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KMID : 0361020130560090594
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Korean Journal of Otolaryngology - Head and Neck Surgery
2013 Volume.56 No. 9 p.594 ~ p.598
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A Case of Cochlear Implantation in Patient with Incomplete Partition Type III Inner Ear Anomaly
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Ban Myung-Jin
Kim Jung-Min
Kwon Tae-Jun
Moon In-Seok
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Abstract
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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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KEYWORD
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Cochlear implantation, Incomplete partition, POU3F4, X-linked deafness
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