Susac Syndrome

The Susac syndrome is a microangiopathy causing small infarcts in the brain, cochlea, and retina. It is characterized by the clinical triad of acute or subacute encephalopathy, sensorineural hearing loss, and retinal branch artery occlusions. This syndrome is probably an immune-mediated endotheliopathy that affects these organs’ microvasculature. The definite diagnosis is made by the presence of the triad of brain, retinal, and vestibulocochlear involvements. If only two domains are affected, a probable diagnosis can be proposed. Treatment of this condition includes immunosuppression with high-dose steroids and intravenous immunoglobulin, and in some cases cytotoxic drugs.