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Usher Syndrome
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Genetics Birth Defects

Usher Syndrome

Usher syndrome is an inherited condition that affects both hearing and vision, representing the most common disorder to impact both senses simultaneously.

Overview

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.

Types of Usher Syndrome

There are three types of Usher syndrome:

  • Type I: People are deaf from birth and have severe balance problems from a young age. Vision problems usually start by age 10 and lead to blindness.
  • Type II: People 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.
  • Type III: People are born with normal hearing and near-normal balance but develop vision problems and then hearing loss.

Management

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.

Frequently Asked Questions

Early signs include hearing loss or delays in speech development, and sometimes balance problems. If you suspect hearing issues, a pediatric audiologist can perform hearing tests, and an ophthalmologist can assess for early retinal changes.
Yes, it is an autosomal recessive disorder. Genetic counseling and testing can determine carrier status in parents and siblings, and help assess risk for future children.
Support groups, low‑vision rehabilitation, Braille instruction, and occupational therapy can help maintain independence. Many organizations, such as the Usher Syndrome Association, offer educational materials and community connections.===
Hearing aids, cochlear implants, and auditory training programs can improve hearing. The choice depends on the type and severity of hearing loss, and a multidisciplinary team can guide the best approach.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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