(A218) Evaluation of auditory outcomes after cochlear implantation in children with CHARGE syndrome

Introduction: the objective of the study is to assess auditory outcomes in children with CHARGE syndrome who received cochlear implants

Methods: Study

Design: The retrospective study included children with CHARGE syndrome who underwent cochlear implantation.
Setting: Tertiary referral center.
Patients: 8 CI children with sensorineural hearing loss following CHARGE syndrome.
Interventions: Minimally invasive cochlear implantation via round window.
Main Outcome Measures: All children underwent Auditory Brainstem Response test before operation. Auditory outcomes were assessed with free-field audiometry test and Automatic Adaptive Speechtest (AAST) about 12 months after CI activation.

Results: Before surgery hearing thresholds in each patient, across 0.5-4 kHz frequencies, were > 90 dB. All patients had anatomical abnormalities of the middle and inner ears. Mean hearing threshold in free-field audiometry test (after operation) was 49 dB. Average AAST results were: (1) in quiet: 43 ; (2) in noise: - 6.125.

Conclusions: Cochlear implantation is a good way to compensate for hearing loss associated with CHARGE syndrome