학술논문

Long term observation of two cases of large vestibular aqueduct syndrome
Document Type
Journal Article
Source
Otology Japan. 2003, 13(1):57
Subject
SLC26A4 (PDS)
gene
large vestibular aqueduct syndrome
Language
English
ISSN
0917-2025
1884-1457
Abstract
Two cases of large vestibular acqueduct syndrome (LVAS) are presented.The first case was a 13-year-old boy, who had been observed 9 years. At the initial examination, hearing threshold was 80dB in the left ear and 60dB in the right ear. He experienced acute hearing deterioration four times, at each time hearing showed recovery by oral administration of steroids, vitamin B12. He was found to have a mutation in the chromosome 7q31.The second case was a 21-year-old women and she had been observed for 14 years. Initially a pure-tone audiogram showed 65dB hearing loss bilaterally. She had experienced acute hearing deterioration accompanied or unaccompanied by vestibular disturbance 11 times.Steroid hormone and vitamin B12 given at each episode were effective. Hearing thresholds at the last pure tone audiogram were 70dB of the left, and 80dB on the right. These findings suggested that hearing deterioration the LVAS could be managed for long term by conservative treatment.