A Rare Cause of Hearing Loss: Susac Syndrome

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A Rare Cause of Hearing Loss: Susac Syndrome.

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 ...

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INTRODUCTION Alström Syndrome is a rare disease caused by mutations in ALMS1 gene. It is characterized by a progressive degeneration of sensory functions, resulting in visual and audiological impairment, as well as metabolic disturbances such as childhood obesity, hyperinsulinemia, and diabetes mellitus type 2. OBJECTIVE To report and discuss the genetic and audiological findings in two sibli...

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Susac syndrome as an example of a rare vasculopathy.

Susac syndrome is a rare vasculopathy affecting small vessels of the brain, the retina and the cochlea. The pathogenesis of this syndrome is unknown. It is characterized by a triad of clinical symptoms, i.e. encephalopathy, visual disorders and hearing disorders. The aim of this work was to review available data about the clinical course, diagnosis and treatment of Susac syndrome.

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Susac syndrome is a readily recognized but often misdiagnosed disorder almost exclusively affecting women in the 20- to 40-year age range. Characterized by the clinical triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss, patients with Susac syndrome are often misdiagnosed with multiple sclerosis (MS). Unlike MS, however, the disease process extends over a ...

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ژورنال

عنوان ژورنال: The Journal of International Advanced Otology

سال: 2015

ISSN: 1308-7649,2148-3817

DOI: 10.5152/iao.2015.1002