Susac’s Syndrome: A Case with Unusual Cardiac Vestibular and Imaging Manifestations

Purpose: Susac syndrome (SS) is a rare autoimmune disorder that affects the brain and the retina and causes unilateral or bilateral sensorineural hearing loss. Although vestibular dysfunction is reported in SS, limited information is available underlying the vestibular pathophysiology. Method: The diagnosis of SS was established based on symptoms and diagnostic tools such as magnetic resonance imaging and fundus fluorescein angiography. The audiovestibular evaluation was done on the seventh day of admission (Session 1) into the emergency unit, whereas the second and third evaluations were done at 3-month (Session 2) and 8-month (Session 3) follow-ups after discharge, respectively. The audiovestibular test battery consisted of routine audiological tests, auditory brainstem response, and vestibular evoked myogenic potentials (VEMPs; both cervical and ocular). Results: We found unilateral sensorineural hearing loss and absent cervical VEMPs(cVEMPs) when testing the left ear during Session 1. In the following sessions, the unilateral hearing loss did not recover; however, cVEMPs were present bilaterally. The ocular VEMPs showed an increase in amplitude during Sessions 2 and 3. Conclusions: SS can selectively disrupt auditory and vestibular structures. It may present with unique findings of audiovestibular tests. A detailed audiovestibular evaluation may be essential in patients with SS. Supplemental Material: https://doi.org/10.23641/asha.21513843

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Cervical and Ocular Vestibular Evoked Myogenic Potential Recovery in Susac Syndrome: A Case Report

Suman

Poovaiah

Rangarajan

et al. 2022

Am J Audiol

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Case report: Susac syndrome—two ends of the spectrum, single center case reports and review of the literature

Cviková,

Štefela,

Všianský

et al. 2024

Front. Neurol.

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Susac syndrome is a rare and enigmatic complex neurological disorder primarily affecting small blood vessels in the brain, retina, and inner ear. Diagnosing Susac syndrome may be extremely challenging not only due to its rarity, but also due to the variability of its clinical presentation. This paper describes two vastly different cases—one with mild symptoms and good response to therapy, the other with severe, complicated course, relapses and long-term sequelae despite multiple therapeutic interventions. Building upon the available guidelines, we highlight the utility of black blood MRI in this disease and provide a comprehensive review of available clinical experience in clinical presentation, diagnosis and therapy of this disease. Despite its rarity, the awareness of Susac syndrome may be of uttermost importance since it ultimately is a treatable condition. If diagnosed in a timely manner, early intervention can substantially improve the outcomes of our patients.

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Susac’s Syndrome: A Case with Unusual Cardiac Vestibular and Imaging Manifestations

Cited by 2 publications

References 11 publications

Cervical and Ocular Vestibular Evoked Myogenic Potential Recovery in Susac Syndrome: A Case Report

Cervical and Ocular Vestibular Evoked Myogenic Potential Recovery in Susac Syndrome: A Case Report

Case report: Susac syndrome—two ends of the spectrum, single center case reports and review of the literature

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