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

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Abstract

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.

Original languageEnglish (US)
Article number101436
JournalMultiple Sclerosis and Related Disorders
Volume37
DOIs
StatePublished - Jan 2020

Keywords

  • Audiogram
  • Corpus callosum
  • Fluorescein angiogram
  • MRI
  • Retinal artery occlusion
  • Sensorineural hearing loss

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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