Hearing loss phenotype and stria vascularis dysfunction in a transgenic mouse strain that robustly overexpresses the mitochondrial 12S ribosomal RNA methyltransferase TFB1M (Tg-mtTFB1). A: Auditory brainstem response (ABR) results in wild-type (WT; closed squares) and Tg-mtTFB1 (Tg-B1; open circles) mice used in this study. Data were analyzed by two-way analysis of variance, and the main effect for genotype is F(1,84) = 38, P < 0.001. Differences at each frequency were tested via post hoc Fisher's least significant difference tests. B: Direct endocochlear potential (EP) measurements of the mice in A. There is a significant EP reduction in Tg-mtTFB1 (Tg-B1, black bars) mice compared with WT (gray bars) controls [Student's t(10) = 3.3, ∗∗P < 0.01]. C: Measurements of the thickness of the stria from hematoxylin and eosin–stained sections. No significant difference exists between WT and Tg-B1 mice [t(45) = 0.9]. ∗P < 0.05, ∗∗P < 0.01, and ∗∗∗P < 0.001. dB SPL, decibels sound pressure level.