Mutation of the Cyba gene encoding p22phox causes vestibular and immune defects in mice
J. Clin. Invest. Yoko Nakano, et al. 118:1176 doi:10.1172/JCI33835 [
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Figure 4Balance organ dysfunction and a lack of otoconia in nmf333 mice. (
A) Balance in WT and homozygous nmf333 mice was examined using swimming tests. Homozygous mutants were unable to orient themselves in water and swirled under the surface. (
B) Balance function based on VsEP waveforms; 2 representative replicates of VsEP waveforms are shown for WT, heterozygous, and homozygous mutant nmf333 mice exposed to linear acceleration stimuli from +6 to –12 dB relative to reference intensity (re) 1.0 g/ms in 3-dB increments, with auditory masking (M) and without auditory masking. Positive-response peaks are labeled with arrowheads. VsEP responses were absent in
nmf333/nmf333 mice. (
C) Summarized VsEP data. Individual VsEP thresholds are shown for WT (open circles;
n = 6), heterozygous (open squares;
n = 6), and
nmf333/333 (filled squares;
n = 4) mice. Mean thresholds are indicated by solid horizontal lines ± 1 SD (dashed lines).
P < 0.0001, 1-way ANOVA; post-hoc Dunnett’s test between WT and heterozygous (
P > 0.05) and between WT and nmf333 groups (**
P < 0.01). (
D) Otoconial presence determined by von Kossa staining. The von Kossa method revealed the calcium salt content characteristic of otoconia (arrows) in the saccule (S) and utricle (U) of a WT mouse. No calcium salts are present in the utricle and saccule of nmf333 mice.
n = 3 per group. Scale bars: 100 μm. (
E) Otoconial presence was investigated by scanning electron microscopy in the saccules of WT and homozygous nmf333 mice. The otoconial crystal layer is completely absent from the nmf333 saccule.