A 64-year-old woman with a history of hypertension, diabetes, hyperlipidemia, and melanoma in remission presented with acute-onset vertigo described as a room-spinning sensation, progressive gait imbalance, vomiting, and a posterior headache. She reported chronic left-sided hearing loss and a remote history of head trauma. Neurological exam revealed eccentric gaze-evoked nystagmus and wide-based, unsteady gait. The HINTS exam indicated a central etiology.

Initial imaging (CT head) was unremarkable. MRI brain however revealed extensive superficial hemosiderin deposition over the cerebellar folia, sylvian fissures, and occipital-temporal lobes, consistent with superficial siderosis. MRA showed no vascular malformations. CSF analysis revealed xanthochromia, elevated red blood cells, and no malignant cells, suggesting chronic bleeding without an active source.

Superficial siderosis most commonly presents with cerebellar ataxia, sensorineural hearing loss, and pyramidal signs. Our patient’s clinical picture and imaging were classic for SS, despite no identifiable bleeding source, however patient reported a significant history of head trauma. Given the irreversible nature of hemosiderin deposition, early diagnosis is crucial.

This case highlights the importance of considering SS in patients with vertigo, hearing loss, and cerebellar signs, especially with a history of trauma. MRI with susceptibility-weighted imaging is critical for diagnosis. While treatment remains limited, identifying and addressing the bleeding source and iron chelation therapy (e.g., deferiprone) may stabilize disease progression. Increased awareness is essential for timely recognition of this rare but impactful condition.