Objective:

To describe a rare case of spinal cerebrospinal fluid-venous fistula (CVF) presenting with sudden isolated hearing loss.

Background:

NA

Design/Methods:

NA

Results:

A 78-year-old man with chronic left-sided hearing loss and tinnitus for 52 years suddenly developed right-sided hearing loss. He denied headache or other postural symptoms. Otolaryngology evaluation was unrevealing. Brain MRI showed diffuse pachymeningeal enhancement around the cerebrum, cerebellum, and internal auditory canals. These findings prompted further evaluation for an infective or inflammatory process, neurosarcoidosis, or occult malignancy. Three attempts at lumbar puncture failed to yield CSF. A fourth attempt 4 months after the decline in hearing showed low-normal opening pressure of 9 cm H2O, mild lymphocytic pleocytosis with nucleated cell count 7 (normal ≤5), elevated protein at 226 mg/dL (normal 15-45), positive xanthochromia, and negative cytology. Biopsy of right frontal parasagittal meninges revealed fibrous dural and arachnoid tissue with dystrophic calcifications without evidence of chronic inflammation. Hemosiderin deposition suggested prior hemorrhage. Prominent thin-walled vascular spaces separated by hypocellular fibrous stroma with meningothelial cell proliferation represented normal dura and arachnoid in the parasagittal regions where arachnoid granulations are most common. At our institution, diffuse pachymeningeal enhancement prompted suspicion for underlying spinal CSF leak, and decubitus CT myelogram showed probable CVFs at the right T9 and T11 neural foramina. Ten months after symptom onset, transvenous embolization at right T9 and T11 resulted in complete resolution of tinnitus and mild improvement in hearing in the right ear.

Conclusions:

Spontaneous spinal CSF leaks, including CVFs, may present with isolated hearing loss, without the classic orthostatic headache. This should be considered in the differential diagnosis of unexplained vestibulocochlear dysfunction or other symptoms when there are radiographic stigmata of intracranial CSF volume depletion such as brain sag or diffuse pachymeningeal enhancement. Advanced myelography is generally necessary to localize a spinal CSF leak and allow targeted, definitive therapy.