Susac’s syndrome : 2 cases without hearing loss

Abstract

Susac’s syndrome is a disease of the microvasculature in the brain, inner ear (cochlea and vestibular apparatus) and retina that consists of a clinical triad of encephalopathy, hearing loss and visual disturbance. It is usually seen in young women. It is first defined by Susac et al. in 1979. Diagnosis is often difficult because the classic triad may take up to 2 years to develop and radiological findings are easily confused with multiple sclerosis. Also, encephalopathy may mask some other findings. We present two cases that we think are particularly instructive because they were male patients, had somewhat unusual presenting symptoms, had no accompanying hearing loss, required relatively less aggressive and less sustained immunosuppression than usual, and followed a relatively short and benign course, without relapse and with excellent outcome.  They also serve as good examples of the apparent efficacy and sufficiency (in their cases) of IVIG.

Keywords

• Susac's Syndrome; Retinal Artery Occlusion; Hearing Loss; IVIG

Susac’s syndrome: 2 cases without hearing loss

Öz

Kaynakça

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