Distinct Auditory Thalamocortical Pathologies Underlie Emerging Neurophysiological Dysfunction in a Cln3 Mouse Model of Batten Disease
This study demonstrates that age- and sex-dependent auditory neurophysiological deficits in a Cln3-/- mouse model of Batten disease are directly linked to region-specific lysosomal storage pathology within the auditory thalamocortical circuit, providing a functional-anatomical framework for developing translational biomarkers.