Usher Syndrome is a rare hereditary disorder. Children suffering from this disorder are born deaf or hard of hearing and apart from night-blindness they also experience a progressive loss of eyesight. Sometimes there are balance problems as well. Eventually, people suffering from Usher Syndrome become both deaf and blind.
There is still no treatment for Usher Syndrome. There are hopeful developments, though. The mission of Stichting Ushersyndroom is: ‘A treatment for Usher Syndrome in 2025.’ This requires a lot of money! Science is faced with major challenges in the search for solutions that have to lead to a treatment for all 400,000 patients in the world.
How do you live your life if you know that one day your world will be quiet and dark? The central figure in the documentary ‘The small world of Machteld Cossee’ knows that she cannot escape her fate. Still, she tries to live a normal life for as long as possible. This results in touching moments as well as frustrations and rows and moments of fear for the future.
He was born deaf. And he will be blind as well. Jeff Horsten sings a self-written song about his impairments on YouTube.
‘I want to inspire people. I do not see myself as a person with impairments. I am just Jeff.’
He sings the song together with singer Jorien Habing. The text is about the ‘tunnel’, as Jeff experiences his life. It refers to his deafness and poor eyesight: Jeff looks through two ‘straws’ of 10 degree. People usually can see 180 degrees.
INTERVIEW WITH DR. RONALD PENNINGS
The speed at which the problems with hearing and seeing develop, may vary per person. Ronald Pennings, MD, PhD, ENT consultant and Usher syndrome specialist, tells more about this syndrome.
Usher Syndrome is not the sum of poor eyesight and bad hearing. The one sense cannot compensate the other.
The limited eyesight and hearing make everyday life with Usher Syndrome a huge challenge. As the impairments increase, the quality of life decreases and there is a large risk of social isolation.