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. 2019 Jun 20;4(12):e128561. doi: 10.1172/jci.insight.128561

Figure 3. Progressive hearing loss in TRIOBP-5–deficient mice.

Figure 3

(A) Average auditory brainstem response (ABR) thresholds in TriobpΔEx8/+ heterozygous control mice (gray/black) and TriobpΔEx8/YHB226 compound heterozygous TRIOBP-5–deficient mice (shades of green). (B) Average ABR thresholds in Triobp+/+ (wild-type) and TriobpΔEx9-10/+ heterozygous controls (gray/black) and in TriobpΔEx9-10/ΔEx9-10 mice (shades of blue). ABRs were measured at 4, 8, and 12 weeks postnatally at frequencies of 8, 16, and 32 kHz. When no response was detected at a maximum stimulus level of 90 dB SPL, the threshold was assigned as 100 dB SPL (dashed line in A and B). Error bars indicate SD. (C) SEM images of P43 mouse IHC and OHC stereocilia bundles from normal-hearing TriobpΔEx8/+ heterozygotes (left) and from deaf TriobpΔEx8/YHB226 (right) compound heterozygotes. (D) SEM images of mouse P35 stereocilia bundles of wild-type and TriobpΔEx9-10/ΔEx9-10 OHCs and IHCs. Note fusion of stereocilia of mutant IHC and loss of stereocilia from longer row in OHC hair bundle. Scale bars: 2 μm in C and right panels in D, and 5 μm in D, left panel.