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Published on: January 4, 2017
Audiological findings in osteogenesis imperfecta.
Joseph P Pillion1, Jay Shapiro
1Department of Audiology, Kennedy Krieger Institute, Baltimore, MD 21231, USA. pillion@kennedykrieger.org
Journal of the American Academy of Audiology
|March 28, 2009
Summary
Hearing loss is common in Osteogenesis Imperfecta (OI) patients, affecting 62% of ears. Older patients have a higher prevalence of hearing loss and middle ear dysfunction, necessitating regular audiological monitoring.
Area of Science:
- Otolaryngology
- Genetics
- Audiology
Background:
- Osteogenesis Imperfecta (OI) is a genetic disorder causing brittle bones and frequent fractures.
- Hearing loss is a frequent comorbidity in individuals with OI.
Purpose of the Study:
- To investigate the relationship between OI type, age, and audiological findings.
- To analyze tympanometry, acoustic reflexes, and otoacoustic emissions in OI patients.
Main Methods:
- Retrospective study of 41 patients with OI, divided into two age groups (<20 and ≥20 years).
- Correlation analysis between audiometric measures, hearing loss, and OI type.
Main Results:
- 62% of ears exhibited hearing loss; 41% sensorineural/mixed, 21% conductive.
- Younger patients showed increased risk of middle ear dysfunction; older patients had more tympanometric abnormalities.
- Hearing loss prevalence was higher in older patients (88%) versus younger patients (38%).
Conclusions:
- Hearing loss is prevalent across all ages in OI patients.
- Close audiological monitoring is recommended for all individuals with OI throughout their lives.
