Outcomes and benefits of pediatric cochlear implantation in children with additional disabilities: a review and report of family influences on outcomes

Figures & data

Table 1 Prevalence data for common comorbid disorders with hearing loss

Comorbid condition	Prevalence of disorder in all children	Sex differences	Prevalence of hearing loss	Prevalence of an additional disability in children with hearing loss
Autism spectrum disorder	One per 68^Citation12	5:1 male to female ratio^Citation12	1%–6% have some degree of sensorineural hearing loss^Citation100	4%^Citation101
Developmental delay	One per 6^Citation20	1.3 male to female ratio^Citation20	10.2% have some degree of sensorineural hearing loss^Citation77	8.8%^Citation14
CHARGE syndrome	One per 9–10,000^Citation102	Equal sex representation^Citation44	34%–38% have severe to profound hearing loss,^Citation43,Citation44 75% have some degree of hearing loss	NR
Cerebral palsy	One per 323^Citation103	1.3: male to female ratio^Citation103	12%–25% have some degree of sensorineural hearing loss^Citation53	NR
Learning disorder	50–150 per 1,000^Citation20	2–3:1 male to female ratio^Citation20	NR	7.20%^Citation14
Usher syndrome	10 per 100,000^Citation62	Equal sex representation^Citation104	100% have some hearing loss^Citation104	3%–6%^Citation105
Waardenburg syndrome	One per 42,000^Citation70	Equal sex representation^Citation70	35%–78% of children with WS I and 55%–91% of children with WS II^Citation71,Citation72	2%–5%^Citation73,Citation77
ADHD	50 per 1,000^Citation20	2:1 male to female ratio^Citation20	NR	5.4%^Citation14

Abbreviations: NR, not reported; ADHD, attention deficit/hyperactivity disorder; WS, Waardenburg syndrome.

Figure 1 Various disabilities discussed in this paper placed on a continuum from minimal potential benefit to maximum potential benefit based on a review of the literature.

Abbreviations: ADHD, attention deficit/hyperactivity disorder; CI, cochlear implant; CP, cerebral palsy; DD, developmental delay.

Table 2 Studies in children with cochlear implantation and additional disabilities

Reference	Date	Sample (n)	Mean age at CI (months)	Mean length of CI use (months)	Outcome measures	Results
Autism spectrum disorder
Hayman and Franck^Citation106	2005	2	Child 1, 12 months; child 2, 135 months	Child 1, 30 months; child 2, N/A	ESP, MLNT	Child 1 had increased social/emotional responsiveness and nonverbal communication Child 2 was in a total communication class for children with CIs
Robertson^Citation17	2013	10	51 (SD 41)	N/A	CAPS, SIR	6 of 10 children used their CI consistently One child used spoken language as their main form of communication, 3 used some speech and some signs, and 6 used nonverbal communication
Beers et al^Citation107	2014	Literature review	N/A	N/A	N/A	Children with ASD and CIs displayed improved social communication, behavioral and environmental awareness, increased vocalizations, eye contact, and reaction to music after implantation
Meinzein-Derr et al^Citation18	2014	14; 10 hearing aid controls	33.6 (SD 21.4)	N/A	PLS-4, CELF	4 children with ASD achieved oral communication skills postimplantation 8 children with ASD used a form of augmented communication (eg, Picture Exchange Communication System)
Donaldson et al^Citation19	2004	7	63.4 (SD 31.5)	25	MAIS, IT-MAIS, GASP-W, GASP-S, PPVT-III, EVT, CDI	Children with ASD experienced increases in vocalization, eye contact, use of sign language, reaction to sound, and response to requests following implantation
Fitzpatrick et al^Citation108	2014	17	N/A	N/A	Retrospective chart review	Higher prevalence of hearing loss in children with ASD than in general population Audiologic profiles of children with ASD were highly variable
Developmental delay
Fukuda et al^Citation35	2003	1	56 (SD N/A)	Assessed at 6, 9, 12, 15, and 24	KSPD, Type 67 Test Battery	The child achieved monosyllable speech ability and audiometric results similar to children without ADs
Holt and Kirk^Citation24	2005	19; 50 CI controls	DD 37.7 (SD 14.6); non-DD 28.84 (SD 13.2)	12	IT-MAIS, GAEL-P, Mr Potato Head Task; PSI, PPVT-III, RDLS	Children with and without DD had significant improvements in speech and language Children with DD performed significantly worse on tests of expressive and receptive language
Edwards et al^Citation28	2006	11; 21 CI controls	28 (SD N/A)	24	SIR, IT-MAIS, E2L	Children with DD were delayed in speech perception or speech production Children with mild DD made progress in areas of speech perception and speech intelligibility 1–2 years postimplantation
Meinzen-Derr et al^Citation109	2011	15; 15 DD controls	21 (SD N/A)	12	PLS-4	Children with ADs had significantly lower receptive and expressive language quotients than children without ADs
Amirsalari et al^Citation78	2012	28: 234 CI controls	N/A	24	CAP, SIR	No significant differences were found between children with motor DD and children without motor DD in CAP and SIR Children with motor DD showed significant improvement on CAP and SIR scores following implantation
Eze et al^Citation110	2013	Literature review	N/A	N/A	N/A	Children with mild DD had comparable outcomes to children with without DD All parents of children with DD stated that they would choose implantation for their child again
Hiraumi et al^Citation22	2013	11; 24 CI controls	37 (SD N/A)	24	CV, PPT	Preoperative DD negatively affected postimplant speech perception outcomes Children with mild DD achieved speech perception scores comparable to children without DD
Wakil et al^Citation27	2014	21	56.4 (SD 2.9)	135.6	IT-MAIS, ESP, GASP, PBK, HINT	Severe DD, limited improvement from CI, although some level of basic auditory skills (sound awareness, association of meaning with sounds, and vocalization behavior) developed from CI use; mild/moderate DD, developed open-set speech recognition; suggests level of DD has impact on effect of CI
CHARGE syndrome
Bauer et al^Citation42	2002	6; 5 CI controls	70 (SD 40)	36	ESP, GASP, IT-MAIS	3 of the 5 children demonstrated improved speech perception All children with CIs experienced improved quality of life after implantation
MacArdle et al^Citation111	2002	4	76.3 (SD 35.7)	48	LSQ-E, MPT, MJWL, SIR	All children showed improvement in receptive language scores postimplantation All children displayed unintelligible speech postimplantation
Au et al^Citation47	2004	1	30 (SD N/A)	Assessed at 12, 24, 36, and 48 months	RDLS-Cantonese version	Speech perception gradually improved after implantation
Buchman et al^Citation112	2004	28	66 (SD 59)	29	ESP, PBK	Children with inner ear malformations were slower to develop speech perception than children without malformations
Lanson et al^Citation44	2007	10	26.4 (SD 10.8)	43.6	IT-MAIS, 9 children tested	All children showed improvements on the IT-MAIS postimplant All children demonstrated increased awareness of environmental sounds and increased vocalizations
Southwell et al^Citation39	2010	3	Child 1, 17 years; child 2, 48 months; child 3, 36 months	6.3	HINT-C	All children reported significant improvements in hearing skills following implantation
Ahn and Lee^Citation45	2013	6	58 (SD 40)	58.4	IT-MAIS, CAP, SIR	Language and speech improved in 5/6 children over time Children with CHARGE syndrome showed varying but steady improvement in auditory performance
Cardoso et al^Citation113	2013	1	52	41	IT-MAIS, MUSS	The child reached highest category of auditory perception The child’s IT-MAIS score rose from 5% preimplant to 90% postimplant
Ricci et al^Citation43	2014	5	37 (SD 16)	29	ESP, IT-MAIS	2 children used oral language as the sole mean of communication; one child started using oral language as main mode of communication 2 children did not develop linguistic abilities
Cerebral palsy
Bacciu et al^Citation53	2009	5	91.6 (SD 24.3)	28.2	ESP, GASP, SIR	All children achieved improved SIR scores 1 year after CI All children improved in speech perception and speech intelligibility over time
Steven et al^Citation52	2011	36	42.6 (SD 23.2)	55.2	SRS	The degree of cognitive impairment was a strong predictor of outcomes postimplantation More severe cognitive impairment led to poorer outcomes, but these children still showed improvement in speech perception and production
Byun et al^Citation51	2013	8; 8 CI controls	21.6 (SD 5.3)	24	CAP, K-Ling, SELSI	4 children with CP had speech and language outcomes comparable with age-matched and sex-matched peers 4 children with severe CP did not produce meaningful speech by 24 months
Learning disability
Issacson et al^Citation114	1996	5; 5 CI controls	LD 64.2 (SD 17.4); non-LD 62 (SD 18.4)	6, 12, 18, 24, and 34 months	DAT, MTS, SERT, WIPI, GASP, MAC, CID, Iowa Medial Vowel and Medial Consonant Tests, NU No 6, PPVT, K-ABC	Children with LD showed speech and language benefits from CI, but their progress was slower and more inconsistent
Usher syndrome
Hinderink et al^Citation115	1994	4	USH 249 (SD 76.2); no-USH 258.6 (SD 101.3)	24	MST, AN, Gestel-Nijmegen Implant Questionnaire	All children demonstrated improvements in speech perception and pattern recognition Children with USH achieved speech perception scores comparable with the control group
Jenison et al^Citation116	1995	2	N/A	36	ESP, NU-CHIPS, GASP	Both children use their CIs full-time and have shown improvement in speech perception skills Both children showed a plateau in improvement during the decline of their vision
Damen et al^Citation67	2006	7; 2 USH controls	75.6 (SD 28.8)	110	NCIQ, SF12, EHL	Children demonstrated increased speech perception abilities after implantation Children expressed greater independence following implantation
Liu et al^Citation61	2008	9	64.8 (SD 37.2)	24	IT-MAIS, ESP, NU-CHIP, MLNT, LNT, PBK, CAP, QACIU	Children with USH type 1 exhibited improvement in speech and auditory perception Children with severe visual impairment benefited most from implantation
Henricson et al^Citation64	2012	7; 33 CI controls; 43 hearing aid controls; 120 normal hearing controls	13 (SD 14)	110	VMPT, NWD, PR, PI, SDM, WM, Word Spotting	Children with USH type 1 performed similarly to the control group on cognitive tests, but demonstrated poorer phonologic working memory
Jatana et al^Citation65	2013	26	39.6 (SD 37.2)	93.6	ESP, MLNT, LNT, PBK, CNC, HINT	The majority of children with USH achieved some level of open-set speech perception 69.2% of children were using oral or primarily oral communication by time of last follow-up
Waardenburg syndrome
Sugii et al^Citation77	2000	1	48 (SD N/A)	24	Ling Sound Test; Open-set word testing	The child achieved 58% on open-set word testing with auditory cues 2 years postimplantation Speech and language abilities were delayed
Daneshi et al^Citation80	2005	6	79 (SD 54.1)	42.8	PAPT/HI, Persian Spondee words test; CAP, SIR	Children with WS demonstrated significant improvement in speech perception and speech intelligibility All children eventually transferred to regular educational settings
Migirov et al^Citation82	2005	5	58.1 (SD 73.2)	52.8	ESP, IT-MAIS	4 children with WS achieved open-set recognition of monosyllabic words, and all 5 children achieved open-set recognition of 2-syllable words Children with WS achieved speech perception outcomes comparable to implanted children without WS
Cullen et al^Citation73	2006	6	37 (SD 20)	69	ESP, PBK	All children achieved some degree of closed-set and open-set speech Overall, children with WS performed extremely well following implantation
Pau et al^Citation76	2006	13	N/A	12	Melbourne Speech Perception Categories	4 children had EABR and lower speech perception scores than the other patients with normal EABR; children were diagnosed with auditory neuropathy
Deka et al^Citation81	2010	4	63.9 (SD 52)	12	CAP, MAIS, SIR	WS group achieved postimplant scores comparable to children without WS All children with WS significantly benefitted from CI
Kontorinis et al^Citation75	2011	25	70.8 (SD 61.2)	99.6	HSM sentence test, FMT, CAP	9 of 25 patients achieved monosyllabic scores better than 80%
Amirsalari et al^Citation78	2012	6; 75 CI controls	26 (SD 15.8)	12	CAP, SIR	Children with WS showed significant improvement postimplantation on CAP and SIR WS group had lower SIR scores than control group
De Sousa Andrade et al^Citation79	2012	7; 261 CI controls	30.6 (SD 9.7)	57.7	IT-MAIS, CAP, MUSS, SIR	Speech perception skills slightly worse in children with WS versus children without WS All children with WS showed significant improvements in speech perception over time
Attention deficit/hyperactivity disorder
Pundir et al^Citation88	2007	2	Child 1, 72 months; child 2, 84 months	9	ISLD	Both cases improved after medication was used to address ADHD-related issues
Moreno Torres et al^Citation87	2010	1	18 (SD N/A)	Dec-60	LAQ, TNI-2, PPVT-III	After 5 years of implant use, the child scored at the mean for children of the same auditory age on expressive and receptive language tests
Retrospective reviews collapsing disabilities
Hamzavi et al^Citation60	2000	10	47.2 (SD 19.3)	1, 3, 6, 12, 18, 24, and 36 months	EARS, MTP	9 of 10 children showed significant improvement in their ability to make speech sounds Children without DD reached a greater mean speech perception level than children with DD at each assessment point postimplant
Pyman et al^Citation25	2000	20; 55 CI controls	38.4 (SD 14.4)	12	Classification of Performance Levels	Children with DD developed closed set speech perception and open-set speech perception with the aid of lip-reading
Waltzman et al^Citation57	2000	29	50.4 (SD 30)	28.8	ESP, NU-CHIPS, GASP, PBK, MLNT, LNT, Common Phrases sentence test	Children with DD benefited from CI, but the development of auditory and linguistic skills was slower and less stable than children without ADs 59% of the children with ADs used oral communication postimplant
Rajput et al^Citation83	2003	85; 21 CI controls	48 (SD 20.4)	N/A	RLIM, SIR	Children with syndromal disease had lower receptive language and speech intelligibility scores than other disability groups postimplant
Dettman et al^Citation33	2004	22; 27 CI controls	DD 29.1; non-DD 26.4 (SD N/A)	37.3	PVT, PBK, CNC, BKB	Children with severe DD had poorer speech perception than those with no DD or mild DD All children showed some benefit from CI
Filipo et al^Citation34	2004	18	76.8 (SD 3.1)	34.7	Listening Progress Profile; Test Abilita Percettive; Test Identificazione Parole Infantili; Bi-Trochee-Polysyllabic word test; Test Abilita Uditive Varese; Common Protocol; Erber’s categories	All children showed improved listening and communication skills following implantation Deaf-blind patients achieved the best outcomes
Wiley et al^Citation59	2005	16	58.8 (SD 56.4)	56.4	Open-ended and close-ended questions	All children had greater awareness of environmental sounds, made progress in speech, and were more attentive following implantation
Wiley et al^Citation58	2006	45	GJB2+ 23 (SD N/A); GJB2–26 (SD N/A); GJB2 NT 55 (SD N/A)	33.5	N/A	62% of GJB2+ children were oral communicators; 67% of GJB2–children were oral communicators; 38% of GJB2 children were oral communicators
Daneshi and Hasanzadeh^Citation56	2007	60	68 (SD 32.2)	12	Revised Leiter International Performance Scale, PAPT/HI	Auditory perception improved in all disability groups Children with CP showed fastest improvement in auditory perception and children with ASD showed slowest improvement
Edwards et al^Citation28	2007	Literature review	N/A	N/A	N/A	Children with ADs, especially a mild cognitive delay, experienced numerous benefits with CI, but those with more severe disabilities showed slower rate of progress
Berrettni et al^Citation31	2008	23	75 (SD 52)	30	PCAP, TIPI 1, TIPI 2	Following implantation, 53% of children attained the highest category of speech perception All children with CI and DD showed improvement in environmental awareness, speaking skills, and interaction with peers
Nikolopoulos et al^Citation37	2008	67; 108 CI controls	ADs 40.8 (SD N/A); No ADs 39.6 (SD N/A)	60	SIR	Children with DD had similar performance on measures of speech intelligibility
Wiley et al^Citation26	2008	14; 21 CI controls	ADs 17; No ADs 16	12	ASC	Children with DD achieved the same rate of auditory skills as children without DD Children with more severe cognitive disabilities improved at half the rate than children with mild cognitive delays
Meinzen-Derr et al^Citation110	2010	20	24 (SD N/A)	28	PLS-4, RI-TLS	Nonverbal cognition, duration with CI, age at hearing loss diagnosis, and number of different therapies were related to post-CI language skills
Beer et al^Citation20	2012	23; 23 CI controls	24 (SD 10)	12	IT-MAIS, PLS-4, Vineland-II	Children with ADs made progress in functional auditory skills within 1 year of implant use Degree of loss, age, and age at implantation did not correlate with progress in functional auditory skills in children with ADs
Birman et al^Citation117	2012	29; 65 CI controls	N/A	12	CAP	Children with ADs had slower progress and poorer results than those without ADs Children with ADs showed greater social engagement/interaction and self-sufficiency over time
Cruz et al^Citation16	2012	157; 31 CI controls	30.1 (SD 15.1)	Assessed at 12, 24, and 36 months	RDLS	Children with ADs have slower rate of growth in oral language than those without ADs; slowest progress was for children with autism Some children with ADs had increases in externalizing behaviors compared with the control group
Wiley et al^Citation118	2012	6	41.7 (SD 45.8)	12	PLS-4	Children with ADs demonstrated improvement in spoken language
Broomfield et al^Citation30	2013	38	39 (SD N/A)	115.5	BKB, SRS	At follow-up, 82% of children were using their CI full time Variable outcomes were found within the syndromic group
Cupples et al^Citation32	2013	34; 85 hearing aid controls	17.5 (SD 7.5)	>10	PLS-4, PPVT-4, DEAP, CDI, PEACH, SIR	Children with ASD, CP, and/or DD had lower receptive and expressive language than those without DD. All children showed improvements in auditory performance after implantation
Janeschik et al^Citation119	2013	115; 148 CI controls	62.1 (SD N/A)	65.4	Mainzer and Gottinger Test for Speech Comprehension in Childhood	Children with syndromal diseases performed significantly worse on all audiologic tests when compared with children with other etiologies

Abbreviations: ESP, Early Speech Perception test; MLNT, Multisyllabic Lexical Neighborhood Test; CAP, Categories of Auditory Performance; SIR, Speech Intelligibility Rating; PLS-4, Preschool Language Scales, Fourth Edition; CELF, Clinical Evaluation of Language Fundamentals; MAIS, Meaningful Auditory Integration Scale; IT-MAIS, Infant-Toddler-MAIS; GASP-W, Glendonald Auditory Screening Procedure for Words; GASP-S, Glendonald Auditory Screening Procedure for Sentences; PPVT-III, Peabody Picture Vocabulary Test; EVT, Expressive Vocabulary Test; CDI, MacArthur-Bates Communicative Development Inventory; KSPD, Kyoto Scale for Psychological Development; GAEL-P, Grammatical Analysis of Elicited Language Pre-Sentence Level Test; PSI, Pediatric Speech Intelligibility Test; RDLS, Reynell Developmental Language Scales; CV, Consonant-Vowel Syllable Perception Test; PPT, Phrase Perception Test; PBK, Phonetically Balanced Monosyllabic Word List -Kindergarten; HINT, Hearing in Noise Test; E2L, E2L Toy Test; LSQ-E, Listening Skills Questionnaire-Environmental sounds; MPT, Manchester Picture Test; MJWL, Manchester Junior Word Lists; HINT-C, Hearing-in-Noise Test; MUSS; Meaningful Use of Speech Scale; SRS, Modified Speech Reception Score; K-Ling, Korean Version of the Ling’s Stage; SELSI, Sequenced Language Scale for Infants; DAT, Discrimination After Training; MST, Monosyllable, Trochee, Spondee Word and Stress Test; SERT, Sound Effects Recognition Test; WIPI, Word Intelligibility by Picture Identification Lists; MAC, Minimal Auditory Capability Battery; CID, Central Institute for the Deaf Everyday Sentences; NU No 6, Iowa Medial Vowel and Medial Consonant Tests; Northwestern University Auditory Test No 6; K-ABC, Kaufman Assessment Battery for Children; AN, Antwerp-Nijmegen Test Battery; NU-CHIPS, Northwestern University Children’s Perception of Speech; NCIQ, Nijmegen Cochlear Implant Questionnaire; SF12, Standard Medical Outcome Study Short-Form 12; EHL, Equivalent HL; LNT, Lexical Neighborhood Test; QACIU, Assessment of Cochlear Implant Use; VMPT, Visual Matrix Pattern Test; NWD, Nonword Discrimination; PR, Phonological Representations; PI, Phoneme Identification; SDM, Semantic Decision Making; WM, Word Mobilization; CNC, Consonant-Nucleus-Consonant words test; PAPT/HI, Persian Auditory Perception Test for the Hearing Impaired; FMT, Freiburg Monosyllabic Test; ISLD, Integrated Scales of Language Development; LAQ, LittlEars Auditory Questionnaire; TNI-2, Test of Nonverbal Intelligence-2; EARS, Evaluation of Auditory Response to Speech; MTP, Monosyllable Trochee Polysyllable Test; RLIM, Receptive Language Improvement score; PVT, Picture Vocabulary Test; BKB, Bamford-Kowal-Bench; PCAP, First Speech Perception Categories; TIPI 1, Test of Identification of Words for Children 1; TIPI 2, Test of Identification of Words for Children 2; ASC, Auditory Skills Checklist; RI-TLS, Rossetti Infant-Toddler Language Scale; Vineland-II, Vineland Adaptive Behavior Scale, Second Edition; DEAP, Diagnostic Evaluation of Articulation and Phonology; CDI, Child Development Inventory; PEACH, Parent Evaluation of Aural/Oral Performance of Children; EABR, electric auditory brainstem response; ADs, additional difficulties; CI, cochlear implant; N/A, not available; SD, standard deviation; ASD, autism spectrum disorder; DD, developmental delay; CP, cerebral palsy; LD, learning difficulty; USH, Usher syndrome; ADHD, attention deficit/hyperactivity disorder; WS, Waardenburg syndrome.

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