Stapes Surgery in Osteogenesis Imperfecta : Results of a New Series

Objective: To evaluate the results of stapes surgery in osteogenesis imperfecta. Study Design: Audiological and clinical analysis of the results of stapes surgery in a consecutive series of 15 ears in 13 patients with osteogenesis imperfecta. Setting: Tertiary referral center. Results: Stapedotomy was successful in 11 of 12 ears in 11 patients with osteogenesis imperfecta type I. Air-bone gaps were within 10 dB in four ears and within 20 dB in the seven remaining ears. In one ear, revision surgery with malleovestibulopexy was needed to achieve this result. In another ear, the initial good result within 20-dB air-bone gap decreased to 28 dB; therefore, it is not considered as a success. In one other patient, revision surgery resulted in only marginal improvement in the hearing level. In one case of, probably, osteogenesis imperfecta type III, surgery resulted in a dead ear. Conclusion: Stapedotomy successfully improved the hearing of patients with osteogenesis type I. These results are in accordance with those reported in a few previous series. It is questionable whether stapes surgery will be as successful in osteogenesis imperfecta types other than type I. Stapedotomy-Osteogenesis imperfecta-Genetic hearing impairment-COLIA1 gene.