Researchers reveal function of novel molecule that underlies human deafness

Hair bundle morphology of immature inner hair cells (IHCs) from normal cochlea
Hair bundle morphology of immature inner hair cells (IHCs) from normal cochlea
Researchers reveal function of novel molecule that underlies human deafness. New research from the University of Sheffield has revealed that the molecular mechanism underlying deafness is caused by a mutation of a specific microRNA called miR-96. The discovery could provide the basis for treating progressive hearing loss and deafness. The research team, led by Dr Walter Marcotti, Royal Society University Research Fellow from the University´s Department of Biomedical Science, in collaboration with Professor Karen Steel at the Sanger Institute in Cambridge, discovered that the mutation in miR-96 prevents development of the auditory sensory hair cells. These cells are located in the inner ear and are essential for encoding sound as electrical signals that are then sent to the brain. The research has been published this week in the Proceedings of the National Academy of Sciences journal and was based on studies of mice, which do not normally hear until about 12 days after birth. Prior to this age their immature hair cells must execute a precise genetic program that regulates the development of distinct types of sensory hair cell, namely inner and outer hair cells.
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