|Nerve and Retina of the Eye|
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- Deaf at birth and receives little or no benefit from hearing aids
- Severe balance problems
- Slow to sit without support
- Rarely learn to walk before age 18 months
- RP begins by age 10 with difficulty seeing at night, and quickly changes to blindness
- Born with moderate to severe hearing loss, and can benefit from hearing aids
- No balance problems
- RP begins in the teenage years
- Born with normal hearing that gets worse in the teenage years and leads to deafness by mid to late adulthood
- Born with near-normal balance
- RP begins in the early teenage years as difficulty seeing at night, and leads to blindness by mid-adulthood
- Visual field test to check side vision
- Test to check for ability to adapt to seeing in the dark
- Test to check sensitivity to color and contrast
- Hearing aids
- Assistive listening devices
- Cochlear implant—a small device surgically put under the skin behind the ear to give deaf people some ability to hear
- Adjustment and career counseling
- Training to help with balance and movement
- Low vision services
- Communications training
- Skills in living independently
National Eye Institute http://www.nei.nih.gov
National Institute on Deafness and Other Communication Disorders http://www.nidcd.nih.gov
Alberta Health http://www.health.gov.ab.ca
The Canadian National Institute for the Blind http://www.cnib.ca
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.
- Reviewer: Rimas Lukas, MD
- Review Date: 03/2014 -
- Update Date: 05/07/2014 -
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