My Child with Usher Syndrome: Charlie’s CI Story

Two weeks ago we introduced you to 10-year-old Charlie, who talked about his passion for singing and playing music, including violin, guitar, and piano. Charlie became profoundly deaf by the time he was three, and received cochlear implants soon afterwards. Last year, Charlie’s eyesight began to deteriorate and he was diagnosed with a condition which will eventually lead to blindness. His mom, Emma, is thankful that “cochlear implants have given Charlie one of his senses back!”

Meet Charlie’s Mom, Emma

Charlie was our first child. My husband and I had been trying for a baby for quite some time, so he was our little miracle. He has a smile to melt a thousand hearts.

Both my husband and I come from families of musicians—it has always been a big part of our lives. Some years ago, my mother turned our farm into a music school, building a concert hall in our back garden and later setting up the Gloucester Academy of Music. My husband and I play in the Carducci Quartet and travel around the world performing together.  I know it sounds awful, but I was so wrapped up in the whirlwind of being a first-time mum and still trying to keep up with my practice and concerts that I didn’t notice anything was wrong with Charlie’s hearing.

Charlie joined us on tour when he was just three weeks old. He spent the first few years of his life sitting in on our practice and he used to dance along and would always giggle when we played pizzicato. He also used to pretend to play the violin and then get us to clap so he could take a bow—quite the performer!

“We realized Charlie had been living in a silent world and felt incredibly guilty that we hadn’t noticed.”

It was my mum, who joined us on many tours that first year, who started to question what he could actually hear. We started doing our own tests—banging saucepans to see if he would turn to look and suddenly banging the door shut, etc. Charlie delighted in these “games”, and used to look at us with a cheeky smile.

We had hospital checkups every 6 months and they usually followed a similar pattern. Charlie wouldn’t turn to sounds as he would intently focus on the toys that the doctors were distracting him with. At the time, we didn’t read too much in to it. Charlie loved to play, so why would he stop to listen? Obviously, looking back, it was clear that his hearing was deteriorating. When we had our daughter Daisy, we were shocked at how many sounds she delighted in and how many little noises she made. We realized Charlie had been living in a silent world and felt incredibly guilty that we hadn’t noticed.

“We were told that he wouldn’t even hear a plane take off next to him.”

After some tests, we learnt that Charlie had profound deafness. We were told that he wouldn’t even hear a plane take off next to him. To say that it was a shock is an understatement—he always seemed to be responding to the music he was hearing. With an extended family full of musicians, I must admit that there was apprehension about Charlie having implants. Aside from the fact that it involved a delicate operation, we were worried about his natural residual hearing, especially when our families’ lives all evolved around our ears. But in the end there was no natural hearing left to save for Charlie, and we had to put his needs first. Charlie had bilateral MED-EL Cochlear Implants at three and a half years old. His speech developed incredibly quickly after getting the CIs, and he was soon able to ask if he could play the violin like daddy!

Usher Syndrome: “…It was a terrifying prospect, and I couldn’t stop thinking about it.”

From a very early age, Charlie would often cry in bright sunlight and would try and turn around in his buggy when the sun was out. The wonders of Google brought Usher Syndrome to my attention. As a condition that affects both the ears and the eyes, it was a terrifying prospect, and I couldn’t stop thinking about it. We pushed to get Charlie tested.

We actually found out the results over the phone just before going on stage to play a concert just after Charlie got his implants. I can remember the doctor telling me that the result was positive for Usher Syndrome type 1B, and my hand shaking as I hung up the phone. My husband Matt was in such disbelief that he made me call the doctor back to double check. We were then called on stage to perform. I played the whole concert without looking over at Matt as I couldn’t trust myself not to break down. The hardest part was phoning our mums to tell them the news. Charlie is the first grandchild on both our sides and our mums always helped look after Charlie backstage and on tour from when he was just a few weeks old. I knew the news would break their hearts.

Last summer, Charlie was diagnosed with the early stages of Retinitis Pigmentosa (a condition of the eye that leads to loss of vision and blindness), and that was tough. He had lots of questions that we tried to answer as honestly as possible without wanting to overload him. Since then, I can honestly say that Charlie’s life has been nothing short of inspirational and he brings a smile to our faces every day.

“To see him chatting happily with friends and to hear him singing at the top of his voice is a constant source of delight.”

Charlie is incredibly resilient and he’s had to be. We’re also fortunate to have supportive families and friends. A fundraising concert we put on raised enough money to cover his Auditory Verbal Therapy which proved invaluable in his early years of rehabilitation. Charlie’s school has also been fantastic, and his teachers are helping him to learn touch-typing and Braille. We are so thankful that Charlie’s cochlear implants have given him one of his senses back. To see him chatting happily with friends and to hear him singing at the top of his voice is a constant source of delight. Last year, he won a speaking competition through Auditory Verbal UK which led to him giving a speech at the House of Commons—a day we will never forget.

Charlie has an incredible zest for life and seizes every opportunity given to him. I’m not sure how I’m going to feel when I see him struggling with loss of vision. What I do know is that he is a fighter and that we have definitely lived a richer life because of the journey we’ve been on.

If you’d like to share a message with Charlie’s mom, Emma, leave a comment below and we will pass it on to her. If you’re looking for support for yourself or your child, the HearPeers Forum can be a great place to talk with others who are also on the cochlear implant journey.

We’re here to support you on every step of your family’s hearing journey. If you’ve just begun your journey of cochlear implants for your child, you can connect with us for local guidance and answers to your questions: My Child and Cochlear Implants

Like this post on Charlie’s journey with Usher Syndrome? Check out Charlie playing violin at Beats of Cochlea 2017!