Over 50,000 children are deaf or have hearing loss in the UK. Half of these children were born deaf and half lost their hearing in childhood because of an infection or being treated with medicines that can cause hearing loss as a side effect.
We want to find ways to prevent children from losing their hearing and lessen the impact it has on them.
Cameron has Norrie disease, which causes hearing loss. His mother, Carla, shares their story:
I can’t imagine being blind and then losing another vital sense which connects me to the world. It’s heart-breaking to think he might be at risk of being completely deaf. Thankfully there is a ray of hope for my son, and other children with Norrie disease, too.”
Our impact in children’s hearing loss
What we’ve done
We’ve supported the development of a rapid genetic test for the NHS that identifies babies at risk of hearing loss. New-born babies, especially premature babies, are particularly vulnerable to developing life-threatening infections such as sepsis, as their immune system isn’t fully developed.
Babies admitted to intensive care with these infections are usually treated with an antibiotic called gentamicin. Gentamicin is an antibiotic that can cause hearing loss as a side effect. Several hundred babies each year develop hearing loss because they’re treated with gentamicin.
Around 1 in 500 babies have a version of a gene that makes them vulnerable to hearing loss if treated with gentamicin. This new rapid genetic test, made by Genedrive, can analyse the DNA of babies to see if they have this version of the gene in 30 minutes. This helps identify which babies would be at risk of hearing loss from gentamicin, so they can be given a different antibiotic.
The test has been approved for use across the NHS. It has been estimated that the test could save the hearing of 200 babies in England every year. We helped kick-start this breakthrough by funding a pilot project in Professor Bill Newman’s lab at the University of Manchester in 2016.
Professor Bill Newman, clinical genetics researcher at the University of Manchester:
“The RNID funding acted as a catalyst to allow us to develop the prototype point of care genetic test. As this type of testing was so novel, RNID’s support was vital to giving other research funding organisations the confidence that we could generate a genetic result at the patient bedside within minutes. This was key to us securing the money that has allowed us to implement the test into routine care of babies on neonatal units.”
What we’re doing now
Currently, we’re funding research to develop gene therapies that could treat hearing loss. Gene therapies have the potential to offer life-changing treatments for hearing loss, particularly inherited forms of hearing loss.
By directly targeting changes in genes that lead to hearing loss, gene therapies may be able to correct the underlying genetic cause, rather than only managing the symptoms. Gene therapies show promise in both preventing hearing loss and restoring lost hearing, offering hope to children and their parents.
Norrie disease is an example of a genetic condition that causes hearing loss. Children with Norrie disease are born blind, and many will begin to lose their hearing from around the age of 12. Currently, children with Norrie disease are offered hearing aids once they develop hearing loss and cochlear implants once their hearing loss becomes severe. However, these devices have limitations and cannot restore natural hearing.
Through research, we are passionate about finding the breakthroughs that can stop hearing loss being inevitable for these children. We’re funding researchers at University College London, led by Dr Jane Sowden, who are developing a gene therapy that can prevent the hearing loss in Norrie disease. This work will provide supporting evidence for the researchers to gain approval to test their new gene therapy in clinical trials.
What we can achieve together in the next 25 years
Our future work into preventing and restoring hearing loss for children includes:
- improved treatments for middle ear infections that reduce the amount of time children have hearing loss because of them and reduce the number of grommet surgeries
- new gene therapies to treat hearing loss for some types of genetic hearing loss – for those who want them.
