Understanding and treating high frequency age-related hearing loss 

In this project, Dr Jing-Yi Jeng at the University of Sheffield explores how a mutation in a gene called Cdh23^ahl can cause accelerated age-related hearing loss by how it affects hair cells in the inner ear.

Project start date: January 2022
Project end date: December 2023

About the project

Many researchers think that age-related hearing loss is partly caused by damage to, and loss of, the sound-sensing cells in the cochlea, known as hair cells. These cells transform sound vibrations from the air into electrical signals and send them to the brain. 

In this project, Jing-Yi will look at the inner ear hair cells in mice to investigate why how a gene called Cdh23^ahl is involved in age-related hearing loss, and why mutations in this gene cause hearing loss to happen faster than normal.

How it works

Jing-Yi will use state-of-the-art experimental approaches, such as electrophysiology, to measure the electrical activity of the hair cells as they respond to sounds of different frequencies, and electron microscopy, which will allow her to visualise tiny structures within the hair cells.

In this way, she will study the impact of the Cdh23^ahl on hair cells in both structural and functional ways. She will focus on the hair cells that respond to high-frequency sounds, to discover why they are more susceptible to damage from ageing than cells that respond to lower-frequency sounds.

Benefit

If successful, this research could tell us whether Cdh23^ahl directly causes damage in high-frequency hair cells, and if gene therapy could reverse age-related hearing loss by providing hair cells with a correct copy of the Cdh23^ahl gene. It will also further our understanding of age-related hearing loss.

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About the researcher

Dr Jing-Yi Jeng is a BBSRC Discovery Fellow at the University of Sheffield. She was awarded an RNID Fellowship award in 2021, co-funded by the Vivensa Foundation.