Susac syndrome: microangiopathy of the retina, cochlea and brain

Clin Exp Ophthalmol. 2000 Oct;28(5):373-81. doi: 10.1046/j.1442-9071.2000.00345.x.


Background: 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.

Methods: Analysis of two male and two female cases of Susac syndrome recognized in Australia.

Results: 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.

Conclusion: 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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use
  • Brain / blood supply
  • Brain Diseases / diagnosis*
  • Brain Diseases / drug therapy
  • Cochlea / blood supply
  • Diagnosis, Differential
  • Electroencephalography
  • Female
  • Fluorescein Angiography
  • Fundus Oculi
  • Glucocorticoids / therapeutic use
  • Hearing Loss, Sensorineural / diagnosis*
  • Hearing Loss, Sensorineural / drug therapy
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Peripheral Vascular Diseases / diagnosis
  • Peripheral Vascular Diseases / drug therapy
  • Retinal Artery Occlusion / diagnosis*
  • Retinal Artery Occlusion / drug therapy
  • Syndrome
  • Vestibular Function Tests
  • Visual Fields


  • Anti-Inflammatory Agents, Non-Steroidal
  • Glucocorticoids