Susac syndrome: ophthalmological and auditory manifestations in a patient with headache: case report




Susac's Syndrome, Retinocochleocerebral Vasculopathy, Retinal Artery Occlusions, Vasculitis, Retinal


The first case reports of Susac Syndrome emerged in 1979 due to two young female patients presenting with the clinical triad of multifocal encephalopathy, occlusion of the central retinal artery branch, and sensorineural hearing loss, as well as histopathological findings of microinfarctions in the gray and white matter of the brain. The clinical features of the syndrome have been documented in approximately 500 cases published worldwide, making it extremely rare. Headache is a prominent complaint by patients with Susac syndrome. Its pathophysiology remains unknown, with immune and genetic factors being most related to the retino-cochleo-cerebral clinical manifestations. Since its first description, the scientific community has been paying greater attention to the syndrome, especially with advances in medical technology and the availability of complementary exams.
Case report
In this study, We present a case report of a woman experiencing progressive pulsatile temporal headaches for two years, associated with decreased visual acuity for three months and simultaneous hypoacusis on the left, who was incorrectly diagnosed and treated for multiple sclerosis.
The literature review discusses Susac Syndrome and its microvascular complications, highlighting the multidisciplinary approach necessary for accurate diagnosis and comprehensive long-term management.


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- Rennebohm R, Susac JO, Egan RA, Daroff RB. Susac’s syndrome: update. J Neurol Sci 2010; 299 (1–2):86–91; Epub 2010 Sep 19. DOI:

- Dawe JA, Green AL. Headache in Susac's Syndrome. Curr Pain Headache Rep. 2021 Mar 18;25(4):25. doi: 10.1007/s11916-021-00939-2 DOI:

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- McLeod DS, Ying HS, McLeod CA, Grebe R, Lubow M, Susac JO, et al. Retinal and optic nerve head pathology in susac’s syndrome. Ophthalmology 2011; 118:548–552. This is the first article to describe the retinal histopathology in Susac syndrome. DOI:

- Petty GW, Engel AG, Younge BR, et al. Retinocochleocerebral vasculopathy. Medicine (Baltimore) 1998; 77:12–40. DOI:

- Venditti L, Rousseau A, Ancelet C, Papo T, Denier C. Susac syndrome following COVID-19 infection. Acta Neurol Belg. 2021 Jun;121(3):807-809. doi: 10.1007/s13760-020-01554-5 DOI:

- Malerbi FK, Schoeps VA, T F Matos K. Paracentral acute middle maculopathy in Susac syndrome after dual exposure to SARS-CoV-2 antigen. BMJ Case Rep. 2022 May 10;15(5):e247159. doi: 10.1136/bcr-2021-247159. PMID: 35537770 DOI:

- Martinet N; Fardeau C ; Adam R; Bodaghi B; Papo T; Piette JC, et al.Fluorescein and indocyanine green angiographies in Susac syndrome. Retina 2007; 27:1238–1242. DOI:

- Bitra RK, Eggenberger E. Review of Susac syndrome. Curr Opin Ophthalmol. 2011 Nov;22(6):472-6. doi: 10.1097/ICU.0b013e32834bbfeb DOI:




How to Cite

Góes LBD, Góes CBD, Almeida CB de, Hübner JLCN, Matos KTF. Susac syndrome: ophthalmological and auditory manifestations in a patient with headache: case report. Headache Med [Internet]. 2024 Jun. 30 [cited 2024 Jul. 14];15(2):111-4. Available from:



Case Report