The research at Sheffield University house discovered that there is a molecular mutation that underlies deafness. Deafness occurs when miR-96 mutates. Dr Walter Marcotti's discovery reveals when this mutation occurs the auditory sensory hair cells within the inner ear do not develop. These sensory hair cells are essential to send electrical signals to the brain allowing it to make sense of sound.
The initial study was conducted on mice because unlike humans they cannot utilise their sense of hearing before they are 12 days old. The reason they can't hear before this time is the fact that they need to develop two dissimilar types of sensory hair cells, the inner and outer cells.
One strain of mice carries a mutation in the miR-96 gene they do not develop the necessary hair cells to hear. The precise function of the miR-96 gene is that it regulates hair cell development in the first instance because it controls the appearance of many different genes all of which are involved in specific stages during the development process.
The miR-96 gene is thought to be a master gene because the mutation has more than one effect. The first effect is at cell apex where the sensitive cells do not form secondly the synaptic structures between the sensory nerves are altered and this prevents the exchange of electrical information to the sensory nerves. The mutation in miR-96 has already been definitively linked to human deafness but the good news is the fact that the molecules can be changed when targeted with drugs which means in theory at a later stage it will be possible to develop treatments for hearing loss. This will be especially useful in treating newborn deaf children and young children.
At the moment further research is necessary to establish a genetic basis for progressive hearing loss the present research will help doctors to understand more completely auditory development rather than hearing loss.
At present children who are born deaf have to undergo a cochlear implant which is a two pronged operation. An internal receiver has to be surgically implanted into the mastoid bone to the large bone behind the ear and electrodes are inserted into the Cochlear. The external part is the speech processor and microphone worn behind the ear connected by a magnet to the electrodes. The signals can then be sent along the auditory nerve to the brain.
Once a child has had a cochlear implant they are still classified as deaf despite the fact that they can both hear and talk. In the UK 800 children are born deaf every year and some children lose their hearing to send diseases such as meningitis. Whilst cochlear implants have improved dramatically in the last 10 years intervention by medicine would be a cheaper and easier solution to deafness. Deaf children would not go on to develop the complex needs that they do at present.
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