Susak syndrome is a rare neurological pathology. It is characterized by the development of microangiopathy with predominant lesions of the cochlea arterioles, retina and brain, which is clinically manifested by hearing, vision disorders and encephalopathy. It is mostly young women who have the disease. In the early stages of the disease, its diagnostics is complicated by the absence of a complete triad of symptoms. The aetiology of vasculopathy is unknown, presumably autoimmune, as confirmed by inflammatory changes in the cochlea, retina and brain, the presence of nonspecific antibodies to endothelial cells found in one-third of patients, and the response to immunomodulatory therapy [1, 2]. The role of vasospasm, coagulopathy and viral infections is assumed, although remains unproven [1, 3–6].

The syndrome was first described in 1979 by Dr John Susak (J.O. Susak) in Neurology magazine, and in 1994 the disease was named after the author [7].

Headache and encephalopathy are the leading symptoms of Susak syndrome, occurring in 75–80 % of patients. Encephalopathy is more often manifested by distraction, memory impairment, less often by behavioural changes, paranoid and gross cognitive disorders [3].

Visual symptoms, i.e. blurred or sudden loss of vision, restricted visual fields, photopsia, scotomas, are caused by occlusion of the retinal artery branches [8].

Hearing impairment, predominantly in the low- and mid-frequency ranges, impairing speech recognition, can be unilateral or bilateral, but usually asymmetrical; damage to the membranous labyrinth is accompanied by marked dizziness and nystagmus [9].

Cases of lesions of muscles, joints and skin (livedo) during Susak syndrome have been described [10].

Susak syndrome can be monophasic, multiphasic, and chronically progressive, and is a potentially disabling disease. The most common is a monophasic disease with an active period of up to two years and spontaneous resolution of the process. In cases of a multiphasic course of the disease, exacerbations occur after two, 10 or more years from the disease onset, in a chronically progressive course, fluctuation of symptoms is observed for two or more years [2, 7, 11].