March 2018 iNews
Charlie Denton has overcome many challenges to play several musical instruments. Being unable to hear since birth has not stopped him from learning the violin, piano, guitar and now even the drums. The 11-year-old is already defying all the odds of being deaf, but is now also facing the possibility he may go blind as well. Charlie has Usher syndrome, an extremely rare genetic disorder caused by a mutation in any one of at least 11 genes resulting in a combination of hearing loss and visual impairment – his parents, Emma and Matthew, both 40, each have the exact same fault in the same gene. It is a leading cause of deafblindness and is at present incurable, although many trials are underway in the search for a cure.
Charlie’s diagnosis of profound deafness was a massive shock to his parents, themselves professional musicians who make up half of the Carducci String Quartet, one of the UK’s most successful string quartets – all the more so as he was their first child together. “He failed his newborn screening but we were told one ear was ok and he was very mildly deaf in the other ear, so it wasn’t going to be a problem,” Emma said at the family home near Gloucester. “But then a friend of ours who used to come on tour with us thought he wasn’t hearing properly, so we started hitting on saucepans and Charlie, who was under a year old at the time, didn’t turn.”
Their doctor prescribed hearing aids a few months later, following a test, but when he was two years old, it was clear they were pointless. Charlie used to regularly throw them off as a toddler, a sign of his frustration that they were not helping him hear at all. “That’s when we were told about cochlear implants, which we’d never heard of before,” says Emma. “For us being musicians, we were all for natural hearing but were told that they could only carry out the operation [to have the implants fitted] when there was no residual hearing left. “We were sent a link so we could hear what Gershwin’s Rhapsody in Blue sounded like through a cochlear implant and it was just distortion, terrible. But, at that time, we didn’t know if Charlie was going to like music and we decided we had to do what’s best for him.” Charlie had his bilateral cochlear implants fitted at Bristol Children’s Hospital when he was just over three and a half, by which point he would not have heard a plane taking off next to him. It was only the second year bilateral implants began to be fitted – prior to then an implant was fitted in just one ear. “It actually took a month for him to heal before we could switch the implants on and we thought ‘how could we subject Charlie to a month of no sound?’ Emma says. “But then realised he has never had any.
The operation was a success though with doctors stressing the earlier the operation takes place in a child, the better. “It’s like learning a language, or music,” Emma says. “The younger you start the easier’s it’s going to be.” “The only thing I can hear without the implants is my tinnitus,” Charlie says. “Which is a bit annoying at night when I don’t wear them, because they fall off if I do.” The device has 36 different frequencies, which Charlie can operate via remote control, if he feels something is too loud – like in a cinema – or too quiet. It is often tricky to get the levels just right, meaning Charlie often suffers from migraines. “He flicks them off if I’m hoovering… or if I’m nagging him,” Emma laughs.
By the time his implants were fitted, he was around two years behind on his speech, but after the operation his progress dramatically improved. Listening to Charlie to speak today you would never know he is totally deaf. His musical aspirations began rather early, having been given a violin when he was just two. “The violin has been the hardest to learn, as I can’t really tell my pitching,” he says, before playing Rustic Dance, a typical piece in the Grade 4 test, which Charlie has passed. “I can’t tell if I’m out of tune or in tune. I usually have 5 practices a week, although have done more recently.” Charlie also took to the piano, passing Grade 1 when he was eight and Grade 2 last year. He has also just won a place at the local grammar school which, given the tests are dominated by verbal reasoning, is another extraordinary achievement. “He set himself the challenge of learning 200 new words over the summer in preparation for the test and he did it,” Emma says. “Plus, it’s just down the road so Charlie figured he could stay in bed a bit longer.”
The couple also have a 9-year-old daughter, Daisy who has been given the all clear from Usher syndrome. “There’s a one in two chance of passing it on, so we were just glad we didn’t find out about that before Daisy was born,” Emma says. Charlie has the most severe type of Usher syndrome, which also causes retinitis pigmentosa in his eyes. Symptoms include trouble seeing at night and decreased peripheral vision with the onset of symptoms generally gradual. Usually, it culminates in tunnel vision, but it can cause blindness in the long run. As a result, Charlie has been learning Braille and touch-type at his primary school. Despite all the talents he possesses, and the determination at overcoming all the challenges that have come his way so far, he says it is too early to know what he wants to do when he is older. “He changes his mind a lot,” Emma says. “It was a forensic scientist last year, but he loves his sport so he may go down that route… I think for us it’s nice he has so many passions, and considering we were told he would have no appreciation of music, just to see him really enjoying playing and listening to music is all we could ask for really.”