Other Names for this Disease
- Retinocochleocerebral vasculopathy
- Retinopathy-encephalopathy-deafness associated with microangiopathy
- SICRET (small infarction of cochlear, retinal, and encephalic tissue) syndrome
- SICRET syndrome
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Affected people may also have branch retinal artery occlusions (BRAO), usually in both eyes. Impairment of vision can occur in some cases; in other cases, people may have no symptoms with the presence of BRAO.
The three main symptoms (encephalopathy, branched retinal arterial occlusions, and hearing loss) are not always present at the onset of the condition, and all three do not necessarily develop in all cases.Susac syndrome can go away on its own, even without treatment. It usually runs its course in two to three years in which people experience recurrent episodes of symptoms. Although it may resolve on its own, some people can develop long-term neurological damage, hearing loss, or vision loss.
The disease is usually self-limiting, and it often stabilizes in 2-4 years. However, it often results in various cognitive, visual and/or hearing problems.
A study published in March 2007 entitled, Long Term Outcome in Susac Syndrome by Aubart-Cohen, et.al. followed 9 patients with Susac syndrome for more than 6 years. In this series, patients did not suffer from severe cognitive sequelae. All but 1 patient returned to work. Vision was usually not seriously impaired. Most of the patients had bilateral hearing loss, with limited disability.
- Susac's Syndrome. National Organization for Rare Disorders (NORD). December 2010; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Susac%27s%20Syndrome. Accessed 4/25/2011.
- Rennebohm RM, Lubow M, Rusin J, Martin L, Grzybowski DM, Susac JO . Pediatr Rheumatol Online J. January 2008; http://www.ped-rheum.com/content/pdf/1546-0096-6-3.pdf. Accessed 4/25/2011.
- Aubart-Cohen, et.al.. Long-Term Outcome in Susac Syndrome. Medicine. March 2007;
- Do TH, Fisch C, Evoy F. AJNR Am J Neuroradiol. 2004 Mar; http://www.ajnr.org/cgi/content/full/25/3/382. Accessed 12/18/2008.
- Susac JO. AJNR Am J Neuroradiol. 2004 Mar; http://www.ajnr.org/cgi/content/full/25/3/351. Accessed 12/18/2008.
- Eggers SDZ, Zee DS. Central Vestibular Disorders. In: Cummings et al. eds. Otolaryngology: Head & Neck Surgery, 4th ed. Philadelphia, Pennsylvania: Mosby, Inc; 2005;
- Rennebohm, Robert; Susac, John. Journal of Neurological Sciences. February 26,2007; http://www.ncbi.nlm.nih.gov/pubmed/17324441.
- Susac JO, Egan RA, Rennebohm RN, Lubow M. J Neurol Sci. June 15, 2007; http://tinyurl.com/yph8kg. Accessed 12/18/2008.
- Jan-Markus Dorr. Susac syndrome. Orphanet. September, 2013; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=838. Accessed 7/21/2014.