COCHLEAR IMPLANTATION IN THE CONGENITAL MALFORMED COCHLEA

Cochlear implantation has proven beneficial for numerous children with profound congenital hearing impairments, Some congenitally deaf ears, however, may have anatomical malformations, This study reports the au thors' recent experience with cochlear implants in patients with conge nital inner ear malformations. Since 1987, 10 patients with congenital abnormal inner ear structures have been implanted at the House Ear Cl inic; 8 with abnormal cochleas and 2 with enlarged vestibular aqueduct s. Nine patients received the Nucleus 22 device and 1 patient was im p lanted with the 3M/House device. Some modifications of the routine sur gical approach were required in 5 patients. A cerebrospinal fluid (CSF ) leak occurred in 4 of the 10 cases. There was a relationship between the degree of abnormality and the number of electrodes that could be inserted. AU 10 patients can perceive sound and are active users of th eir implants.