In 1971, Nance et al.4first reported X‐linked mixed deafness with congenital fixation of the stapes and perilymphatic gusher. Mutations in the POU3F4 gene were first described in 1995 after its localization to the X chromosome in 1988 (Xq21 band).5, 6. Here, POU3F4 is linked to deafness.