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.