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:
  • 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 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