Karen Steel is an RNID-funded researcher at the Wolfson Centre for Age-Related Diseases at King’s College, London. We have awarded Karen a number of grants, including an International Project Grant to study the genetic basis of the variation in human hearing ability using mouse models.
Karen’s main research interest is the genetics of deafness. She uses mouse mutants to better understand the genes and molecules involved in the normal development and function of hearing.
Karen wants to understand the process of deafness at the molecular (genetic) level through to hearing function at the level of the whole organism. Starting with mouse mutants with hearing loss, her team defines the underlying pathology and identifies the mutant gene. Then they collaborate with human geneticists to identify mutations in the same genes in humans who are deaf or have hearing loss.
After a first degree from Leeds University, a PhD at UCL studying the genetics of deafness, and postdoctoral positions at the MRC (Medical Research Council) Institute of Hearing Research in Nottingham and the Technical University in Munich, Karen returned to the MRC in Nottingham to establish the genetics of deafness programme there.
In 2003, she moved to the Wellcome Trust Sanger Institute to set up the Mouse Genetics Project, a large- scale programme that generates new mouse mutants and screens them for many disease features, including deafness. In 2009, she moved to King’s College London to the Wolfson Centre for Age-Related Diseases – a strong neuroscience environment that supports her current focus on progressive (age-related) hearing loss.
You can find out more about Karen’s work on her lab webpage.
What do you think will be the next big step forward?
The next step will be a molecular understanding of the pathological processes underlying progressive age-related hearing loss. This is critical to the development of treatments to slow or stop the progression of hearing loss.
What are the biggest problems facing hearing research?
Human hearing loss is hugely varied and complex: it’s very hard to study directly. There are probably over 900 genes involved in deafness, any one of which can carry variants leading to hearing loss. Then there are all the environmental ‘insults’ like noise, infections and certain types of drug – they can all damage hearing.
The inaccessibility of our inner ear is a problem, and the fact that we have to analyse very small amounts of tissue available from experimental animals, makes this research challenging.
What does RNID funding mean to you?
It’s made a major contribution to the research we are able to carry out, by providing support for a major study of genes implicated in hearing loss in the human population. It also funds a PhD student to analyse the role of lipids in normal hearing, and our two Pauline Ashley awards mean we have two young ‘investigators’ working in my group, developing their own research ideas.
Why do you work in hearing research?
Because it’s fascinating! It includes many different scientific disciplines, so it’s intellectually challenging and, at the same time, it means I can contribute to resolving a considerable human problem in communication.
What do you want your research to achieve?
We are already building a solid grounding of understanding of the molecular and pathological basis of progressive hearing loss. I anticipate that we can provide an experimental proof-of-principle that hearing loss can be prevented or reversed. This will be the tipping point that persuades industry to invest in developing new drug-based treatments.
My hope is that this research will lead to diagnostics and treatments available for people with progressive hearing loss, and that hearing loss will no longer be regarded as an inevitable part of growing older.