Outcomes of Cochlear Implantation in Auditory Neuropathy Spectrum Disorder and the Role of Cortical Auditory Evoked Potentials in Benefit Evaluation.

Objective: To compare the outcomes of cochlear implantation (CI) in children with auditory neuropathy spectrum disorder (ANSD) and age-matched controls with profound sensorineural hearing loss, using categories of auditory performance (CAP), speech intelligibility rate (SIR), meaningful auditory integration scale (MAIS), and meaningful use of speech scale (MUSS), and to determine the role of Cortical Auditory Evoked Potentials (CAEP) in benefit evaluation after CI.

Methods: Ten patients (8 males and two females) with ANSD who underwent CI were included in the study. Auditory and speech scores were compared between baseline and after 12 months of habilitation in children with ANSD. Post CI speech scores in children with ANSD were compared with the control group (age-matched children with profound sensorineural hearing loss) at 12 months of habilitation. P1 latency of CAEP has a good correlation with auditory and speech scores in children with ANSD in the study group.

Results: Significant benefits were seen in children with ANSD who underwent CI compared to the baseline CAP and SIR scores and one year after habilitation. There is no statistically significant difference in outcomes between the two groups with CI (ANSD and profound sensorineural hearing loss) (p-value: CAP=1.00, SIR=0.84, MAIS=0.33, MUSS=0.08). Speech perception in noise test (SPIN) scores in children with ANSD were 63% and 80% with 0 dB signal noise ratio (SNR) and +10dB SNR, respectively. P1 wave of CAEP has a good correlation with the subjective outcomes.

Conclusion: CI in children with ANSD has showed benefits comparable to children with profound sensorineural hearing loss. CAEP is a useful tool in objectively assessing cortical maturity in children with ANSD following CI.