Case report| Volume 37, 101436, January 2020

Progressive sensorineural hearing loss many years preceding completion of Susac's syndrome triad: A case report

Published:October 07, 2019DOI:


      • Susac's syndrome can manifest without the full triad.
      • Symptoms may be separated in time, even by years.
      • It can be a chronic, progressive disorder, leading to significant disability.
      • Treatment may require aggressive immune therapy for disease control.


      Susac's syndrome (SuS) is a rare disorder with a clinical triad of encephalopathy, sensorineural hearing loss, and branch retinal artery occlusions. We report a 7-year-old girl who presented with chronic, progressive sensorineural hearing loss, who, years later, presented with encephalopathy and vision loss. Such prolonged period between symptoms is unusual and to our knowledge, this is the longest interval between onset of hearing loss and completion of the full triad in SuS. In addition, she had a protracted disease course, requiring multiple immune therapies for disease control.


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