Page URL:

Gene therapy treats deafness in mice

1 September 2008
Appeared in BioNews 473

A team of US scientists have used gene therapy to regenerate inner ear 'hair' cells - the culprit in many forms of deafness. The team, lead by John Brigande, who is himself profoundly deaf, injected mice with a version the Atoh1 gene modified so that it fluoresces green. Atoh1 is thought to orchestrate the formation of cochlear hair cells, which are fundamental to hearing and involved in producing brain signals in response to sound waves.

The mice, who were injected with the gene whilst still in the womb, converted non-sensory cells into cells producing green fluorescent hairs in exactly the right segment of the cochlea. The researchers then went on to prove that the hair cells were functional - converting air movement into electrical signal.

A similar study carried out in 2003 successfully used gene therapy with Atoh1 to revive hearing in chemically deafened guinea pigs, but there was controversy at the time as to the origin of the new hair cells produced. The new study proves that the cells generated are as a result of gene therapy.

Whilst excited about the findings, Brigande urged caution.

'I'd like to hear, and I would love to be a member of the research team or community that does define an efficacious therapy, but I think it needs to be approached with enormous caution,' he says.

Stanford University cell biologist Stefan Heller, who specialises in hearing research, agrees: 'No human subject committee would allow this kind of experiment to be done, given that the process would involve injecting a foetus with a foreign gene.'

Deafness can be the result of prolonged exposure to loud noise, infection, old age or gene mutations damaging the 'cochlear hair cells', with the only current treatment being a cochlear implant which bypasses defective hair cells tapping directly into auditory nerves, giving users the sensation of sound. Although not a cure for deafness, it is hoped that these findings will contribute to scientist's understanding of deafness, perhaps helping with the development of new drugs to treat the condition, offering the hope of one day finding a cure.

Cure for deafness now within reach
The Daily Telegraph |  27 August 2008
Gene therapy 'may repair hearing'
BBC News Online |  27 August 2008
Milestone reached in search for deafness cure
New Scientist |  27 August 2008
Researchers regrow crucial hair in inner ear
Reuters |  27 August 2008
8 October 2012 - by Dr Sarah Spain 
US researchers have identified a genetic mutation responsible for the hearing loss that occurs with the condition Usher syndrome type I...
17 September 2012 - by Dr Greg Ball 
Stem cells have been used to partially restore hearing in deaf gerbils through replacement of their auditory nerve cells...
5 December 2011 - by George Frodsham 
Two separate studies have successfully transplanted neurons into the brains of mice. The transplanted neurons are able to send and receive electrical impulses, and can be used to compensate for faulty brain cells, restoring normal function. Both studies sourced the transplanted neurons from embryos – mouse embryos in one case, human embryonic stem cells were used in the other...
15 February 2005 - by BioNews 
US and Japanese researchers have used gene therapy to restore the hearing of deaf guinea pigs, an achievement they say is 'the first time anyone has biologically repaired the hearing of an animal'. The team, based at the University of Michigan in Ann Arbour, delivered a gene to the inner...
to add a Comment.

By posting a comment you agree to abide by the BioNews terms and conditions

Syndicate this story - click here to enquire about using this story.