Auditory outcomes after cochlear implantation in children with additional disabilities: A grading-based evaluation of inner ear malformations.

This study looked at cochlear implants in children who have autism or other disabilities alongside hearing loss. Most children (60%) had autism. Children with normal or mildly abnormal inner ear structure did much better with their implants than those with severely abnormal ears. Early implantation helped, even though autism can be hard to diagnose before surgery.

The research shows cochlear implants can help children with multiple disabilities, but outcomes vary based on each child's unique situation.

This retrospective study examined cochlear implant outcomes in 58 children with additional disabilities, including autism spectrum disorder (60% of participants). Researchers evaluated how inner ear malformation severity affected auditory and speech development over a median follow-up of 86.5 months. Children with mild inner ear malformations (Grade 0-I) achieved significantly better auditory outcomes than those with severe malformations (Grade II+), with 60% versus 13% reaching conversational speech understanding levels. Intellectual disability and severe inner ear malformations were independent predictors of poorer outcomes, while older age at assessment correlated with better results.

The study supports early cochlear implantation for children with additional disabilities despite diagnostic challenges.

Clinicians should consider inner ear anatomy grading and cognitive status when counseling families about cochlear implant expectations. Early implantation remains important despite diagnostic challenges with autism. Individualized habilitation planning is essential given variable outcomes in children with multiple disabilities.

Single-center retrospective design limits generalizability. Study type not specified. No control group comparison. Potential selection bias in cochlear implant candidacy. Limited detail on autism severity or other disability characteristics that may affect outcomes.

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. 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. 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.

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.