A 39 year-old-female with a chief complaint of hearing impairment was found to have van Buchem'sdisease which is a rare malformation of the bone, characterized by abnormal subperiosteal bone hyperplasia.
The disease can be differentiated from marble bone disease and other hypertrophic bone diseases byradiographical findings.
This present case, the third reported case in Japan, was described with special references to otorhinolaryngologicalfindings together with a review of the literature.
