Coexistence of acute hearing loss with retinal artery occlusion and encephalopathy

Abstract 

We characterize 2 cases with sensorineural hearing loss and ophthalmologic findings. The clinicopathologic features revealed diagnosis of Susac's syndrome, a rare microangiopathy with cochlea, retinal, and brain affection. Diagnosis may be difficult because most specialists are not familiar with this angiopathy. However, the characteristic symptoms can mimic different pathologies, which may result in attention of radiologists, ophthalmologists, neurologists and otolaryngologists. In this report, we present 2 women with Susac's syndrome unveiled by audiometry, magnetic resonance imaging of the brain, and ophthalmologic findings. The course of the illness and a review of literature are presented.