Exploring the genetic landscape of hearing loss: towards new therapies for deafness

Professor Steve Brown FRS FMedSci, Director, MRC Harwell Institute

The mouse is a formidable tool for identifying and dissecting the genes involved with hearing loss. Large-scale mutagenesis studies in the mouse have identified an extensive and unexplored genetic landscape involved with auditory dysfunction. The genetic basis for one common childhood form of deafness, chronic inflammatory middle ear disease or otitis media (OM), has eluded geneticists for many years, despite strong evidence for a significant genetic component. Mouse models of chronic OM identified from mutagenesis screens have provided new insight into the molecular pathogenesis of OM and opened up new opportunities for therapy.

Professor Steve Brown did his PhD at Cambridge University and before he joined the MRC, he was Professor of Genetics at Imperial College, London. His research interests cover mouse functional genomics, including the use of large-scale mouse mutagenesis and comparative genomic analysis to study the genetic basis of disease and to develop pre-clinical disease models. A particular focus has been the use of mouse models to study the molecular basis of genetic deafness. Along with Karen Steel, he discovered myosin VIIA as the gene underlying the shaker1 mutant, one of the first deafness genes to be identified. Subsequently, he has developed interests in the protein complexes that are involved with stereocilia elongation in hair cells in the inner ear. In addition, over the last ten years he has led a substantial research effort in the genetics of otitis media or glue ear, a common cause of hearing loss in children, employing mouse models to elaborate the key genetic pathways involved and develop novel therapeutic strategies.

He has served on numerous advisory boards and his current appointments include the Advisory Council for the National BioResource Centre, Japan; SAB for the Centre Intégratif de Génomique (CIG), University of Lausanne; Strategic Policy Committee, Institut de Génétique et de Biologie Moléculaire et Cellulaire (IGBMC) Strasbourg; and a member of the UK - China Joint Steering Group on promoting scientific and technological innovation and cooperation in Laboratory Animal Science. He is the current Chair of the International Mouse Phenotyping Consortium (IMPC) Steering Committee. He is a Fellow of the Royal Society, a Fellow of the Academy of Medical Sciences, a member of the European Molecular Biology Organisation and in 2009 was the recipient of the Genetics Society Medal.