Of these patients, 101 (23 %) received a genetic diagnosis implicating an NSHL mimic, which included Usher syndrome (59 patients), Pendred syndrome (29 patients), Deafness-infertility syndrome (6 males and 1 female with NSHL), Alström syndrome (1 patient), autosomal dominant non-ocular Stickler syndrome (1 patient), branchiootorenal syndrome (BOR) (2 patients), MYH9-associated disease (1 patient), and Wolfram syndrome (1 patient) (Table S4). This evidence concerns the gene MYH9 and deafness-infertility syndrome.