Usher Syndrome

Usher Syndrome

Your IALVS eye doctor helps with vision loss from Retinitis Pigmentosa (RP)/Usher Syndrome

Usher syndrome is a rare genetic disorder that causes progressive deafness and vision loss and can also interfere with balance. Research has estimated that approximately 25,000 people in the United States have Usher syndrome. Across the world, this disease is the leading cause of combined blindness and deafness.

In general, the symptoms and development of Usher syndrome vary. Scientists have classified three different types of the disease based on:

  • Degree of hearing and vision loss
  • Age when vision loss occurs
  • How rapidly the disease progresses
  • Presence of balance problems

Vision loss & Usher syndrome

Loss of vision in people with Usher syndrome is caused by a gene mutation that leads to retinitis pigmentosa (RP), a degenerative retinal condition. The retina is the light-sensitive layer of tissue at the back of the eye, responsible for converting light into electrical signals for the brain to process as images. As RP deteriorates, the person first loses peripheral sight. Gradually, vision loss spreads to affect central vision too.

Who gets Usher syndrome?

This disease is an autosomal recessive disorder, which means that both parents must carry the mutated gene – but they do not have Usher syndrome. To inherit the condition, a child needs to receive a mutated gene from each parent.

What happens during an eye exam to check for vision loss from Usher syndrome?

The vision problems of Usher syndrome may remain unnoticed when children with the condition are very young, until the child complains or parents pick up on something. An eye exam will inspect the retina for the following signs: any changes in the blood vessels, clumps of dead retinal cells, a waxy appearance of the surface of the retina. Visual field testing will also be done to assess peripheral vision, and a specialized test called an electroretinogram (ERG) will be performed to confirm the diagnosis of retinitis pigmentosa.

How can low vision assistance from IALVS help?

A qualified IALVS eye doctor is knowledgeable about all the visual aids and strategies that can maximize your remaining vision with RP. We can help you to maintain your independence while doing the daily activities you enjoy doing, such as:

  • Reading
  • Watching TV or a movie
  • Walking safely around your home and other places
  • Driving
  • Recognizing the faces of your loved ones

We will meet with you to learn about your lifestyle and the tasks that you want to do. Your IALVS eye doctor is dedicated to making the most of your quality of life, and we’ll recommend the most effective low vision aids to achieve that goal.

Some low vision devices that can help you cope with vision loss from RP include:

  • Telescopic glasses to sharpen distance vision and widen your field of vision, making it possible to read a street sign or identify faces
  • Bioptic eyeglasses may make it possible for you to watch TV and see your computer
  • Hand-held and stand magnifiers with illumination can help with reading, writing, and using your smartphone screen
  • Side-vision awareness glasses may enable you to drive and watch sports games
  • Prismatic lenses can make it possible to read and do many up-close, hand-held tasks
  • Using a computer or smartphone

What is the treatment for Usher syndrome?

Many research studies are underway to discover a cure for Usher syndrome, but presently it has not yet been found. Treatment therefore focuses on helping people with this condition to adapt to their hearing and vision loss in order to enjoy the fullest life possible. Low vision services can significantly enhance the quality of life for people with Usher syndrome, helping to maximize remaining vision and channel it towards the individual’s preferred activities.

An extended study conducted by the National Eye Institute and the Foundation for Fighting Blindness showed that taking high doses of vitamin A palmitate can slow the development of vision loss due to RP. To find out if this vitamin therapy can help you or your child with Usher syndrome, consult with an IALVS eye doctor.

How can you benefit from visiting an IALVS eye doctor?

If you’re interested in maintaining your day-to-day independence and doing the tasks you want to do, such as reading, using a computer or smartphone, cooking, or taking a walk safely, an IALVS eye doctor can integrate the most effective low vision devices into your lifestyle!

After a personalized consultation, your IALVS eye doctor will recommend the best advanced low vision aids to optimize your vision with Usher syndrome.