Average ABR thresholds at two ages for (A) Coch−/− and (B) CochG88E/G88E mouse models. At 13 months of age, the Coch−/− mice (n=6) showed no significant elevation of ABR thresholds in comparison to the Coch+/+ (n=5) and Coch−/+ (n=6) littermates. At 21 months, Coch−/− mice (n= 11) showed elevated ABR thresholds or absent ABRs at the highest frequency tested as compared to Coch+/+ (n=9) and Coch−/+ (n=12) littermates, with 9 of 11 Coch−/− mice showing absent ABRs at 41.2 kHz. In the CochG88E/G88E mouse model, ABR thresholds were elevated for all frequencies at 21 months of age, with 4 of 8 showing absent ABR. In contrast to Coch−/+ mice, CochG88E/+ mice show ABR threshold elevations similar to those of the CochG88E/G88E mice. Error bars represent standard error. [Color figure can be viewed in the online issue, which is available at http://www.sciencedirect.com/science/journal/03785955.]