Nager症候群の中耳所見—症例と文献考察

Nager Syndrome: A Case Report

I．緒言

　第一・第二鰓弓由来の形成不全に上肢橈側の形成不全を合併するNager症候群と考えられる症例を経験し，手術的に耳小骨奇形を確認し聴力改善に成功したので，文献的考察を加えて報告する。

　The authors presented a case of Nager syndrome, which is characterized in the first and second branchial arch syndrome with dysplasia of the radial ray.

　This is the first otological report of Nager syndrome in ,Japan.

　The patient was a 7-year-old girl, and audiometric evaluation showed a bilateral conductive hearing loss. A left middle ear exploration was performed and the following anotnalies were observed: defect of long process of the incus, monopodal stapes, and fixation of the footplate. The conduction chain was reconstrio by a sliced cartilage, TORP, and perichondrium after stapedectomy. Hlearing improvement of 30 dB was obtained.