Longitudinal analysis of the audiological phenotype in osteogenesis imperfecta: a follow-up study.

Objective: This prospective study involved a longitudinal analysis of the progression of hearing thresholds in patients with osteogenesis imperfecta. Methods: Audiometric results from 36 osteogenesis imperfecta patients (age range, 6–79 years) were compared between two test times with an average interval of 4 years. Audiometric evaluation included acoustic admittance measurements, acoustic stapedial reflex measurements, pure tone audiometry and otoacoustic emissions testing. Results: Air conduction pure tone average, corrected for sex and age, and bone conduction pure tone average increased significantly in the study population (p < 0.05 and p < 0.001, respectively). In 14.3 per cent of the evaluated ears, an alteration in type and/or severity of hearing loss was observed. Conclusion: After an average time interval of four years, significant changes in hearing status occurred in a population of osteogenesis imperfecta patients. These findings highlight the importance of regular audiological follow up in osteogenesis imperfecta patients, including audiometry, and measurements of acoustic admittance, acoustic stapedial reflexes and otoacoustic emissions. [ABSTRACT FROM AUTHOR]