Fig. 1

Four-frequency (0.5/1/2/4 KHz) pure-tone threshold averages for ears with enlargement of the vestibular aqueduct. Each data point represents one ear displayed according to SLC26A4 genotype status (M0, M1 or M2) and haplotype status (C, CEVA; R, reference (most common haplotype)). The CEVA haplotype is trans to the SLC26A4 mutation in the M1 group. **p ≤ 0.01, ***p ≤ 0.001, ****p ≤ 0.0001, Mann-Whitney Rank Test. M0 data points shown as a triangle (▲) correspond to CEVA homozygotes with one allele in cis with p.M775 T, a hypofunctional variant thought to be pathogenic only in trans with a mutation affecting the coding region or splice sites