青色鞏膜患者の難聴

概要

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Audiometer tests on hearing ability of one brother and two younger sisters who had blue seleras and his cousins were perfomed. Two cousins had normal hearing, but the brother and two sisters had pure perceptive deafness of abrupt high tone loss. Since their mother had blue sclera also, the authors diagnosed their perceptive deafness as one symptom of van der Hoeve's syndrom. The origin of deafness complicated with blue sclera is considered as being due to otosclerosis. According to this hypothesis, the audiogram findings showing pure perceptive type can be explained by the interpretation that characteristic otosclerotic focus invaded not the vestibular window but only the perceptive system.
一般社団法人日本耳鼻咽喉科学会の論文