Summary

Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder that causes your vision to get worse over time. It is the most common condition that affects both hearing and vision.

There are three types of Usher syndrome:

• People with type I are deaf from birth and have severe balance problems from a young age. Vision problems usually start by age 10 and lead to blindness.
• People with type II have moderate to severe hearing loss and normal balance. Vision problems start in the early teens and get worse more slowly than in type I.
• People with type III are born with normal hearing and near-normal balance but develop vision problems and then hearing loss.

There is no cure. Tools such as hearing aids or cochlear implants can help some people. Training such as Braille instruction, low-vision services, or auditory training can also help.

NIH: National Institute on Deafness and Other Communication Disorders

Learn More

• Retinitis Pigmentosa (Foundation Fighting Blindness)
• Usher Syndrome (Genetic and Rare Diseases Information Center) Also in Spanish
• Usher Syndrome (National Eye Institute)
• Usher Syndrome (National Institute on Deafness and Other Communication Disorders) Also in Spanish

Clinical Trials

• ClinicalTrials.gov: Usher Syndromes (National Institutes of Health)

Journal Articles References and abstracts from MEDLINE/PubMed (National Library of Medicine)

• Article: Outcomes of genetic testing for Usher syndrome in a diverse population...
• Article: Functional Vision Assessment Over 4 Years in USH2A Using the Veteran...
• Article: Detailed Clinical, Ophthalmic, and Genetic Characterization of MYO7A-Associated Usher Syndrome.
• Usher Syndrome -- see more articles

Patient Handouts

• Retinitis pigmentosa (Medical Encyclopedia) Also in Spanish