Craniometaphyseal dysplasia (CMD) is a genetic bone disorder involving an abnormality of modeling of the long bones and sclerosis of the cranium. Both conductive and sensorineural hearing may be frequently associated with this syndrome. Conductive hearing loss is due to attic fixation of the lateral ossicular chain and hyperostosis formation with stapes ankylosis. Two patients with CMD were treated surgically. Two of three ears operated on had closure of the air-bone gap, whereas the third ear failed ossicular reconstruction because of hyperostosis of the promontory with deepening of the oval window.