Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings

Middle ear ossicle malformations are an uncommon event. Among them, the congenital absence of the stapes is a very rare condition that is seldom described in the literature. We report the cases of two women, aged 19 and 22 , who presented with a long history of conductive deafness. An exploratory ty...

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Main Authors:	Jose Ignacio Undabeitia, José Undabeitia, Laura Cianci, Luis Padilla, Eduardo Petreñas, Antonio Municio
Format:	Article
Language:	English
Published:	Hindawi Limited 2014-01-01
Series:	Case Reports in Otolaryngology
Online Access:	http://dx.doi.org/10.1155/2014/901672

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spelling	doaj-900d207b4e354bf38df60c8d089eabb82020-11-24T21:01:43ZengHindawi LimitedCase Reports in Otolaryngology2090-67652090-67732014-01-01201410.1155/2014/901672901672Bilateral Congenital Absence of the Stapes Superstructure in Two SiblingsJose Ignacio Undabeitia0José Undabeitia1Laura Cianci2Luis Padilla3Eduardo Petreñas4Antonio Municio5Otolaryngology, Head and Neck Surgery Department, Cruces University Hospital, Plaza de Cruces s/n, Barakaldo, 48903 Bizkaia, SpainNeurosurgery Department, Donostia University Hospital, Calle Doctor Begiristain 117, San Sebastián, 20080 Gipuzkoa, SpainOtolaryngology, Head and Neck Surgery Department, Cruces University Hospital, Plaza de Cruces s/n, Barakaldo, 48903 Bizkaia, SpainOtolaryngology, Head and Neck Surgery Department, Cruces University Hospital, Plaza de Cruces s/n, Barakaldo, 48903 Bizkaia, SpainOtolaryngology, Head and Neck Surgery Department, Cruces University Hospital, Plaza de Cruces s/n, Barakaldo, 48903 Bizkaia, SpainOtolaryngology, Head and Neck Surgery Department, Cruces University Hospital, Plaza de Cruces s/n, Barakaldo, 48903 Bizkaia, SpainMiddle ear ossicle malformations are an uncommon event. Among them, the congenital absence of the stapes is a very rare condition that is seldom described in the literature. We report the cases of two women, aged 19 and 22 , who presented with a long history of conductive deafness. An exploratory tympanotomy was performed and the absence of the stapes superstructure and an abnormal position of the facial nerve could be observed. A bone anchored hearing aid (BAHA) was implanted in both patients with good results. It is believed that stapes agenesis is related to an abnormal development of the facial nerve, which by the 5th to 6th week of gestation would interpose between the otic capsule and the stapes blastema, preventing these structures from contacting. A long history of nonprogressive hearing loss from birth or early childhood is the key to reach a diagnosis. Several treatment options have been described. The authors opted for a hearing aid due to the high risk of facial nerve lesion, with good functional results.http://dx.doi.org/10.1155/2014/901672
collection	DOAJ
language	English
format	Article
sources	DOAJ
author	Jose Ignacio Undabeitia José Undabeitia Laura Cianci Luis Padilla Eduardo Petreñas Antonio Municio
spellingShingle	Jose Ignacio Undabeitia José Undabeitia Laura Cianci Luis Padilla Eduardo Petreñas Antonio Municio Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings Case Reports in Otolaryngology
author_facet	Jose Ignacio Undabeitia José Undabeitia Laura Cianci Luis Padilla Eduardo Petreñas Antonio Municio
author_sort	Jose Ignacio Undabeitia
title	Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings
title_short	Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings
title_full	Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings
title_fullStr	Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings
title_full_unstemmed	Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings
title_sort	bilateral congenital absence of the stapes superstructure in two siblings
publisher	Hindawi Limited
series	Case Reports in Otolaryngology
issn	2090-6765 2090-6773
publishDate	2014-01-01
description	Middle ear ossicle malformations are an uncommon event. Among them, the congenital absence of the stapes is a very rare condition that is seldom described in the literature. We report the cases of two women, aged 19 and 22 , who presented with a long history of conductive deafness. An exploratory tympanotomy was performed and the absence of the stapes superstructure and an abnormal position of the facial nerve could be observed. A bone anchored hearing aid (BAHA) was implanted in both patients with good results. It is believed that stapes agenesis is related to an abnormal development of the facial nerve, which by the 5th to 6th week of gestation would interpose between the otic capsule and the stapes blastema, preventing these structures from contacting. A long history of nonprogressive hearing loss from birth or early childhood is the key to reach a diagnosis. Several treatment options have been described. The authors opted for a hearing aid due to the high risk of facial nerve lesion, with good functional results.
url	http://dx.doi.org/10.1155/2014/901672
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Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings

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