In LER (CD−M2), Ush1c, Otogl, Gjb2, and Eps8 were associated with NSHL; Ush1c and Clrn1 with Usher syndrome, Six1 with branchiootorenal syndrome; Gjb2 with skin phenotype and HL; Gata3 with hypoparathyroidism, sensorineural hearing loss, and renal dysplasia; and Col9a2 with Stickler syndrome. The gene discussed is OTOGL; the disease is renal dysplasia.