Objective: To present a case with bilateral-hearing loss, hyperacusia, and dizziness as components of spontaneous intracranial mimicking labyrinthine hydrops.

Methods: Herein, in addition to a literature review, we present a case of spontaneous intracranial hypotension syndrome that was the cause of otologic symptoms, which was successfully treated with a high-volume epidural blood patch.

Results: A 40-year-old woman presented with orthostatic headache, emesis, dizziness, bilateral ear fullness, hyperacusia, and hearing loss. Audiography showed bilateral low-frequency hearing loss. Cranial magnetic resonance imaging (MRI) and MRI myelography showed increased pachymeningeal contrast enhancement, subdural collections, venous engorgement of the internal acoustic canals, and an extrathecal cerebrospinal (CSF) fluid leak. Lumbar puncture opening pressure in the lateral decubitus position was 50 mmH(2)O. CSF protein, microscopic examination, and culture were normal. The patient was diagnosed with spontaneous intracranial hypotension syndrome. After conservative treatment failed a high-volume epidural blood patch was applied. The patient had no otologic complaints after this treatment and had normal audiogram results. Additionally, follow-up cranial MRI showed minimal pachymeningeal contrast enhancement and the absence of subdural collections.

Conclusion: Spontaneous intracranial hypotension syndrome, which is curable, should be considered in patients with orthostatic headache and symptoms mimicking labyrinthine hydrops.