Deafblindness is a unique disability affecting a wide range of people from babies to the older generation. It can be congenital (from birth) or acquired (occurs later in life) and varies in its severity and impact on the person’s access to communication, mobility and their environment.
What is Usher Syndrome?
Usher Syndrome is a leading cause of acquired Deafblindness across the world, with approximately 10,000 people with the condition in the UK alone (data from 2010). Advancements in genetic testing and these tests being more readily available are resulting in more babies and young children being diagnosed with Usher. It is a condition that causes varying degrees of Deafness from birth with sight loss occurring later in life, and often affects the young person’s balance.
The type of Deafness associated with Usher is sensorineural Deafness which is a problem with how the inner ear or auditory nerve works. Some people are fitted with cochlear implants or hearing aids to support this. Retinitis Pigmentosa is the visual impairment associated with Usher Syndrome. This is a progressive visual loss whereby the young person will first show signs of challenges in dim light or at night (night blindness) progressing to loss of peripheral vision.
The extent to which a young person with Usher Syndrome will lose their vision is very unique to each individual. Some may retain some degree of useful central vision, whilst others may go blind with no functional vision.
What are the different types of Usher Syndrome?
| Type 1 | Type 2 | Type 3 |
| Profound hearing loss or deafness at birth. | Moderate to severe hearing loss at birth. | Progressive hearing loss in childhood or early teens. |
| Decreased night vision by age 10, progressing to severe vision loss by midlife. | Decreased night vision by adolescence, progressing to severe vision loss by midlife. | Varies in severity and age of onset; night vision problems often begin in teens and progress to severe vision loss by midlife. |
| Balance problems from birth. | Normal balance. | Normal to near-normal balance in childhood; chance of later problems. |
Children and young adults are diagnosed with 1 of 3 types of Usher Syndrome, often identified through genetic testing. The severity of Deafness and severity and onset of vision loss is different with each type.
More recently a fourth type of Usher Syndrome is being diagnosed which is characterised by late onset vision loss (from midlife) and late onset Deafness (from late teens to midlife). This type is not thought to be linked to challenges with balance.
Living with Usher Syndrome
Even though a young person is diagnosed as having a certain type of Usher, each person will go on a completely different journey, experiencing sensory change and loss at different times, and processing and adapting to these changes in different ways. No two people with Usher Syndrome are the same.
Children with Usher Syndrome are often late to walking due to the challenges faced by their Deafness or by the Vestibular Dysfunction linked to the condition.
Living with a lifelong, changing, progressive condition is a rollercoaster of emotions for the young person and their families. They often say that just as they are accepting the condition, their vision changes and they are “right back to square one again”, having to relearn skills and find different ways of doing things. Challenges change as the young people move through their education and into adult life but with the right support in place, people with Usher Syndrome are capable of living successful, independent and fulfilling lives.
Here at Seashell, we do our bit to offer support for ordinary living for those with sensory related disabilities and even offer sensory training services to help people living with sensory impairments.
How Seashell can help
Seashell Trust is a UK-leading disability charity and specialist in supporting children and young people with complex difficulties, disabilities and additional communication needs. We have harnessed this unique expertise to create a range of specialist assessment, support and disability training services that we provide to families and organisations across the education, health and care sectors.
To find out more about what we do and who we are, don’t hesitate to get in touch on 0161 610 0100 or email us at info@seashelltrust.org.uk.
