Stapes surgery in osteogenesis imperfecta in Finland

Abstract

We present the surgical findings and audiometric results of ear surgery performed between 1961 and 2002 on 33 Finnish patients (43 operations) with osteogenesis imperfecta (OI). The mean age at the time of the first operation was 30.1 years. The typical surgical findings were a thick, fixed, or obliterated footplate, thick and vascular mucosa with an excessive tendency to bleed, and elastic, fractured, or atrophic stapes crura. As compared with previous studies, the hearing gain was poorer and the remaining postoperative gap was greater for the 43 operations analyzed. The results of this nationwide study, however, may not be directly comparable with operative results of non-population studies. On the other hand, the hearing gain in our study was better in university hospitals than in central hospitals and, furthermore, was comparable with that of previous studies after surgery performed by a single surgeon in a university hospital. Conductive hearing loss related to OI may be successfully treated with surgery in most patients. The rarity of the disease, leading to small annual numbers of operations, the variable surgical findings, and the profuse bleeding tendency of the middle ear, as well as the audiometric results in this study, support centralization of ear surgery in OI patients.