목적: Pendrin, encoded by SLC26A4 gene, is a carrier protein expressed in inner ear epithelial cells that secrete HCO3 into endolymph. Details of vestibular ocular reflex (VOR) and anatomical abnormality of the lateral semicircular canal have not been characterized. Here we report the quantitative VOR responses and vestibular anatomy in pendrin deficient mice strain. 방법:Total 40 mice of wild type (Slc26a4+/+, N=9), Slc26a4 heterozygous knockout (Slc26a4Δ/+, N=10), transgenic mice with doxycycline- inducible partial expression of Slc26a4 (Tg(+)Slc26a4Δ/Δ, N=13) and Slc26a4 homozygous knockout (Slc26a4Δ/Δ, N=8) are used. Pendrin deficient mice were evaluated by auditory brainstem response (ABR), VOR, histology of the horizontal canal. Rotation test is performed more than 10 cycles with the maximal angular velocity of and 100 degree/sec in 0.1, 0.2, 0.5 Hz. 결과:Mean ABR thresholds of Slc26a4Δ/Δ mice were significantly elevated compared to Tg(+)Slc26a4Δ/Δ, Slc26a4Δ/+ and Slc26a4+/+ mice. In VOR testing, Slc26a4Δ/Δ mice had minimal response to any of the stimuli tested, whereas Tg(+)Slc26a4Δ/Δ and Slc26a4Δ/+ had an intermediate response compared to Slc26a4+/+. The mean gains of horizontal rotation at 0.1 Hz were 0.03 0.02 in Slc26a4Δ/Δ, 0.57 0.03 in Tg(+)Slc26a4Δ/Δ, 0.75 0.02 in Slc26a4Δ/+ and 0.87 0.03 in Slc26a4+/+mice, showing pendrin-dose dependent formation of VOR. A substantial portion of vestibular hair cells in the lateral canal from Slc26a4Δ/Δ are replaced by vacuoles, while there is no apparent difference between Slc26a4+/+ and Slc26a4Δ /+. 결론:Vestibular function through VOR of horizontal canal is manifested with a pendrin dose-dependent pattern, while auditory function shows all-or-none phenotype in pendrin deficient mice. Mutations of SLC26A4 gene induce anatomical and functional deficits of vestibular hair cells of lateral semicircular canals.