Osteogenesis Imperfecta and Hearing Loss

Abstract

Objective: 1) Determine the incidence and prevalence of hearing loss in the pediatric population with osteogenesis imperfecta. 2) Determine the average age of onset of hearing loss. 3) Determine the type and severity of hearing loss.

Method: This is a retrospective chart review of audiological data from 127 patients, 19 years or younger, with osteogenesis imperfecta who were seen in the orthopedic clinic at Childrens Hospital. Data in the form of pure tone audiometry, sound field audiometry, tympanometry, and otoacoustic emissions was collected and reviewed. The prevalence, incidence, age of onset, type, and severity of hearing loss were determined.

Results: The prevalence of hearing loss was 24% and the incidence was 9%. The mean age of onset was 5.8 years. A conductive hearing (50-56%) loss was more common than sensorineural (39-44%) or mixed (11%) hearing loss. The majority of patients had a mild hearing loss (67%).

Conclusion: Hearing loss was found in 24% of patients. The average age of onset was 5.8 years. Our data supports screening children with osteogenesis imperfecta for hearing loss starting at a young age.