Hearing impairment is the most common sensorineural deficit worldwide. Deafness has a major genetic component, and understanding how genetic variation impacts hearing needs to be extensively studied. The importance of this work is reflected in the review by Lufkin: “the sense of hearing is one of the most crucial senses endowed to a living organism and its loss can have many ramifications.” This special issue about the Genetics of Deafness contributes to these studies by describing new mutations in genes important in hearing, by exploring the clinical implications of treatment based on genotype as well as reviewing of the literature.