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.

Usher Syndrome is a leading cause of acquired Deafblindness in the UK and worldwide, 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 persons balance.

The type of Deafness associated with Usher is sensori-neural 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 (nightblindness) 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. 

Type 1Type 2Type 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. 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.

Danielle Wise
Qualified Teacher of Multi-Sensory Impairments/ Deafblindness
Seashell Sensory Support Team

The Sensory Support Team at Seashell offer Usher Awareness Training to schools, colleges, universities and Local Authority Sensory Support Services.

We are able to carry out Section 7 Deafblind Guidance Assessments to ensure the needs of this very unique group are met within their local area.

We can also offer advisory support into colleges and further education settings.

Discover more of our MSI Support services available here
For more information please contact outreach@seashelltrust.org.uk