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Treacher-Collins综合征患者植入骨锚式助听器的结果。

Results of the implantation of bone-anchored hearing aids in patients with treacher-collins syndrome.

作者信息

Oliveira Alexandra Kolontai de Sousa, Ferro Lília Pereira Abreu, da Silva Jaiede Nicacio, Okada Daniel Mochida

机构信息

Resident Doctor in the Department of Otolaryngology at the Institute of Medical Assistance to the State's Public Service, Sao Paulo.

Speech Therapist at the Institute of Medical Assistance to the State's Public Service, Sao Paulo.

出版信息

Int Arch Otorhinolaryngol. 2013 Apr;17(2):222-6. doi: 10.7162/S1809-97772013000200018.

DOI:10.7162/S1809-97772013000200018
PMID:25992018
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4423323/
Abstract

INTRODUCTION

Treacher-Collins syndrome is characterized by craniofacial malformations, narrowing of the external auditory canal (EAC), and, in 30% of cases, agenesis of the canal and ossicular chain defects. The use of hearing aids (HA) is not possible in cases in which agenesis or stenosis of the EAC accompanies conductive deafness. In contrast, bone conduction implants such as the Bone Anchored Hearing Aid (BAHA(®)) allow direct stimulation of the cochlea and are thus superior to conventional hearing aids in cases of severe conductive hearing loss.

OBJECTIVE

To present 2 cases of patients with Treacher-Collins syndrome who underwent implantation of BAHA(®).

CASES REPORTS

The first patient was a 52-year-old woman diagnosed with Treacher-Collins syndrome who presented with severe bilateral mixed hearing loss and a history of unsuccessful previous use of a bone contact conduction device. The BAHA(®) implantation was uneventful, and the post-operative results were good. The second patient was a 14-year-old girl who was also diagnosed with Treacher-Collins Syndrome with bilateral moderate conductive hearing loss by audiometry. The use of a bone vibrator contact device did not improve her hearing; however, implantation of a BAHA(®) resulted in a decreased gap postoperatively. Final comments: BAHA(®) hearing devices provide adequate rehabilitation and consequent improvement of the quality of life in patients with Treacher-Collins syndrome.

摘要

引言

特雷彻 - 柯林斯综合征的特征为颅面畸形、外耳道狭窄，30%的病例存在外耳道发育不全及听骨链缺陷。当外耳道发育不全或狭窄伴有传导性耳聋时，无法使用助听器。相比之下，骨传导植入物如骨锚式助听器（BAHA(®)）可直接刺激耳蜗，因此在严重传导性听力损失的情况下优于传统助听器。

目的

介绍2例接受BAHA(®)植入的特雷彻 - 柯林斯综合征患者。

病例报告

首例患者为一名52岁女性，诊断为特雷彻 - 柯林斯综合征，表现为严重双侧混合性听力损失，既往使用骨接触传导装置未成功。BAHA(®)植入手术顺利，术后效果良好。第二例患者为一名14岁女孩，也被诊断为特雷彻 - 柯林斯综合征，听力测试显示双侧中度传导性听力损失。使用骨振动器接触装置未改善其听力；然而，植入BAHA(®)后术后差距减小。总结：BAHA(®)听力装置可为特雷彻 - 柯林斯综合征患者提供充分的康复治疗，从而改善其生活质量。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf4c/4423323/b41830e7e3c8/10-7162-s-1809-97772013000200018-i170218-5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf4c/4423323/9208abc8cdca/10-7162-s-1809-97772013000200018-i170218-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf4c/4423323/4152781b2aff/10-7162-s-1809-97772013000200018-i170218-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf4c/4423323/097119b78e79/10-7162-s-1809-97772013000200018-i170218-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf4c/4423323/b6d53b44bd5c/10-7162-s-1809-97772013000200018-i170218-4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf4c/4423323/b41830e7e3c8/10-7162-s-1809-97772013000200018-i170218-5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf4c/4423323/9208abc8cdca/10-7162-s-1809-97772013000200018-i170218-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf4c/4423323/4152781b2aff/10-7162-s-1809-97772013000200018-i170218-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf4c/4423323/097119b78e79/10-7162-s-1809-97772013000200018-i170218-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf4c/4423323/b6d53b44bd5c/10-7162-s-1809-97772013000200018-i170218-4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf4c/4423323/b41830e7e3c8/10-7162-s-1809-97772013000200018-i170218-5.jpg

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本文引用的文献

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Staged reconstruction for adult complete Treacher Collins syndrome.成人完全性特雷彻·柯林斯综合征的分期重建。
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