单侧内耳Mondini畸形并发脑脊液耳漏1例报告及文献复习

目的：探讨单侧内耳Mondini畸形并发脑脊液耳漏患者的发病机制、临床表现、诊断和手术方法，提高对其临床特征的认识。方法：收集1例单侧内耳Mondini畸形并发脑脊液耳漏患者的临床资料，结合国内外相关文献，分析其临床特征、影像学表现、诊断和手术方法。结果：患者因"左侧鼻腔流水、头痛20年，加重1个月"就诊。查体见左耳鼓膜内陷、完整，左耳听力下降。鼻内镜下，颅内压增加时左侧咽鼓管咽口有脑脊液流出。结合患者相关检查结果和临床表现初步考虑左耳Mondini畸形并脑脊液耳漏。行左脑脊液耳漏修补术，患者术后听力良好，无鼻腔流液和头痛不适。随访1年，患者未复发脑膜炎。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，手术是主要的治疗方法。

Unilateral inner ear Mondini malformation complicated with cerebrospinal fluid ear leakage: A case report and literature review

Objective: To discuss the pathogenesis, clinical manifestation, diagnosis and operation methods of the patients with unilateral inner ear Mondini malformation complicated with cerebrospinal fluid ear leakage, and to improve the understanding of the clinical features. Methods: The clinical materials of one patient diagnosed as unilateral inner ear Mondini malformation complicated with cerebrospinal fluid ear leakage were collected, and the clinical features, imageological performance and diagnosis and treatment methods were analyzed combined with the relevant literatures. Results: Because of left nasal discharge, headache for 20 years, aggravation for 1 month,the patient went to our hospital. The physical examination results showed that the left tympanic membrane was invaginated and intact, and there was hearing loss in the left ear.The endoscopic examination results showed that when the intracranial pressure was increased, the cerebrospinal fluid flowed out from the left eustachian tube orifice. The Mondini malformation and cerebrospinal fluid otorrhea in the left ear were initially considered in combination with the clinical manifestations, and relative examination results of the patient. The left cerebrospinal fluid otorrhea repair was performed, the postoperative hearing was good, and there was no nasal flow, headache and discomfort. The patient was followed up for 1 year and there was no recurrence of meningitis. Conclusion: The patient with recurrently unclear meningitis would be firstly considered as congenital malformation of inner ear. The diagnosis of the disease is mainly based on the examinations of temporal bone CT and MRI; the tymanic exploration and repair surgery might be an effective method,and operation is the main treatment method.