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Longitudinal analysis of the audiological phenotype in osteogenesis imperfecta: a follow-up study

Published online by Cambridge University Press:  18 June 2018

S Martens ,
I J M Dhooge  and
F K R Swinnen
S Martens
Affiliation:
Department of Speech, Language and Hearing Sciences, Faculty of Medicine and Health Sciences, Ghent University, Belgium
I J M Dhooge
Affiliation:
Department of Otorhinolaryngology, Ghent University Hospital, Belgium
F K R Swinnen*
Affiliation:
Department of Otorhinolaryngology, Ghent University Hospital, Belgium
*
Author for correspondence: Dr Freya Swinnen, Department of Otorhinolaryngology (2P1), Ghent University Hospital, De Pintelaan 185, B-9000 Ghent, Belgium E-mail: Freya.Swinnen@UGent.be Fax: +32 9 332 49 93
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Abstract

Objective

This prospective study involved a longitudinal analysis of the progression of hearing thresholds in patients with osteogenesis imperfecta.

Methods

Audiometric results from 36 osteogenesis imperfecta patients (age range, 6–79 years) were compared between two test times with an average interval of 4 years. Audiometric evaluation included acoustic admittance measurements, acoustic stapedial reflex measurements, pure tone audiometry and otoacoustic emissions testing.

Results

Air conduction pure tone average, corrected for sex and age, and bone conduction pure tone average increased significantly in the study population (p < 0.05 and p < 0.001, respectively). In 14.3 per cent of the evaluated ears, an alteration in type and/or severity of hearing loss was observed.

Conclusion

After an average time interval of four years, significant changes in hearing status occurred in a population of osteogenesis imperfecta patients. These findings highlight the importance of regular audiological follow up in osteogenesis imperfecta patients, including audiometry, and measurements of acoustic admittance, acoustic stapedial reflexes and otoacoustic emissions.

Keywords

Osteogenesis ImperfectaHearing LossLongitudinal Studies
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 132 , Issue 8 , August 2018 , pp. 703 - 710
Copyright
Copyright © JLO (1984) Limited, 2018 

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Footnotes

Dr F Swinnen takes responsibility for the integrity of the content of the paper

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