Abstract
Spontaneous cerebrospinal fluid (CSF) leakage through the temporal bone in adults is thought to be rare1,2,3 and therefore misdiagnosis or failure to make an early diagnosis is common.4 It is defined as one that has no identifiable cause such as trauma, surgery, infection, neoplasia or congenital abnormalities.5 Most commonly these defects occur in the tegmen tympani or tegmen mastoideum and less commonly in the posterior fossa plate. The meningeal defects are either meningoencephalocoeles or simply holes in the dura.1 Patients usually present in adulthood with aural fullness and hearing loss and the most common signs are a clear middle ear effusion and clear tympanostomy tube otorrhoea.6 In this review of the literature we discuss the pathogenesis, natural history, salient investigations and the surgical management of the condition.