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Susac syndrome.
Otol Neurotol 2004; 25(4):470-3ON

Abstract

OBJECTIVE

The objective of this study was to describe the clinical manifestations; radiographic, audiometric, and retinal fluorescein angiography findings; pathogenesis and treatment of Susac syndrome with review of the literature.

STUDY DESIGN

We conducted a retrospective case review.

SETTING

This study was conducted at a tertiary referral center.

PATIENT

A 50-year-old woman presented with recurrent episodes of neurologic symptoms, bilateral sensorineural hearing loss, and silent retinal artery occlusion.

INTERVENTIONS

The patient underwent complete evaluation, including magnetic resonance image studies, audiometric tests, and retinal fluorescein angiography. She was treated initially with corticosteroids and later with other immunosuppressive agents.

RESULTS

The patient was initially diagnosed with left sudden sensorineural hearing loss. Despite comprehensive clinical and laboratory studies that did not reveal systemic disease, 3 weeks later, the patient developed vertigo, sensorineural hearing loss, and tinnitus in the opposite ear. The neurologic involvement and the bilateral audiologic manifestations raised the possibility of Susac syndrome.

CONCLUSION

Susac syndrome is a rare disorder of unknown origin characterized by the triad of encephalopathy, fluctuating hearing loss, and visual loss resulting from microangiopathy of the brain, cochlea, and retina. The multiple organ involvement seen in Susac syndrome raises a differential diagnosis ranging from autoimmune disease, through systemic vasculitis, to multiple sclerosis. Otolaryngologists should be aware of this syndrome as a result of the vestibulocochlear manifestations and the multidisciplinary evaluation that is required.

Authors+Show Affiliations

Department of Otolaryngology/Head & Neck Surgery, Hadassah-Hebrew University Hospital, Jerusalem, Israel. drgrossm@hotmail.comNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

15241223

Citation

Gross, Menachem, et al. "Susac Syndrome." Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, vol. 25, no. 4, 2004, pp. 470-3.
Gross M, Banin E, Eliashar R, et al. Susac syndrome. Otol Neurotol. 2004;25(4):470-3.
Gross, M., Banin, E., Eliashar, R., & Ben-Hur, T. (2004). Susac syndrome. Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, 25(4), pp. 470-3.
Gross M, et al. Susac Syndrome. Otol Neurotol. 2004;25(4):470-3. PubMed PMID: 15241223.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Susac syndrome. AU - Gross,Menachem, AU - Banin,Eyal, AU - Eliashar,Ron, AU - Ben-Hur,Tamir, PY - 2004/7/9/pubmed PY - 2005/2/11/medline PY - 2004/7/9/entrez SP - 470 EP - 3 JF - Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology JO - Otol. Neurotol. VL - 25 IS - 4 N2 - OBJECTIVE: The objective of this study was to describe the clinical manifestations; radiographic, audiometric, and retinal fluorescein angiography findings; pathogenesis and treatment of Susac syndrome with review of the literature. STUDY DESIGN: We conducted a retrospective case review. SETTING: This study was conducted at a tertiary referral center. PATIENT: A 50-year-old woman presented with recurrent episodes of neurologic symptoms, bilateral sensorineural hearing loss, and silent retinal artery occlusion. INTERVENTIONS: The patient underwent complete evaluation, including magnetic resonance image studies, audiometric tests, and retinal fluorescein angiography. She was treated initially with corticosteroids and later with other immunosuppressive agents. RESULTS: The patient was initially diagnosed with left sudden sensorineural hearing loss. Despite comprehensive clinical and laboratory studies that did not reveal systemic disease, 3 weeks later, the patient developed vertigo, sensorineural hearing loss, and tinnitus in the opposite ear. The neurologic involvement and the bilateral audiologic manifestations raised the possibility of Susac syndrome. CONCLUSION: Susac syndrome is a rare disorder of unknown origin characterized by the triad of encephalopathy, fluctuating hearing loss, and visual loss resulting from microangiopathy of the brain, cochlea, and retina. The multiple organ involvement seen in Susac syndrome raises a differential diagnosis ranging from autoimmune disease, through systemic vasculitis, to multiple sclerosis. Otolaryngologists should be aware of this syndrome as a result of the vestibulocochlear manifestations and the multidisciplinary evaluation that is required. SN - 1531-7129 UR - https://www.unboundmedicine.com/medline/citation/15241223/Susac_syndrome_ L2 - http://Insights.ovid.com/pubmed?pmid=15241223 DB - PRIME DP - Unbound Medicine ER -