What is Usher syndrome?
Usher syndrome is a genetic condition which affects vision, hearing and, in some cases, balance. It is broadly split into three types.
Although Usher syndrome brings challenges, people with Usher lead fulfilling and independent lives. People with Usher can and do have relationships, get married, have children, go to university, travel, go to work and enjoy numerous leisure pursuits.
The time of onset as well as the degree to which vision, hearing and balance is affected varies between individuals. However, the main mechanism behind the impairments remains similar.
Vision loss caused by Usher syndrome is due to a condition called retinitis pigmentosa. The hearing loss is due to a condition called sensorineural deafness. Additionally, many people with Usher experience problems with balance.
How common is Usher syndrome? (Prevalence)
The prevalence of specific Usher types varies geographically. However, overall Usher syndrome, in developed countries, is believed to have a prevalence rate from 3.2 to 6.2 per 100,000 people.
However, this rate is believe to be much higher, particularly cases of Usher type 2 and 3, due to undiagnosed cases or misdiagnosis of Usher syndrome as another condition. A study from 2010 suggested a prevalence rate of 15 per 100,000 people which would mean that there are approximately 9,750 people in the UK with Usher syndrome.
It is believed that Usher syndrome is the underlying cause for :
3% to 6% of the congenitally deaf population
18% of the population with retinitis pigmentosa
About our Usher syndrome information
The information created in this section has been created as a result of feedback from people with Usher and is intended for that audience. It is hopefully also useful to anyone else, such as family members, with an interest. All of the pages can be navigated using the pane on the left hand side of the page.
As well as this Usher-specific information, the Sense website has lots of additional information which can be useful to those with Usher, such as:
- The law and social care
- Sense's Children's Specialist Services
- Your rights to support in education
- Welfare benefits
- Disability discrimination
- Clinical assessment of hearing, vision and balance
- Assistive technology
The Sense Usher Service specialises in the field of Usher syndrome and supports people with Usher, their families and professionals. Support covers advice, assessments, training, mentoring and professional support. Contact firstname.lastname@example.org or email@example.com for more information. You can also follow the Usher Service on Facebook.
Research on well-being and quality of life for people with Usher syndrome
In 2016, Sense supported research to determine whether psychosocial well-being is associated with the health-related quality of life of people with Usher syndrome. The study found that loneliness is predictive of poor mental health related quality of life. Social support is also predictive of better mental health and loneliness correlates with higher depressive symptoms in people who are deafblind. The full findings of the research can be read online.
The Usher Peer Support Project is for people, aged 18 and older, who have Usher syndrome, or for adult family members of those with Usher Syndrome across England, Wales and Northern Ireland. We are looking for people who will use their experience of living with Usher syndrome to give support to other individuals to enable them to make changes in their lives.
We would also like to hear from anyone who is looking for support from those who know what it’s like to live with Usher syndrome.
If you are interested in finding out more about the Usher Peer Support Project, please contact Ann Copson in the Sense Usher Service Team.
Created: February 2016
Review due: February 2018
First published: Friday 8 June 2012
Updated: Friday 15 September 2017