Susac's syndrome: a rare cause of fluctuating sensorineural hearing loss

J Laryngol Otol. 1997 Nov;111(11):1072-4. doi: 10.1017/s0022215100139374.


Susac's syndrome is extremely rare and is characterized by the clinical triad of encephalopathy, retinal artery occlusion and deafness. A case of this rare syndrome is presented and for the first time the fluctuating nature of the hearing loss is demonstrated. The site and nature of the hearing loss has been investigated. The value of neural hearing loss as a method of monitoring the clinical severity is discussed.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Audiometry, Pure-Tone
  • Brain / pathology
  • Central Nervous System Diseases / pathology*
  • Diagnosis, Differential
  • Female
  • Fundus Oculi
  • Hearing Loss, Sensorineural / pathology*
  • Humans
  • Magnetic Resonance Imaging
  • Multiple Sclerosis / diagnosis
  • Retinal Artery Occlusion / pathology*
  • Syndrome