Abstract: 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.

Key words: unilateral inner ear Mondini malformation, cerebrospinal fluid ear leakage, tymanic exploration and repair surgery