Assessing Neural Synchrony in the Cochlear Nerve to Electrical Stimulation in Children With Auditory Neuropathy Spectrum Disorder.

Objectives: This study reported phase locking values (PLVs) that quantified the trial-to-trial phase coherence of electrically evoked compound action potentials in children with auditory neuropathy spectrum disorders (ANSD) and children with Gap Junction Beta 2 (GJB2) mutations, a patient population without noticeable cochlear nerve damage.

Design: PLVs were measured at three electrode locations in 11 children with ANSD and 11 children with GJB2 mutations. Smaller PLVs indicated poorer neural synchrony. A linear mixed-effects model was used to compare PLVs measured at different electrode locations between participant groups.

Results: After controlling for the stimulation level effect, children with ANSD had smaller PLVs than children with GJB2 mutations at all three electrode locations.

Conclusions: Cochlear-implanted children with ANSD show poorer peripheral neural synchrony than children with GJB2 mutations.