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Mouse  Tmc1  and  Tmc2  are required for sensory transduction in cochlear and vestibular hair cells. Homozygous  Tmc1  ∆/∆  mice are deaf,  Tmc2  ∆/∆  mice have normal hearing, and double homozygous  Tmc1  ∆/∆ ;  Tmc2  ∆/∆  mice have deafness and profound vestibular dysfunction. These phenotypes are consistent with their different spatiotemporal expression patterns.  Tmc1  expression is persistent in cochlear and vestibular hair cells, whereas  Tmc2  expression is transient in cochlear hair cells but persistent in vestibular hair cells. On the basis of these findings, we hypothesized that persistent  Tmc2  expression in mature cochlear hair cells could restore auditory function in  Tmc1  ∆/∆  mice. To express  Tmc2  in mature cochlear hair cells, we generated a transgenic mouse line, Tg[P  Tmc1  :: Tmc2 ], in which  Tmc2  cDNA is expressed under the control of the  Tmc1  promoter. The Tg[P  Tmc1  :: Tmc2 ] transgene slightly but significantly restored hearing in young  Tmc1  ∆/∆  mice, though hearing thresholds were elevated with age. The elevation of hearing thresholds was associated with deterioration of sensory transduction in inner hair cells and loss of outer hair cell function. Although sensory transduction was retained in outer hair cells, their stereocilia eventually degenerated. These results indicate distinct roles and requirements for  Tmc1  and  Tmc2  in mature cochlear hair cells.

Tmc2 expression partially restores auditory function in a mouse model of DFNB7/B11 deafness caused by loss of Tmc1 function