Susac's syndrome: Intratympanic therapy for hearing loss and a review of the literature

Crawley, Brianna K.; Close, Abby; Canto, Cecilia; Harris, Jeffrey P.

doi:10.1002/lary.20040

Cited by 12 publications

(6 citation statements)

References 17 publications

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“…Intratympanic steroid injection for SNHL has been performed, with mixed results reported in case studies 10 , 22 . One of our presented patients had no improvement with three rounds of IT dexamethasone.…”

Section: Treatmentmentioning

confidence: 73%

“…Intratympanic steroid injection for SNHL has been performed, with mixed results reported in case studies. 10,22 One of our presented patients had no improvement with three rounds of IT dexamethasone. Rehabilitation of hearing loss should be addressed with amplification when appropriate and an understanding of the potential for progression of SNHL.…”

Section: Treatmentmentioning

confidence: 76%

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Scoping review of cochlear implantation in Susac's syndrome

Perez

McCall

Hirsch

2021

World j. otorhinolaryngol.-head neck surg.

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Objective Scoping review of published literature to establish clinical characteristics and audiologic outcomes in patients diagnosed with Susac’s Syndrome(SS) who have undergone cochlear implantation (CI). Data sources All published studies of CI in SS and contribution of two of our own patients who have not been reported previously. Methods A comprehensive search of MEDLINE (via PubMed) was carried out in March 2020 using the following keywords and related entry terms: Susac’s Syndrome, Cochlear Implantation. Results Our search identified a total of five case reports of CI in SS. With the addition of our two patients reported here, we analyzed characteristics and outcomes in seven patients. Mean age at implantation was 30 years old (range 19–46), with six women and one man implanted. Mean time from onset of hearing loss to implantation was 17 months (range three months to four years). Best reported postoperative speech understanding was reported via different metrics, with six of seven patients achieving open set speech scores of 90% or better, and one subject performing at 68%. Vestibular symptoms were present preoperatively in four of seven patients (57%), with vestibular testing reported in two patients, and showing vestibulopathy in one patient. No complications were reported following cochlear implantation. Conclusion Cochlear implantation is a viable option for hearing rehabilitation in patients with SS, with levels of attainment of open set speech comparable to other populations of CI candidates.

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Section: Treatmentmentioning

confidence: 73%

Section: Treatmentmentioning

confidence: 76%

Scoping review of cochlear implantation in Susac's syndrome

Perez

McCall

Hirsch

2021

World j. otorhinolaryngol.-head neck surg.

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“…One week into the course he developed hearing loss, which was treated with the addition of intratympanic steroids with moderate improvement. The use of intratympanic steroids has been reported on occasion [ 15 , 16 ]. Although it appears to have been moderately successful in our patient, it remains unclear whether the improvement was related to this local treatment, systemic therapy, or both.…”

Section: Discussionmentioning

confidence: 99%

Susac syndrome with prominent dermatological findings and a prompt response to intravenous immunoglobulin, steroids, and rituximab: a case report

Gertner

Rosenbloom²

2016

J Med Case Reports

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BackgroundSusac syndrome (retinocochleocerebral vasculopathy) is an autoimmune endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear. It presents with encephalopathy, branch retinal artery occlusions, and hearing loss. The condition is often under recognized because the clinical symptoms may present at different times and physicians may be unfamiliar with the syndrome. Peripheral findings would be helpful in early diagnosis. There are numerous treatment regimens proposed with varying effectiveness.Case presentationWe report the case of a 22-year-old Caucasian man in whom there were prominent skin findings, including livedo reticularis and a micropapular eruption which responded promptly to treatment suggesting that skin involvement may facilitate earlier diagnosis. Rituximab has occasionally been used in more refractory disease. We observed a prompt response to the combination of intravenous immunoglobulin, corticosteroids, and rituximab instituted immediately after diagnosis.ConclusionsA careful search for dermatological manifestations may help with earlier diagnosis. Skin findings may be another marker of endothelial cell involvement. Early use of rituximab as part of the therapeutic regimen may be warranted.

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“…Susac syndrome is a rare diagnosis. It is likely misdiagnosed, and therefore underdiagnosed, for three reasons: a) Susac syndrome mimics other diseases, such as Multiple Sclerosis (MS) [5];…”

Section: Introductionmentioning

confidence: 99%

The Rehabilitation of a Susac Syndrome Patient: A Case Report and Clinical Review

Cao

Baczuk

Chen

et al. 2019

BJSTR

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Susac syndrome is a rare condition involving the brain, retina, and cochlea vasculature causing encephalopathy, visual loss due to retinal branch occlusion, and sensorineural hearing loss. Most evidence support that this disease is an acquired autoimmune disorder. Early diagnosis and aggressive treatment are important to prevent irreversible neurological damage, hearing loss, or vision loss. The objective of this case study and clinical review is to emphasize the recognition of this underdiagnosed syndrome and its treatment. Especially, the adjunct of rehabilitation treatment to pharmacological treatment may promote functional recovery.

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Susac's syndrome: Intratympanic therapy for hearing loss and a review of the literature

Cited by 12 publications

References 17 publications

Scoping review of cochlear implantation in Susac's syndrome

Scoping review of cochlear implantation in Susac's syndrome

Susac syndrome with prominent dermatological findings and a prompt response to intravenous immunoglobulin, steroids, and rituximab: a case report

The Rehabilitation of a Susac Syndrome Patient: A Case Report and Clinical Review

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