X-Linked sensorineuronal hearing loss (SNHL) is a rare but recognized cause of bilateral SNHL associated with CSF gusher. We report a case presenting a varied imaging spectrum and clinical scenario of a one year old boy with unilateral profound hearing loss and recurrent meningitis due to spontaneous oval window gusher. Imaging showed a unilateral abnormality, with the fistula leaking from the oval window into the middle ear cleft.
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