Spontaneous intracranial hypotension (SIH) is a rare pathology caused by unexplained and variably localized leakage of cerebrospinal fluid (CSF). The prime symptom is orthostatic headache, although other less specific clinical signs may predominate, and mislead diagnosis.

A 47-year-old man presented with Ménière-like symptoms of sudden onset. Secondary orthostatic headache led to the performance of cerebral MRI, which found signs suggestive of intracranial hypotension. A blood-patch was immediately carried out, and was followed by consciousness disorder associated with onset of bilateral subdural hematoma, which required iterative neurosurgical drainage. Myelo-CT confirmed CSF leakage facing the right 12th dorsal nerve root sheath. Radio-guided sealing with biologic glue provided complete regression of all symptoms.

Auditory signs may predominate in the clinical presentation of SIH. Their orthostatic character is suggestive. The present case is of a rare severe form. The role of neurosurgery in such cases remains to be defined.