Since the identification of the first deafness gene in the '90s, a relevant number of genes have been identified. This finding led to a significant increase on the molecular knowledge of the processes responsible for hearing and balance and of the corresponding pathological mechanisms. But there is still a long way to go and many genes remain to be identified. The elucidation of the exact function of genes for which only a putative function has been hypothesized and of genes with an unknown function, remains a great challenge. In this chapter an up-to-day overview on the genetics of hearing loss is provided to the reader.
