Annual hearing screening in children with osteogenesis imperfecta: Results from the first five years in glasgow

Background: Hearing loss is common in people with osteogenesis imperfecta (OI), although exactly how common is unknown. The prevalence of hearing loss in children with OI has been reported to be anything from 0 to 77 %. Brittle Bone Society guidelines suggest that, unless there are ear symptoms, children with OI should have their hearing tested every three years starting at age three. There is limited evidence to support this recommendation. We postulate that annual hearing screening would be easier to manage and would have a worthwhile pick-up rate. Methods: In March 2019 we began a programme of annual hearing screening for all children (ages 0-16) with OI. We collected data on age, genotype, otoscopy findings, tympanometry findings, audiometric test results and subsequent outcomes for the first five years of our programme (2019-2024). Results: Nineteen children with OI participated in the screening programme. Only one abnormality was found: a unilateral mild hearing impairment with a type B tympanogram, suggesting middle ear effusion. This was present in year 2 of the programme but resolved by year 3. Conclusion: The screening programme has a low pickup rate (5 %) for new otological problems in the paediatric population. However, we believe that the low cost and small workload associated with the screening programme justifies continuing it until further conclusions can be drawn.