Orphanet: Susac syndrome

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Susac syndrome

Disease definition

A rare systemic or rheumatologic disease characterized by the triad of central nervous system (CNS) dysfunction, branch retinal artery occlusions (BRAOs) and sensorineural hearing loss (SNHL) due to autoimmune-mediated occlusions of microvessels in the brain, retina, and inner ear.


Classification level: Disorder
  • Synonym(s):
    • RED-M
    • Retinocochleocerebral vasculopathy
    • Retinopathy-encephalopathy-deafness associated with microangiopathy
    • Retinopathy-encephalopathy-hearing loss associated with microangiopathy
    • SICRET syndrome
    • Small infarctions of cochlear, retinal and encephalic tissue
  • Prevalence: Unknown
  • Inheritance: Unknown 
  • Age of onset: All ages
  • ICD-10: I67.7
  • OMIM: -
  • UMLS: C2717757
  • MeSH: D055955
  • GARD: 7713
  • MedDRA: 10071573

Detailed information

Article for general public


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