Spontaneous intracranial hypotension with endolymphatic hydrops: A case report

Abstract

Spontaneous intracranial hypotension may be defined as a postural headache syndrome without preceding major head trauma or dural puncture, associated with low cerebrospinal fluid volumes and opening pressure. Orthostatic headache is a cardinal clinical manifestation, associated symptoms may include neck stiffness, nausea, diplopia, vertigo, tinnitus and deafness in spontaneous intracranial hypotension. We describe a woman presented with a 1 week history of headache who was diagnosed with endolymphatic hydrops after cerebrospinal fluid analysis. A 40 years old woman presented with a 1 week history of headache. The pain was described as generalized non-throbbing and complain a mild febrile sensation. She admitted a local hospital but her symptoms were continued. On admission, she had severe orthostatic headache, nausea, vomiting and both ear fullness, tinnitus. No photophobia, phonophobia, and other autonomic dysfunction were exist. She had never been previous head or back trauma and cerebrospinal fluid tapping history. No abnormality in laboratory result. cerebrospinal fluid exam showed 4cmH2O pressure, RBC 780, WBC 0, glucose 39 (serum 83), protein 95. Brain MRI showed diffuse prominent dural enhancement by gadolinium. Pure tone audiogram: decreased hearing in low tone. It suggested endolymphatic hydrops. She treated by hydration and symptomatic treatment and symptoms were improved. In conclusion, a compensatory expansion of the endolymphatic compartment would induce endolymphatic hydrops and in the result, it causes low tone hearing loss. Because spontaneous intracranial hypotension may cause endolymphatic hydrops, it is highly probable that audiometry is necessary to the orthostatic hypotension patient with ear fullness.