In this project, Professor Karen Steel at King’s College London explores whether a specific kind of hearing loss can be reversed by changing a gene in a mouse model.
Project start date: March 2021
Project end date: August 2025
Read about the project outcomes here.
About the project
The researchers will investigate whether it is possible to reverse a specific type of hearing loss in an adult ear. To do this, they’ll study mice which have a specific change in a gene called Pex3, developing progressive hearing loss as a result. The Pex3 protein is important for many normal cell functions and is found in structures called peroxisomes, which protect the sound-sensing cells of the inner ear from loud noise.
How it works
The researchers will try to reverse the hearing loss in the Pex3 ‘mutant’ mice by correcting the gene and thus reactivating its function. They’ll also study whether there’s a time limit after which this reversal is no longer possible. This would suggest that there is a “critical period” after which it is no longer possible to improve hearing.
How will this research benefit people with hearing loss?
There are currently no treatments that can reverse hearing loss or slow down its progression. Researchers around the world have been working to develop these treatments, including using gene therapy. However, as yet, no research has conclusively shown that it is possible to reverse hearing loss once the inner ear has matured.
If reactivating the Pex3 gene reverses hearing loss in these mice, this will show that hearing loss can be reversed even after the inner ear has fully developed. This will encourage the development of genetic treatments for hearing loss by providing proof that it is possible.
What we’ve learned so far
The researchers found that activating the Pex3 gene in mutant adult mice did not reverse the hearing loss. This suggests that any critical period for avoiding hearing loss caused by changes to Pex3 may require treatment before the auditory system is fully mature.
Given that a gene therapy treatment in humans would be given at a time when hearing is mature (as humans are born with a mature auditory system), these findings suggest that such a treatment would not be successful in this instance.
About the researcher
Karen Steel is Professor of Sensory Function at the Wolfson Centre for Age-Related Diseases, King’s College London. She is also a Fellow of the Academy of Medical Sciences and a Fellow of the Royal Society. An RNID Discovery Research Grant was awarded to her in 2021.
I would like my research to lead to the availability of drugs that can slow or halt the progression of hearing loss, and even better, reverse hearing loss.
RNID funding has played a critical role in enabling my research at the boundaries of the field.”
