Progressive Bilateral Sensorineural Hearing Loss and Ataxia Caused by Superficial Siderosis Secondary to Chronic Spontaneous Intracranial Hypotension

Superficial siderosis (SS) is an uncommon neurodegenerative disorder resulting from persistent or repeated bleeding into the subarachnoid space, which causes hemosiderin to accumulate along the subpial surfaces of the brain, brainstem, spinal cord, and cranial nerves. Chronic spontaneous intracranial hypotension (SIH) due to a dural tear is an uncommon but treatable condition. We report a 51-year-old man with a seven-year history of progressive bilateral sensorineural hearing loss, disabling gait ataxia, vertigo, tinnitus, and facial numbness. Ten years earlier, he had experienced a prolonged, classical orthostatic headache lasting four to six months, which resolved after unspecified treatment. Magnetic resonance imaging (MRI) of the brain revealed features of SS involving the posterior fossa, and an MRI whole-spine survey with fat-suppressed sampling perfection with application-optimized contrasts (SPACE) sequence identified a cervicodorsal, longitudinally extensive extradural cerebrospinal fluid (CSF) collection. The brain MRI also demonstrated a reduced ponto-mesencephalic angle, consistent with intracranial hypotension. A diagnosis of SS secondary to chronic SIH from a spinal dural tear was made. He underwent an autologous epidural blood patch, followed by a second patch, which resulted in symptomatic stabilization, along with iron chelation and corticosteroid therapy. This case highlights the importance of recognizing SS as a delayed complication of untreated SIH. Timely identification and repair of dural defects can halt progression and potentially reverse neurological deficits.