Usher Syndrome

What is Usher syndrome?

Usher syndrome is an inherited disorder that involves both a hearing impairment and a vision impairment called retinitis pigmentosa. Some people also have varying problems with balance.

Usher syndrome is passed from parents to their children genetically.

What are the different types of Usher syndrome?

There are three types of Usher syndrome:

US type 1 (US1) - characteristics include:
- profoundly deaf from birth
- do not usually benefit from hearing aids
- severe balance problems
- vision problems begin by age 10
- blindness eventually occurs
US type 2 (US2) - characteristics include:
- moderate to severe hearing problems
- usually benefit from hearing aids
- use speech to communicate
- normal balance
- retinitis pigmentosa begins in teenage years
US type 3 (US3) - characteristics include:
- born with normal hearing
- hearing problems develop in teenage years
- near normal balance
- deafness by late adulthood
- retinitis pigmentosa begins around puberty
- blindness by mid-adulthood

How is Usher syndrome diagnosed?

Special tests assist in the diagnosis of Usher syndrome, including:

electronystagmography (ENG) to detect balance problems
electroretinography (ERG) to detect retinitis pigmentosa

Treatment for Usher syndrome:

Specific treatment for Usher syndrome will be determined by your physician based on:

your age, overall health, and medical history
extent of the disease
your tolerance for specific medications, procedures, or therapies
expectations for the course of the disease
your opinion or preference

At present, there is no known cure for Usher syndrome. The best treatment, however, is early identification so that education programs can begin as soon as possible. Treatment may include:

adjustment counseling
career counseling
assistive devices, such as hearing aids
orientation and mobility training
communication services
independent living training
low vision services
auditory training

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Online Resources of Otolaryngology

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