Susac syndrome is a rare vasculopathy caused by an autoimmune reaction against endothelial or intramural structures of small arteries in the inner ear, retina and brain. Manly young women are affected. It is characterized by a triad of sensorineural hearing loss, vision loss and neurological symptoms. Tinnitus and dizziness can also occur. The main differential diagnosis is Multiple Sclerosis. A fast start of immunosuppressive therapy is essential to prevent a progression of the disease. Worldwide around 300 cases are described, whereas the estimated number of unpublished cases is much higher. Due to a wide range of symptoms with variable intensity this autoimmune illness is an interdisciplinary challenge which demands a close cooperation between ENT, Neurology and Ophthalmology.
In the last 3 years two female patients with Susac syndrome were treated in our clinic. Based on their case reports the Susac syndrome will be highlighted to sensitize ENT doctors for this disease.
