About Usher syndrome

What is Usher syndrome?

Usher syndrome is a rare genetic condition which causes hearing loss, progressive vision loss, and, in some cases, balance difficulties (vestibular dysfunction).Vision loss in Usher syndrome is caused by retinitis pigmentosa (RP), which affects the area at the back of the eye which senses light (the retina). The first symptom is usually difficulty seeing in the dark, followed by progressive loss of peripheral vision (reduced visual field). Usher syndrome is thought to affect between 4-17 individuals per 100,000 in the population. We estimate there are approximately 1,000 children and young people aged 0-25yrs with Usher syndrome living in the UK.

This video, produced in partnership with Retina UK, gives basic information about Usher syndrome. This may be helpful in providing wider family members or professionals supporting a child with Usher syndrome with an understanding of the condition. If you’d like more detailed information you can access the sections below (ears, eyes and balance) to find out more about the different aspects of Usher syndrome.

Types of Usher syndrome

There are 3 main clinical types of Usher syndrome, each associated with different genetic changes.

Type 1

Hearing Loss
From birth – severe to profound
Vision Loss
Usually affects individual by age 10 to 20
Balance difficulties
From birth

Type 2

Hearing Loss
From birth – moderate to severe
Vision Loss
Usually affects individual by age 20 to 30
Balance difficulties
Usually not affected

Type 3

Hearing Loss
Progressive – moderate to severe
Vision Loss
Variable
Balance difficulties
Variable

Ears

In this section we will give information about the structure and function of the ears, and how hearing is affected by Usher syndrome. We will also cover some of the options which exist for children and young people with hearing loss.

Find out more

eye

Eyes

In this section we will give information about the structure and function of the eyes, and how vision is affected by Usher syndrome.

Find out more

Balance

In this section we will give information about the ways in which balance can be affected by Usher syndrome, and strategies which can be helpful.

Find out more

Research

For an overview of current areas of research, check out this webinar with our trustee Professor Mariya Moosajee.

Usher Kids UK works in close partnership with the following organisations which focus on research into Usher syndrome and potential treatments.

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