Literature DB >> 12185873

[Susac syndrome as a cause of sensorineural hearing loss].

J D Cubillana Herrero1, A Soler Valcárcel, I Albaladejo Devis, B Rodríguez González-Herrero, N Minguez Merlos, J A Jiménez Cervantes-Nicolás.   

Abstract

Susac's syndrome is an extremely rare clinical manifestation characterized by the triad of fluctuating sensorineural hearing loss, sudden visual loss and encephalopathy. Probably underdiagnosed, it affects young women who start the clinical history with headache, visual and hearing disturbances, with neurological findings in MRI. With unknown aetiology, pathogenesis is based on arteriolar microinfarcts in retina, cochlea, and grey and white matter in the brain. Treatment is, as stated in the bibliography and our experience, intravenous high doses of steroids followed by oral steroids together with hyperbaric oxygen to minimize ischaemic lesions. Aspirin associate to nimodipine has been useful to date in the treatment of our patient. We present a case and review the existing literature.

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Year:  2002        PMID: 12185873     DOI: 10.1016/s0001-6519(02)78323-4

Source DB:  PubMed          Journal:  Acta Otorrinolaringol Esp        ISSN: 0001-6519


  2 in total

Review 1.  Characteristics of Susac syndrome: a review of all reported cases.

Authors:  Jan Dörr; Sarah Krautwald; Brigitte Wildemann; Sven Jarius; Marius Ringelstein; Thomas Duning; Orhan Aktas; Erich Bernd Ringelstein; Friedemann Paul; Ilka Kleffner
Journal:  Nat Rev Neurol       Date:  2013-04-30       Impact factor: 42.937

2.  Cyclophosphamide and methotrexate in Susac's Syndrome: a successful sequential therapy in a case with involvement of the cerebellum.

Authors:  Eleonora Catarsi; Veronica Pelliccia; Chiara Pizzanelli; Ilaria Pesaresi; Mirco Cosottini; Paola Migliorini; Antonio Tavoni
Journal:  Clin Rheumatol       Date:  2014-05-03       Impact factor: 2.980

  2 in total

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