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 child(ren) genetically.
What are the different types of Usher syndrome?
There are three types of Usher syndrome:
• US type 1 (US1) - characteristics include:
o profoundly
deaf from birth
o do not
usually benefit from hearing aids
o severe
balance problems
o vision
problems begin by age 10
o blindness
eventually occurs
• US type 2 (US2) - characteristics include:
o moderate
to severe hearing problems
o usually
benefit from hearing aids
o use
speech to communicate
o normal
balance
o retinitis
pigmentosa begins in teenage years
• US type 3 (US3) - characteristics include:
o born
with normal hearing
o hearing
problems develop in teenage years
o near
normal balance
o deafness
by late adulthood
o retinitis
pigmentosa begins around puberty
o 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 will be determined by the physician(s) based
on:
• patient's age, overall health, and medical history
• extent of the disease
• expectations for the course of the disease
• patient's tolerance for specific medications, procedures,
or therapies
• patient's (or family's) 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
