Journal
NEUROLOGICAL SCIENCES
Volume 43, Issue 1, Pages 683-686Publisher
SPRINGER-VERLAG ITALIA SRL
DOI: 10.1007/s10072-021-05581-9
Keywords
Susac syndrome; Hearing loss; Encephalopathy; Branch retinal artery occlusions; Corpus callosum
Categories
Funding
- Ministry of Science and Technology of Taiwan [MOST 110-2314-B-016036-MY2]
- Tri-Service General Hospital, Taiwan [TSGH-E-110195]
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Susac syndrome is a rare disease with a classic triad of symptoms, including sensorineural hearing loss, encephalopathy, and branch retinal artery occlusions. Early symptoms may lead to misdiagnosis, making neurological and retinal examinations crucial for accurate diagnosis. Case reviews and treatment guidelines are available for Susac syndrome presenting as sudden sensorineural hearing loss.
Susac syndrome is a rare disease presenting with a classic triad of symptoms. These are sensorineural hearing loss, encephalopathy, and branch retinal artery occlusions. Initial presentation is usually headache and symptoms of encephalopathy. Hearing loss is unusual in the early stages but, when it does present, can often lead to a misdiagnosis of sudden sensorineural hearing loss. Hence, neurological and retinal examinations are essential to an accurate diagnosis. In this study, we aimed to raise awareness of Susac syndrome among physicians and facilitate recognition of its manifestation, especially in those patients presenting with hearing loss alone. Identifying Susac syndrome that presents as sudden sensorineural hearing loss can be challenging but a number of case reviews have been reported in recent years and treatment guidelines are available.
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