5 30 25 25 30 30 25
8 M 28.75 30 30 30 25 30 30
9 M 28.75 25 30 30 30 25 25
10 M 25 20 20 25 25 30 25
11 M 32.5 30 30 35 35 30 30
12 F 26.25 25 15 30 25 35 35
13 M 28.75 30 30 25 30 30 30
14 F 28.75 25 25 30 30 30 30
66 H-Y. Youm et al.

7
6 *
5
4 *

CAP
3
2 * Mild ID group

1 Moderate ID group
Control group
0
Pre 3 months 6 months 12 months

Figure 3. Categories of Auditory Performance (CAP) scale in the mild and moderate intellectual disability (ID) groups compared to that in the
control group. The CAP score in the ID group was signicantly lower than that in the control group at all times and this difference was
signicant (*p = 0.038). The median CAP score in the mild ID group was 1 before cochlear implantation (CI) and 4 at the last follow-up
(*p < 0.001), while it changed from 0 to 2 in the moderate group (*p = 0.034). No signicant difference was identied between the two groups.

moderate ID showed improvement only in CAP score
and median score changed from 0 to 2 (p = 0.034).
Individual CAP growth curves for the children with
ID in the mild and moderate groups are displayed
in Figure 5. In the moderate group, development in
phonetic and phonologic levels of acquisition was not
signicant (p = 0.436): the scores were both 0 before
implantation, and 1 at 12 months postoperatively
(Figure 6). None of the test variables, including the
CAP score and K-Ling, were different between the
mild and moderate groups. Longitudinal changes in
communication mode between the experimental and
control groups were tested using GEE and revealed a
signicant difference between the two groups
(p = 0.009) and according to the time after CI
(p = 0.001) (Figure 7). The interaction between group
and time was not signicant (p = 0.348).
Discussion
In a previous study, Uziel et al. reported that about
5% of the deaf population are children with additional
handicaps; blindness was the most common disability
and ID was second [15]. The presence of an addi-
tional handicap such as ID in hearing-impaired
children makes it difcult to predict developmental
outcomes after CI. Developmental delay and poor
communication skills before implantation are poor
prognostic factors for language development after
CI [16]. On the other hand, Hamzavi et al. concluded
that cochlear implants provide substantial benet for
multiply handicapped children. They insisted that
cochlear implants change the lifestyle of prelingually
deaf children with severe performance disturbances or
intellectual decits [6]. Furthermore, Waltzman et al.
stated that multiply handicapped children also
develop communication skills, social interactions,
and a general connectedness with the environment
in addition to auditory skills with the use of a cochlear
implant [7]. Another study by Meinzen-Derr and
colleagues reported post-cochlear implant language
outcomes of 20 children with heterogeneous devel-
opmental disabilities: they have made measurable
progress in the area of both receptive and expressive
language skills after CI, which offers an opportunity
for auditory stimulation and subsequent oral commu-
nication [10]. It is important to understand the gains
in auditory and speech development that these
children will experience if they undergo CI. One
must keep in mind that this group of children is

7 7
6 6 *
Phonologic level
Phonetic level

5 * 5
K-Ling

K-Ling

4 4 *
3 * 3
2 Mild ID group
2
Moderate ID group
1 1
Control group
0 0
Pre 3 months 6 months 12 months Pre 3 months 6 months 12 months

Figure 4. The Korean version of Lings model (K-Ling) score in the mild and moderate intellectual disability (ID) groups compared to that in
the control group. Although phonetic and phonological development was not different between the experimental and control groups, their
development was apparent in both groups. Mean phonetic development in the mild group went from 1 to 3 and phonologic development went
from 2 to 4 (*p < 0.001 and *p = 0.001, respectively). On the other hand, the change was not signicant in those with moderate ID (p = 0.436).
*Statistical signicance.
 Auditory performance of children with intellectual disability after CI 67

7 7
6 6
5 5
4 4
CAP

3 3
2 2
1 1
0 0
Preoperative 1 year post CI Preoperative 1 year post CI

Mild group (n = 9)* Moderate group (n = 5)*

Figure 5. Individual Categories of Auditory Performance (CAP) growth curves of the children with mild vs moderate intellectual disabilities
(IDs). Median CAP development between preoperative and 1 year post-implantation evaluation in the children with mild ID was from 1 (0, 4)
to 4 (3, 5) (*p < 0.001), while that in the moderate group was 0 (0, 2) to 2 (1, 4.5) (*p = 0.034). Both groups of children were able to make
progress during the rst year post implant.
*Statistical signicance.

very different from those who are simply hearing
impaired, and they may achieve lower scores than
those without ID. To our knowledge, the present
study is the rst to enroll and assess auditory and
speech performance in children with ID coexisting
with a profound sensorineural hearing loss and
age- and gender-matched controls without additional
disabilities simultaneously. The experimental group
was relatively homogeneous, making outcome
prediction more easier.
In our study, children with intellectual impairment
showed poorer average warble tone threshold after CI
compared with that of the control group. Never-
theless, we found that children in the ID group could

7 7

6 6
5 5
Phonetic level

4 4
3 3

2 2

1 1
0 0
Preoperative 1 year post CI Preoperative 1 year post CI

Mild group (n = 9)* Moderate group (n = 5)

7 7

6 6
Phonologic level

5 5

4 4
3 3

2 2

1 1
0 0
Preoperative 1 year post CI Preoperative 1 year post CI

Figure 6. Individual K-Ling score changes of the children with mild vs moderate intellectual disabilities (IDs). In the mild group, phonetic and
phonologic development was signicant: from 1 to 3 (*p < 0.001) and 2 to 4 (*p = 0.001), respectively. In the moderate group, changes in
phonetic and phonologic levels of acquisition were not signicant (p = 0.436).
*Statistical signicance.
68 H-Y. Youm et al.

p = 0.001*

Before implanation After implanation

14
14
13
13
12 12
Communication mode

11 11
10 10
9 9
8 8
7 7
6 6
5 5
4 4
3 3
2 2
1 1
0 0
Control group ID group Control group ID group

p = 0.009*

Figure 7. Communication mode before and after cochlear implantation (CI) in the intellectual disability (ID) and control groups.
Communication mode in the two groups revealed a signicant difference (*p = 0.009) and according to the time after CI (*p = 0.001).
Light gray, non-verbal; deep gray, vocalization; black bar, oral communication.
*Statistical signicance.

also achieve a favorable threshold (mean, 30 dB) in
the sound eld with the use of a cochlear implant,
which means that they can hear sound using the
implanted device. However, processing the sound
to perceive sounds as speech and produce them as
a language is another issue. This sound processing
needs higher cognitive function. Previous study on
children with cognitive delays has reported some form
of measurable post implant gains in speech perception
and word or sentence recognition, although progress
was often slower than in typically developing children
with cochlear implants [17]. We aimed to determine
the auditory perception and speech outcome in these
children with ID, by using CAP and K-Ling.
Children with ID also showed lower scores on CAP
measures. However, both hearing-impaired children
with ID and those without such disability demon-
strated signicant improvements in auditory percep-
tion over time on every test administered, and the
results were statistically signicant. Furthermore,
both the simply hearing-impaired group and the
experimental group of children also revealed signi-
cant advancements in phonetic and phonological
development during the follow-up period. These
results are in accordance with previous results that
even children with additional intellectual handicaps
can show a demonstrable benet after CI [8,9].
In addition, we analyzed main communication
mode to identify the real-life benet of CI in these
handicapped children. This study presents various
types of vocalizations as a means of communication
in infants and young children for the rst time, which
were categorized into nonverbal communication,
vocalization, and oral communication. In our series,
the children with ID developed their communication
skills with the aid of CI and a perioperative rehabi-
litation program. Of interest, two-thirds of children
with intellectual impairment could communicate
orally at 1 year following CI, which means that a
signicant proportion of these handicapped children
can be members of the oral communication society
with CI.
When we performed a post hoc analysis after
stratication to determine the effect of the severity
of accompanying ID on postoperative outcome,
children in the mild group showed signicant
improvement in all test parameters regarding auditory
perception and language acquisition performance
after CI, as we expected. On the contrary, children
in the moderate group obtained limited benet
despite the use of CI; only a tendency for improve-
ment in the CAP score was evident in the ve subjects
after implantation, but the differences were not
signicant, and the average score did not exceed 2.
These ndings suggest that the children with
profound hearing loss and accompanying ID,
especially mild disability, can benet obviously
from CI. For children with more severe intellectual
impairment, however, the benet from CI would be
minimal as compared to those with mild disabilities.
This study would have been strengthened had it been
designed as a prospective setting to include the same
number of children in the mild and moderate groups,
whose demographic variables do not differ. The
current study subjects were composed of nine
children in the mild group and ve children in the
 Auditory performance of children with intellectual disability after CI
moderate group. Their mean age at implantation was
5.6 (SD, 2.7) and 7.0 years (SD, 3.7), respectively,
and this difference was not statistically signicant. It is
possible that some children in the moderate group
received their implants later than children in the mild
or control groups according to the medical necessities
of each child. A number of factors contributed to a
later age at implantation in our patients. Two children
required life-sustaining surgery and one needed
medical intervention; therefore they needed some
time to be suitable for cochlear implantation.
Our ndings suggest that although auditory,
speech, and linguistic gains may be inuenced by
their underlying cognitive abilities, these limitations
do not appear to preclude benet from cochlear
implant stimulation, as assessed by CAP, K-Ling,
which is our newly validated measure of speech and
language development, and communication mode.
Providing children that have IDs with cochlear
implants can result in substantial benets regarding
auditory skills and expressive language, and they
should not be contraindicated for CI. Therefore,
we recommend that hearing-impaired children with
IDs are considered for CI, although all are not
typically considered good candidates.
Acknowledgments
The authors thank the children and their families who
participated in the research. This work was supported
by a grant from the Strategic Technology Deve-
lopment Program of the Ministry of Knowledge
Economy (10031764), and a grant from the Seoul
R&D Program (SS100022).
Declaration of interest: The authors report no
conicts of interest. The authors alone are responsible
for the content and writing of the paper.
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