Superficial siderosis of the meninges and its otolaryngologic connection: A series of five patients

Objective: To study the otolaryngologic disorders in patients with superficial siderosis of the meninges. Background: Superficial siderosis of the central nervous system is a rare disorder characterized by progressive bilateral hearing loss and ataxia caused by recurrent bleeding into the subarachnoid space. The cerebellum, eighth cranial nerve, and olfactory nerve are particularly susceptible to the deposition of hemosiderin, which is responsible for the symptoms. The diagnosis is confirmed by magnetic resonance imaging. Methods: The clinical notes of five patients with superficial siderosis of the meninges were reviewed with the intent of reporting the otolaryngologic symptoms and signs, the clinical investigations, and treatments. Results: Four of the five patients had sensorineural deafness, two had smell disturbances, and three had gait abnormalities. Magnetic resonance imaging was the most important investigation used to identify the condition. Conclusions: Superficial siderosis of the meninges is an important differential diagnosis in patients with progressive sensorineural deafness.