Actin in hair cells and hearing loss

Hereditary deafness is genetically heterogeneous such that mutations of many different genes can cause hearing loss. This review focuses on the evidence and implications that several of these deafness genes encode actin-interacting proteins or actin itself. There is a growing appreciation of the contribution of the actin interactome in stereocilia development, maintenance, mechanotransduction and malfunction of the auditory system. ► Many human deafness genes encode proteins that interact with actin. ► Proteins of the actin interactome are essential for stereocilia development and function. ► Mouse models have been instrumental in unraveling the pathology of human hereditary deafness. ► Either β-actin alone or γ-actin alone is sufficient to establish hearing. ► Both β-actin and γ-actin are essential to maintain hearing.