Norrie Disease is a rare genetic disorder, mainly affecting boys, that leads to blindness and, in most cases, progressive hearing loss as well. Other symptoms include autism and cognitive impairment.
As my son was born totally blind, it quickly became 'our normal'. Despite being told all the possibilities of additional needs Norrie Disease brings, one thing we never mentally prepared ourselves for was for Cameron to progressively lose his hearing. Over the months, we noticed a change in his behaviour: going out in noisy crowded places became a huge problem and would quickly send him into a sensory-overload meltdown.
It was difficult to manage his frustrations, and his severely delayed communication skills were showing no progress. After years of recurrent ear infections and traumatising pain, I began to notice certain sounds were somewhat painful to his ears. After being closely monitored by audiologists every 6 months, we decided to have grommets inserted - where it was then discovered that Cameron had completely lost his hearing in one ear at the age of 3. The grommets were completely life changing, helping to manage the pain and help keep his one hearing ear clear of fluid and congestion.
Still, my son has to work ten times harder to locate sounds and process them compared to other children. He can’t see what is making the noise so doesn’t know where it is coming from or what it is, and his hearing is severely affected when there is too much background noise. Having a multisensory impairment (sight and hearing loss) has caused my son to have very complex additional needs, so much more than if he had just blindness or hearing loss alone. When one sensory loss accompanies the other, we feel the disability is far greater and much more difficult to overcome.
When people learn about Cameron’s blindness, the first thing that everybody says, without fail, is how his 'super senses' will compensate for his vision, especially his hearing. But this isn’t the case. For Cameron, the most important sense is touch. Having dual sensory loss can prove difficult at times: he is still unable to form communication and language skills and we have to adapt our surroundings and environment to help him reach his full potential.
Every day we live in fear that the progressive hearing loss will affect his other ear before he develops the skills to talk - something I so long to hear. It’s a ticking time bomb in the corner of the room, just waiting to happen.
Research into hearing loss is so important to our family. Finding a treatment that works for Cameron would open up a whole new world to encourage communication skills. His smile lights up his face when he hears his favourite music or his sisters’ voices, but knowing that this one vital sense is already being taken away from him too soon makes us feel sad and frustrated. No one wants their child to feel isolated, living in a world where they cannot see and they cannot hear. We want to find a way to stop that, so that Cameron’s smile never fades.
We depend on your donations so we can fund the best research into hearing loss around the world. Donate today and help us continue our vital work into hearing treatments, so that people like Cameron and his family can live life to the full again.
You can find out more about the research we’re funding in our biomedical research section. If you’re interested in finding out more about the latest developments in hearing research, sign up to receive our Soundbite newsletter. It’s a monthly email, filled with the latest news about hearing and tinnitus research.
For more information about Norrie Disease and support available you can visit The Norrie Disease Foundation’s website.
Hearing loss caused by excessively loud music and audio from personal listening devices is an increasing problem. In the latest version of their Health app, Apple are introducing new features to tackle the issue. They will be available as part of their iOS13.1 software update.
Researchers at University College London (UCL) are investigating the effect of noisy listening environments on children’s ability to understand speech and would like to invite your child to take part.
Certain medicines can harm hearing as a side-effect. We funded research to understand how a new drug might protect hearing when someone has to take one of these medicines. Tracey, from our Research team, explains in her blog post.
With the help of our funding, researchers at the UCL Ear Institute have shown that structures called stress granules, which form when a cell is damaged or otherwise stressed, can protect hair cells from the damage caused by ototoxic (ear-toxic) medicines, such as aminoglycoside antibiotics. Dr Ralph Holme, from our Research team, tells us more.