There are several hyperostosis diseases of the skull that are accompanied by hearing impairment. In this article a case of Engelmann disease is presented. Currently > 130 cases of Engelmann disease are reported in literature. About 18% of these cases reported hearing impairment, but it is possible that this figure may be higher, because many of the authors concentrated on other aspects of the disease and made no reference to hearing loss in their reports. The etiology of deafness is the narrowing of the internal auditory canals caused by bony encroachment on nerves and vessels. This leads to progressive perceptive hearing loss and narrowing of the tympanic cavities. The result is fixation or adhesion of ossicles to tympanic walls, and narrowing of the bony part of auditory tubes, which causes serous otitis media.