Usher Syndrome is a genetic disorder. People who have Usher Syndrome are usually born deaf or hard of hearing, then gradually lose their sight due to Retinitis Pigmentosa (RP).

RP is an eye condition which is generally diagnosed when the sufferer notices problems with night vision. As RP progresses, the field of vision narrows and "tunnel vision" develops.

Other symptoms include difficulty adjusting to a change in lighting conditions and problems seeing when there is low contrast (e.g. concrete or carpeted stairs with no edge markings can be difficult to distinguish).

Usher Types

There are three known types of Usher Syndrome which are summarised below:

Type 1

Born with a profound hearing loss
Balance problems
Vision starts to deteriorate due to RP at around 8 - 12 years old, or in some cases even earlier
Many people with Type 1 Usher use sign language

Type 2

Born with moderate to severe hearing loss
Vision starts to deteriorate due to RP at around 20 - 25 years old or sometimes as early as the teens
No balance problems in childhood
Many people with Type 2 Usher use speech and lip-reading to communicate

Type 3

Hearing loss develops and worsens over time
Vision starts to deteriorate due to RP at around 20 - 30 years old. (The hearing loss often develops or deteriorates at about the same time)
May have balance problems in later life
Generally communicate using speech and lip-reading
Type 3 Usher is very rare

How you can help

There are a number of things you can do to help people with Usher Syndrome make the most of their remaining sight and hearing.

Try and pick a well-lit area in which to communicate and make sure the light is shining on YOU

Avoid patterned or bright clothing, wear dark plain clothes and try to position yourself in front of a plain contrasting background.

Stay in one place, don't move around.

If you are signing to a person with tunnel vision, ask them about the extent of their field of vision and confine your signs to this space. Fingerspelling can sometimes be particularly difficult to see.

Always ask the person with Usher Syndrome what the best distance for their vision is and what lighting conditions they need. Seat them accordingly.

Keep the floor clear of bags, bins and boxes, and be prepared to warn the person of low furniture, obstacles or unexpected steps.

Sign language users will find it difficult to watch an interpreter AND follow information displayed on an overhead projector, blackboard or TV. Remember to provide appropriate printed material before meetings and try to use a blackboard rather than a whiteboard, as the shiny surface can be distracting.

Use a loop system in a quiet room for people who have a 'T' setting on their hearing aid. Remember to speak clearly without shouting.

More Information

If you have any questions, please e-mail our enquiry service at This e-mail address is being protected from spambots. You need JavaScript enabled to view it , or contact us at:

RAD Head Office
Walsingham Road
Colchester
CO2 7BP
Tel: 01206 509509
Text: 01206 577090
Fax: 01206 769755
Videophone: 01206 710064
E-mail: This e-mail address is being protected from spambots. You need JavaScript enabled to view it

Alternatively, you may find the following organisations helpful:

Hearing and Sight Impaired (HIS)
(Incorporating UHAU - the Usher Hearing Aid Users Group). HIS is a group of people who are mainly but not exclusively Usher types 2 or 3, who tend to use speech and hearing aids rather than sign.

Usher Resources provide a wide-range of information on Usher syndrome - from the effects of Usher syndrome through to communication, mobility, genetics and mental health.

For all the above, contact:

Sense
Usher Resources
11-13 Clifton Terrace
Finsbury Park
London N4 3SR
Tel: 020 7272 7774
Text: 020 7272 9648
Fax: 020 7272 6012
E-mail: This e-mail address is being protected from spambots. You need JavaScript enabled to view it
Web: www.sense.org.uk

UsherLife is a truly dedicated website for people who have Usher syndrome. The name UsherLife has a double meaning ­ we have an Usher life and the website fulfils life in the Usher community. UsherLife also facilitates an informal group for people to meet online or outside.

Deafblind UK offer support and information to people with Usher Syndrome.

Contact them at:

Deafblind UK Head Office
National Centre for Deafblindness
John and Lucille van Geest Place
Cygnet Road
Hampton
Peterborough
Cambridgeshire, PE7 8FD.
Telephone: 01733-358100
Fax: 01733-358356

This guide was prepared by Linda Isaac - Head of Information, with the kind assistance of Marylin Kilsby - Usher Resources, and Roy Staines.