Bilateral Sensorineural Hearing Loss Related to a Skull Base Spontaneous Cerebrospinal Fluid Leak: A Case Report and Literature Review.

Spontaneous intracranial hypotension (SIH) is a rare condition characterized by orthostatic headache, pulsatile tinnitus, vertigo, nausea, and fluctuating hearing loss; this latter seems to be due to the development of cochlear endolymphatic hydrops following negative cerebrospinal fluid (CSF) pressure transmitted to the perilymphatic space through a patent cochlear aqueduct. We here describe a case of bilateral progressive sensorineural hearing loss (SNHL) due to intracranial hypotension caused by an undiagnosed spontaneous CSF leak from a skull base defect in a middle-aged woman. To the best of our knowledge, this is the first report describing a SNHL in a patient affected by SIH secondary to sphenoidal CSF leak.