Identification and characterization of genes for human retrocochlear hearing disorders

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The genetic analysis of hereditary forms of hearing impairment in human families and diverse animal models has led to the identification of a large number of genes/proteins that are indispensable for hearing. This holds especially true for molecules which are primarily expressed and functionally relevant in cells and structures of the cochlea itself. On the contrary, there is only very limited knowledge about the genetic and molecular basis of retrocochlear hearing disorders like for example for the so called auditory neuropathy and central auditory processing disorders. The main goal of this project is to identify and - in collaboration with other members of this priority programme - further characterize genes involved in the development of such retrocochlear human hearing disorders. To achieve this aim we will apply systematic and genome-wide linkage and sequencing approaches in families/patients affected by different forms of neural hearing disorders and subsequently investigate the identified genes/genetic variants in suited cell or animal models. The identification of underlying disease genes will help to improve our understanding about the molecular and physiological basis of central auditory information processing in humans.