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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。
In order to summarize the diagnosis and treatment of inner ear deformity, five cases (6 ears) of Mondini inner ear deformity were reported. Four patients (5 ears) with cerebrospinal fluid ear and rhinorrhea (3 cases with Klippel-Feil syndrome) were successful in traumatic surgery and 1 case was associated with otosclerosis. Combined with the characteristics of pathological diagnosis and surgical treatment focus on: ① diagnostic basis in addition to hearing tests, with special emphasis on CT for the diagnosis of imaging information provided; ② Son-dini inner ear malformation caused by perilymph fistula in the vestibular or near the window, And round window parts; ③ spontaneous cerebrospinal fluid ear, rhinorrhea treatment can be taken from the tympanic cavity of the ear exploration and fistula repair, perforation of the mucosa should be thoroughly scratched, the selection of mesodermal tissue filling the vestibular window, the stuffing should be Incarcerated.