Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome

Nat Rev Neurol. 2009 Dec;5(12):683-8. doi: 10.1038/nrneurol.2009.176.


Background: A 23 year-old female presented to a neurology department with a 3 year history of recurrent episodes involving hearing loss, encephalopathy, focal neurological deficits, and visual field deficits. In the 3 years before presentation, the patient had been treated with methylprednisolone for suspected acute demyelinating encephalomyelitis and peripheral otogenic dysfunction from which she made a complete recovery, and for a visual defect in both eyes caused by bilateral branch retinal arterial occlusion, from which she partially improved and commenced long-term treatment with acetylsalicylic acid.

Investigations: Detailed history, clinical examination, extensive laboratory work-up, cerebrospinal fluid analysis, cerebral and spinal MRI, periventricular single-voxel (1)H magnetic resonance spectroscopy, retinal fluorescence angiography, optical coherence tomography, audiometry, neurophysiological work-up (EEG, evoked potentials).

Diagnosis: Susac syndrome, characterized by a combination of encephalopathy, branch retinal artery occlusions, and hearing loss.

Management: Long-term immunosuppressive treatment with azathioprine (150 mg/day) and prednisolone (10 mg/day), and inhibition of thrombocyte function with acetylsalicylic acid (100 mg/day).

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aspirin / therapeutic use
  • Auditory Perceptual Disorders / etiology*
  • Brain / pathology*
  • Cerebral Angiography / methods
  • Diagnosis, Differential
  • Disease Progression
  • Female
  • Fibrinolytic Agents / therapeutic use
  • Hearing Loss / etiology*
  • Humans
  • Magnetic Resonance Imaging / methods
  • Susac Syndrome / complications*
  • Susac Syndrome / diagnosis
  • Susac Syndrome / drug therapy
  • Vision Disorders / etiology*
  • Young Adult


  • Fibrinolytic Agents
  • Aspirin