Our Story

Evie received her implant in June 2012 - the following is her story prior to implantation.

I tell my little girl every day how much I love her but she has never once heard me.

Evie was born in September 2010 without any real complications.  I noticed that she had a small dermoid (white lump) in her right eye and a small skin tag on her right cheek but we were told these were simply cosmetic. The hospital were unable to obtain a clear result in her newborn hearing screen but we were assured this was often due to glue ear and not something we should worry about.  I knew in my heart something was wrong even on that first night as she didn't seem to quieten to the sound of my voice as my others had but I put my worries down to hormones.

When she was three weeks old we underwent further tests and were given the devastating news that our little girl was profoundly deaf .  We went home reeling - I had no idea how I would be able to tell her brothers and sister that the stories they had made up for her and the songs they had sung to her had quite literally fallen on those small perfectly formed deaf ears.

Over the next twelve months we attended a specialist nursery and underwent various tests to try to establish the cause of her hearing loss.  Evie wore hearing aids and we all started learning sign language. As part of the many tests we discovered she also had marked astigmatism in her right eye and would need to wear glasses.  Eventually she was given an MRI under sedation which revealed significant abnormalities on her spine and questions regarding her hearing nerves.  We were told the right nerve was absent and the left was very thin.  This set of results led to a confirmed diagnosis of Goldenhar a very rare condition for which there is no known cause  but which affects the eyes, ears and spine.  Its technical name is Oculo-Auriculo-Vertebral (OAV) syndrome) and  approximately only one in every 500,000 live births are affected by it.

We were put onto the cochlear implant team in Southampton but I just didn't have any confidence when we met the surgeon that he had enough experience or knowledge of Evie's particular case.  We therefore decided to obtain a second opinion in London and were then informed that in fact she had no hearing nerves and no balance nerves on either side and that the nerve they had seen in Southampton was not a thin hearing nerve but a facial nerve in the wrong channel.  A cochlear implant would not be an option as there is nothing for the sound to travel through - like having a set of speakers and a stereo but no lead to connect them.

If we wanted Evie to have a world with sound our only option would be an Auditory Brainstem Implant - a bionic ear. If it sounds scary it's because it is!  Amazingly a handful of specialist surgeons worldwide are able to implant a tiny device into the actual brainstem where hearing is processed.  If implanted early enough a child with no hearing can access sounds, enough to allow that child to be able to understand speech with lip reading and in some cases even without lip reading. 

As a family we are all learning BSL and Evie happily signs to communicate her needs but I am very aware that signing is not my first language and therefore Evie's ability to learn BSL is inevitably going to be slower than a child whose parents' first language is BSL. 

Despite Evie's spine abnormalities and her lack of balancing nerves she is a little girl who absolutely loves life and is the first to jump head first into anything new! She walks and runs like any other child but with a few more wobbly moments as she uses her sight and surroundings to balance rather than any balancing nerves!

Once the implant is in place it will need to be regularly programmed as some of the electrodes may stimulate other parts of the brain and cause tingling or even pain.  This process is called "mapping" and will take place over the weeks and months after the implant and then annually.  Only a few specialists world wide are able to do this.  Evie will also require specialist speech therapy and to give Evie the best chance of success with her ABI it would be amazing if we could take her to The John Tracey clinic in America who offer a summer residential placement of three weeks  for children that need this specialist type of speech therapy. The cost of the placement is 900 US Dollars.

We have established a Trust called Hope for Hearing.  It doesn't have charitable status as it is set up with Evie as the beneficiary and a charity has to be for a wider group of beneficiaries, not just one.