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Usher Syndrome

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by Rosenblum L
 

Definition

Usher syndrome is a rare, inherited disorder that involves loss of hearing and sight. Hearing loss is due to the inability of the auditory nerves to send sensory input to the brain. This is called sensorineural hearing loss.
The vision loss, called retinitis pigmentosa (RP), usually happens after age ten. RP is a deterioration of the retina. The retina is a layer of light-sensitive tissue that lines the back of the eye. It changes visual images into nerve impulses in the brain that allow us to see. RP slowly gets worse over time.
Nerve and Retina of the Eye
Normal Anatomy of the Eye
Copyright © Nucleus Medical Media, Inc.
Three types of Usher syndrome have been identified: types I, II, and III. The age of onset and severity of symptoms separate the different types.
 

RESOURCES

National Eye Institute
http://www.nei.nih.gov

National Institute on Deafness and Other Communication Disorders
http://www.nidcd.nih.gov

 

CANADIAN RESOURCES

Alberta Health
http://www.health.gov.ab.ca

The Canadian National Institute for the Blind
http://www.cnib.ca

 

References


Dammeyer J. Children with Usher syndrome: Mental and behavioral disorders. Behav Brain Funct. 2012; 8:16.


Usher syndrome. Boys Town National Research Hospital website. Available at: http://www.boystownhospital.org/research/molecularstudies/pages/ushersyndrome.aspx. Accessed May 20, 2013.


Usher syndrome. National Institute on Deafness and Other Communication Disorders website. Available at: www.nidcd.nih.gov/health/hearing/pages/usher.aspx. Updated February 2008. Accessed May 20, 2013.


What is Usher syndrome? Foundation Fighting Blindness website. Available at: http://www.blindness.org/index.php?option=com%5Fcontent&view=article&id=56&Itemid=81. Accessed May 20, 2013.

 

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