Financial Disclosure: None.
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.
Doi T, Nakazawa H, Adachi M, Kaneko T, Munemoto Y, Komeda M, Ikeda H, Kitajiri M, Yamashita T. Familial Isolated Stapes-Pyramidal Fixation by a Bony Bar With Normal Stapedius Tendon. Arch Otolaryngol Head Neck Surg. 2005;131(4):349-352. doi:10.1001/archotol.131.4.349