抄録
In 1987, we have reported the temporal bone histopathology from a patient with CHARGE association (a 5-month-old girl) showing unusual but characteristic inner ear anomalies as in the other two reported cases. Aplasia of the pars superior (absence of the entire semicircular canals and utricle) and hypoplasia of the pars inferior was observed in both temporal bones. In addition to the two cases of CHARGE association, a review of the ilterature revealed six previous reports of the unusual form of aplasia of the pars superior. Two of the 6 cases were observed to have similar clinical features to the CHARGE association. According to Schuknecht's classification (1967), this anomaly should be classified with Mondini dysplasia, although it is close to the Michel from of aplasia. As proposed by Guyot et al (1987), this specific form of anomaly (aplasia of the pars superior) may become new category in the classification of inner ear anomalies.
