Stapes surgery in osteogenesis imperfecta: retrospective analysis of 34 operated ears

Objectives : In Osteogenesis Imperfecta (OI) a conductive hearing loss may arise during childhood or young adulthood, which is usually due to an otosclerosis-like pathology involving stapes footplate fixation whether or not in combination with atrophic or fractured crura. Usually the hearing loss evolves to a mixed hearing loss over time. The benefits of stapes surgery, partially or totally replacing the stapes by a prosthesis, have been a controversial matter in the OI population because of the progressive course of the sensorineural component. We present the short- and long-term postoperative audiometric results in a new series of stapes surgery in OI and compare them with the results from other series in literature.Methods : Surgery reports and preoperative, short-term postoperative and long-term (U+2265 12 months) postoperative audiometric results from 34 operated ears originating from 22 Dutch and Belgian OI patients operated at different institutes were obtained retrospectively. A primary stapes operation was performed in 29 ears, and from 5 other ears we obtained the results of revision stapes surgery.Results : At surgery all ears demonstrated fixation of the stapes footplate in the oval window, impeding the normal transmission of sound waves to the inner ear. Additionally, footplates were mentioned thickened or fragile, stapes crura atrophic or fractured, and the middle ear mucosa thickened or hypervascularized. The post-operative audiometric follow-up period ranged from 1 month to 37 years. Postoperative audiometry in the short-term revealed improved hearing and reduced air-bone gaps in 28 out of 29 ears with primary stapes surgery and in all ears with revision surgery after a second revision operation was performed in one ear. In all the 22 ears with long-term postoperative follow-up, which had an average duration of 15 years, a hearing gain was still measurable at the latest audiometric evaluation.Conclusions : Beneficial results are obtained in the majority of OI patients undergoing stapes surgery for the reduction of a conductive hearing loss component caused by stapes footplate fixation. Despite the progressive course of the concomitant sensorineural hearing loss component, the hearing gain remains beneficial over the years.