West African Centre for Cell Biology of Infectious Pathogens (WACCBIP), University of Ghana, Accra, Ghana.
Division of Human Genetics, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa.
Public Health Genomics. 2020;23(5-6):184-189. doi: 10.1159/000512121. Epub 2020 Dec 10.
Genetic factors significantly contribute to the burden of hearing impairment (HI) in Ghana as there is a high carrier frequency (1.5%) of the connexin 26 gene founder variant GJB2-R143W in the healthy Ghanaian population. GJB2-R143W mutation accounts for nearly 26% of causes in families segregating congenital non-syndromic HI. With HI associated with high genetic fitness, this indicates that Ghana will likely sustain an increase in the number of individuals living with inheritable HI. There is a universal newborn hearing screening (UNHS) program in Ghana. However, this program does not include genetic testing. Adding genetic testing of GJB2-R143W mutation for the population, prenatal and neonatal stages may lead to guiding genetic counseling for individual and couples, early detection of HI for at-risk infants, and improvement of medical management, including speech therapy and audiologic intervention, as well as provision of the needed social service to enhance parenting and education for children with HI. Based on published research on the genetics of HI in Ghana, we recommend that the UNHS program should include genetic screening for the GJB2-R143W gene variant for newborns who did not pass the initial UNHS tests. This will require an upgrade and resourcing of public health infrastructures to implement the rapid and cost-effective GJB2-R143W testing, followed by appropriate genetic and anticipatory guidance for medical care.
遗传因素在加纳听力障碍(HI)的负担中起着重要作用,因为在健康的加纳人群中,间隙连接蛋白 26 基因的 GJB2-R143W 创始人变体的携带频率很高(1.5%)。GJB2-R143W 突变几乎占家族性先天性非综合征 HI 原因的 26%。由于 HI 与高遗传适应性相关,这表明加纳可能会有更多遗传性 HI 的个体。加纳有一个普遍的新生儿听力筛查(UNHS)计划。然而,该计划不包括基因检测。对人群进行 GJB2-R143W 突变的基因检测,在产前和新生儿阶段,可能会导致为个人和夫妇提供遗传咨询,早期发现高危婴儿的 HI,并改善医疗管理,包括语言治疗和听力干预,以及提供必要的社会服务,以提高 HI 儿童的养育和教育水平。基于加纳 HI 遗传学的已发表研究,我们建议 UNHS 计划应包括对未通过初始 UNHS 测试的新生儿进行 GJB2-R143W 基因突变的基因筛查。这将需要升级和提供公共卫生基础设施资源,以实施快速且具有成本效益的 GJB2-R143W 检测,随后进行适当的遗传和预期指导以进行医疗护理。