Topic: sound localization deficits

Aims: SCA13 patients exhibit an extracerebellar auditory phenotype characterized by sound localization deficits, despite normal audiograms. This phenotype is caused by the R420H point mutation in the KCNC3 gene which encodes for Kv3.3 subunits of high-voltage gated potassium channels. Here, we investigate how this mutation leads to disruption of temporal processing along the auditory pathway.Methods: The R420H mouse model was created using CRISPR-Cas9. Whole-cell patch-clamp electrophysiology and afferent synaptic stimulation in acute mouse brain slices were used to characterize neural firing