Mouse Models for Deafness: Lessons for the Human Inner Ear and Hearing Loss

In Brief In the field of hearing research, recent advances using the mouse as a model for human hearing loss have brought exciting insights into the molecular pathways that lead to normal hearing, and into the mechanisms that are disrupted once a mutation occurs in one of the critical genes. Inaccessible for most procedures other than high-resolution computed tomography (CT) scanning or invasive surgery, most studies on the ear in humans can only be performed postmortem. A major goal in hearing research is to gain a full understanding of how a sound is heard at the molecular level, so that diagnostic and eventually therapeutic interventions can be developed that can treat the diseased inner ear before permanent damage has occurred, such as hair cell loss. The mouse, with its advantages of short gestation time, ease of selective matings, and similarity of the genome and inner ear to humans, is truly a remarkable resource for attaining this goal and investigating the intrigues of the human ear. The mouse genome and ear are quite similar to their human counterparts. Because of these and other reasons, mouse models represent a remarkable resource to investigate how the human ear works. This article reviews mouse genetics, mouse models for deafness, and how they have contributed to our understanding of human deafness and cochlear physiology.