Severe cochlear dysplasia causing recurrent meningitis: a surgical lesson.

Meningitis may be the sole presenting sign of a cerebrospinal fluid (CSF) fistula of the temporal bone. An eight-year-old boy suffering from recurrent meningitis was found to have bilateral severe cochlear dysplasia. Bilateral tympanotomies were performed, planning to obliterate each vestibule. In the right ear a stapedectomy was performed, resulting in a torrential 'CSF gusher' and difficulty in packing the vestibule. CSF rhinorrhoea requiring revision surgery and two episodes of gram-negative bacterial meningitis complicated the post-operative management, resulting in a prolonged hospital stay. Subsequently, the left ear was managed in a different fashion, leaving the stapes in situ, with grafts placed to seal the oval window niche. We would recommend this alternative procedure in cases of severe cochlear dysplasia, where abnormalities of the vestibule and basal turn of the cochlea mean that performing a stapedectomy to pack the vestibule may result in a severe 'CSF gusher', by opening directly into the subarachnoid space.