Mondini畸形伴脑脊液耳漏的外科治疗

目的：探讨Mondini畸形伴脑脊液耳漏患者的外科治疗方法及预防措施。方法：对我院确诊为Mondini畸形伴脑脊液耳漏的15例患者采用鼓室探查并经卵圆窗、圆窗及其他漏孔进行填塞修补。结果：手术探查发现卵圆窗处有脑脊液漏出，圆窗及附近其他部位也有漏孔发生。15例患者中，10例经漏孔填塞修补1次治愈；5例经2次或3次填塞术后治愈。术后随访10个月～7年无脑脊液漏或脑膜炎复发。结论：经鼓室进路手术修补为该病的有效治疗方法，避免致畸因素的影响是预防本病发生的重要措施，佩带助听器是治疗手段之一，部分患者可考虑电子耳蜗植入。

Surgical management of Mondini dysplasia with cerebrospinal fluid leakage

Objective:To investigate the etiology,clinical presentation,surgical treatment and prevention of Mondini dysplasia with cerebrospinal fluid otorrhea.Method:Fifteen cases of Mondini dysplasia were collected from our department during 1998~2004.The clinical presentations,surgical treatment and image characters were analysed.Surgical therapy methods included: checking fenestra vestibuli,fenestra cochlear or other fistula and repairing the fistulas by plugging with fascial tissue via tympanoplastic approach with endaural incision.Result:Severe sensorineural hearing loss and spontaneous recrudescent meningitis were the characteristics of Mondini dysplasia.The leakage was from fenestra vestibuli,fenestra cochlear or other fragile region nearby.In these cases,ten cases underwent one plugging operation and cured,five cases underwent two or three operation and cured.All these cases had no leakage or meningitis recrudescence after ten months follow up.Conclusion:Repairing fistula via tympanoplastic approach is an effective theraputic method.Avoiding nosogenesis,fitting hearing aid or taking cochlear implantation are important means of prevention and cure.