Korean J Otorhinolaryngol-Head Neck Surg.
2013 Sep;56(9):594-598. 10.3342/kjorl-hns.2013.56.9.594.
A Case of Cochlear Implantation in Patient with Incomplete Partition Type III Inner Ear Anomaly
- Affiliations
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- 1Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Korea. ismoonmd@yuhs.ac
- 2Department of Biology, College of Natural Sciences, Kyungpook National University, Daegu, Korea.
- KMID: 1855842
- DOI: http://doi.org/10.3342/kjorl-hns.2013.56.9.594
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.
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