Observed Frequency and Characteristics of Hearing Loss in Osteogenesis Imperfecta

ABSTRACT Background: Osteogenesis imperfecta (OI) is a rare group of genetic disorders affecting connective tissue, with consequent bone fragility, frequent fractures and skeletal deformity. Depending on the type, patients can have blue sclera, dentinogenesis imperfecta, and hearing loss. Aim: To...

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Autores principales:	Waissbluth,Sofia, Lira,Karina, Aracena,Karina, Oyarzun,Javier, Willson,Matias, Seiltgens,Cristián
Lenguaje:	English
Publicado:	Sociedad Médica de Santiago 2020
Materias:	Hearing Loss Osteogenesis Imperfecta Otosclerosis Reflex, Acoustic
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872020001201781
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spelling	oai:scielo:S0034-988720200012017812021-04-04Observed Frequency and Characteristics of Hearing Loss in Osteogenesis ImperfectaWaissbluth,SofiaLira,KarinaAracena,KarinaOyarzun,JavierWillson,MatiasSeiltgens,Cristián Hearing Loss Osteogenesis Imperfecta Otosclerosis Reflex, Acoustic ABSTRACT Background: Osteogenesis imperfecta (OI) is a rare group of genetic disorders affecting connective tissue, with consequent bone fragility, frequent fractures and skeletal deformity. Depending on the type, patients can have blue sclera, dentinogenesis imperfecta, and hearing loss. Aim: To determine the frequency, type and audiometric characteristics of hearing loss in a group of patients with OI. Material and Methods: A prospective cohort study was completed. A clinical and diagnostic hearing evaluation with tympanometry, acoustic stapedial reflex, pure-tone and speech audiometry were performed. Results: Thirty patients completed the study; mean age of 22 years (range 6-63 years). Sixty seven percent had a type I OI. Overall, nine (30%) patients had hearing loss (15/60 ears). Of these, six had bilateral hearing loss. Of the 15 affected ears, six showed conductive hearing loss, five sensorineural hearing loss, and four mixed hearing loss. Patients with hearing loss were older than patients with normal hearing. Only one pediatric patient developed hearing loss. Of the ears without hearing loss, 13% did not have an acoustic stapedial reflex. Conclusions: In this group of patients with OI, 30% had hearing loss and among those ears with normal hearing, 13% did not have an acoustic stapedial reflex. Patients with OI should be monitored for hearing loss.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.148 n.12 20202020-12-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872020001201781en10.4067/S0034-98872020001201781
institution	Scielo Chile
collection	Scielo Chile
language	English
topic	Hearing Loss Osteogenesis Imperfecta Otosclerosis Reflex, Acoustic
spellingShingle	Hearing Loss Osteogenesis Imperfecta Otosclerosis Reflex, Acoustic Waissbluth,Sofia Lira,Karina Aracena,Karina Oyarzun,Javier Willson,Matias Seiltgens,Cristián Observed Frequency and Characteristics of Hearing Loss in Osteogenesis Imperfecta
description	ABSTRACT Background: Osteogenesis imperfecta (OI) is a rare group of genetic disorders affecting connective tissue, with consequent bone fragility, frequent fractures and skeletal deformity. Depending on the type, patients can have blue sclera, dentinogenesis imperfecta, and hearing loss. Aim: To determine the frequency, type and audiometric characteristics of hearing loss in a group of patients with OI. Material and Methods: A prospective cohort study was completed. A clinical and diagnostic hearing evaluation with tympanometry, acoustic stapedial reflex, pure-tone and speech audiometry were performed. Results: Thirty patients completed the study; mean age of 22 years (range 6-63 years). Sixty seven percent had a type I OI. Overall, nine (30%) patients had hearing loss (15/60 ears). Of these, six had bilateral hearing loss. Of the 15 affected ears, six showed conductive hearing loss, five sensorineural hearing loss, and four mixed hearing loss. Patients with hearing loss were older than patients with normal hearing. Only one pediatric patient developed hearing loss. Of the ears without hearing loss, 13% did not have an acoustic stapedial reflex. Conclusions: In this group of patients with OI, 30% had hearing loss and among those ears with normal hearing, 13% did not have an acoustic stapedial reflex. Patients with OI should be monitored for hearing loss.
author	Waissbluth,Sofia Lira,Karina Aracena,Karina Oyarzun,Javier Willson,Matias Seiltgens,Cristián
author_facet	Waissbluth,Sofia Lira,Karina Aracena,Karina Oyarzun,Javier Willson,Matias Seiltgens,Cristián
author_sort	Waissbluth,Sofia
title	Observed Frequency and Characteristics of Hearing Loss in Osteogenesis Imperfecta
title_short	Observed Frequency and Characteristics of Hearing Loss in Osteogenesis Imperfecta
title_full	Observed Frequency and Characteristics of Hearing Loss in Osteogenesis Imperfecta
title_fullStr	Observed Frequency and Characteristics of Hearing Loss in Osteogenesis Imperfecta
title_full_unstemmed	Observed Frequency and Characteristics of Hearing Loss in Osteogenesis Imperfecta
title_sort	observed frequency and characteristics of hearing loss in osteogenesis imperfecta
publisher	Sociedad Médica de Santiago
publishDate	2020
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872020001201781
work_keys_str_mv	AT waissbluthsofia observedfrequencyandcharacteristicsofhearinglossinosteogenesisimperfecta AT lirakarina observedfrequencyandcharacteristicsofhearinglossinosteogenesisimperfecta AT aracenakarina observedfrequencyandcharacteristicsofhearinglossinosteogenesisimperfecta AT oyarzunjavier observedfrequencyandcharacteristicsofhearinglossinosteogenesisimperfecta AT willsonmatias observedfrequencyandcharacteristicsofhearinglossinosteogenesisimperfecta AT seiltgenscristian observedfrequencyandcharacteristicsofhearinglossinosteogenesisimperfecta
_version_	1718437172231536640

Observed Frequency and Characteristics of Hearing Loss in Osteogenesis Imperfecta

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