We conducted a retrospective chart review at a university hospital on a family that exhibited a rare cause of conductive hearing loss. Four male patients of this family had congenital conductive hearing loss. Three of these family members underwent bilateral ossiculoplasty, a father and two of his three sons. All three were found to have mild or moderate conductive hearing loss due to fixation of the head of the stapes by a bony bar that extended from the head of the stapes to the posterior ear canal wall. Surgical removal of the bony bar improved hearing in all cases. This family exhibits autosomal-dominant inheritance of this abnormality. Fixation of the head of the stapes by a bony bar that extends to the posterior ear canal wall is a rare cause of congenital, autosomal-dominant, mild or moderate conductive hearing loss that can be surgically corrected.