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Annual hearing screening in children with osteogenesis imperfecta: Results from the first five years in glasgow
Emmett Lui1, Owen Conlan2, Karen Hunter3
1Department of Child Health, University of Glasgow, Glasgow, G12 8QQ, Scotland, UK; Department of Otolaryngology, Royal Hospital for Children, 1345 Govan Road, Glasgow, G51 4TF, Scotland, UK.
Insights
Annual hearing screening for children with osteogenesis imperfecta (OI) identified few new ear problems. This low-cost screening is recommended to continue due to its manageable workload and potential for early detection.
Area of Science:
- Pediatric Otolaryngology
- Genetics and Rare Diseases
Background:
- Hearing loss is a frequent concern in osteogenesis imperfecta (OI), with reported prevalence in children ranging widely from 0% to 77%.
- Current guidelines recommend hearing tests every three years for children with OI, starting at age three, but evidence supporting this frequency is limited.
- Annual screening is proposed as a more manageable approach with a potentially valuable detection rate.
Purpose of the Study:
- To evaluate the effectiveness and yield of an annual hearing screening program for children diagnosed with osteogenesis imperfecta (OI).
Main Methods:
- An annual hearing screening program was implemented for children (ages 0-16) with OI from March 2019 to 2024.
- Data collected included patient age, genotype, otoscopy, tympanometry, audiometric results, and outcomes.
- Analysis focused on the first five years of the screening program.
Main Results:
- Nineteen children with OI participated in the screening.
- One case of unilateral mild hearing impairment with middle ear effusion (type B tympanogram) was detected in year two, which resolved by year three.
- The overall pickup rate for new otological issues was low, approximately 5%.
Conclusions:
- The annual hearing screening program demonstrated a low incidence of newly identified otological problems in the pediatric OI population.
- Despite the low pickup rate, the program's minimal cost and workload support its continuation for further data collection.
- Continued screening may offer long-term benefits in monitoring hearing health in children with OI.
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.
