Spontaneous Intracranial Hypotension May Be an Under-recognized Cause of Endolymphatic Hydrops

Objective: We describe three rare cases of spontaneous intracranial hypotension (SIH) presenting with symptoms of endolymphatic hydrops (EH) and perform a literature review to bring attention to a rare link between SIH and EH.

Patient: A 59-year-old female presented with postural headache, aural fullness, vertigo, hearing loss, and abnormal electrocochleography after being diagnosed with SIH by magnetic resonance imaging. The site of cerebrospinal fluid leak was identified in this individual. Two additional patients with vertigo, hearing loss, and SIH were identified by retrospective chart review.

Intervention: All patients underwent blood patches. One patient also had diuretic treatment while another had fibrin glue injection.

Main outcome measures: The outcomes of interest were resolution of headache, vertigo, aural fullness, and hearing loss.

Results: All patients eventually improved with time. Literature review suggests that overall outcome is excellent.

Conclusions: SIH may be an under-recognized cause of EH. We support the theory that negative intracranial pressure transmitted through the cochlear aqueduct and perilymph leads to EH. Despite the variations in treatments, the overall prognosis is excellent.