Living with Usher syndrome

This page covers practical steps you can take to live with and manage Usher syndrome to live as fully and independently as possible. 

Topics covered include talking to your GP, registering your sight and heaaring loss, eye clinic liaison officers, local authority support, state and other benefits, and looking after your physical and mental health. 

On this page:

I’ve had a diagnosis of Usher syndrome: what do I do? 

Immediately after diagnosis, you may feel a range of emotions, including shock, disbelief, fear, anxiety, guilt, anger and more.  

No two people react in the same way and there’s no right or wrong way to feel.  

Living with Usher syndrome can be challenging. You’ll have good days and bad days. 

At present, there’s no cure for Usher syndrome, but there are some treatment options.

There are also a number of important steps you can take to make living with your condition easier and lead as independent and fulfilling a life as possible. 

Talk to your GP

First, talk to your GP. They’re responsible for referring you to any specialists you might need to see, including ophthalmologists (eye specialists) and audiologists (hearing specialists). 

You’ll need a referral to an ophthalmologist or audiologist if you want to register your sensory loss.

GPs can also refer you to counsellors, therapists, psychologists and psychiatrists. 

It’s important that you can communicate easily with your GP.  

Under the Equality Act 2010and the Accessible Information Standard, GPs must provide the communication support you need. 

Register your sensory loss 

Register your sight loss  

If your sight deteriorates to a certain level, you can choose to register with the NHS as visually impaired. Speak to your GP for a referral to an ophthalmologist. 

Depending on the severity of your vision loss, you’ll either be registered as sight impaired (previously ”partially sighted”) or severely sight impaired (previously “blind”). 

The ophthalmologist will decide whether you’re eligible to be certified as sight impaired or severely sight impaired.  

If you are, they will complete an official certificate with the results of your eye examination. 

In England and Wales this certificate is called the Certificate of Vision Impairment (CVI). 

In Scotland, it’s called the CVI (Scotland) form, and in Northern Ireland it’s called an A655. 

Your ophthalmologist will send a copy of the certificate to you, one to your GP and one to your local social services department.  

Your local social services team will then contact you to ask whether you want to be added to its register of visually impaired people.  

Once you’re registered, social services will contact you again to assess what help you need to remain independent, such as help with cleaning and cooking, or help with mobility and transport. 

Register your hearing loss

Speak to your GP for a referral to an audiologist.  

An audiologist at your hospital will issue an audiogram if they decide your hearing loss is significant. 

They will send a copy to your local social services who will contact you to discuss whether you want to be on their register. 

The register is voluntary and confidential. 

Benefits of registering sensory loss

Registering sensory loss isn’t compulsory, but, depending on your needs, it can help you to get a range of benefits, including: 

  • Benefits to help with any costs relating to your disability or illness. 
  • A reduction in the TV licence fee. 
  • Help with NHS costs. 
  • Help with Council Tax and tax allowances. 
  • Leisure discounts. 
  • Reduced fees on public transport. 
  • Parking concessions. 

Eye clinic liaison officers

If a referral to an eye clinic liaison officer (ECLO) isn’t offered, you can ask to be referred to one if you feel that you need additional assistance due to your sight loss. 

ECLOs are based in eye departments supporting people with sight loss or impeding sight loss and their families and/or carers.   

ECLOs provide practical and emotional support to help you understand your diagnosis, cope with the challenges of sight loss presents and maintain your independence.   

ECLOs form a bridge between the eye clinic and social care and community services, ensuring people with sight loss get the services and benefits they need.   

ECLOs usually accept referrals from ophthalmologists and low-vision clinics.  

Get support from your local authority 

If you have a combined sight and hearing impairment, you are entitled to an assessment under the Department of Health’s deafblind guidance

This assessment may help you to get some or all of the following: 

Get the benefits you’re entitled to 

People with disabilities are entitled to a range of benefits.  

Visit gov.uk to find out more about financial help if you’re disabled.

Look after your physical health

A healthy body will make it easier to cope with stress and changes to your condition. 

Eat well, sleep well and exercise 

It helps to eat a healthy, balanced diet and have good routines for sleeping and relaxing. 

Exercise if you can. Not only is it good for fitness and maintaining a healthy weight, but it also helps to improve sleep and boosts feel-good brain chemicals such as serotonin and endorphins. 

Being overweight does not directly affect any particular eye or hearing condition, but it does put you at risk of other health problems, such as high blood pressure, Type 2 diabetes, heart disease and some cancers. 

These problems could lead to eye conditions such as glaucoma and cataracts, which affect your sight. 

Annual eye tests 

Regular eye check-ups are important to monitor any changes in your sight. They will also help to detect any further complications, such as cataracts. 

Eye safety in the sun 

UVA and UVB rays can harm your eyes and increase the risk of cataracts and age-related macular degeneration.  

Wearing sunglasses or glasses with a built-in UV filter will protect your eyes.  

You should only use sunglasses that have a CE mark or carry British Standard BSEN 1836:1997. 

Stop smoking 

Smoking can damage the eye tissue and should be avoided. It may not contribute to retinitis pigmentosa, but it doubles the likelihood of developing age-related macular degeneration, putting any remaining sight at serious risk. 

Vitamin A and fish oil supplements 

There are claims that vitamin A supplements and/or fish oil supplements can slow the progression of retinitis pigmentosa.  

These claims stem from a single trial in the USA in 1993, but unfortunately nobody has been able to prove this conclusively in human clinical trials.

While vitamin A and oily fish have health benefits in the natural quantities found in some foods, supplements can be dangerous for people under 18, pregnant women and women considering pregnancy.  

We recommend that you first discuss the use of either supplement with your GP. 

Vitamin E supplements 

Some research suggests that the use of vitamin E supplements may actually speed up sight loss in people with retinitis pigmentosa.  

We strongly recommend that you do not take vitamin E supplements. 

Look after your mental health

A healthy, balanced diet, good routines for sleeping, relaxing and exercising are just as important for looking after your mind as your body. 

But the changes and adjustments in your life from sight and hearing loss can be a serious challenge to your mental wellbeing. 

“With my deterioration in vision, I feel like I have just been diagnosed all over again. The feelings are the same. I can’t do now what I could a month or two ago.”

Person with Usher syndrome

You may feel like withdrawing from others, but isolation can make things worse.  

If you’ve retreated from relationships that were once important to you, make the effort to reconnect. 

Support from family, friends or professionals can help you to remain positive and move forward. 

Talking to someone who has received the same diagnosis can help you to deal with any new emotions you are feeling. 

If you do feel you need professional support, speak first to your GP about referral to a counsellor, therapist, psychologist or psychiatrist.  

Keep in mind that there is often a waiting list for NHS services. How long you might have to wait depends on how urgent your need is and what’s available in your area. 

If you can afford private mental healthcare, you can also ask your GP to advise you. 

Share your experience 

It can be helpful to share stories, achievements, ideas, experiences and strategies for coping with Usher with other people who also have the condition. 

A problem you’re experiencing has probably been faced and solved before, and solutions you’ve found could be helpful to others. 

For some people, raising awareness of Usher is a way of changing the helplessness they feel. 

Top tips for people who know you have Usher syndrome 

If you have family members, friends or colleagues who would like some tips about what to do and what not to do around you, you can direct them to this handy lists of Dos and Don’ts. 

If you know someone with Usher syndrome… 

Please do: 

  • Feel free to ask if the person needs help. 
  • Tap on or gently hold the top of their arm and remain where you are standing or sitting when you want to get their attention. 
  • Allow them to decide the best distance for them to follow signing, lipreading or fingerspelling. About 4–5ft is a reasonable distance. 
  • Say your name when you start talking with the person, and when the conversation is finished, tell them that you are leaving. 
  • If somebody else wants to join the conversation, or speak to them, tell the person with Usher that person’s name and where they are. 
  • To have conversation in a well-lit area, keep the direction of the light at the side or behind the person with Usher. 
  • Keep your fingerspelling and signs to chest level.
  • If you refer to someone or something, let the person with Usher know who it is or what it is, and where the person or thing is located. For example, ‘the bar is behind you’, ‘Alex is sitting on your left side’. 
  • Be ready to warn the person of any low furniture, obstacles or unexpected steps in their path 
  • Gently offer your arm to help guide them in the dark. Try saying something like, “Need help?” 

Please don’t: 

  • Be afraid to ask if the person needs help. 
  • Wave at the person from the side when you are trying to get their attention. 
  • Point at someone else who is trying to get the person with Usher’s attention or join in the conversation. 
  • Point vaguely in the general direction of someone or something you’re talking about. 
  • Talk with the light coming from directly behind you, whatever the source, e.g. sunlight, bright doorway, lamplight, etc. 
  • Try to talk with the person in a poorly lit room. 
  • Stand too close to the person when finger spelling, signing or speaking.
  • Use large, wide movements when using sign language.
  • Assume the person with Usher sees low furniture, obstacles or unexpected steps. 
  • Grab the person’s arm when they need help in the dark. 

Become a Sense Member

Find out here about the benefits of becoming a Sense member, including: 

  • Feeling connected to more people with complex disabilities. 
  • Our membership magazine Talking Sense. 
  • Regular emails with updates from across the Sense community.

Sense support 

The Sense Usher Service provides support to people with Usher syndrome.

You can get in touch with use directly at: [email protected]

To Skype the Sense Usher Service, contact us for an appointment, Monday to Thursday, 10am to 3pm, letting us know if you use sign language. 

Visit the Sense Usher Service group on Facebook, and find us on Twitter: @SenseUsher.

More help from Sense to live fully and independently 

You’ll also find much more information on the following pages about the many ways of communicating for people with complex disabilities, and about hearing, sight and assistive technologies: 

Need help finding the right support?

Sense is here for everyone who is deafblind and everyone with complex disabilities. Get in touch to find out how we can support you.

This content was last reviewed in April 2022. We’ll review it again next year.