Mondini畸形伴脑脊液耳漏2例临床分析

Clinical Analysis of Two Cases of Mondini Dysplasia with Cerebrospinal Fluid Otorrhea

目的:探讨Mondini畸形伴脑脊液耳漏的临床表现及外科手术方法,提高对其临床特征的认识。方法:分析2例Mondini畸形患者的临床特征、影像学表现及手术情况并进行文献复习。结果:Mondini畸形伴脑脊液耳漏患者,常以反复发作的脑膜炎为主要症状,易误诊,尤其是单侧畸形,因对侧听力正常,常易漏诊。2例患者皆一次手术修补成功,随访至今无脑脊液漏或脑膜炎复发。结论:反复发作不明原因的脑膜炎患者应考虑先天性内耳畸形的可能,颞骨薄层CT扫描与MRI检查是确诊的主要手段,鼓室探查修补术是填补漏口的有效方法。

Objective： To evaluate the clinical manifestation and surgical technique of Mondini dysplasia with cerebrospinal fluid otorrhea, and to deepen the understanding of congenital malformation of inner ear with cerebrospinal fluid otorrhea. Methods： The clinical manifestation and the examina- tion of CT and MRI and surgical treatment of two Mondini dysplasia cases were discussed and the literatures were also reviewed. Results： Mondini dysplasia with cerebrospinal fluid otorrhea was difficult to be diagnosed when it accompanied by recurrent meningitis. Especially, if the defect was unilateral, it was unrecognized frequently. The leakages were all stopped by the primary sur- gical closure and had no recurrent symptoms. Conclusion： The patients with recurrently unclear cerebrospinal fluid otorrhea and meningitis would be considered as congenital malformation of in- ner ear. The diagnosis of the disease is mainly based on the examination of temporal bone CT and MRI. A transtympanic closure might be an effective method.