CASE REPORT Susac Syndrome: Retinocochleocerebral Vasculopathy

Susac syndrome is a rare microangiopathy of cochlea, retina, and brain. We report a case of a 30-year-old man with Susac syndrome. The patient initially suffered from unilateral hearing loss associated with peripheral vestibular syndrome, and followed with recurrent arterial retinal occlusions and encephalopathy. The patient underwent clinical, laboratory, and neuroradiological examination. Laboratory tests were negative for systemic inflammatory or infectious disease. Signs of encephalopathy and vestibular syndrome regressed after 6 weeks, retinal obstructions were partially improved, and deafness remained unchanged. Two unexplained epileptic seizures had been documented 7 years before the development of typical clinical course. The etiology is still unknown and diagnosis was suggested by the clinical triade of bilateral sensorineural hearing loss on low frequency on audiology, recurrent bilateral retinal branch artery occlusions, and small multiple areas of signal hyperintensity in the white and gray matter on brain magnetic resonance T2-weighted images. The clinical course is self-limited and treatment options are not codified. Epileptic seizures, as those in our patient, may extend the clinical spectrum of Susac syndrome. This case also documents the possibility of multiphasic disease course.