Congenital Malformations of the Ear

Congenital middle ear malformations are rare. Most part of them are usually associated with other malformations, such as aural atresia, microtia, and dysmorphic craniofacial features. A clinical case of a 24-year-old male with a right-sided conductive hearing loss since his childhood, without craniofacial malformation, is presented. He was proposed for exploratory tympanotomy under the suspicious diagnosis of otosclerosis. The surgery revealed an abnormal location of stapes' superstructure, which was attached to the promontory and had an isolated and mobile osseous footplate in the oval window. A stapes prosthesis was inserted and resulted in closure of the air-bone gap by 25 dB. A review of the literature was also performed using MEDLINE. Two theories diverge on the embryologic origin of the stapes. Our findings seem to be in favour of the theory that defines two different embryologic origins to the stapes.

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“…These minor middle ear abnormalities can appear to be isolated or in combination, involving more than one structure [2, 3]. …”

Section: Introductionmentioning

confidence: 99%

“…Minor abnormalities appear with a normal tympanic membrane, EAC, and pinna and may involve fixations, defects, or absence of middle ear structures (malleus, incus, stapes, oval window, and round window). These minor middle ear abnormalities can appear to be isolated or in combination, involving more than one structure [ 2 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

Abnormal Congenital Location of Stapes’ Superstructure: Clinical and Embryological Implications

Henriques

Teles

Sousa

et al. 2016

Case Reports in Otolaryngology

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Aktuelle Diagnostik und Therapie bei Ohrmuscheldysplasien und Gehörgangsfehlbildungen

Brandt,

Bodmer

2023

HNO

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ZusammenfassungAngeborene Fehlbildungen der Ohrmuschel und des äußeren Gehörgangs können mit schweren ästhetischen und funktionellen Defiziten einhergehen. Neben dem Verständnis embryologischer Grundlagen ist auch die Klassifikation derartiger Anomalien für die Behandlung essenziell. Die frühzeitige Erkennung einer Fehlbildung sowie die Einleitung zeitgerechter Diagnostik sind essenziell, um durch eine adäquate Therapie langfristige Einschränkungen zu verhindern. Ohrmuscheldysplasien werden heute meist operativ, seltener mittels Epithese korrigiert. Die Methoden des „tissue engineering“ sind seit Langem in der Erprobung und könnten in Zukunft eine wesentliche Rolle spielen. Die Behandlung von Gehörgangsstenosen und -atresien bezweckt neben einer Hörverbesserung auch die Verhinderung von Folgeerkrankungen. Darunter finden sich u. a. Cholesteatome oder rezidivierende Infekte. Die Hörrehabilitation umfasst konventionelle und implantierbare Hörgeräte, wobei der Trend zu Letzteren tendiert.

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Congenital Aural Atresia: Hearing Rehabilitation by Bone-Anchored Hearing Implant (BAHI)

Ricci¹,

Stadio²,

Gambacorta³

et al. 2020

Advances in Rehabilitation of Hearing Loss

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Auris atresia (AA) is a congenital pathology characterized by aplasia or hypoplasia of the external ear with associated middle ear malformation. The AA has a different degree of severity, and the severe form of the disorder presents no identifiable ear canal (complete atresia) and absence or significative underdevelopment of the middle ear structures. Sometimes AA is associated with a malformation of the ear called "microtia." The alterations of the external auditory canal and of the middle ear structures are responsible for the conductive hearing loss which affects the patients. The hearing restoration procedures may recreate a normal external and middle ear anatomy to favor the recovery of the hearing function, or the surgeon may simply restore the hearing capacity through bypassing the malformed structures by boneanchored hearing implants (BAHIs). The restoring of normal anatomy is generally associated with episode of restenosis of the external ear canal due to bony regrowth. The formulation of a therapeutic strategy may be supported by using Jahrsdoerfer classification to identify the severity of malformation. In the chapter we discuss various bone anchoring prostheses currently used (Baha, Ponto, Alpha2 by Sophono, Bonebridge) and the results that can be obtained by the use of these implants.

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Congenital Malformations of the Ear

Cited by 3 publications

References 145 publications

Abnormal Congenital Location of Stapes’ Superstructure: Clinical and Embryological Implications

Abnormal Congenital Location of Stapes’ Superstructure: Clinical and Embryological Implications

Aktuelle Diagnostik und Therapie bei Ohrmuscheldysplasien und Gehörgangsfehlbildungen

Congenital Aural Atresia: Hearing Rehabilitation by Bone-Anchored Hearing Implant (BAHI)

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