Objective
Auditory Neuropathy Spectrum Disorder (ANSD) affects nearly 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.
Results
Children with ANSD exhibited differences in central auditory maturation. Overall, two-thirds of children revealed present P1 CAEP responses. Of these, approximately 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.
Conclusions
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.
Results of this national clinical trial showed significant improvements in speech perception and quality of life following cochlear implantation. SmartSound™ 2 signal processing features showed a significant benefit of FOCUS when listening in noise, although preference of signal processing feature did not correlate with performance. Significant correlations were observed between speech perception tests. The findings of this study can be applied in clinical assessment, programming, and follow-up for CI candidates and recipients.
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