Zakzouk Siraj
Department of ENT, Security Forces Hospital, Riyadh, Saudi Arabia.
J Laryngol Otol. 2002 Oct;116(10):811-6. doi: 10.1258/00222150260293628.
Consanguineous marriage is a tradition which is commonly practised among Asian, African, and Latin American communities whether they are living in their own countries or settled in Europe or the USA. These communities, in addition to their custom of interrelated marriage, have large families and are a rapidly growing population. The siblings of consanguineous marriages have a significantly higher incidence of autosomal recessive diseases including hearing impairment. Two epidemiological surveys were carried out 10 years apart. There were 6,421 subjects from Riyadh City and 9,540 from all other parts of the Kingdom of Saudi Arabia. A random sample was examined otologically and a questionnaire was filled in that included age, sex, family relation, number of siblings, etc. ENT examination and audiological assessment were performed. Consanguinity was found among 22 per cent as first cousins and 23 per cent as second cousins in the first survey. In the second survey 19 per cent were first cousins and 28 per cent second cousins. The rate of consanguinity was 45 per cent in the first survey and 47 per cent in the second. The prevalence of hereditary sensorineural hearing loss (SNHL) was 66.07 per cent and 36.6 per cent in the first and second survey respectively. The incidence of hereditary hearing impairment is very high in developing countries compared to developed countries. Prevention is essential to reduce the incidence of genetic hearing loss. Consanguinity should be discouraged through health education of the public about the adverse effect of interrelated marriage. Genetic counselling, premarital and antenatal screening are to be applied whenever possible, at least for those at risk of developing genetic diseases including hearing impairment.
近亲结婚是一种在亚洲、非洲和拉丁美洲社区普遍存在的传统，无论这些社区的人们是生活在自己的国家，还是定居在欧洲或美国。这些社区除了有近亲结婚的习俗外，家庭规模较大，人口增长迅速。近亲结婚所生子女患常染色体隐性疾病（包括听力障碍）的几率显著更高。相隔10年进行了两项流行病学调查。一项调查中有来自利雅得市的6421名受试者，另一项调查中有来自沙特阿拉伯王国其他地区的9540名受试者。对随机抽取的样本进行了耳科检查，并填写了一份包含年龄、性别、家庭关系、兄弟姐妹数量等信息的问卷。同时进行了耳鼻喉科检查和听力评估。在第一次调查中，发现22%的人为一级表亲近亲结婚，23%为二级表亲近亲结婚。在第二次调查中，19%为一级表亲，28%为二级表亲。第一次调查中的近亲结婚率为45%，第二次调查为47%。第一次和第二次调查中遗传性感音神经性听力损失（SNHL）的患病率分别为66.07%和36.6%。与发达国家相比，发展中国家遗传性听力障碍的发病率非常高。预防对于降低遗传性听力损失的发病率至关重要。应通过对公众进行健康教育，使其了解近亲结婚的不良影响，从而不鼓励近亲结婚。只要有可能，就应提供遗传咨询、婚前和产前筛查，至少针对那些有患包括听力障碍在内的遗传性疾病风险的人群。
