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Here, we performed a systematic review of literature on the genetic profile of HI in Africa. The study protocol was registered on PROSPERO, International Prospective Register of Systematic Reviews with the registration number \u201cCRD42021240852\u201d. Literature search was conducted on PubMed, Scopus, Africa-Wide Information, and Web of Science databases. A total of 89 full-text records was selected and retrieved for data extraction and analyses. We found reports from only 17\/54 (31.5%) African countries. The majority (61\/89; 68.5%) of articles were from North Africa, with few reports found from sub-Saharan Africa. The most common method used in these publications was targeted gene sequencing (<jats:italic>n<\/jats:italic>\u2009=\u200966\/111; 59.5%), and only 13.5% (<jats:italic>n<\/jats:italic>\u2009=\u200915\/111) used whole-exome sequencing. More than half of the studies were performed in families segregating HI (<jats:italic>n<\/jats:italic>\u2009=\u200951\/89). <jats:italic>GJB2<\/jats:italic> was the most investigated gene, with <jats:italic>GJB2<\/jats:italic>: p.(R143W) founder variant only reported in Ghana, while <jats:italic>GJB2<\/jats:italic>: c.35delG was common in North African countries. Variants in <jats:italic>MYO15A<\/jats:italic> were the second frequently reported in both North and Central Africa, followed by <jats:italic>ATP6V1B1<\/jats:italic> only reported from North Africa. Usher syndrome was the main syndromic HI molecularly investigated, with variants in five genes reported: <jats:italic>USH2A<\/jats:italic>, <jats:italic>USH1G<\/jats:italic>, <jats:italic>USH1C<\/jats:italic>, <jats:italic>MYO7A<\/jats:italic>, and <jats:italic>PCDH15<\/jats:italic>. <jats:italic>MYO7A<\/jats:italic>: p.(P1780S) founder variant was reported as the common Usher syndrome variant among Black South Africans. This review provides the most comprehensive data on HI gene variants in the largely under-investigated African populations. Future exomes studies particularly in multiplex families will likely provide opportunities for the discovery of the next sets of novel HI genes, and well as unreported variants in known genes to further our understanding of HI pathobiology, globally.<\/jats:p>","DOI":"10.1007\/s00439-021-02376-y","type":"journal-article","created":{"date-parts":[[2021,10,5]],"date-time":"2021-10-05T18:19:34Z","timestamp":1633457974000},"page":"505-517","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Hearing loss in Africa: current genetic profile"],"prefix":"10.1007","volume":"141","author":[{"given":"Samuel Mawuli","family":"Adadey","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edmond","family":"Wonkam-Tingang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elvis Twumasi","family":"Aboagye","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Osbourne","family":"Quaye","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gordon A.","family":"Awandare","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1420-9051","authenticated-orcid":false,"given":"Ambroise","family":"Wonkam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,10,5]]},"reference":[{"key":"2376_CR1","doi-asserted-by":"publisher","first-page":"569","DOI":"10.1089\/gte.2008.0063","volume":"12","author":"O Abidi","year":"2008","unstructured":"Abidi O, Boulouiz R, Nahili H, Bakhouch K, Wakrim L, Rouba H, Chafik A, Hassar M, Barakat A (2008) Carrier frequencies of mutations\/polymorphisms in the connexin 26 gene (GJB2) in the Moroccan population. 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