Susac Syndrome (Retinal Vasculitis, Hearing Loss, and Encephalopathy)

2021 ◽  
pp. 277-280
Author(s):  
Ryan A. Shields ◽  
Neil Onghanseng ◽  
Muhammad Hassan ◽  
Quan Dong Nguyen
Keyword(s):  
Hearing Loss ◽  
Retinal Vasculitis ◽  
Susac Syndrome

Vestibular function assessment of Susac syndrome patients by the video head impulse test and cervical vestibular-evoked myogenic potentials

2020 ◽  
Vol 30 (6) ◽  
pp. 393-399
Author(s):  
Yahav Oron ◽  
Ophir Handzel ◽  
Zohar Habot-Wilner ◽  
Keren Regev ◽  
Arnon Karni ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Vestibular Function ◽  
Pure Tone Audiometry ◽  
Sensorineural Hearing ◽  
Vestibular Evoked Myogenic Potentials ◽  
Head Impulse Test ◽  
Video Head Impulse Test ◽  
Head Impulse ◽  
Susac Syndrome

BACKGROUND: Susac syndrome (retino-cochleo-cerebral vasculopathy, SuS) is an autoimmune endotheliopathy characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. In contrast to data regarding auditory function, data measuring vestibular function is sparse and the cervical vestibular-evoked myogenic potentials (cVEMPs). OBJECTIVE: To determine whether the video head impulse test (vHIT) can serve as a confirmatory assessment of vestibulocochlear dysfunction in cases of suspected SuS. METHODS: Seven patients diagnosed with SuS underwent pure tone audiometry, a word recognition test, cVEMPs and the vHIT. RESULTS: Five patients were diagnosed with definite SuS, and two with probable SuS. Two patients were asymptomatic for hearing loss or tinnitus, and no sensorineural hearing loss was detected by audiograms. Four patients complained of tinnitus, and three patients reported experiencing vertigo. Three patients had abnormal cVEMPs results. All seven patients’ vHIT results were normal, except for patient #2, who was one of the three who complained of vertigo. The calculated gain of her left anterior semicircular canal was 0.5, without saccades. CONCLUSIONS: This is the first study to describe the results of the vHIT and cVEMPs among a group of patients with SuS. The results suggest that the vHIT should not be the only exam used to assess the function of the vestibular system of SuS patients.

scholarly journals Susac syndrome and pregnancy: a review of published cases and considerations for patient management

2021 ◽  
Vol 14 ◽  
pp. 175628642098135
Author(s):  
Barbara Willekens ◽  
Ilka Kleffner
Keyword(s):  
Hearing Loss ◽  
Family Planning ◽  
Postpartum Period ◽  
Management Strategy ◽  
Patient Management ◽  
Treatment Options ◽  
Young Females ◽  
Susac Syndrome ◽  
Retinal Artery

Susac syndrome (SuS) is a rare autoimmune endotheliopathy leading to hearing loss, branch retinal artery occlusions and encephalopathy. Young females are more frequently affected than males, making counselling for family planning an important issue. We reviewed published cases on SuS during pregnancy or in the postpartum period, and selected 27 reports describing the details of 33 patients with SuS. Treatment options and implications for pregnancy and breastfeeding are discussed. We propose new areas for research and suggest a management strategy.

scholarly journals Anecdotal Response to Amphotericin in a Patient with Probable Susac Syndrome: Implications in the Pathogenesis

2019 ◽  
Vol 32 (11) ◽  
pp. 727
Author(s):  
Raquel Gil-Gouveia ◽  
Natália Marto ◽  
Pedro Vilela ◽  
Ana Catarino
Keyword(s):  
Hearing Loss ◽  
Fungal Infection ◽  
Infectious Agent ◽  
Brain Biopsy ◽  
High Dose ◽  
Radiological Findings ◽  
Susac Syndrome ◽  
Infection Treatment ◽  
Immune Mediated

Susac syndrome is a rare, probably immune-mediated endotheliopathy presenting with encephalopathy, sensorineural hearing loss and retinal arterial occlusions. A 33-year-old female with Susac syndrome was worsening despite high-dose steroids so a brain biopsy was performed which suggested a possible fungal infection. Treatment with amphotericin B resulted in prompt reversal of symptoms and radiological findings, and no further symptoms occurred during 8 years of follow-up. A diagnosis of fungal infection was not confirmed. The etiology of Susac syndrome is unknown and this anecdotal observation suggests that an infectious agent susceptible to amphotericin might have caused or triggered Susac syndrome in this patient.

scholarly journals Susac syndrome presenting with acute hemibody paraesthesia

2019 ◽  
Vol 8 ◽  
pp. 204800401984468
Author(s):  
Philip Campbell ◽  
Diego Kaski ◽  
Tabish A Saifee
Keyword(s):  
Hearing Loss ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Branch Retinal Artery Occlusion ◽  
Stroke Management ◽  
Susac Syndrome ◽  
Hyperacute Stroke ◽  
Appropriate Treatment ◽  
Retinal Artery ◽  
Clinical Triad

Susac syndrome is an orphan disease characterised by encephalopathy, branch retinal artery occlusion and sensorineural hearing loss. As the clinical triad is rarely present at symptom onset, it is often initially misdiagnosed and appropriate treatment is often delayed. Herewith, we report a case of Susac syndrome in a 47-year-old man presenting with acute hemisensory loss and highlight the challenges of early diagnosis, particularly relevant in the era of hyperacute stroke management.

Vestibular function assessment of Susac syndrome patients by the video head impulse test and cervical vestibular-evoked myogenic potentials

2020 ◽  
pp. 1-7
Author(s):  
Yahav Oron ◽  
Ophir Handzel ◽  
Zohar Habot-Wilner ◽  
Keren Regev ◽  
Arnon Karni ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Vestibular Function ◽  
Pure Tone Audiometry ◽  
Sensorineural Hearing ◽  
Vestibular Evoked Myogenic Potentials ◽  
Head Impulse Test ◽  
Video Head Impulse Test ◽  
Head Impulse ◽  
Susac Syndrome

BACKGROUND: Susac syndrome (retino-cochleo-cerebral vasculopathy, SuS) is an autoimmune endotheliopathy characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. In contrast to data regarding auditory function, data measuring vestibular function is sparse. OBJECTIVE: To determine whether the video head impulse test (vHIT) can serve as a confirmatory assessment of vestibulocochlear dysfunction in cases of suspected SuS. METHODS: Seven patients diagnosed with SuS underwent pure tone audiometry, a word recognition test, cervical vestibular-evoked myogenic potentials (cVEMPs), and the v-HIT. RESULTS: Five patients were diagnosed with definite SuS, and two with probable SuS. Two patients were asymptomatic for hearing loss or tinnitus, and no sensorineural hearing loss was detected by audiograms. Four patients complained of tinnitus, and three patients reported experiencing vertigo. Three patients had abnormal cVEMPs results. All seven patients’ vHIT results were normal, except for patient #2, who was one of the three who complained of vertigo. The calculated gain of her left anterior semicircular canal was 0.5, without saccades. CONCLUSIONS: This is the first study to describe the results of the vHIT and cVEMPs among a group of patients with SuS. The results suggest that the vHIT should not be the only exam used to assess the function of the vestibular system of SuS patients.

scholarly journals A Rare Cause of Hearing Loss: Susac Syndrome

2015 ◽  
Vol 11 (2) ◽  
pp. 167-169 ◽  
Author(s):  
Busra Yurtsever ◽  
Murat Cabalar ◽  
Hakan Kaya ◽  
Betul Tugcu ◽  
Zahide Mine Yazici ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Susac Syndrome

Encephalopathy, visual disturbance and hearing loss—recognizing the symptoms of Susac syndrome

2009 ◽  
Vol 5 (12) ◽  
pp. 683-688 ◽  
Author(s):  
Jan Dörr ◽  
Helena Radbruch ◽  
Markus Bock ◽  
Jens Wuerfel ◽  
Anne Brüggemann ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Visual Disturbance ◽  
Susac Syndrome

scholarly journals Retinal vasculitis in Susac syndrome: case report

2009 ◽  
Vol 72 (3) ◽  
pp. 397-399 ◽  
Author(s):  
Gherusa Helena Milbratz ◽  
Francisco Azevedo Marquardt ◽  
Heriberto Pinto Guimarães Neto ◽  
Daniel Azevedo Marquardt ◽  
Eduardo Soares Maia Vieira de Souza
Keyword(s):  
Case Report ◽  
Retinal Vasculitis ◽  
Susac Syndrome

scholarly journals Susac syndrome misdiagnosed as multiple sclerosis with exacerbation by interferon beta therapy

2017 ◽  
Vol 31 (2) ◽  
pp. 207-212 ◽  
Author(s):  
Hussein Algahtani ◽  
Bader Shirah ◽  
Muhammad Amin ◽  
Eyad Altarazi ◽  
Hashem Almarzouki
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Interferon Beta ◽  
Young Patients ◽  
Oligoclonal Bands ◽  
Resonance Imaging ◽  
Susac Syndrome ◽  
Retinal Artery

Susac syndrome is a rare autoimmune disorder characterised by the clinical triad of encephalopathy, retinopathy (branch retinal artery occlusions) and hearing loss. The diagnosis of Susac syndrome may be difficult initially, and it is not uncommon for patients with Susac syndrome to be misdiagnosed with multiple sclerosis. In this case report, we describe a patient who was diagnosed as having multiple sclerosis for three years, with further deterioration after starting treatment with interferon beta-1a. The patient had the triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. She had the classic magnetic resonance imaging appearance, with normal magnetic resonance imaging of the spinal cord and absence of oligoclonal bands in the cerebrospinal fluid. Our patient responded well to treatment with a combination therapy and discontinuation of interferon beta-1a. Our observations raise awareness about the importance of the early and correct diagnosis of Susac syndrome, which usually affects young patients, with an excellent prognosis if treated aggressively at an early stage of the disease. Susac syndrome is underdiagnosed and is not uncommonly misdiagnosed as multiple sclerosis. Susac syndrome is a great mimicker of multiple sclerosis, and establishing diagnostic criteria for this syndrome is very useful. In any patient presenting with a progressive disabling neurological disorder associated with callosal lesions and/or hearing loss, and/or visual loss especially in women, Susac syndrome should be suspected.

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