Peer-reviewed article
Auditory outcomes after cochlear implantation in children with additional disabilities: A grading-based evaluation of inner ear malformations.
Minami S, Enomoto C, Kato H, Tachibana N, Ihara M et al. ·
Summary
Written by AI, not yet AICS's view · claude-haiku-4-5-20251001This medical study evaluates hearing outcomes in 58 children with both severe hearing loss and additional disabilities (autism, intellectual disability, ADHD) who received cochlear implants. Children with less severe inner ear malformations achieved better auditory outcomes than those with more severe anatomical abnormalities. The study suggests cochlear implants can help children with these combined conditions, though results depend on the degree of cognitive and inner ear differences.
Key points
- 60% of children had autism spectrum disorder and 83% had intellectual disability
- Children with Grade 0-I inner ear malformations had significantly higher auditory performance scores than those with Grade II or higher (median 8 vs. 2)
- 60% of those with mild inner ear malformations achieved conversational speech understanding, compared to only 13% with severe malformations
- Mild intellectual disability and severe inner ear malformations were independent negative predictors of better outcomes
- Age at assessment was positively associated with better auditory outcomes
Limitations
This is a retrospective study of children treated at a single center with no comparison group of children with disabilities who did not receive implants. The study cannot determine whether observed outcomes are better than natural development without intervention, nor can it identify which specific aspects of the intervention contribute most to outcomes.
Abstract
Objective This study aimed to evaluate auditory and speech outcomes after cochlear implantation (CI) in children with additional disabilities (ADs), including autism spectrum disorder (ASD), with a particular focus on the impact of inner ear malformation (IEM) grading based on modiolus and cochlear nerve integrity. Methods We retrospectively reviewed 58 children with bilateral severe-to-profound sensorineural hearing loss and at least one AD who underwent CI between 2007 and 2022 at a national cochlear implant center. ADs included ASD, intellectual disability (ID), and attention-deficit/hyperactivity disorder (ADHD). IEMs were classified into Grades 0-III based on radiological evaluation. Auditory outcomes were assessed using the Revised Categories of Auditory Performance (R-CAP) and Speech Intelligibility Rating (SIR) scales. Statistical analyses included non-parametric tests and multivariable logistic regression. Results Among the 58 children, 60 % had ASD and 83 % had ID. IEMs of Grade II or higher were observed in 26 %. Median age at implantation was 29.5 months, and median age at evaluation was 86.5 months. R-CAP scores were significantly higher in children with Grade 0- I IEM than in those with Grade ≥ II (median 8 vs. 2, p < 0.01). R-CAP ≥8, indicating conversational speech understanding, was achieved by 60 % of Grade 0- I cases but only 13 % of those with more severe IEMs. Multivariable analysis showed that mild ID and severe IEMs were independent negative predictors of achieving R-CAP ≥8, while age at assessment was positively associated with better outcomes. Conclusion CI can provide meaningful auditory development for children with ADs, but outcomes vary by cognitive and anatomical factors. Early implantation remains essential despite challenges in diagnosing conditions like ASD before surgery, supporting the need for ongoing developmental surveillance and individualized habilitation planning.