Every year, the world’s leading hearing and tinnitus researchers gather at the Association for Research in Otolaryngology (ARO) Midwinter Meeting – the largest global event dedicated to advancing our understanding of hearing.
Earlier this month, our Director of Research, Ralph Holme, and Research Grants Manager, Ellie Weston, joined thousands of experts for five days of new discoveries and global collaboration.
Here, Ralph shares some of the highlights.
The power of collaboration
The meeting opened by shining a spotlight on the power of collaboration. Gone are the days when individual labs would study a few genes at a time. Advances in technology now mean scientists can study the activity of thousands of genes at the level of a single cell generating huge datasets.
Collaborative platforms are now helping researchers bring these large datasets together in one place, where they can easily share, compare and analyse the information. By working together like this and combining resources, faster progress can be made towards developing treatments.
Gene therapies showing real promise
Gene therapy continued to be a hot topic at the conference, with researchers sharing encouraging progress across several areas of research, including:
- Otoferlin gene therapy trials.
Updates were given on the latest results from clinical trials of gene therapies to treat a rare form of deafness caused by a fault in the otoferlin gene. Back in 2024, the initial results from these trials were presented at ARO, showing that within 30 days of treatment, children went from being profoundly deaf to having only mild to moderate hearing loss. Two years on, hearing is maintained, showing that the treatment appears to be long lasting and safe. - Developing treatments for GJB2-related hearing loss.
Progress is being made towards developing gene therapies for other types of genetic hearing loss. Sensorion, a French biotech company, is working on a gene therapy for hearing loss caused by faults in a gene called GJB2, which is the most common cause of genetic hearing loss.
They showed that in mice mimicking this particular type of hearing loss, their gene therapy can improve hearing, reaches the correct cells within the inner ear, and is safe. They are now preparing to move this treatment into clinical trials. - New progress on Norrie disease.
Dr Patel presented the latest results from a project jointly funded by RNID and Fondation Pour l’Audition to develop a gene therapy for Norrie disease. Children affected by this condition are blind from birth and progressively lose their hearing during childhood. Early results are promising: in mice mimicking Norrie disease, the team have been able to halt the loss of hearing.
Most of the gene therapies being developed are aimed at treating cells deep inside the cochlea. However, hearing loss in Norrie disease is caused by defects in the network of blood vessels that surround the cochlea. So, although Norrie disease is rare, the technology being developed to study and treat the cochlear vascular system in this project could have wider applications, opening the door to treatments for other types of hearing loss.
Type 2 diabetes and hearing difficulties
We’ve known for some time that hearing loss is associated with an increased risk of developing dementia, and there were many posters on research to better understand why this is the case. But cognitive decline is not the only condition that has been associated with hearing loss.
Type 2 diabetes has also been linked to an increased risk of hearing difficulties. Results of a large study of 241 people with type 2 diabetes who all underwent a series of hearing tests were also presented.
Interestingly, the prevalence of hearing loss as measured by the audiogram was no higher than in the general population, but difficulties in perceiving speech in noise were much more prevalent.
The researchers suggest that this is because type 2 diabetes is thought to damage the connections between the hair cells that detect sound and the auditory nerve that carries the signal to the brain. This type of damage has less impact on the ability to hear a quiet tone but can make carrying out complex listening tasks, like following a conversation in a noisy restaurant, much harder.
It is often referred to as hidden hearing loss. The study highlights the importance of looking beyond standard audiometry when assessing hearing.
Laboratory models for hearing research
A major challenge in developing treatments for hearing loss has been accessing human inner ear tissue to study. Unlike other parts of the body, it is not possible to take tissue samples from the inner ear of people to study. So, it was encouraging to see how researchers are tackling this problem in a session dedicated to this topic.
A breakthrough that holds huge potential is around the ability to take cells from the skin or blood and reprogramme them to an embryonic-like state. This is done by activating specific genes. The embryonic-like cells, called induced pluripotent stem cells, have the potential to give rise to all the different cell types that make up the body.
We heard how researchers have developed ways of taking these stem cells and, by exposing them to various factors, guide them into developing into the cell types that make up the cochlea.
This now gives researchers a ready supply of human cochlear cells to study. They can even be grown so that they form clumps of cells called organoids that mimic 3D tissue in the cochlea. This allows researchers to study how different cell types interact with one another.
How these models are advancing hearing research
These new human cell models are already being used in a wide range of research, such as studying how cytomegalovirus causes hearing loss. This is a human virus, so hard to study in animal cells, and is a common cause of hearing loss in newborns.
Other research groups are exploiting the fact that large quantities of human inner ear cells can now be grown in the lab to screen large libraries of chemical compounds to identify those that are able to protect auditory neurons or promote their regeneration. This is accelerating early-stage drug discovery.
RNID at the heart of hearing research
I was proud to see so many RNID-funded researchers sharing their latest findings and to see the impact our support is having.
We spoke to lots of researchers at our exhibition stand, learning more about how their research interests might contribute to efforts to discover and develop treatments, and to explain how our funding and support can help them achieve their goals.
I left the ARO Midwinter Meeting feeling inspired by the energy and dedication the research community has towards finding solutions to prevent hearing loss, improve hearing and silence tinnitus.
