内耳Mondini畸形临床分析

Case Study of Mondini Dysplasia

江刚;郭梦和;钱宇虹;谢民强;

1:南方医科大学珠江医院耳鼻咽喉-头颈外科

摘要

目的探讨内耳Mondini畸形的病因、临床特征、诊断和治疗。方法分析2例Mondini畸形患者的临床资料并复习相关文献。结果 Mondini畸形属于常染色体显性遗传病;主要表现为听力下降,形成脑脊液漏后可伴发反复发作的脑膜炎,单侧畸形者,因对侧听力正常,常就诊于其他科室易漏诊;其诊断需结合病史、听力学及影像学检查;手术修补对脑脊液漏效果好,本文2例术后随访1年余,均未见复发。结论听力下降并反复发作不明原因的脑脊液漏伴脑膜炎者应疑为内耳Mondini畸形,CT与MRI检查是确诊该病的主要依据,对伴发脑脊液耳漏的患者,鼓室探查修补术是目前唯一有效的方法。

关键词

Mondini畸形;脑脊液耳鼻漏;复发性脑膜炎;外科手术

基金项目(Foundation):

作者

江刚;郭梦和;钱宇虹;谢民强;

参考文献

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Mondini畸形脑脊液耳鼻漏复发性脑膜炎外科手术

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江刚郭梦和钱宇虹谢民强