Figure 2.
Auditory phenotypes and cochlear histology. (A) Heterozygous Slc26a4+/C565Y, homozygous Slc26a4C565Y/C565Y, and compound heterozygous Slc26a4919-2A>G/C565Y mice showed normal hearing thresholds across different frequencies, similar to that of wild-type Slc26a4+/+ mice. (B) Cochlear histology harvested at 3 months. Abnormal histological phenotypes observed in Slc26a4919-2A>G/919-2A>G mice, including dilatation of scala media (Cochlea panel; RM, Reissner’s membrane) and degeneration of cochlear hair cells (Organ of Corti panel), which were not observed in Slc26a4+/+, Slc26a4C565Y/C565Y, and Slc26a4919-2A>G/C565Y mice (bar = 150 μm). (C) Histology of cochlear hair cells harvested from mice at three months. Myosin-VIIA expression was normal in Slc26a4+/+, Slc26a4C565Y/C565Y, and Slc26a4919-2A>G/C565Y mice when compared to diminished expression in Slc26a4919-2A>G/919-2A>G mice (bar = 50 μm).