Hostname: page-component-586b7cd67f-dlnhk Total loading time: 0 Render date: 2024-11-24T13:09:30.741Z Has data issue: false hasContentIssue false
  • English
  • Français

Susac's syndrome: a rare cause of fluctuating sensorineural hearing loss

Published online by Cambridge University Press:  29 June 2007

Neil D. Bateman ,
Ian J. M. Johnson  and
Kevin P. Gibbin
Neil D. Bateman*
Affiliation:
Department of Otolaryngology – Head and Neck Surgery, University Hospital, Nottingham, UK
Ian J. M. Johnson
Affiliation:
Department of Otolaryngology – Head and Neck Surgery, University Hospital, Nottingham, UK
Kevin P. Gibbin
Affiliation:
Department of Otolaryngology – Head and Neck Surgery, University Hospital, Nottingham, UK
*
Address for correspondence: Mr Neil Bateman, Department of Otolaryngology – Head and Neck Surgery, University Hospital, Queen's Medical Centre, Nottingham NG7 2UH
Get access Rights & Permissions [Opens in a new window]

Abstract

Susac's syndrome is extremely rare and is characterized by the clinical triad of encephalopathy, retinal artery occlusion and deafness. A case of this rare syndrome is presented and for the first time the fluctuating nature of the hearing loss is demonstrated. The site and nature of the hearing loss has been investigated. The value of neural hearing loss as a method of monitoring the clinical severity is discussed.

Keywords

Hearing loss, sensorineural
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 111 , Issue 11 , November 1997 , pp. 1072 - 1074
Copyright
Copyright © JLO (1984) Limited 1997

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Cane, M. A., Lutman, M. E., O'Donoghue, G. M. (1994) Transiently evoked otoacoustic emissions in patients with cerebellopontine angle tumours. American Journal of Otology 15: 207–216.Google Scholar
Copetto, J. R., Currie, J. N., Monteiro, M. L. R., Lessell, S. A. (1984) Syndrome of arterial occlusive retinopathy and encephalopathy. American Journal of Ophthalmology 98: 189–202.CrossRefGoogle Scholar
Gordon, D. L., Hayreh, S. S., Adams, H. P. (1991) Microangiopathy of the brain, retina and ear: improvement without immunosuppressive therapy. Stroke 23: 903–907.Google Scholar
Heiskala, H., Somer, H., Kovanen, J., Poutianen, E., Karli, H., Haita, M. (1988) Microangiopathy with encephalopathy, hearing loss and retinal arteriolar disease: two new cases. Journal Neurological Science 86: 43–46.CrossRefGoogle ScholarPubMed
Mass, M., Bourdette, D., Bernstein, W., Hammerstad, J. (1988) Retinopathy, encephalopathy, deafness associated microangiopathy (the RED M syndrome): three new cases [abstract]. Neurology 38 (Suppl 1): 215.Google Scholar
Monteiro, M. L. R., Swanson, R. A., Coppeto, J. R., Cuneo, R. A., Dearmond, S. J., Prusiner, S. B. (1985) A microangiopathic syndrome of encephalopathy, hearing loss and retinal arteriolar occlusions. Neurology 35: 1113–1121.CrossRefGoogle ScholarPubMed
Pfaffenbach, D. D., Hollenhorst, R. W. (1973) Microangiopathy of the retinal arterioles. Journal of the American Medical Association 225: 480–483.Google Scholar
Schwitter, J., Agosti, R., Ott, P., Kalman, A., Waespe, W. (1992) Small infarctions of cochlear, retinal and encephalic tissue in young women. Stroke 23: 903–907.CrossRefGoogle ScholarPubMed
Susac, J. O. (1994) Susac's syndrome: The triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology 44: 591–593.CrossRefGoogle ScholarPubMed
Susac, J. O., Hardimann, J. M., Selhorst, J. B. (1979) Microangiopathy of the brain and retina. Neurology 29: 313–316.Google Scholar