Hearing loss is the most common sensorineural disorder worldwide and is associated with more than1000 mutations in more than 90 genes. While mutations in genes such as GJB2 （gap-junction protein β 2）and GJB6 （gap-junction protein 13 6） are highly prevalent in Caucasian, Asian, and Middle Eastern pop-ulations, they are rare in both native African populations and those of African descent. The objective ofthis paper is to review the current knowledge regarding the epidemiology and genetics of hearing loss inAfrican populations with a focus on native sub-Saharan African populations. Environmental etiologiesrelated to poor access to healthcare and perinatal care account for the majority of cases. Syndromicetiologies including Waardenburg, Pendred and Usher syndromes are uncommon causes of hearing lossin these populations. Of the non-syndromic causes, common mutations in GJB2 and GJB6 are rarelyimplicated in populations of African descent. Recent use of next-generation sequencing （NGS） hasidentified several candidate deafness genes in African populations from Nigeria and South Africa that areunique when compared to common causative mutations worldwide. Researchers also recently describeda dominant mutation in MYO3a in an African American family with non-syndromic hearing loss. The useof NGS and specialized panels will aid in identifying rare and novel mutations in a more cost- and time-effective manner. The identification of common hearing loss mutations in indigenous African populationswill pave the way for translation into genetic deafness research in populations of African descentworldwide.