Analysis of speech perception outcomes among patients receiving cochlear implants with auditory neuropathy spectrum disorder.

Objective: Cochlear implantation (CI) is currently the main device option for children with auditory neuropathy spectrum disorder (ANSD) who receive minimal benefit from conventional amplification. This study examines potential prognostic factors associated with post-CI speech performance in this population.

Study Design: Retrospective chart review.

Setting: Academic center.

Patients: ANSD patients without inner ear abnormalities implanted with unilateral or bilateral CI between 1998 and 2010.

Intervention: CI and speech perception testing.

Main Outcome Measure: Post-CI speech perception testing at 50 dBHL. "Good" performers were defined as patients with greater than 70% speech perception and "poor" performers less than 70%. Medical comorbidity, educational information, and social history were gathered.

Results: Twenty-seven patients met inclusion criteria. Mean age at diagnosis, first CI, and second CI in good performers were 2.5 ± 3.4, 3.4 ± 3.6, and 3.8 ± 1.6 years, respectively, compared with 9.7 ± 7.8, 14.8 ± 12.9, and 8.9 ± 3.5 in poor performers. Mean speech perception after first and second implantation for good performers trended at 85% and 90%, respectively, compared with 36% and 73% in poor performers. Better pre-CI PTA correlated with better post-CI speech perception. Patients with bilateral CI demonstrated better speech perception outcomes compared with unilateral CI use. Poor performers had later age of implantation, lower socioeconomic status, and lack of family support compared with good performers.

Conclusion: ANSD patients who do not benefit from conventional amplification do well when implanted at a young age with proper access to education and habilitation training. Medical, social, and economic information may be helpful in predicting positive outcomes.