RT Journal Article
SR Electronic
T1 Characterization of Auditory and Binaural Spatial Hearing in a Fragile X Syndrome Mouse Model
JF eneuro
JO eNeuro
FD Society for Neuroscience
SP ENEURO.0300-19.2019
DO 10.1523/ENEURO.0300-19.2019
VO 7
IS 1
A1 Elizabeth A. McCullagh
A1 Shani Poleg
A1 Nathaniel T. Greene
A1 Molly M. Huntsman
A1 Daniel J. Tollin
A1 Achim Klug
YR 2020
UL http://www.eneuro.org/content/7/1/ENEURO.0300-19.2019.abstract
AB The auditory brainstem compares sound-evoked excitation and inhibition from both ears to compute sound source location and determine spatial acuity. Although alterations to the anatomy and physiology of the auditory brainstem have been demonstrated in fragile X syndrome (FXS), it is not known whether these changes cause spatial acuity deficits in FXS. To test the hypothesis that FXS-related alterations to brainstem circuits impair spatial hearing abilities, a reflexive prepulse inhibition (PPI) task, with variations in sound (gap, location, masking) as the prepulse stimulus, was used on Fmr1 knock-out mice and B6 controls. Specifically, Fmr1 mice show decreased PPI compared with wild-type mice during gap detection, changes in sound source location, and spatial release from masking with no alteration to their overall startle thresholds compared with wild-type mice. Last, Fmr1 mice have increased latency to respond in these tasks, suggesting additional impairments in the pathway responsible for reacting to a startling sound. This study further supports data in humans with FXS that show similar deficits in PPI.