불완전 분할 3형 내이기형 환자에서 시행한 인공와우 이식술 1예

Abstract

Among the inner ear anomalies, incomplete partition type III (IP III) is a rare finding. The X-linked deafness type 3 (DFN3), the most common type of X-linked hearing loss of its kind, results from mutations in the POU3F4 gene and accounts for -50% of all families carrying X-linked non-syndromic hearing loss. Pathognomonic temporal bone deformities, recently classified as IP III, have been associated with DFN3. We report a patient with IP III carrying a mutation in the POU3F4 gene who experienced cerebrospinal fluid gusher during and after cochlear implantation.