Figure 6

Gfi1Cre inducible expression of Tm[Tmc2] in absence of Tmc1 restore mild ABR sensitivity. Auditory brainstem responses were recorded in knockin mice crossed to Gfi1Cre expressing mice to obtain Tm[Tmc2] expression in absence of Tmc1 (A), Tmc2 (B) or both (C). ABR were detected for sound levels above 80 dB in mice that expressed the Tm[Tmc2] in absence of Tmc1 or Tmc1 and Tmc2 at P16. Responses gradually disappeared and the mice were profoundly deaf by 6 weeks of age (A,C). While mild high frequency hearing loss was observed in mice expressing Tm[Tmc2] in presence of Tmc1, the loss was also evident in Gfi1Cre control mice (Tmc1+/+Tmc2+/+Gfi1cre/+) demonstrating that this feature resulted from background strains rather than expression of Tm[Tmc2] itself (B). Responses observed at P16 had longer latencies which resulted in altered ABR (D). Mean ± S.E.M.