Outcomes and Time to Emergence of Auditory Skills After Cochlear Implantation of Children With Charge Syndrome.

Objective: Review perioperative complications, benefits, and the timeframe over which auditory skills develop in children with CHARGE syndrome who receive a cochlear implant (CI). Study Design: IRB-approved retrospective chart review of children with CHARGE syndrome who had at least 12 months of cochlear implant use. Setting: Tertiary care children's hospital. Patients: Twelve children, seven males and five females. Mean age implant = 3.5 years (1.7-8.2 yr); mean duration follow-up = 4.7 years (1.5-10.1 yr). Intervention: Cochlear implantation. Main Outcome Measures: Auditory skills categorized into four levels, temporal bone imaging findings, perioperative complications, time to emergence of speech perception, expressive communication mode. Results: All children imaged with magnetic resonance imaging had cochlear nerve deficiency in at least one ear. Speech awareness threshold improved with the CI compared with aided preoperative in 83% of children, with means of 51.7 dB SAT preoperative and 27.1 dB with the CI (p

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