Abstract
A case of osteopetrosis (marble bone disease) was encountered, in which (a) osteosclerosis centering in the craniofacial bone, (b) chronic sinusitis due to osteosclerosis and stenosis of ostium maxillare, and (c) mixed deafness caused by narrowing of the internal auditory canal and middle ear osteosclerosis were noted. Hearing impairment due to disorder of conductive components was found to be ascribable to not only exudative otitis media caused by narrowing of the auditory canal (CT finding) but also osteosclerosis of the auditory ossicle (surgical finding). Tympanoplasty was performed by modified Type III approach, where the posterior wall was conserved, while myringoplasty was performed by underlaying temporal fascia. Because the tubal function had been decreased due to marked tubal stenosis, a ventilation tube was indwelt. After operation, left hearing was improved from the preoperative value of 67.5 dB to the postoperative value of 31.3 dB. Within our literature survey, there were no reports of cases operated on or cases with osteosclerosis of auditory ossicles in association with osteopetrosis. In treatment of patients with hearing impairment associated with osteopetrosis, we think that the middle ear cavity should be actively opened to observe the pathological state, followed by surgical approach if deemed necessary.
