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Hearing loss in children with osteogenesis imperfecta
K Kuurila1, R Grénman, R Johansson
1Department of Otorhinolaryngology-Head and Neck Surgery, Turku University Central Hospital, Finland. kaija.kuurila@vshp.vaasa.fi
European Journal of Pediatrics
|August 3, 2000
Summary
Hearing loss is less common in children with osteogenesis imperfecta (OI) than previously thought. Regular audiology screenings are recommended for children with OI starting at age 10.
Area of Science:
- Genetics
- Otolaryngology
- Pediatrics
Background:
- Osteogenesis imperfecta (OI) is a genetic connective tissue disorder.
- Progressive hearing loss affects approximately 50% of adult OI patients.
- Childhood hearing loss in OI exacerbates disabilities and can be managed with early treatment.
Purpose of the Study:
- To determine the prevalence and characteristics of hearing loss in a cohort of Finnish children with OI.
- To evaluate the necessity of audiological screening in pediatric OI patients.
Main Methods:
- A nationwide search identified 254 Finnish patients with OI.
- Forty-five children (aged 4-16) with OI underwent audiological evaluation, including pure tone audiometry.
- OI types were determined by a clinical geneticist.
Main Results:
- Two sporadic cases (4.4%) of OI type IV with conductive hearing loss were identified.
- One case of OI type I presented with severe sensorineural deafness, likely unrelated to OI.
- Hearing loss was less frequent in this pediatric OI cohort than generally suspected.
Conclusions:
- Hearing loss in children with osteogenesis imperfecta is less frequent than commonly believed.
- Routine audiological screening is advised for all children with OI, starting at age 10.
- Screenings should be repeated every three years for early detection and management.