Spontaneous cerebrospinal fluid (CSF) leak via the oval window is uncommon and can result in recurrent bacterial meningitis. Current understanding of spontaneous CSF otorrhoea is reviewed and a diagnostic algorithm is presented.

A seven-year-old boy presented with bilateral congenital deafness and recurrent meningitis. High-resolution computed tomography (HRCT) of the temporal bone showed a labyrinthine deformity and communication between the internal auditory canal (IAC) and the cochlea. Subtotal petrosectomy with closure of the external acoustic meatus and eustachian tube was performed. Post-operatively, the child had no further episodes of meningitis.

This rare and obscure cause of recurrent childhood meningitis requires a high index of suspicion and the use of diagnostic tools, especially HRCT.