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.
    Polskie archiwum medycyny wewnȩtrznej 01/2009; 119(1-2):80-2. · 2.12 Impact Factor
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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.
    Clinical and Experimental Ophthalmology 11/2000; 28(5):373-81. · 2.35 Impact Factor
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    ABSTRACT: Susac syndrome, also called SICRET syndrome (small infarction of cochlear, retinal, and encephalic tissue) is a rare condition difficult to diagnose. Sudden deafness may be the inaugural sign. A female patient developed subacute encephalopathy, bilateral sensorineural hearing loss, and ischemic retinopathy. The patient was given cyclophosphamid and methylprednisolone for six months, followed by prednisone for eight months. Signs of encephalopathy had totally regressed by month 14 and retinal arteries were free of obstruction. Deafness remained unchanged. Diagnosis of this microangiopathy involving the inner ear, the brain, and the retina is suggested by the clinical triad and established on the basis of tonal audiometry, fundus examination, fluorescein angiography, examination of the cerebrospinal fluid, magnetic resonance imaging. Multiple sclerosis is the main differential diagnosis. The pathogenesis remains unknown. We observed transient-evoked otoacoustic emissions. There is no consensus concerning treatment. Many advocate combining corticosteroids and immunosuppressors. Otolaryngologists should be aware that an ophthalmological examination is required for patients with central or visual disorders associated with hearing loss.
    Annales d Otolaryngologie et de Chirurgie Cervico-Faciale 03/2003; 120(1):49-53.
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