Susac syndrome is a rare disorder caused by the development of microangiopathic lesions in the inner ear, retina, and central nervous system. Although relatively few cases have been reported in the literature, this diagnosis is becoming more frequent as physician awareness increases. A high index of suspicion is needed to make the diagnosis because the signs and symptoms of Susac syndrome are those of a wide range of differential diagnoses. Otolaryngologists should be familiar with this syndrome because hearing loss may be the initial presenting symptom. We describe a new case of Susac syndrome to further promote physician awareness, and we discuss the options for diagnosis and treatment.