A familial case of Engelmann disease with hearing impairment

Abstract

Engelmann's disease is an uncommon genetic disease characterized by symmetrical hyperostosis of the diaphysis of the long bone and skull base. A patient with this disease and concomitant hearing loss in the family are presented. The propositus was a 44-year-old man who complained of bilateral hearing loss and tinnitus but without leg pain. He had had mild conductive hearing loss in the right ear due to bony involvement of ossicle, and mild sensorineural hearing loss in the left ear due to acoustic nerve compression in the internal auditory canal. A 23-year-old woman in the same family had a 12-year history of severe bilateral leg pain. She also demonstrated complete deafness in the left ear due to acoustic nerve compression in the internal auditory canal.