Characterizing sound-localization deficits in a mouse model of spinocerebellar ataxia type 13 (SCA13)
Theo Cerciat, Mihai Stancu, Luna A. Studer, Ian D. Forsythe, Conny Kopp-Scheinpflug · FENS Forum 2024
Aims: SCA13 patients carrying the R420H mutation in Kv3.3 also have compromised sound localization based on inferior processing of interaural differences in time and intensity of sound. These patients show no pure-tone deficit in the audiogram and their peripheral auditory system seems unaffected. The Kv3.3 subunit of voltage-gated potassium channels is highly expressed in fast-firing neurons of the auditory pathway, including the medial nucleus of the trapezoid body (MNTB) and the lateral superior olive (LSO) and is often co-expressed with the Kv3.1 subunit. Here, we investigate how this Kv3.
ePoster · Neuroscience