COCHLEAR IMPLANTATION IN CHILDREN WITH INNER-EAR MALFORMATIONS

We performed a case study and intervention study, with follow-up of 1 to 5 years, in 4 children with inner ear malformations who underwent i mplantation of a multichannel cochlear implant (Nucleus, Cochlear Corp oration) at ages 3 to 12 years. Malformations included a common cavity deformity, 2 incomplete partitions, and 1 case of isolated bilateral vestibular aqueduct enlargement. One child had a single-channel implan t placed at 3 years of age, and this was exchanged for a 22-channel im plant at age 9. One child had her implant placed at age 4.5 years, but due to complications from a cerebrospinal fluid (CSF) leak had the in itial implant removed and replaced at age 5 years during repair of the CSF leak. Intraoperative findings included a CSF leak at the time of surgery in 3 patients. One patient contracted bacterial meningitis 7 m onths postimplantation that was thought to be secondary to acute otiti s media in the unoperated ear. Bilateral CSF leaks were noted in the m iddle ear by a lumbar puncture radionuclide and fluorescein dye study. Successful repair of the CSF leaks and reimplantation of the cochlear implant was carried out in this patient. Mapping and programing of th e implant was found to be challenging in each of these patients. All p atients demonstrated improved performance after implantation. Two pati ents demonstrated some open-set speech perception. One patient demonst rates improved use of temporal cues in a structured closed set. One pa tient has achieved no significant speech recognition at this time, but does have improved sound detection and awareness. Cochlear implantati on in children with congenital inner ear abnormalities can be a succes sful method of rehabilitation. It should be recognized that the postop erative speech perception results may be highly variable among patient s, and that intraoperative complications may occur.