Fig. 8. Purkinje cell quantification and hearing threshold in NPC cats administered IC HPβCD. Purkinje cell quantification and hearing threshold in NPC.

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Fig. 8. Purkinje cell quantification and hearing threshold in NPC cats administered IC HPβCD. Purkinje cell quantification and hearing threshold in NPC cats administered IC HPβCD. (A) Untreated 24-week-old NPC cats showed significantly fewer Purkinje cells per unit length compared to age-matched normal control cats (*P < 0.05). Cats (24 weeks old) receiving 30 mg of IC HPβCD or greater showed significantly greater Purkinje cell numbers compared to untreated NPC cats (†P < 0.05). In contrast, 24-week-old cats treated postsymptomatically with 120 mg of IC HPβCD at 16 weeks of age showed significantly fewer Purkinje cells compared to normal control cats (*P < 0.05). (B) Untreated 24-week-old NPC cats showed no significant difference in hearing threshold compared to normal control cats. In contrast, cats receiving 7.5 or 120 mg of HPβCD developed significant increases in hearing threshold compared to untreated NPC cats (†P < 0.05). Individual cats receiving lower doses of HPβCD also showed elevated hearing thresholds, although, as a group, these were not significant. (C) At 76 weeks of age, all NPC cats receiving 30 mg of HPβCD or greater showed significant elevations in hearing threshold. *P < 0.001. Charles H. Vite et al., Sci Transl Med 2015;7:276ra26 Copyright © 2015, American Association for the Advancement of Science