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Wednesday, 21 November, 2001, 00:45 GMT
Gene linked to hearing loss
Low frequency hearing loss may be more common than previously thought
Scientists say they have discovered a gene defect that causes an unusual type of hearing loss.

An international research team, led by scientists from the University of Michigan Medical School in the US, found that a mutated form of the WFS1 gene caused low frequency sensorineural hearing loss.

This discovery has to be welcomed as it adds another piece to the jigsaw

Dr John Lowe
There are believed to be more than 50 genes involved in hearing loss, and approximately 20 have been identified so far.

It is hoped the discovery of the WFS1 gene may shed light on more common types of hearing loss and conditions such as Meniere's Disease.

The researchers found that children who inherited a mutated form of WFS1 gradually lost their ability to hear low-frequency sounds.

Hearing aids

The hearing loss became much worse over time and eventually resulted in hearing aids.

But the researchers found that many people who are unable to hear low-frequency sounds may not be aware of it because their ability to understand speech is not affected.

Lead scientist, Dr Marci Lesperance, said: "It is possible that this type of hearing loss is more common than we think.

"However, discovering a new gene and its related protein gives scientists another piece of information to increase their understanding of inner ear development and function.

"These proteins are produced in tiny amounts in the inner ear or cochlea - an area that is inaccessible for tissue sampling and difficult to study."

Dr Lesperance believes there may be a connection between mutations in WFS1 and the more common form of progressive sensorineural hearing loss involving high-frequency sounds like human speech.

She said people who had low-frequency hearing loss as children often lost their ability to hear high-frequency sounds as they got older.

"High-frequency hearing loss is caused by ageing, noise exposure or drug toxicity, but mutations in WFS1 might make people more susceptible," she said.

Welcome discovery

Dr John Low, Director of Research and Technology at the Royal National Institute for the Deaf (RNID), said: "RNID is encouraged that leading research centres are focusing on deafness and hearing loss which has devastating effects on so many people across the world.

"This discovery has to be welcomed as it adds another piece to the jigsaw.

"RNID actively supports international research in genetics, and is particularly focused on enabling international consortia to be formed."

Scientists believe that if they can identify the genes involved in hearing loss, new methods of intervention can then be explored.

See also:

18 Oct 01 | Health
Breakthrough for deaf children
20 Sep 01 | Health
Children will 'learn to hear'
25 Mar 01 | Health
Ear cells 'could restore hearing'
28 Jun 00 | Health
Hearing tests for all newborns
18 Jan 00 | Health
Hi-tech hearing aids free on NHS
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