Mondini内耳畸形的诊断与治疗

为总结内耳畸形的诊断与治疗特点，报告５例（６耳）Ｍｏｎｄｉｎｉ内耳畸形。４例（５耳）伴发脑脊液耳、鼻漏（其中３例伴发Ｋｌｉｐｐｅｌ－Ｆｅｉｌ综合症）经手术补漏成功，１例伴发耳硬化症。结合病理诊断及手术处理特点重点讨论：①诊断依据除听力检查外，特别强调ＣＴ为确诊提供的影像学资料；②Ｍｏｎ－ｄｉｎｉ内耳畸形所致外淋巴瘘主要在前庭窗或其附近，镫骨底板及圆窗部位；③自发性脑脊液耳、鼻漏处理可采取耳内切口的鼓室探查及瘘孔修补术，瘘孔周围粘膜应彻底搔刮，选用中胚叶组织填塞前庭窗，填塞物应呈嵌顿状。

Diagnosis and management of Mondini malformation

Owing to the development of imaging technology and audiology, some of the sensorineural hearing loss cases that were previcusly considered to be of unknown cause have been found to be inner ear malformation. Five cases of Mondini malformation are reviewed in this paper. CSF otorhinorrhea occurred in four cases, Klippel Feil syndrome in three, and concurrent otosclerosis in one patient. In discussion, the authors point out that: 1) CT scanning is an supplement to audiologic tests for such patients; 2) perilymph fistula in Mondini malformation is often found at the oval window and its vicinity, the foot plate and the round window; 3) the fistula can be treated by plugging with fascial tissue via tympanoplastic approach with endaural incision. The mucosa around the fistula should be stripped away and the plugging tissue should be of dumb bell shape.