Susac's syndrome: A rare cause of fluctuating sensorineural hearing loss

Department of Otolaryngology-Head and Neck Surgery, University Hospital, Nottingham, UK.
The Journal of Laryngology & Otology (Impact Factor: 0.67). 12/1997; 111(11):1072-4. DOI: 10.1017/S0022215100139374
Source: PubMed


Susac's syndrome is extremely rare and is characterized by the clinical triad of encephalopathy, retinal artery occlusion and deafness. A case of this rare syndrome is presented and for the first time the fluctuating nature of the hearing loss is demonstrated. The site and nature of the hearing loss has been investigated. The value of neural hearing loss as a method of monitoring the clinical severity is discussed.

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    ABSTRACT: 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.
    Preview · Article · Jan 2009 · Polskie archiwum medycyny wewnȩtrznej
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    ABSTRACT: Susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea. Analysis of two male and two female cases of Susac syndrome recognized in Australia. In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleo-vestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of Susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus erythematosis (SLE), Behçet's syndrome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro-otologic features are most frequently misdiagnosed as multiple sclerosis. Susac syndrome, first described in 1979, is becoming an increasingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent cognitive, audiologic and visual sequelae.
    Full-text · Article · Nov 2000 · Clinical and Experimental Ophthalmology
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    ABSTRACT: Susac's syndrome is a rare disorder characterized by the triad of microangiopathy of the brain and retina with hearing loss. More than 50 affected individuals have been reported worldwide, all Caucasians. We herein identify the first Japanese patient with Susac's syndrome. A 36-year-old man developed recurrent subacute encephalopathy, bi- a lateral sensorineural hearing loss, and retinal arteriolar occlusions, caused by microangiopathy from a year previously. T2-weighted MRI showed multiple high-signal lesions ti predominantly in the periventricular white matter. During the exacerbated phase both high-dose intravenous methyl-prednisolone and oral prednisone therapy produced beneficial effects. He showed definite remission within 2 years from the disease onset.
    No preview · Article · Mar 2001 · Internal Medicine
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