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This study aimed to investigate <i>GJB2</i> (MIM: 121011) and <i>GJB6</i> (MIM: 604418) variants associated with familial non-syndromic hearing impairment (HI) in Senegal. We investigated a total of 129 affected and 143 unaffected individuals from 44 multiplex families by segregating autosomal recessive non-syndromic HI, 9 sporadic HI cases of putative genetic origin, and 148 control individuals without personal or family history of HI. The DNA samples were screened for <i>GJB2</i> coding-region variants and <i>GJB6</i>-D3S1830 deletions. The mean age at the medical diagnosis of the affected individuals was 2.93 ± 2.53 years [range: 1–15 years]. Consanguinity was present in 40 out of 53 families (75.47%). Variants in <i>GJB2</i> explained HI in 34.1% (<i>n</i> = 15/44) of multiplex families. A bi-allelic pathogenic variant, <i>GJB2</i>: c.94C>T: p.(Arg32Cys) accounted for 25% (<i>n</i> = 11/44 families) of familial cases, of which 80% (<i>n</i> = 12/15) were consanguineous. Interestingly, the previously reported “Ghanaian” founder variant, <i>GJB2</i>: c.427C>T: p.(Arg143Trp), accounted for 4.5% (<i>n</i> = 2/44 families) of the families investigated. Among the normal controls, the allele frequency of <i>GJB2</i>: c.94C>T and <i>GJB2</i>: c.427C>T was estimated at 1% (2/148 ∗ 2) and 2% (4/148 ∗ 2), respectively. No <i>GJB6</i>-D3S1830 deletion was identified in any of the HI patients. This is the first report of a genetic investigation of HI in Senegal, and suggests that <i>GJB2</i>: c.94C>T: p.(Arg32Cys) and <i>GJB2</i>: c.427C>T: p.(Arg143Trp) should be tested in clinical practice for congenital HI in Senegal.