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