We reviewed the CT examinations of the temporal bone, performed with 1-mm-thick contiguous sections, of seven patients with the CHARGE association. We found abnormalities of the incus and stapes, with ossicular chain fixation, absence of the stapedius muscle and oval window, hypoplasia or dysplasia of the vestibule and absence of the semicircular canals in all ears. The pyramidal eminence and tympanic sinus were absent and there were anomalies of the cochlea in 13 of 14 ears. Absence of the semicircular canals is the most specific change in patients with the CHARGE association.