Dereymaeker A M, Fryns J P, Ars B, Andrescescou J, Van den Berghe H
Centre for Human Genetics, University of Leuven, Belgium.
Acta Otorhinolaryngol Belg. 1991;45(3):283-91.
In this paper we present the results of an etiological study in 155 hearing-impaired children and young adults, born between 1960 and 1984, inmates of the IRSA institute (Royal Institute for Deaf and Blind children). Special reference to genetic factors was given. One third of the inmates of this institute were children of immigrants, the majority Italians and North-Africans. The study revealed the following classification for the Belgian pupils: hereditary deafness 22.9%; acquired deafness 44.9% and deafness of unknown origin 32.1%; in the group of immigrants, it was 39.1%, 45.6% and 15.2%, respectively. Six of the 45 patients with a genetic form of deafness presented a distinct syndrome, Usher syndrome (2 patients) being the most frequent. Autosomal dominant inheritance was present more often in the deaf population than in the hearing-impaired population (hearing loss below 80 db), but the difference was not statistically significant. Even after 1980 congenital rubella remained the most frequent exogenous factor causing congenital deafness and, furthermore, we documented for the first time in a retrospective study cytomegalovirus infection as a cause of hearing loss. Atypical retinal abnormalities not related to congenital rubella syndrome were found in 10 inmates by systematic ophthalmological examination: 7 in the unknown group, 1 in the genetic group and 2 in the acquired (meningitis) group.
在本文中，我们展示了一项针对155名听力受损儿童和年轻人的病因学研究结果，这些人出生于1960年至1984年之间，是IRSA研究所（皇家聋哑和盲人儿童研究所）的住院患者。特别提及了遗传因素。该研究所三分之一的住院患者是移民的子女，大多数是意大利人和北非人。研究揭示了比利时学生的以下分类情况：遗传性耳聋占22.9%；后天性耳聋占44.9%，不明原因的耳聋占32.1%；在移民群体中，分别为39.1%、45.6%和15.2%。45例遗传性耳聋患者中有6例表现出一种独特的综合征，其中尤塞综合征（2例）最为常见。常染色体显性遗传在聋人群体中比听力受损人群（听力损失低于80分贝）中更常见，但差异无统计学意义。即使在1980年之后，先天性风疹仍然是导致先天性耳聋最常见的外部因素，此外，我们在一项回顾性研究中首次记录了巨细胞病毒感染是听力损失的一个原因。通过系统的眼科检查，在10名住院患者中发现了与先天性风疹综合征无关的非典型视网膜异常：不明原因组7例，遗传组1例，后天性（脑膜炎）组2例。
