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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