Higashi K, Matsuki C
Department of Otolaryngology, Akita University, Japan.
Am J Otol. 1996 Jan;17(1):26-9.
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 cause by bony encroachment on nerves and vessels. This lends to progressive perceptive hearing loss and narrowing of the tympanic cavities. The result is fixation or adhesion of ossicle to tympanic walls, and narrowing of the bony part of auditory tubes, which causes serous otitis media.
有几种颅骨骨肥厚疾病伴有听力障碍。本文报告1例恩格尔曼病。目前文献报道的恩格尔曼病病例超过130例。其中约18%的病例报告有听力障碍，但这个数字可能更高，因为许多作者关注的是该疾病的其他方面，在报告中未提及听力损失情况。耳聋的病因是内耳道因骨质侵犯神经和血管而变窄。这导致进行性感音神经性听力损失和鼓室腔变窄。结果是听小骨与鼓膜壁固定或粘连，以及咽鼓管骨性部分变窄，进而引起浆液性中耳炎。
