Abstract
Bilateral congenital deafness was observed in a 9-year-old girl with recurrent meningitis. A careful workup, including polytomograph, CT scanning and Radioisotope (RI)-cisternography revealed an inner ear dysplasia of the Mondini type, viz. dilation of the lateral semicircular canal, vestibule and cystic degeneration of cochlea on both ears. RI-cisternography demonstrated an abnormal accumulation of radioisotope in the left mastoid region, suggesting spontaneous cerebrospinal fluid (CSF) otorrhea. At exploratory tympanotomy of the left ear, the CSF leak was found from a round defect in the center of the stapedial foot plate. The CSF leak was treated successfully by packing the vestibule with temporal fascia. Spontaneous CSF otorrhea is seldom the cause of meningitis but has to be considered especially if associated with a dead ear and with recurrent meningitis. The diagnosis and management are discussed.
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