According to the most recent research dated 2010 there are/were only 9750 people in the UK living with Usher Syndrome and according to the Government Digital Service, as of October 2017 there were 11 million deaf or hard of hearing people in the UK of which 151,000 use BSL (British Sign Language) to communicate (1.37%). There will be a smaller percentage using tactile signing. The vast majority are oral.
According to this research only 0.09% of the 11 million deaf or hard of hearing have Usher Syndrome. People living with Usher Syndrome/deafblind have considerably more challenges than those deaf alone. Those with Usher Syndrome most born deaf then acquire progressive blindness (Retinitus Pigmentosa). The onset of blindness affects communication.
Visual strategies adopted by the deaf include lipreading, facial gesture and body language. These strategies become very challenged or impossible from the onset of the progressive blindness that forms a part of Usher Syndrome. Currently the NHS accepts that those with a dual sensory impairment should have 2 pairs of hearing aids and if appropriate 2 pairs of glasses, sadly in some areas this is denied, due to funding! In comparison deaf people are provided with 1 pair of hearing aids.
Having made this differentiation it is accepted that dual sensory impairment requires different measure. Of the 9750 people living with Usher Syndrome some will chose to use no hearing aids, others have cochlear implants so therefore hearing aid provision is not appropriate. The actual number requiring hearing aids is unclear but would be around two thirds of those with the condition. Those with Usher Syndrome and profoundly deaf benefit hugely from bilateral cochlear implants. They enable not just best hearing experience for but very importantly location of sound, imperative with progressive sight loss /blindness. Location of sound enables access to safety and inclusion in every day life.
Those with Usher Syndrome with deafness varying between mild, moderate, severe and profound who wear hearing aids also need access to sound with clarity, locational sounds to enable access to safety and inclusion in every day life. Hearing aid technology that enables this is available but not provided by the NHS. Today’s smart hearing aids enable those with Usher Syndrome/deafblind to direct stream sound. To experience clarity of sound, to locate sound. As a result enable access to a plethora of assistive technology aurally. This often enables those living with Usher Syndrome to continue living their lives as sight loss progresses.
The Molly Watt Trust champions the possibilities assistive technologies and digital accessibility can bring for a more inclusive world for those living with Usher Syndrome. Our previous projects have resulted in the funding of various items of assistive technology to enable those living with deafblindness. In our biggest and most impactful project yet we are carrying out case studies on hearing aid users living with Usher Syndrome. Each of of our case studies will be fitted with smart hearing aids.
Each will provide in depth feedback on:
- Initial findings
- Impact on daily living
- Monthly updates
- Regular analyses and validation
MWT will assess the above, providing regular feedback to our supporters.