Rare Cause of Hearing Loss: Susac Syndrome

Susac's syndrome is a rare autoimmune disease, which is characterized by microangiopathic changes that affects the brain, retina, and cochlea. It is mainly characterized by asymptomatic cerebral infarctions, low-and mid-frequency sensorineural hearing loss, and bilateral distal retinal artery occlusions. Otolaryngologists should be familiar with Susac's syndrome because hearing loss may be the initial presenting symptom. The recommended treatment options vary from antithrombotic to immunomodulatory drugs. Although in its early stage, remission from the disease or self-limiting clinical course may be observed. Residual disabilities such as blindness, deafness, and dementia may also be presented in its late stages. Awareness of the condition and suspicion in selected patients will provide early diagnosis and treatment, which are both important to prevent the development of severe sequelae.