家族性に見られた高位頚静脈球と拡大内耳道を伴う第1,第2鰓弓発生異常による複合奇形

概要

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This is a report of three siblings, a 14-year-old girl, a 12-year-old boy and an 11-year-old boy, having the first and second branchial arch syndrome. They all had mixed deafness, bilateral aural fistulas and bilateral lateral cervical fistulas. In addition, an abnormally wide internal auditory canal and an abnormally high jugular bulb were revealed in all the children by radiographic studies including polytomography. A probe tympanotomy done on the sister disclosed that the high jugular bulb was completely blocking the round window, which may have been a factor contributing to the conductive hearing loss. The review of the literature suggested that the abnormally wide internal auditory canal had something to do with the developmental anomaly of the first and second branchial arches.
耳鼻咽喉科臨床学会の論文