Integrins are heterodimeric transmembrane receptors that mediate bidirectional interactions between the intracellular cytoskeletal array and the extracellular matrix. This review focuses on recent advances regarding the function of integrin alpha 8 beta 1 (α8β1) during organ development, with a particular interest in kidney and inner ear development. The paper discusses α8β1’s role in injury and disease, its importance for mesenchymal to epithelial transition during cancer development, and its potential as a diagnostic and therapeutic tool for hearing function and disease elimination.