Abstract
We report a case of unilateral inner ear malformation that presenting with recurrent meningitis and bilateral deafness. A 8-months-old boy with congenital left-side hearing impairment, diagnosed in newborn screening, suffered bacterial meningitis twice within 7 months of birth, becoming bilateral totally deaf there after. Temporal bone computed tomography (CT) indicated left-side inner ear malformation, Mondini dysplasia, and a cerebrospinal fluid (CSF) fistula. CSF from a stapes footplate defect was repaired surgically. Cochlear implantation was successful in the right ear 3 months after the first operation. In the 14 months since the second operation, he has had no bacterial meningitis and has begun speaking two-word sentences. Early diagnosis and prompt surgical intervention are thus crucial in avoiding meningitis sequelae.
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