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. 2018 Jul 9;128(8):3382–3401. doi: 10.1172/JCI94351

Figure 9. AAV2/8-mediated delivery of clarin-1 in Clrn1ex4fl/fl Myo15-Cre+/– mice leads to long-term preservation of hearing.

Figure 9

(A) Comparative tone-burst ABR thresholds (audiogram, mean ± SEM and individual points for each frequency) in AAV2/8-Clrn1–injected (purple, n = 7) and untreated (red, n = 10) inner ears of P22–P24 Clrn1ex4fl/fl Myo15-Cre+/– mice and control (black, n = 6) mice. (B) Similar comparative audiograms for AAV2/8-Clrn1–injected (purple, n = 5) and untreated (red, n = 6) inner ears of P60 Clrn1ex4fl/fl Myo15-Cre+/– mice and P60 control (black, n = 6) mice. (C) Mean click ABR waves at P60 in control and in untreated and AAV2/8-Clrn1–injected Clrn1ex4fl/fl Myo15-Cre+/– mice. (D) Scanning electron microscopy view of stereocilia from untreated (left) and AAV2/8-Clrn1–injected (right) ears of P120 Clrn1ex4fl/fl Myo15-Cre+/– mice (n = 3). By contrast to the untreated ears, the injection of AAV2/8-Clrn1 resulted in apparently normal hair bundles still visible on P120 (n = 3). However, despite the persistence of V-shaped bundles, the stereocilia in the short and middle rows are heterogeneous in length, and many of these stereocilia are missing. **P < 0.01, ***P < 0.001 (unpaired Student’s t test).Scale bars: 1 μm.