Abstract
A case of craniometaphyseal dysplasia (CMD), which is one type of inborn cranium dysplasia, is herein reported. A 4-year-old boy visited our clinic complaining of a facial spasm. Although the pure-tone audiometry findings were with the normal range, elongations in the latencies of both the I- and V-waves, and of the I-V inter-peak latency were observed in ABR. Both external canals had become narrow but the eardrums could be observed and showed no significant problems. On a temporal bone CT scan, however, cranium cortical hyperplasia, the disappearance of the paranasal sinus, and the stenosis of both external and internal auditory canals were noted. The patient revisited our clinic at 6 year of age with a complaint of a progressive hearing loss that was confirmed by pure tone audiometry. Neither side of both eardrums could be observed due to progressive stenosis of the external canal. A re-examination of the temporal bone CT findings showed progressive stricture in both the external and internal auditory canals. The widened metaphyses of the long bones of the upper arm and the crura was observed, thus leading to a diagnosis of CMD. A bone conductive hearing aid was therefore applied to improve his hearing loss. The symptoms in such cases have been reported to progress after operative intervention. In addition, no post-operative long-term findings have yet been reported. In the follow-up, neither infection nor cholesteatoma have been observed.
