Otologic manifestations of Susac syndrome

Susac syndrome, a rare autoimmune disorder first described as a classic triad (encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss) in 1979 by renowned physician John O. Susac, has been an advancing area of clinical interest and scientific research over the last several decades. This comprehensive review aims to succinctly highlight the breadth and detail of this enigmatic disease, with a primary focus on otologic manifestations. Topics discussed include epidemiology, pathophysiology, clinical manifestations, differential diagnoses, classification schema, laboratory investigations, characteristic audiometric findings, high-yield radiographic imaging, temporal bone histopathology, treatment strategies and overall prognosis.