New findings into biology of progressive hearing loss - Action On Hearing Loss: RNID
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New findings into biology of progressive hearing loss

Posted on 16/08/2016

New research with funding from UK charity Action on Hearing Loss has led to the discovery of a new biological mechanism involved in the progressive loss of hearing which could lead to new approaches to treating this common form of hearing loss.

It’s known that gradual loss of sound-detecting sensory hair cells within the inner ear is associated with progressive hearing loss, but now researchers from King’s College London, the Wellcome Trust Sanger Institute, and University College London have shown that defects in a structure within the ear called the stria vascularis, can also cause progressive hearing loss.

The stria vascularis is essential for normal hearing and is involved in maintaining the endocochlear potential - a difference in charged molecules between compartments of the inner ear which acts like a battery to power the transmission of sound signals from the ear to the brain.

Researchers made the discovery while investigating why mice with a specific mutation in a gene called S1pr2 have a progressive loss of hearing, and found degeneration of the stria vascularis and a low endocochlear potential in these mice correlated with their loss of hearing. Significantly, the gene in humans is also associated with changes in people’s ability to hear - making this discovery relevant to progressive hearing loss in the human population.

Professor Karen Steel at King’s College London who led the research said: ‘Our finding suggests that designing treatments to boost the function of the stria vascularis could be important in treating some forms of progressive hearing loss. What is needed now are accurate ways of diagnosing what part of the ear is affected so that in the future the most appropriate treatment can be administered.’

Dr Ralph Holme, Head of Biomedical Research at Action on Hearing Loss said: ‘Hearing Loss affects as many as 1 in 6 people in the UK and all too often isolates people from friends and family. By funding cutting edge research to increase our understanding of progressive hearing loss we aim to bring the development of urgently needed treatments a step closer.’

Action on Hearing Loss runs the world’s largest donor-supported hearing research programme dedicated to funding research into better treatments and cures for hearing loss and tinnitus.


Contact for media enquiries:
Gorki Duhra, Senior PR Officer, telephone: 020 7296 8057, out of hours: 07944 038 635 or email: Follow Action on Hearing Loss on Twitter @hearinglosspr

  • Notes to Editors
    Action on Hearing Loss helps people to confront deafness, tinnitus and hearing loss to live the life they choose.  Action on Hearing Loss enables them to take control of their lives and remove the barriers in their way. Action on Hearing Loss gives people support and care, develop technology and treatments, and campaign for equality.
  • The research paper is available via the following link, Ingham et al. S1PR2 variants associated with auditory function in humans and endocochlear potential decline in mouse. Sci Rep. 2016 Jul 7; 6:28964.
  • For more information about Action on Hearing Loss’s Biomedical Research programme, go to
  • Action on Hearing Loss spokespeople and case studies are available for interview upon request.
  • Hearing loss research is woefully underfunded, compared to the scale of the issue, which affects one in six people in the UK. In 2014, hearing loss research received less than a tenth of the funding per person affected (£1.11 per person) compared to sight loss (£11.35 per person affected) and a fraction of funding for research into other conditions such as diabetes, and heart disease.
  • Hearing loss, if undiagnosed or mismanaged, can lead to isolation, depression and reduce employment opportunities. Investment in research has the potential to not only improve the quality of life for millions of people across the UK, but save the economy millions of pounds.

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