Any child who develops unexplained meningitis(particularly recurrent meningitis) in the presence of a hearing loss must be suspected of having a perilymphatic fistula and an inner ear anomaly, therefore appropriate radiologic studies should be performed. In recent years, radiological studies such as polytomography and computed tomography scan have permitted the clinical diagnosis of these disorders. The authors experienced 2 cases of hearing-impaired children with Mondini dysplasia where developed unexplained recurrent meningitis. The cases are presenteed with a review of literature. Emphasis will be placed on the pathophysiology and current diagnosis strategies.