Supporting students with Usher syndrome

Usher syndrome is a degenerative condition that combines deafness with a visual impairment called Retinitis Pigmentosa (RP). RP is an eye disease of the retina that affects peripheral vision and causes night blindness in most cases. There are 3 types of Usher: 

• Usher Type 1 – Children are born profoundly/severely deaf, develop RP and have problems with their balance.  
• Usher Type 2 – Children are born severely/moderately deaf, develop RP and have no problems with their balance.  
• Usher Type 3 – Children are born hearing or with moderate hearing loss, develop RP and in 50 per cent of cases will also have poor balance.

As with most syndromes the age of which children or young adults develop symptoms will vary, as will the severity and speed of which onset may occur. Many people with Usher may also have extra eye problems such as cataracts.  Having Usher can present many challenges as the child's eyesight and hearing changes. The deterioration in their vision or hearing can affect them in different ways in different environments and on different days. For example, if a child is tired, in a new environment, with a supply teacher, all of this could affect the child’s ability to communicate, access information and move around a space safely.

Children and young people will need access to specialist support and adaptations to their learning if they are to make the most of the opportunities their education gives them. There is specific guidance in planning for and supporting students in the Special Educational Needs and Disability (SEND) code of practice that teaching professionals should be familiar with. It is important to remember that one size does not fit all and the most successful education placements are flexible in accommodating the young person's needs and preferences.

Young people with Usher may not meet the threshold for an Education Health and Care plan, but may still have complicated and multiple needs. Local offer should specify local support services and how to access them.  Students may require 1:1 support in lessons, this may include an interpreter, intervenor, communicator-guide or note takers, while others have found buddy/guide dogs helpful.

Students may also require advisory teaching support in settings, e.g. Hearing Impairment, Vision Impairment and Multisensory Impairment teachers. Students with Usher may also benefit from peer to peer mentoring as well as buddying services. It’s important to remember that support needs are likely to change over the course of the students’ school career. If you notice a child or young person with Usher slowing down near stairs, posts or obstacles, if they are having difficulty focusing or not responding to questions, it may be that they are experiencing subtle changes in what they can see or hear. You should advise parents or carers to seek professional advice, a re-assessment or a visit from a MSI specialist.

1. Never assume what is most suitable for a child with Usher – always ask them.  For example a young adult with Usher could struggle with exams – automatically offering extra time might not be the answer. It could be font sizes, colour contrast, even getting to the exam room could be a problem.
   
2. The more information a child receives or understands about their Usher, the more in control they will feel.
   
3. If a child looks like they are always in a ‘day dream’ in a class, it may be the child is finding it difficult to lip-read a teacher – maybe they are sitting too far away? They might be next to a window, or a bright light behind the teacher? Maybe there is not enough light, or very uneven lighting? It could even be the wrong clothes, like a white shirt the same as a white wall? Talk to a child privately about these possibilities.
   
4. If a child wants to talk about their Usher, encourage them to talk openly and just listen. They are probably not looking for solutions or answers.
   
5. In some cases families do not want a child to know about their condition. Try to encourage the family to be open with a child.
   
6. If a child wants to talk about their Usher to promote awareness amongst peers they may need some help from professionals but it can prove very successful.
   
7. During PE lessons, if a child cannot see the ball, do not stop a child playing or trying different sport such as netball or volleyball. Try using a bright coloured ball or tops. Good colour contrast will always help, but remember some Usher types have problems with their balance so this will affect some sports.
   
8. Children with Usher can achieve going onto further education, university, travelling, employment, having a family just like their peers. It is important that school believes this as well as the child.
   
9. If a child wants to meet another person with Usher, organisations such as Sense can help arrange this. They could join a support group or Facebook groups.
   
10. Every child with Usher is individual – no two children, not even siblings are the same.

Many professionals will only come across children who are deafblind very occasionally and may be uncertain how best to support their individual needs. Sense offers advice and professional support via its Children’s Specialist Services team and Usher Service team. Contact [email protected] or [email protected] for more information.

About the authors

Steve Rose, Head of Children’s Specialist Services

Steve Rose is a Health and Care Professions Council registered speech and language therapist specialising in working with people with deafblindness.   He has worked as a Specialist Speech and Language therapist in special schools in North London with children with physical disabilities, sensory impairments, autistic spectrum disorders and learning difficulties. In 2011, his dissertation focused on differences between deafblindness and Autism, as part of his MEd in deafblindness at Birmingham University. Currently, he is the lead professional advisor for deafblind children and young people and Head of Children’s Specialist Services at Sense.

Emma Boswell, National Usher Co-ordinator

Emma is National Usher Co-ordinator at deafblind charity Sense. Emma has Usher type 1 and is a bilateral cochlear implant user which allows her to bring a unique personal perspective to the role. Emma is Chair of the International Usher Network and has delivered various plenary presentations both in the UK and Internationally. In 2012, she gained her postgraduate Deafblind course and in her spare time she is a voluntary Chair of the Deaf Cancer Support Group.