家族性ストマイ難聴の三家系とその遺伝について

概要

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Three families in which 2 or more members have a streptomycin-induced hearing loss are reported. These three families and families which have reported by several authors (Takafuji et al. 1953, Esaki et al. 1957, Kawaguchi 1957, Ohuchi et al. 1957, Horiguchi and Moriyama 1957, Prazic and Subotic 1964, Johnsonbaugh 1974, Tsuiki 1976) were analyzed.Whether the susceptibility of cochlea to streptomycin is transmitted to the next generation by males or females in each family was examined. In 22 families, the trait which makes the person who possesses it vulunerable to streptomycin is transmitted only by mothers to their sons and daughters. There were only two families, in which fathers transmitted the trait to their offsprings. In the remaining 13 families, the decision whether the trait was transmitted by males or females was impossible, because in these families only members of the same siblings were affected.Tsuiki and Murai (1971) suggested autosomal dominant inheritance of this trait, but such cannot explain the lack of transmission by males.To explain this unusual inheritance, two hypotheses are possible. One possibility is autosomal dominant inheritance with a special mechanism which decreases the possibility of male-to-male and male-to-female inheritance. Another possibility is extranuclear inheritance, and such is more probable, at least in our patients.