耳瘻孔•難聴症候群の中耳,内耳所見

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A family with the earpits-deafness syndrome is reported.The proband was a 22-years-old male, who suffered from bilateral deafness since his childhood. He has earpits, cervical fistulas and deformed auricles on both sides. His audiogram showed mixed hearing loss, about 60dB, bilaterally.His mother had also bilateral earpits and cervical fistulas. Her auricles were normal in size and shape but with accessory ears. She had bilateral hearing impairment, sensorineural in the right, conductive type in the left.Probands younger sister had right-sided deformed auricle, right deafness and bilateral cervical fistulas.The exploration of the probands left middle ear revealed the following findings.a. Hypoplasia of the anterior crus of the stapes.b. Fixation of the stapes foodplate.c. Absence of the stapedius muscle.d. High juglar bulb.Tomography and CT-scan of the temporal bone showed hypoplasia of the petrous bone. The internal auditory canals and the labyrinths were placed more medially than the normal position. The internal auditory canals were widened. Only the basal turn of the cochlea was identified. The loops of the horizontal canals were approximately two-third smaller than normal.
社団法人日本耳鼻咽喉科学会の論文