Familiar apearance of unilateral total deafness in children.
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概要
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One hundred eighty three family lines with juvenile unilateral total deafness treated in the authors' clinic to children with sensori-neural hearing loss during 15 years from 1970 to 1985 were studied.Their clinical history and the results of hearing, tomography, caloric tests and pedigree patterns were investigated. A total of 553 relatives were examined for their hearing by the audiometry.Following results were obtained.1. In the 183 family lines with juvenile unilateral total deafness, there were 10 cases of 5 family lines (or 2.73%). Their family lines were 3 cousin's families, 1 brother's family and 1 aunt and boy's family. 1 case of 10 cases was found to have inner ear deformity in the side of hearing loss.2. The total of family lines with sesori-neural hearing loss relatives were 75 family lines (or 41.0%). The family lines with unilateral deafness relatives were 37 (or 20.2%). The family lines with bilateral sesori-neural hearing loss cases were 45 (or 24.6%).3. The occurring rate of hearing loss in relatives with unilateral total deafness was higher than in normal hearing cases. That rate in lower children was 3-7% (0% in normal hearing cases), and that in the age of 30-40 years old was 14-35% (4-10% in normal hearing cases).4. Summing up, it may be concluded that the occurrence of unilateral total deafness is related to the hereditary nature, and there are hereditary unilateral total deafness cases.
- 一般社団法人 日本耳鼻咽喉科学会の論文