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はじめに
胎生4週から5週における第一,第二鰓弓の発生異常により難聴,耳瘻孔,側頸部瘻孔,耳介奇形,副耳,下顎骨発育不全などの奇形が生じ,様々な複合奇形として発現することは周知のことであるが,このうち難聴(伝音性,感音性あるいは混合性),耳瘻孔,側頸部瘻孔を呈するものは,特にbranchio-oto syndromeと呼ばれており1),本邦ではこれまで16例の報告を認めるのみである2〜13)。今回われわれは,両側内耳道の著明な拡大を呈したbranchio-oto syndromeの1症例を経験したので,若干の考察を加えて報告する。
Branchio-oto syndrome (BOS) was defined by Melnick et al as a clinical entity that has preaur-icular fistula, cervical fistula or cyst and hearingimpairment. We described a 22 year old male who presented with bilateral mixed hearing loss, bilat-eral preauricular fistulas and bilateral cervical fis-tulas diagnosed as BOS. He also presented bilateral enlargement of the internal auditory canals. II min in width and oval shape on polytomographv. He also presented a deformity of the external auditory canal but no auricular abnormalities, malformation of osseous labyrinth, facial palsy, microgenia lacrimal duct stenosis as reported in other articles. There was no BOS in his family history.
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