Pediatric cochlear implantation in cochlear malformations.
OBJECTIVE: This study aimed to present relevant information about pediatric cochlear implantation in malformed cochleas based on the experience gathered with 12 implanted children. STUDY DESIGN: A retrospective analysis was performed. SETTING: All patients were diagnosed and implanted at the Medical University of Hannover. Medical check-ups were performed regularly. The rehabilitation concept was developed by the Cochlear Implant Center of Hannover. PATIENTS: All children were female and were between 2 and 13 years of age at the time of implantation, with the average age being 4 years and 2 months. Only patients who were younger than 14 years of age and implanted between September 1992 and October 1995 were evaluated. INTERVENTION: Diagnostic computed tomographic scans including three-dimensional reconstructions and magnetic resonance imaging images were performed. In all cases, Nucleus devices (Mini 22 or 20 + 2) were implanted. Medical University of Hannover standard surgical technique was used, although in most cases, facial nerve monitoring and electrically evoked auditory brain stem responses were additionally recorded. Total or partial obliteration of the middle ear had occurred in two cases. An anteroposterior approach was used four times. The implantation was followed by the standard rehabilitation procedure for children. RESULTS: No serious complications occurred. All children responded to acoustic stimuli and showed improvement in their speech production. However, one autistic child performed poorly, and for another child suffering from a CHARGE syndrome, results still are pending. CONCLUSIONS: Given suitable preconditions, cochlear implantation is feasible with an acceptable risk of complications. Implantation appears to be beneficial in most cases with cochlear malformations provided that eighth nerve and cochlear lumen are present.