MicroRNA-96 mutations effect changes in hair cell gene expression, hair cell degeneration and progressive hearing loss. (a) MicroRNA-96 sequence and mutations within the seed region (blue) found in humans (green) and the diminuendo mouse (red). (b) The balance of hair cell gene expression is offset by the diminuendo mutation in miR-96, where primary effects on target gene expression appear less in magnitude than secondary effects on the expression of other genes crucial to hair cell function. (c) Loss of crucial hair cell protein expression in diminuendo mouse contributes to hair cell degeneration and ultimate demise. The normal subcellular localization of hair cell proteins absent in diminuendo mouse is indicated.