Table 3.
All1 | Cases2 | Population3 | Counsyl | OR | 95% CI | Z | P value |
---|---|---|---|---|---|---|---|
Total number of individuals tested for GJB2 | 17635 | 802339 | 654426 | ||||
Total number of alleles tested for GJB2 | 35270 | 1604678 | 1308852 | ||||
Total number of individuals with p.Val37Ile | 464 | 10233 | 7609 | ||||
Total number of p.Val37Ile heterozygotes | 313 | 9887 | 7370 | ||||
Total number of p.Val37Ile homozygotes | 151 | 346 | 239 | 20 | 17–24 | 31 | <0.0001 |
Total number of p.Val37Ile compound heterozygotes4 | 115 | N/A5 | 96 | ||||
Total number of p.Val37Ile alleles | 615 | 10488 | 7848 | ||||
Overall p.Val37Ile allele frequency | 0.0174 | 0.0065 | 0.0060 | ||||
Number of Asian individuals tested for GJB2 | 2066 | 75857 | 60355 | ||||
Number of Asian alleles tested for GJB2 | 4132 | 151714 | 120710 | ||||
Number of Asian individuals with p.Val37Ile | 197 | 6173 | 4023 | ||||
Number of Asian p.Val37Ile heterozygotes | 113 | 5898 | 3852 | ||||
Number of Asian p.Val37Ile homozygotes | 84 | 275 | 171 | 12 | 9.1–15 | 19 | <0.0001 |
Number of Asian p.Val37Ile compound heterozygotes4 with another GJB2 pathogenic allele | 49 | N/A5 | 466 | 196 | 15–246 | 266 | <0.00016 |
Number of Asian p.Val37Ile alleles | 281 | 6360 | 4194 | 1.7 | 1.5–1.9 | 8.1 | <0.0001 |
p.Val37Ile allele frequency in Asians | 0.0680 | 0.0419 | 0.0347 |
Only probands (unrelated individuals) were counted. However, we could not rule out the possibility of related cases from different sites because cases were de-identified before being shared. Nevertheless, the likelihood of such occurrence would be low and would not significantly impact the conclusion.
The total number of cases included in statistical analyses did not include BCH, DY, and GDWC where the total number of individuals tested at these sites were not available.
The total population data were from Counsyl, CUHK, TAU, UNC, and gnomAD.
Compound heterozygosity was presumed in individuals with a second pathogenic or likely pathogenic variant in GJB2 that had never been reported to have occurred in cis.
NA: Not available, because individual allele state information is not available from gnomAD.
Analyses involving compound heterozygotes were performed using Counsyl data as the population control (see Methods).