Cortical maturation and behavioral outcomes in children with auditory neuropathy spectrum disorder

Abstract Objective. Auditory neuropathy spectrum disorder (ANSD) affects approximately 10% of patients with sensorineural hearing loss. While many studies report abnormalities at the level of the cochlea, auditory nerve, and brainstem in children with ANSD, much less is known about their cortical development. We examined central auditory maturation in 21 children with ANSD. Design. Morphology, latency and amplitude of the P1 cortical auditory evoked potential (CAEP) were used to assess auditory cortical maturation. Children's scores on a measure of auditory skill development (IT-MAIS) were correlated with CAEPs. Study Sample. Participants were 21 children with ANSD. All were hearing aid users. Result. Children with ANSD exhibited differences in central auditory maturation. Overall, two-thirds of children revealed present P1 CAEP responses. Of these, just over one third (38%) showed normal P1 response morphology, latency and amplitude, while another third (33%) showed delayed P1 response latencies and significantly smaller amplitudes. The remaining children (29%) revealed abnormal or absent P1 responses. Overall, P1 responses were significantly correlated with auditory skill development. Conclusion: Our results suggest that P1 CAEP responses may be: (i) A useful indicator of the extent to which neural dys-synchrony disrupts cortical development, (ii) A good predictor of behavioral outcome in children with ANSD.