Susac's syndrome (SS) is a clinical triad of encephalopathy, branch retinal artery occlusion and sensorineural hearing loss and maybe due to an immune-mediated endotheliopathy. Because of its rarity and some similarities to other common neurological conditions such as multiple sclerosis and acute disseminated encephalomyelitis, it is often misdiagnosed and therefore mistreated. To the best of o...

Susac's syndrome is a rare autoimmune disease, which is characterized by microangiopathic changes that affects the brain, retina, and cochlea. It is mainly characterized by asymptomatic cerebral infarctions, low-and mid-frequency sensorineural hearing loss, and bilateral distal retinal artery occlusions. Otolaryngologists should be familiar with Susac's syndrome because hearing loss may be the ...

1 MD, PhD (Professor of the Department of Otolaryngology, School of Medicine, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil). 2 MD, Otolaryngologist (Otolaryngology and Head and Neck Surgery Department of Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil) 3 MD, Otorhinolaryngologist. MSc Department of otorhinolaryngology Medical School of the Federal University of Rio...

We describe aggressive immunosuppressive treatment of an adolescent with Susac's syndrome (SS), a disease of the microvasculature in the brain, retina, and inner ear. Because the immunopathogenesis of SS appears to have much in common with that of juvenile dermatomyositis (JDM), the patient was treated with an approach that has been effective for severe JDM. The patient's outcome provides evide...

1978 21. Susac JO, Hoyt WF, Daroff RB: Clinical spectrum of ocular bobbing. J Neurol Neurosurg Psychiat 33: 771-775, 1970 22. Suzuki K, Nakajima K: A case of pontine hemorrhage showing ocular bobbing with electroencephalographic changes. Jpn J Stroke 2: 221-225, 1980 23. Bosch EP, Kennedy SS, Archembrener CA: Ocular bobbing: The myth of its localizing value. Neurol 25: 949-953, 1975 24. Imabaya...