Here, we report the identification of a new deafness gene, which encodes an ELMO/CED 12 domain containing protein, ELMOD3. Our biochemical studies demonstrated that ELMOD3 possesses GAP activity against a small GTPase in the Arf family, Arl2, providing a functional link between Arf family signaling pathways and stereocilia actin-based cytoskeletal architecture. This evidence concerns the gene ELMOD3 and deafness.