Medical Research Council - London Institute of Medical Sciences

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Hearing Hairs
04 April 2017

Hearing Hairs

These sprouting fronds are tiny protrusions called sterocilia emanating from sensory hair cells within a mouse’s ear. Such cilia are also present in human ears where they detect and transmit both sound- and balance-related stimuli to the brain. In people with Usher syndrome – characterised by hearing loss, blindness and dizziness – the stereocilia are often stumpy because of defects in proteins responsible for the cilia’s normal development. Mice with a mutation in one of these developmental proteins – called whirlin – also have shortened cilia together with balance and hearing problems making them ideal for studying Usher syndrome and for developing potential treatments. Using a viral vector to deliver functional whirlin to the inner ears of these mice, scientists have been able to restore cilia growth and improve the animals’ hearing and balance for a period of months. This encouraging result now paves the way for a similar gene therapy approach in humans.

Written by Ruth Williams

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BPoD stands for Biomedical Picture of the Day. Managed by the MRC London Institute of Medical Sciences (the new name for the MRC Clinical Sciences Centre) the website aims to engage everyone, young and old, in the wonders of biomedicine. Images are kindly provided for inclusion on this website through the generosity of scientists across the globe.

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