耳真菌病:病因、病理因素和真菌学谱的研究

A. Krishnan
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摘要

摘要先天性中耳听骨链畸形是一种少见的畸形,其表现形式多种多样。在这些畸形中,先天性镫骨缺失(CAS)是一种非常罕见的情况,在英语科学文献中很少被描述。1955年,麦卡斯基尔和沙利文首次在两名传导性听力损失患者身上报道了这种情况。患者男,19岁,自幼双侧听力困难,非进行性。家族史没有任何耳科或遗传问题。听力图显示双侧传导性听力损失,两侧平均气骨间隙为60 dB。颞骨CT显示双侧镫骨上部结构缺失,面神经位置异常。2017年先行右侧内镜探查性鼓室切开术,2018年(6月)行左侧听骨重建术。显然,CAS是一种罕见的实体,文献中仅报道了12例。在所有病例中,CAS表现为传导性听力损失,外耳道正常,鼓膜完整。确切的病因尚不清楚,有人认为镫骨和卵圆窗的畸形或发育不全与面神经发育异常有关。随着时间的推移,人们描述了各种管理方法。这包括广泛的选择,从探查手术和重建假体到放大。结论在所有可能的中耳异常中,镫骨发育不全是传导性听力损失的鉴别诊断之一。1955年至2017年,英国科学文献中仅发表了12例病例。到目前为止,印度还没有公布病例。超过一半的CAS患者有异常的面神经,这可能使手术复杂化。该患者面神经管前下移位,但我们已成功地进行了双侧内窥镜重建手术,未损伤面神经。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Otomycosis: Study of Etiopathological Factors and Mycological Spectrum
Introduction The congenital anomalies of ossicular chain of the middle ear are an uncommon event that can present in a variety of ways. Among these malformations, the congenital absence of stapes (CAS) is a very rare condition that is seldom described in the English scientific literature. It was first reported by Mcaskile and Sullivan in 1955 in two patients with conductive hearing loss. Case Presentation A 19-year-old male patient presented with complaints of bilateral hard of hearing since childhood, nonprogressive type. Family history was negative for any otologic or genetic problem. The audiogram showed bilateral conductive hearing loss with an average air–bone gap of 60 dB on both sides. CT temporal bone revealed the absence of stapes superstructure bilaterally, and an abnormal facial nerve location. Endoscopic exploratory tympanotomy and ossicular reconstruction were done on right side first, in the year 2017, and on left side in the year 2018 (June). Discussion The CAS is obviously a rare entity with only 12 cases reported in the literature. In all cases, CAS present as a conductive hearing loss with normal external auditory canal and intact tympanic membrane. The exact etiology is still not known and it has been suggested that malformation or agenesis of the stapes and oval window is related to the abnormal development of the facial nerve. Various managements have been described through time. This includes a broad range of options from exploratory surgery and reconstruction prosthesis to amplification. Conclusion Among all possible middle ear anomalies, stapes agenesis is rarely a part of the differential diagnosis for conductive hearing loss. Only 12 cases have been published in the English scientific literature (1955–2017). No cases have been published in India, till date. More than half of the patients with CAS have an abnormal facial nerve, which may complicate surgery. This patient has an anteroinferior displacement of facial nerve canal but we have done bilateral endoscopic reconstruction surgery successfully without any injury to facial nerve.
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