In cases of isolated congenital stapes superstructure fixation, a bony bar between the stapes and the pyramidal eminence and a normal stapedius tendon is unusual.1 Hearing loss, however, rarely seems to occur as a result; to our knowledge, only 3 cases have been reported to date.2,3 Kinsella and Kerr3 reported 2 of these cases, in which removal of the bony bar resulted in normal hearing for 2 family members. We also report familial cases spanning 3 generations.