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先天性耳聋与聋哑:历史视角

Congenital Deafness and Deaf-Mutism: A Historical Perspective.

作者信息

Cozza Andrea, Di Pasquale Fiasca Valerio Maria, Martini Alessandro

机构信息

Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, 35128 Padua, Italy.

Section of Otorhinolaryngology-Head and Neck Surgery, Department of Neurosciences, University of Padua, 35128 Padua, Italy.

出版信息

Children (Basel). 2023 Dec 30;11(1):51. doi: 10.3390/children11010051.

Abstract

Hearing loss is the most common sensory deficit and one of the most common congenital abnormalities. The estimated prevalence of moderate and severe hearing loss in a normal newborn is 0.1-0.3%, while the prevalence is 2-4% in newborns admitted to the newborn intensive care unit. Therefore, early detection and prompt treatment are of utmost importance in preventing the unwanted sequel of hearing loss on normal language development. The problem of congenital deafness is today addressed on the one hand with hearing screening at birth, on the other with the early (at around 3 months of age) application of hearing aids or, in case of lack of benefit, by the cochlear implant. Molecular genetics, antibody tests for some viruses, and diagnostic imaging have largely contributed to an effective etiological classification. A correct diagnosis and timely fitting of hearing aids or cochlear implants is useful for deaf children. The association between congenital deafness and "mutism", with all the consequences on/the consideration that deaf mutes have had since ancient times, not only from a social point of view but also from a legislative point of view, continued until the end of the nineteenth century, with the development on one side of new methods for the rehabilitation of language and on the other of sign language. But we need to get to the last decades of the last century to have, on the one hand, the diffusion of "universal newborn hearing screening", the discovery of the genetic causes of over half of congenital deafness, and on the other hand the cochlear implants that have allowed thousands of children born deaf the development of normal speech. Below, we will analyze the evolution of the problem between deafness and deaf-mutism over the centuries, with particular attention to the nineteenth century.

摘要

听力损失是最常见的感觉缺陷之一,也是最常见的先天性异常之一。正常新生儿中,中度和重度听力损失的估计患病率为0.1%-0.3%,而入住新生儿重症监护病房的新生儿患病率为2%-4%。因此,早期发现和及时治疗对于预防听力损失对正常语言发育产生的不良后果至关重要。如今,先天性耳聋问题一方面通过出生时的听力筛查来解决,另一方面通过早期(约3个月大时)使用助听器来解决,若使用助听器无效,则通过人工耳蜗植入来解决。分子遗传学、针对某些病毒的抗体检测以及诊断成像在很大程度上有助于进行有效的病因分类。对失聪儿童而言,正确诊断并及时佩戴助听器或植入人工耳蜗很有帮助。先天性耳聋与“缄默症”之间的关联,以及自古以来聋哑人所面临的所有后果,不仅从社会角度,而且从立法角度来看,一直持续到19世纪末,与此同时,一方面出现了新的语言康复方法,另一方面出现了手语。但直到上世纪最后几十年,一方面“普遍新生儿听力筛查”得以普及,超过半数先天性耳聋的遗传病因被发现,另一方面人工耳蜗植入使得成千上万先天性失聪儿童能够正常说话。下面,我们将分析几个世纪以来耳聋与聋哑问题的演变,尤其关注19世纪。

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