Is it necessary to screen for hearing loss in the paediatric population with osteogenesis imperfecta?

The aim of the study was to assess the necessity of a screening service to detect early hearing loss in the paediatric population with osteogenesis imperfecta. Twenty-two children were assessed over a 5-year period. Five children (22.7%) had normal hearing. Fourteen (63.6%) had conductive hearing loss, with 12 children in this group having otitis media with effusion (OME); all had resolution of hearing loss with appropriate therapy. Two children had persistent conductive losses unrelated to OME. Three children (13.6%) had sensorineural hearing loss, with one being detected at the age of 1 year. Existing evidence suggests that hearing loss associated with osteogenesis imperfecta has its onset in the second to third decade of life. Contrary to this, hearing loss was detected in 77.3% (17) of this population with a median and mean age of 9 years. This study would suggest that routine screening is worthwhile in children with osteogenesis imperfecta.