Osteogenesis imperfecta and hearing loss: an analysis of patients attended at a benchmark treatment center in southern Brazil

Abstract

Objective: To characterize the audiological findings of a sample of patients with osteogenesis imperfecta (OI) in southern Brazil.

Methods: This was a cross-sectional, observational, quantitative study. Research was carried out at a hospital which is considered to offer benchmark treatment for patients with OI in southern Brazil. Seventy-seven patients were recruited, at ages between 5 and 55 years; the mean age was 21.9 ± 14.3 years. Patients were divided into three age groups: 10 and under, 10-19 and over 19. During our study, peripheral audiological assessments were performed (pure tone testing and acoustic immittance measurements). The main outcome measures taken into account were airway thresholds, bone conduction, air-bone gap and compliance values between compared frequencies. Data were analyzed per ear.

Results: Normal hearing thresholds were found in 96 (64.4%) ears of the total sample. When analysis was stratified into age groups, normal hearing thresholds were found in 81.3%, 65%, and 54.4%, of the children, adolescent and adult groups, respectively. Concerning hearing impairments, there was a predominance of mixed type hearing loss in adults (21.1%) whereas adolescents presented conductive hearing loss or a conductive loss factor, while maintaining airway thresholds within the bounds of normality (30%). Ears with hearing loss showed superior compliance means than ears without hearing loss (p = 0.002).

Conclusions: Overall, the majority of the subjects in this patient sample presented normal hearing thresholds. When present, hearing impairments were more prevalent in the adult group than in the adolescent or children's groups.

Keywords: Hearing; Hearing loss; Osteogenesis; Osteogenesis imperfecta.