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.
Over nine million people in the UK are either always or often lonely. We know that hearing loss can be sometimes be socially isolating, so this Loneliness Awareness Week we want to encourage anyone with hearing loss who feels lonely to take action. We’ve products and technology to help you, or a loved one, join in again and enjoy a better quality of life.
If you wear hearing aids but still struggle to follow close-range conversations and what’s happening on TV, a hearing loop could be the answer. It only amplifies the sounds you want to hear, while cutting out background noise. It’s ideal if your hearing aids have a loop or ‘T’ setting. We have hearing loops for the home and portable loops for when you’re out and about. Here’s a roundup of our top five hearing loops, with 10% off between 13 - 21 June 2019.
A team of researchers and clinicians in London and Sheffield are looking for participants to take part in a clinical trial of a new drug treatment for sudden hearing loss.
Do you struggle to follow what’s going on because you can’t always hear what people are saying? If so, a conversation listener will help you join in again. It’s ideal for day-to-day situations, such as family get-togethers, shopping trips, meetings or meals out. You’ll hear more clearly, whether or not you have hearing aids. From 30 May to 7 June, enjoy 10% off in our Conversation Listeners Sale.