Royal Society Fellow awarded the € 1 million Brain Prize 2012

12 March 2012

Professor Karen Steel FRS has been announced as winner of The Brain Prize 2012. She shares the award with Professor Christine Petit for their pioneering work on the genetics of hearing and deafness.

The Brain Prize is awarded by Grete Lundbeck European Brain Research Foundation, a charitable, non-profit organization. It is a personal prize awarded to one or more scientists who have distinguished themselves by an outstanding contribution to European neuroscience.

Professor Steel FRS and Professor Petit receive the award ‘for their unique, world-leading contributions to our understanding of the genetic regulation of the development and functioning of the ear, and for elucidating the causes of many of the hundreds of inherited forms of deafness’.

Inherited conditions render one in a thousand children deaf at birth, and cause as many again to become deaf before maturity, leading to delay or failure in the acquisition of speech, and frequently to disadvantages in communication and learning. Genetic anomalies also contribute to many age-related and progressive forms of hearing loss. About one-tenth of the population in the developed world suffers from significant hearing impairment, which has an enormous impact on individuals and on society.

Professor Steel FRS and Professor Petit are at the forefront of efforts to understand the molecular mechanisms of the specialised hair cells in the inner ear, whose extraordinary sensitivity to mechanical stimulation underpins the senses of hearing and balance.

Professor Colin Blakemore, Oxford University, Chairman of the Selection Committee said: 

“Together, the work of these two Europeans scientists illustrates the value and power of interdisciplinary approaches in neuroscience, and the way in which cutting-edge fundamental research is needed to understand complex clinical problems and to accelerate benefit for patients.

We are delighted that The Brain Prize for the best of European neuroscience goes, in its second year, to two women scientists. We are sure that the award will be applauded by female researchers around the world, and by all those who are concerned that young women are given every encouragement to consider careers in science.”

Professor Steel FRS is Principal Investigator for the Genetics of Deafness and founder of the Mouse Genetics Programme at the Wellcome Trust Sanger Institute.  She has pioneered the use of mouse models, starting with her painstaking description of the characteristics of mutant mice with disorders of balance and hearing.  Her early work on cochlear function and hair cell degeneration in the mutant deafness mouse led to identification of the gene calledTmc1, the human homologue of which is mutated in several forms of hereditary deafness. She and her colleagues went on to identify causative genes in nearly 30 forms of hearing disorder in mice, most of which have been linked to human conditions. In particular, she described the genes for myosin-7a and cadherin-23, which are key components in the transduction mechanism in hair cells, and mutations of which underlie forms of Usher Syndrome.

The prize lectures and award ceremony will take place 9th May in Copenhagen, Denmark. The Prize will be presented by Her Majesty the Queen of Denmark.