Usher Syndrome and Vision Loss

The number of Americans with Usher syndrome is estimated at approximately 25,000. While there is no cure for it, there are low vision devices and strategies that can improve visual function.

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What is usher syndrome?

Usher syndrome is a rare genetic disorder that affects both hearing and vision. It causes an eye condition known as retinitis pigmentosa (RP). 

Retinitis pigmentosa occurs when the retina deteriorates over time, causing a gradual loss of vision. When a patient is diagnosed with retinitis pigmentosa, they become 'night blind,' meaning that they cannot see in dim light at first. Their visual field becomes progressively smaller over time, eventually leading to tunnel vision (similar to looking through a straw).

While there is no cure for this condition, there are ways that can help prevent progression, as well as make daily life easier for you.

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How can low vision devices enhance your quality of life?

Low-vision device may be helpful for you if you have Usher syndrome, particularly retinitis pigmentosa. You can maximize your remaining vision with the help of low vision aids and vision rehabilitation services. You can maintain your independence by using low vision assistive devices prescribed by our low vision optometrists so that you can read, write, navigate maps, watch television, walk safely around your home, drive and recognize the faces of your loved ones. 

Following are a number of low vision devices you can use to cope with vision loss from retinitis pigmentosa, including:

  • The use of bioptic eyeglasses could enable you to view TV and computer screens from a distance. Additionally, you can use these eyeglasses to drive and see traffic signs from a distance
  • Reading, writing and using your smartphone screen is easier with hand-held and stand magnifiers with illumination
  • By wearing prisms, people with side vision loss (loss of peripheral vision) can perform daily activities like driving more safely.
  • With prismatic lenses, it's possible to read close-up and do many other hand-held tasks such as using a computer or a phone.
  • Tinted lenses can be used to reduce glare, improve contrast, and improve visual function.
  • There are also a variety of digital devices that can improve visual function
What are the different signs and symptoms of Usher syndrome?

What are the different signs and symptoms of Usher syndrome?

There are several types and subtypes of this syndrome. The symptoms of Usher syndrome vary based on its type. The syndrome may cause the following symptoms:

  • Hearing problems or deafness
  • Problems with balance
  • A progressive loss of vision that can result in blindness
  • Night vision loss that begins in teens and progresses into middle age
  • Tunnel vision (loss of peripheral vision)
What are the different signs and symptoms of Usher syndrome?

What are the different signs and symptoms of Usher syndrome?

Type I

Children born with type I of Usher syndrome begin to lose their vision as a result of Retinitis Pigmentosa (RP). It appears during pre-adolescence (around age 10).

Type II

In this type, vision loss typically occurs later (in teens and young adults) and progresses more slowly than in Type I.

Type III

Children born with Type III usually begin to show symptoms during their teenage years. Vision loss varies in severity from individual to individual.

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How does an eye doctor diagnose Usher syndrome?

How does an eye doctor diagnose Usher syndrome?

A person with this condition is affected from birth, but they are diagnosed during their childhood or teenage years. An optometrist uses various imaging tests during a vision exam to diagnose Usher syndrome. The most commonly used are described below:

  • Using an electroretinogram (ERG), a retinal response to light can be measured, which is commonly used to diagnose Usher syndrome.
  • The retina is examined along with other structures in the back of the eye during a retinal exam.
  • Visual field testing is done to measure the peripheral vision of the child.
  • Optical coherence tomography (OCT) provides detailed images of the layers of the optic nerve and retina, allowing our eye doctor to diagnose disease.

How is Usher syndrome treated?

There is currently no cure for Usher syndrome or for retinitis pigmentosa (RP). Usher syndrome is treated based on the specific symptoms that each patient exhibits. 

The treatment may involve the coordination of efforts of a number of medical professionals, including pediatricians, internists, otolaryngologists, audiologists, optometrists, specifically low vision optometrists among others.

Vitamin A

Vitamin A has been found to slow the progression of certain forms of retinitis pigmentosa, but high intake of vitamin A may lead to worsening of other eye conditions. You can discuss the risks and benefits of Vitamin A with our eye doctor and learn how much Vitamin A you should take safely. A high intake of vitamin A can be harmful, and vitamin A is not proven to affect retinitis pigmentosa (RP) progression significantly. Consequently, vitamin A supplements are not recommended at the present time.

Gene therapy

Many forms of RP and Usher syndrome are being subjected to gene therapy trials. FDA approved the first gene therapy for RP (linked to the RPE65 gene) in 2017. During this treatment, a normal copy of the disease-causing gene is implanted inside the retina. As a result of the normal gene, the retina works more efficiently.

What are the different signs and symptoms of Usher syndrome?
What are the different signs and symptoms of Usher syndrome?

What can you do to improve your child's academic performance with Usher syndrome?

Here are some suggestions and strategies that may help patients succeed academically:

  • Make sure your home has adequate lighting
  • Get rid of sources of glare. Glare can be reduced with tints prescribed by a low vision optometrist.
  • Increase the width of the text cursor, which is usually done at the operating system level.
  • Ensure your monitor's brightness and color are set to provide the best contrast, since bright light can interfere with reading for some people with low vision.
  • Increasing font size and/or enlarging software may help some people to properly read letters.
  • Use non glare papers
  • Use closed-circuit television (CCTV) to read difficult texts
  • Teach them to use dark ink when writing on paper (never red)
  • It can be difficult to read if the computer screen has a light background. Changing the background to a darker color could help.
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Common Questions

Usher syndrome is a genetic condition. Therefore, the gene change must be passed down from both parents. Those who inherit the changed gene from one parent do not have the syndrome, but are carriers. Children born to two Usher syndrome carriers have a one-in-four chance of developing the syndrome.
Usher syndrome affects about 4 to 17 out of 100,000 babies, and it is the underlying cause of deafness in 3% to 6% of all deaf children. A gene that causes this disease must be carried by both parents in order for you to inherit it.
Usher Syndrome and Vision Loss
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If you wish to maintain your independence every day and do the tasks you wish, such as reading, using a computer, or your smartphone, cooking, or walking safely, your low vision optometrist can help you find the best low vision device for your needs. After a low vision exam, they will prescribe the best advanced low vision aids to maximize your vision.

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