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Susac syndrome

Disease definition

Susac syndrome (SS) is a rare disorder characterized by the triad of central nervous system (CNS) dysfunction, branch retinal artery occlusions (BRAOs) and sensorineural hearing loss (SNHL). It is presumably due to autoimmune-mediated occlusions of microvessels in the CNS, the retina, and the inner ear.

ORPHA:838

  • Synonym(s):
    • RED-M
    • Retinocochleocerebral vasculopathy
    • Retinopathy-encephalopathy-deafness 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

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