A bone conduction implantable device as a functional treatment option in unilateral microtia with bilateral stapes ankylosis: A report of two cases

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Abstract

Objective: Congenital defects/diseases Background: Implantable devices have been proposed as an alternative to hearing aids and auditory canal reconstruction in patients with microtia (congenital aural atresia), which includes a malformation of the external and middle ear. This report is of two rare cases of microtia associated with congenital stapes ankylosis treated with an implantable device and describes the treatment outcomes. Case Report: Two siblings from Ecuador, a 29-year-old woman, and her 35-year-old brother, were born with unilateral type II microtia with bilateral external auditory canal atresia and conductive hearing loss. Pre-operatively, high-resolution computed tomography (HRCT) imaging was performed using FastView software to allow placement of a bone conduction-floating mass transducer (BC-FMT) to couple a Bonebridge bone conduction implant (BCI) system in both patients. Pure-tone audiometry (PTA) testing and speech audiology were performed. The Abbreviated Profile of Hearing Aid Benefit (APHAB) and the Speech, Spatial and Qualities (SSQ) of hearing scale questionnaires and scoring systems were used. Following activation of the implantable device, both patients achieved improved bilateral conductive hearing with sound-field (field-free) thresholds >25 dB, and speech recognition scores >90%. In both cases, hearing improvement remained at three years following surgery. Conclusions: To our knowledge, these are the first reported cases of microtia with congenital stapes ankylosis successfully treated with a bone conduction implantable device. Patients with microtia and stapes ankylosis who are reluctant to undergo surgery may benefit from unilateral or bilateral, short-term or long-term use of a Bonebridge bone conduction implantable device.

Original languageEnglish
Pages (from-to)82-89
Number of pages8
JournalAmerican Journal of Case Reports
Volume19
DOIs
Publication statusPublished - Jan 23 2018

Fingerprint

Bone Conduction
Stapes
Ankylosis
Equipment and Supplies
Hearing
Hearing Aids
Siblings
Audiology
Conductive Hearing Loss
Pure-Tone Audiometry
Ecuador
External Ear
Therapeutics
Ear Canal
Middle Ear
Transducers
Software
Tomography
Congenital Microtia

Keywords

  • Bone conduction
  • Congenital abnormalities
  • Correction of hearing impairment
  • Ear, external
  • Hearing aids
  • Hearing loss, bilateral

ASJC Scopus subject areas

  • Medicine(all)

Cite this

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title = "A bone conduction implantable device as a functional treatment option in unilateral microtia with bilateral stapes ankylosis: A report of two cases",
abstract = "Objective: Congenital defects/diseases Background: Implantable devices have been proposed as an alternative to hearing aids and auditory canal reconstruction in patients with microtia (congenital aural atresia), which includes a malformation of the external and middle ear. This report is of two rare cases of microtia associated with congenital stapes ankylosis treated with an implantable device and describes the treatment outcomes. Case Report: Two siblings from Ecuador, a 29-year-old woman, and her 35-year-old brother, were born with unilateral type II microtia with bilateral external auditory canal atresia and conductive hearing loss. Pre-operatively, high-resolution computed tomography (HRCT) imaging was performed using FastView software to allow placement of a bone conduction-floating mass transducer (BC-FMT) to couple a Bonebridge bone conduction implant (BCI) system in both patients. Pure-tone audiometry (PTA) testing and speech audiology were performed. The Abbreviated Profile of Hearing Aid Benefit (APHAB) and the Speech, Spatial and Qualities (SSQ) of hearing scale questionnaires and scoring systems were used. Following activation of the implantable device, both patients achieved improved bilateral conductive hearing with sound-field (field-free) thresholds >25 dB, and speech recognition scores >90{\%}. In both cases, hearing improvement remained at three years following surgery. Conclusions: To our knowledge, these are the first reported cases of microtia with congenital stapes ankylosis successfully treated with a bone conduction implantable device. Patients with microtia and stapes ankylosis who are reluctant to undergo surgery may benefit from unilateral or bilateral, short-term or long-term use of a Bonebridge bone conduction implantable device.",
keywords = "Bone conduction, Congenital abnormalities, Correction of hearing impairment, Ear, external, Hearing aids, Hearing loss, bilateral",
author = "Diego Zanetti and {Di Berardino}, Federica",
year = "2018",
month = "1",
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doi = "10.12659/AJCR.904907",
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T1 - A bone conduction implantable device as a functional treatment option in unilateral microtia with bilateral stapes ankylosis

T2 - A report of two cases

AU - Zanetti, Diego

AU - Di Berardino, Federica

PY - 2018/1/23

Y1 - 2018/1/23

N2 - Objective: Congenital defects/diseases Background: Implantable devices have been proposed as an alternative to hearing aids and auditory canal reconstruction in patients with microtia (congenital aural atresia), which includes a malformation of the external and middle ear. This report is of two rare cases of microtia associated with congenital stapes ankylosis treated with an implantable device and describes the treatment outcomes. Case Report: Two siblings from Ecuador, a 29-year-old woman, and her 35-year-old brother, were born with unilateral type II microtia with bilateral external auditory canal atresia and conductive hearing loss. Pre-operatively, high-resolution computed tomography (HRCT) imaging was performed using FastView software to allow placement of a bone conduction-floating mass transducer (BC-FMT) to couple a Bonebridge bone conduction implant (BCI) system in both patients. Pure-tone audiometry (PTA) testing and speech audiology were performed. The Abbreviated Profile of Hearing Aid Benefit (APHAB) and the Speech, Spatial and Qualities (SSQ) of hearing scale questionnaires and scoring systems were used. Following activation of the implantable device, both patients achieved improved bilateral conductive hearing with sound-field (field-free) thresholds >25 dB, and speech recognition scores >90%. In both cases, hearing improvement remained at three years following surgery. Conclusions: To our knowledge, these are the first reported cases of microtia with congenital stapes ankylosis successfully treated with a bone conduction implantable device. Patients with microtia and stapes ankylosis who are reluctant to undergo surgery may benefit from unilateral or bilateral, short-term or long-term use of a Bonebridge bone conduction implantable device.

AB - Objective: Congenital defects/diseases Background: Implantable devices have been proposed as an alternative to hearing aids and auditory canal reconstruction in patients with microtia (congenital aural atresia), which includes a malformation of the external and middle ear. This report is of two rare cases of microtia associated with congenital stapes ankylosis treated with an implantable device and describes the treatment outcomes. Case Report: Two siblings from Ecuador, a 29-year-old woman, and her 35-year-old brother, were born with unilateral type II microtia with bilateral external auditory canal atresia and conductive hearing loss. Pre-operatively, high-resolution computed tomography (HRCT) imaging was performed using FastView software to allow placement of a bone conduction-floating mass transducer (BC-FMT) to couple a Bonebridge bone conduction implant (BCI) system in both patients. Pure-tone audiometry (PTA) testing and speech audiology were performed. The Abbreviated Profile of Hearing Aid Benefit (APHAB) and the Speech, Spatial and Qualities (SSQ) of hearing scale questionnaires and scoring systems were used. Following activation of the implantable device, both patients achieved improved bilateral conductive hearing with sound-field (field-free) thresholds >25 dB, and speech recognition scores >90%. In both cases, hearing improvement remained at three years following surgery. Conclusions: To our knowledge, these are the first reported cases of microtia with congenital stapes ankylosis successfully treated with a bone conduction implantable device. Patients with microtia and stapes ankylosis who are reluctant to undergo surgery may benefit from unilateral or bilateral, short-term or long-term use of a Bonebridge bone conduction implantable device.

KW - Bone conduction

KW - Congenital abnormalities

KW - Correction of hearing impairment

KW - Ear, external

KW - Hearing aids

KW - Hearing loss, bilateral

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