Susac's Syndrome

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Susac's Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Susac syndrome

Disorder Subdivisions

  • None

General Discussion

Susac's syndrome is a rare disorder characterized by impaired brain function (encephalopathy), blockage (occlusion) of the arteries that supply blood to the retina (branched retinal arterial occlusion [BRAO]), and hearing loss. Two main forms of Susac's syndrome have been identified. In one form, encephalopathy is the main finding; in the other form, BRAO and hearing loss occur without signs of brain disease. The specific symptoms and severity of Susac's syndrome vary from one person to another. The encephalopathic form of Susac's syndrome often improves spontaneously even without treatment (self-limited); the other form is frequently a chronic disorder. Although considered rare, Susac's syndrome is being recognized more often worldwide and its true frequency in the general population is unknown.

Susac's syndrome is considered an autoimmune endotheliopathy. An autoimmune disorder occurs when the body's own immune system mistakenly attacks healthy tissue. An endotheliopathy is any disorder that primarily involves the endothelium, which is the thin layer of cells that line the walls of blood vessels. In Susac's syndrome, the smallest blood vessels (microvasculature) of the circulatory system such as the capillaries, venules and arterioles are affected. Specifically, the microvasculature of the brain, retinas and inner ears are involved.

Resources

American Autoimmune Related Diseases Association, Inc.
22100 Gratiot Avenue
Eastpointe, MI 48021
Tel: (586)776-3900
Fax: (586)776-3903
Tel: (800)598-4668
Email: aarda@aarda.org
Internet: http://www.aarda.org/

Better Hearing Institute
515 King Street, Suite 420
Suite 420
Alexandria, VA 22314
United States
Tel: (703)684-3391
Fax: (703)684-6048
Tel: (800)327-9355
Email: mail@betterhearing.org
Internet: http://www.betterhearing.org

National Institute of Neurological Disorders and Stroke (NINDS)
P.O. Box 5801
Bethesda, MD 20824
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
TDD: (301)468-5981
Email: me20t@nih.gov
Internet: http://www.ninds.nih.gov/

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223
Email: ordr@od.nih.gov
Internet: http://rarediseases.info.nih.gov/Default.aspx

American Academy of Audiology
11730 Plaza America Drive, Suite 300
Reston, VA 20190
Tel: (703)790-8466
Fax: (703)790-8631
Tel: (800)222-2336
Email: infoaud@audiology.org
Internet: http://www.audiology.org

AutoImmunity Community
Tel: (919) 552-9057
Email: bandrews@autoimmunitycommunity.org
Internet: http://autoimmunitycommunity.org

Hearing Loss Association of America
7910 Woodmont Avenue
Suite 1200
Bethesda, MD 20814
Tel: (301)657-2248
Fax: (301)913-9413
Email: info@hearingloss.org
Internet: http://www.hearingloss.org

For a Complete Report

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

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