This page covers treatments for Usher syndrome types 1, 2 and 3, and for retinitis pigmentosa.
It also includes useful information for older people with Usher, and on specialist centres for genetic eye disease and dual sensory clinics.
On this page:
- Is there a cure for Usher syndrome?
- Treatments for type 1 Usher
- Treatments for types 2 and 3 Usher
- Older people and Usher
- Treatment for retinitis pigmentosa
- Usher syndrome and SEND
- Specialist centres for genetic eye disease
- Dual sensory clinics
Is there a cure for Usher syndrome?
No, there is presently no cure for Usher syndrome, but research continues into the genetic causes of the condition and potential new treatments.
Treatments for type 1 Usher
The standard treatment for Usher type 1, which is apparent from birth, is to receive cochlear implants in both ears within the first two years of life.
This makes it easier for children to develop speech and understand what people are saying without visual clues.
Because Usher syndrome, especially type 1, is being recognised earlier in children, cochlear implants are being fitted much earlier.
As a result, speech tends to develop normally in these children.
Treatments for types 2 and 3 Usher
Patients with Usher types 2 and 3 benefit from conventional hearing aids, although they may get cochlear implants later in life if their hearing deteriorates further.
Older people and Usher
For many older patients, whose hearing loss was not diagnosed in childhood, cochlear implants and hearing aids are not an option.
Instead, many of them use sign language to communicate.
For those with severe sight loss who can’t use conventional sign language, hands-on signing is an alternative.
Treatment for retinitis pigmentosa
Treatment for retinitis pigmentosa focuses on relieving symptoms and making the best of someone’s remaining sight. This includes:
- Regular monitoring of eye function and prescribing glasses, if needed.
- Referral to low vision services, which are often located in a hospital. Staff at the clinic can help you understand your condition and come to terms with your diagnosis.
- Using visual aids and a wide range of assistive technologies to improve quality of life.
- Having a healthy diet consisting of fresh fruit and green leafy vegetables.
- Using blue light screen protectors on mobile devices or computer screens (as a precaution)
- Wearing hats and sunglasses with UV protection, and placing sunlight diffusers at the back window of cars to ease light sensitivity.
- Regular check-ups to monitor for other eye conditions linked to retinitis pigmentosa, such as cataracts and macular oedema.
Usher syndrome and SEND
Children with Usher syndrome should be assessed for special educational needs and disabilities (SEND) support.
This will allow for changes to be made at home and extra help in early years and school settings so that children can fulfil their potential and achieve independence in life.
Specialist centres for genetic eye disease
There are various specialist centres for genetic eye disease throughout the UK.
These centres can offer more comprehensive management of conditions, including:
- Genetic testing and genetic counselling.
- Development assessment for children.
- Evaluation for taking part in research or clinical trials.
The Moorfields Eye Hospital NHS Foundation Trust in London has the largest genetic eye service in the UK and has dedicated clinics for all forms of eye disease.
How do I get a referral to a specialist centre?
You have two options:
- NHS referral.
- Private referral.
For an NHS referral, speak to your GP.
If you want to be seen as a private patient, you can book an appointment with a genetic eye specialist at one of the following:
- Moorfields Private Eye Hospital, London.
- Nuffield Health Oxford Hospital.
- Spire Southampton Hospital.
Some specialist centres offer virtual consultations and arrange genetic testing remotely by sending blood or saliva kits to your home.
Dual sensory clinics
Dual sensory clinics are being established to improve the overall clinical experience for children with Usher syndrome who are usually seen in separate eye and hearing clinics.
Dual sensory clinics offer faster and more accurate diagnosis through extensive genetic testing and detection of visual symptoms at an earlier stage.
Being able to see all relevant specialists in one clinic visit also reduces the stress of multiple appointments with different specialists.
One of the first to offer a dual sensory clinic for children and young people with Usher syndrome is Great Ormond Street Hospital for Children in London.
How do I get a referral to Great Ormond Street dual sensory clinic?
If your child is already a patient at Great Ormond Street Hospital, talk to your consultant to get an internal referral.
If your child isn’t a patient at Great Ormond Street Hospital, talk to your GP or local paediatrician.
The Sense Usher Service
With the right support, you can overcome the challenges presented by Usher syndrome, and live a full, connected and independent life.
This content was last reviewed in April 2022. We’ll review it again next year.