Hearing relies on the proper functioning of auditory hair cells and on actin-based cytoskeletal structures. Diaphanous-related formins (DRFs) are evolutionarily conserved cytoskeletal proteins that regulate the nucleation of linear unbranched actin filaments. They play key roles during metazoan development, and they seem particularly pivotal for the correct physiology of the reproductive and auditory systems. Indeed, in , a single diaphanous (dia) gene is present, and mutants show sterility and impaired response to sound. Vertebrates, instead, have three orthologs of the diaphanous gene: , , and . In humans, defects in and have been associated with different types of hearing loss. In particular, heterozygous mutations in are responsible for autosomal dominant deafness with or without thrombocytopenia (, MIM #124900), whereas regulatory mutations inducing the overexpression of cause autosomal dominant auditory neuropathy 1 (, MIM #609129). Here, we provide an overview of the expression and function of DRFs in normal hearing and deafness.