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Usher Syndrome in Children

What is Usher syndrome?

Usher syndrome is a disorder that is passed down through families (inherited). A syndrome is a group of symptoms that happen together. Usher syndrome involves both hearing loss and vision loss. The hearing loss may be mild to complete. The vision problem is called retinitis pigmentosa. The retinas of the eyes are slowly damaged over time. The retinas receive light and play a very important part in being able to see. Usher syndrome is the most common childhood condition that affects both vision and hearing.

What causes Usher syndrome?

Usher syndrome is passed on from parents to their children. It may be inherited when both parents are carriers of an abnormal gene. If both parents have the gene, they have a 1 in 4 chance of having a child with Usher syndrome with each pregnancy.

What are the symptoms of Usher syndrome?

Symptoms depend on the type of Usher syndrome. There are several types and many sub-types of the syndrome. Symptoms of the syndrome may include:

  • Problems with hearing or deafness
  • Problems with balance
  • Problems with eyesight or blindness

The problems with eyesight are caused by an eye disorder called retinitis pigmentosa. This disorder includes a group of eye diseases that leads to gradual loss of eyesight.

How is Usher syndrome diagnosed?

All newborn babies are screened for hearing problems. If a hearing problem is found in the newborn, the baby will have follow-up testing. Your child's healthcare provider will test hearing, eyesight, and balance to diagnose Usher syndrome. The tests include:

  • Electronystagmography (ENG). This test checks eye movements to help diagnose balance problems.
  • Vision testing. Different tests are used to test the eyesight of very young children.
  • Eye exam. An eye doctor will look at the retina during an eye exam.
  • Electroretinography (ERG). This test checks how the retina reacts to light. It is used to diagnose retinitis pigmentosa.
  • Hearing evaluation. A hearing specialist will examine the ears and do a series of tests to check your child’s hearing.

One a child is diagnosed, he or she may have genetic testing to find out the type of Usher syndrome.

How is Usher syndrome treated?

Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is.

There is no known cure for Usher syndrome. Spotting it early is very important so that support and education can begin as soon as possible. Treatment may include:

  • Cochlear implants
  • Hearing aids
  • Hearing or auditory training
  • Low vision support
  • Speech, physical, and occupational therapy
  • Orientation and mobility training to help with balance
  • Counseling to help you and your child deal with a long-term health problem

How is Usher syndrome managed?

The hearing, vision, and balance problems may get worse over time. Your child will need ongoing care and support, as his or her needs change.

When should I call my child’s healthcare provider?

Call your child’s healthcare provider if you notice your child is having trouble with his or her eyesight, hearing, or balance.

Key points about Usher syndrome

  • Usher syndrome is an inherited problem with both hearing loss and vision loss.
  • Usher syndrome is passed on from parents to their children.
  • If both parents are carriers, they have a 1 in 4 chance of having a child with Usher syndrome with each pregnancy.
  • Hearing, vision, and balance tests are used to diagnose Usher syndrome.
  • There is no known cure for Usher syndrome.
  • Finding the syndrome early is important so that support and education programs can begin as soon as possible.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:
  • Before your visit, write down questions you want answered.
  • At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you for your child.
  • If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.
Usher Syndrome in Children - WellSpan Health

Online Medical Reviewer: Berry, Judith, PhD, APRN
Online Medical Reviewer: Holloway, Beth, RN, M.Ed.
Last Review Date: 2015-06-15T00:00:00
Last Modified Date: 2015-11-18T00:00:00
Published Date: 2015-11-18T00:00:00
Last Review Date: 2007-03-30T00:00:00
© 2016 WellSpan Health. All Rights Reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.

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