Figure 5.

Ehd1 and inner ear. (A) Localization of Ehd1 (green) in stria vascularis (SV) (inner ear). In wildtype (WT) mice, Ehd1 was strongly expressed in the SV. In homozygous knockout (B) and knockin mice (C) the labeling of SV and Reissner membrane (RM) was absent or grossly diminished, respectively. (SM) scala media (containing endolymph); scale bar 50 µm. (D) Auditory brainstem response measurements of mice revealed a high-frequency hearing impairment in homozygous knockin mice (Ehd1^R398W/R398W, n=6, red symbols) compared with wildtype mice (Ehd1^WT/WT, n=7, black symbols). Please note the reverse scaling of the y axis to facilitate comparison with the hearing phenotype of the patient shown in Figure 1. * indicates P≤0.05 between groups (t test with Bonferroni–Dunn correction for multiple testing).