Cochlear implantation in children with CHARGE association.

OBJECTIVE To explore the anomalies of the temporal bone found on radiologic examination, technical challenges in cochlear implantation, and audiologic benefit derived from cochlear implantation in a series of children with CHARGE association. DESIGN Case series report. SETTING Tertiary referral children's hospital pediatric cochlear implant program. PATIENTS Six children with CHARGE association and sensorineural hearing loss. INTERVENTION All patients were evaluated and followed up by the cochlear implant team. Cochlear implantation was attempted in all 6 children. MAIN OUTCOME MEASURES Computed tomographic scans and cochlear implantation operative records were reviewed, and their findings were correlated. Audiometric and speech perception data before and after cochlear implantation were compared. RESULTS Five children with CHARGE association received implants. A sixth child did not because of an aberrant course to the facial nerve. The 5 children receiving implants obtained varying degrees of measurable benefit from their implants. All 6 children had temporal bone abnormalities seen on their computed tomographic scans and documented at the time of surgery. CONCLUSIONS Variations in the temporal bone anatomy of patients with CHARGE association can lead to increased technical challenges and risk to the facial nerve during cochlear implantation. Individual outcomes after implantation may vary; our patients receiving implants obtained benefit. Parents should be counseled thoroughly and have appropriate expectations before proceeding with implantation.