Mondini畸形的临床表现及处理(附1例报告)

The clinical manifestation and management of Mondini dysplasia

目的:提高对Mondini畸形的认识。方法:对1例Mondini畸形患者的临床特征、影像学表现及手术情况等临床资料进行分析。结果:Mondini畸形形成脑脊液漏后可伴发脑膜炎,常反复发作,患者大多就诊于其他科室,不易确诊,尤其是单侧畸形,因对侧听力正常,常易漏诊。结论:反复发作不明原因的脑脊液漏伴脑膜炎者应疑及此病,CT与MRI检查是确诊的主要依据。对伴发脑脊液耳漏的患者,鼓室探查修补术是有效的方法。

Objective： To improve the possibility of diagnosis of the Mondini dysplasia. Method：The clinical manifestation and the examination of CT and MRI and surgical treament of the Mondini dysplasia were discussed. Result：Because Mondini dysplasia with cerebrospinal fluid leak would occur recurrent meningitis, the patients were often difficult to be diagnosed. Especially. if the defect was unilateral, it was frequently unrecognized. Conclusion： The patients with recurrently unclear cerebrospinal fluid leak and meningitis would be suspected. The diagnosis of the disease is based on the examination of the temporal bone CT and MRI. To the patients with cerebrospinal fluid leak, a transtympanic closure is one of very effective management.