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目的 :熟悉先天性内耳畸形的临床表现及处理原则。方法 :对Mondini畸形 1例进行分析 ,并结合文献进行讨论。结果 :CT检查有利于该病的诊断。结论 :不明原因感音性聋伴或不伴脑脊液耳鼻漏 ,均应考虑本病可能。瘘口周围粘膜充分搔刮是自发性脑脊液耳鼻漏修补成功的关键。
Objective: To familiarize the clinical manifestations and treatment principles of congenital inner ear deformity. Methods: One case of Mondini malformation was analyzed and discussed in the literature. Results: CT examination is conducive to the diagnosis of the disease. Conclusion: Sensitive deafness with or without unexplained cerebrospinal fluid rhinorrhea, should consider the possibility of this disease. Scraping of mucosa around the full scratch is the key to success of spontaneous cerebrospinal fluid rhinorrhea repair.