In this project, Professor Angelika Doetzlhofer at Johns Hopkins University, USA uses gene therapy to test potential new ways to reproduce inner ear hair cells. These hair cells are essential for hearing and cannot currently be replaced once lost.
Project start date: June 2020
Project end date: May 2023
About the project
Hair cells in the inner ear help convert sound waves from the air into electrical signals that the brain processes as sound. They are formed from a type of cell called ‘precursors’, and these precursors cannot be replenished or reproduced. If hair cells are damaged, they cannot be replaced, leading to permanent hearing loss.
However, birds can replace their hair cells and restore their hearing by using ‘neighbouring supporting cells’. Professor Doetzlhofer’s team will investigate if it’s possible to activate similar precursor-like features in supporting cells in mammals, using mice.
How it works
The researchers will activate a protein called LIN28B in the supporting cells. LIN28B plays an important role in hair cell precursors, helping hair cells regenerate when hearing is still developing. They will grow a miniature organ from a mixture of cells in a dish (a process called ‘organoid culture’) to see if they can re-activate the ability of supporting cells to regenerate hair cells in adult mice.
How will this research benefit people with hearing loss?
If the researchers are successful, their work could be the first step towards a new treatment to restore lost hair cells, and therefore hearing, in people.
About the researcher
Professor Angelika Doetzlhofer is Professor of Neuroscience at Johns Hopkins University, USA. She was awarded an RNID Discovery Research Grant for this project in 2020.