Atypical auditory cortical lateralization and hearing loss-associated temporal volume reductions in youth with chromosome 22q11.2 deletion syndrome: Preliminary implications for psychosis risk.

Chromosome 22q11.2 deletion syndrome (22q11.2DS) is a complex neurodevelopmental disorder with significantly elevated risk of schizophrenia spectrum disorders but a poorly understood etiopathology. Hearing loss affects 40 to 65% of individuals with 22q11.2DS, and auditory processing abnormalities are implicated in psychosis. We examined structural MRI in youth with 22q11.2DS (n = 23; 11 with hearing loss, 12 without) and typically developing controls (n = 21), assessing auditory cortical volumes by hearing status and psychological functioning. After controlling for total cerebral volume (TCV) and age, no regional volumes survived FDR correction between groups. Within 22q11.2DS, participants with hearing loss showed smaller right lateral superior temporal gyrus volumes than those without (d = 1.41, bootstrap p = 0.003), though this did not survive FDR correction. Altered planum temporale and planum polare lateralization relative to controls was bootstrap-supported (uncorrected). The 22q11.2DS group showed elevated anxiety, atypicality, attention problems, internalizing symptoms, and withdrawal, and lower functional communication, social skills, and cognitive indices (all ps < 0.05 FDR corrected). Brain and behavior correlations did not survive correction. Given small subgroups and multiple comparisons, the neuroimaging findings are preliminary and hypothesis-generating, suggesting that hearing loss may modulate auditory cortical development with implications for psychosis-risk etiopathology.