Familial isolated stapes-pyramidal fixation by a bony bar with normal stapedius tendon.

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 Kerr 3 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.