A Case of Cochlear Implantation in Patient with Incomplete Partition Type III Inner Ear Anomaly.
10.3342/kjorl-hns.2013.56.9.594
- Author:
Myung Jin BAN
1
;
Jung Min KIM
;
Tae Jun KWON
;
In Seok MOON
Author Information
1. Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Korea. ismoonmd@yuhs.ac
- Publication Type:Original Article
- Keywords:
Cochlear implantation;
Incomplete partition;
POU3F4;
X-linked deafness
- MeSH:
Cochlear Implantation;
Cochlear Implants;
Congenital Abnormalities;
Deafness;
Ear, Inner;
Hearing Loss;
Humans;
Lifting;
Temporal Bone
- From:Korean Journal of Otolaryngology - Head and Neck Surgery
2013;56(9):594-598
- CountryRepublic of Korea
- Language:Korean
-
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.
