小儿Mondini's聋106例报告

Mondini's deafness

报告106例（199耳）Mondini’s聋的临床资料。患儿多自幼出现重度耳聋。高分辨颞骨CT扫描示单纯耳蜗畸形4耳，耳蜗、前庭或半规管畸形90耳，耳蜗形态正常仅前庭或半规管畸形105耳（称前庭型或不典型Mondini’s聋）；199耳中伴前庭导水管扩大81耳。并对该病病因、临床表现及防治进行讨论。

Mondini's deafness was the congenital malformation of inner ear. We studied 199 ears of 106 cases. Most of them were found deaf when they were babies. High resolution computed tomography of temperal bone was described:cochlea malformation 4 ears;malformation of cochlea, vestibule and semicircular canal 90 ears; vestibule of semicircular malformation without cochlea rnalformation 1 05 ears,this type was called the vestibule malformation or untypically Mondini's. Of all the 199 ears there were 81 ears with enlagement of the vestibule aqueducts. The pathogeny,cli-nical manifestation and treatment were discussed.