Background Definition primary metabolic bone disease of the otic capsule and ossicles It causes fixation of the ossicles(stapes u It results in conductive or mixed hearing loss It is genetically-mediated via autosomal dominant transmissionBackground ◼ Definition ◼ primary metabolic bone disease of the otic capsule and ossicles ◼ It causes fixation of the ossicles (stapes) ◼ It results in conductive or mixed hearing loss. ◼ It is genetically-mediated via autosomal dominant transmission