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

  • Otology
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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.

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Funding

For examinations and appointments, this research was funded by the institution's Research and Events Incentive Fund, which is linked to the Institution's Research and Graduate Group. Funding was provided by Hospital de Clínicas de Porto Alegre (Grant no. 2016-0455)

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Correspondence to Andressa Colares da Costa Otavio.

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The authors declare that they have no conflict of interest.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee (Research Ethics Committee of the Institution—CAAE 58621916.0.0000.5327, approval number 1718791) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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da Costa Otavio, A.C., Teixeira, A.R., Félix, T.M. et al. Osteogenesis imperfecta and hearing loss: an analysis of patients attended at a benchmark treatment center in southern Brazil. Eur Arch Otorhinolaryngol 277, 1005–1012 (2020). https://doi.org/10.1007/s00405-020-05799-x

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