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Is it necessary to screen for hearing loss in the paediatric population with osteogenesis imperfecta?
P Imani1, S Vijayasekaran, F Lannigan
1Princess Margaret Hospital for Children, University Department of Otolaryngology-Head and Neck Surgery, University of Western Australia.
Insights
Routine hearing screening is recommended for children with osteogenesis imperfecta. Early detection of hearing loss, including sensorineural and conductive types, is crucial in this population.
Area of Science:
- Pediatric audiology
- Genetics and rare diseases
Background:
- Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by brittle bones.
- Hearing loss is a known complication of OI, but its typical onset is reported in adulthood.
Observation:
- This study evaluated hearing in 22 children with OI over 5 years.
- A significant majority (77.3%) of children exhibited some form of hearing loss.
- Commonly observed were conductive hearing losses, often associated with otitis media with effusion.
Findings:
- Conductive hearing loss was prevalent (63.6%), frequently resolving with treatment for otitis media with effusion.
- Sensorineural hearing loss was identified in 13.6% of the children, with one case detected at 1 year of age.
- The median age of hearing loss detection (9 years) was significantly earlier than previously suggested for OI.
Implications:
- Early and routine hearing screening in pediatric OI populations is essential.
- Timely identification and management of hearing loss can prevent long-term auditory complications.
- Findings challenge the traditional understanding of hearing loss onset in osteogenesis imperfecta.
Abstract:
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.