TY - JOUR
T1 - Progressive sensorineural hearing loss many years preceding completion of Susac's syndrome triad
T2 - A case report
AU - Wang, Yujie
AU - Burkholder, Bryn
AU - Newsome, Scott D.
N1 - Publisher Copyright:
© 2019 Elsevier B.V.
Copyright:
Copyright 2020 Elsevier B.V., All rights reserved.
PY - 2020/1
Y1 - 2020/1
N2 - 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.
AB - 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.
KW - Audiogram
KW - Corpus callosum
KW - Fluorescein angiogram
KW - MRI
KW - Retinal artery occlusion
KW - Sensorineural hearing loss
UR - http://www.scopus.com/inward/record.url?scp=85073166632&partnerID=8YFLogxK
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U2 - 10.1016/j.msard.2019.101436
DO - 10.1016/j.msard.2019.101436
M3 - Article
C2 - 32173001
AN - SCOPUS:85073166632
VL - 37
JO - Multiple Sclerosis and Related Disorders
JF - Multiple Sclerosis and Related Disorders
SN - 2211-0348
M1 - 101436
ER -