Patients with auditory neuropathy spectrum disorder (ANSD) exhibit altered neural synchrony in response to auditory stimuli. It has been hypothesized that a slower rate of electrical stimulation in programming strategies for cochlear implant (CI) users with ANSD may enhance development of neural synchrony and speech perception abilities.Study Design
Retrospective case series.Setting
Tertiary otologic practice.Patients
Twenty-two patients with ANSD underwent CI. Patients with complete postoperative audiometric data and at least 2 years of follow-up were included in further analysis.Intervention
Thirteen patients patients met inclusion criteria. Five “poorly performing” CI recipients with ANSD who had not developed closed-set speech perception abilities despite at least 2 years of implant use underwent implant programming to lower the neural stimulation rate.Main Outcome Measures
Speech perception abilities over time using parent questionnaire, closed-set testing, and open-set measures.Results
A high incidence of comorbid conditions was present in the poor performers, including cognitive delay (n = 2), motor delay (n = 3), and autism spectrum disorder (n = 1). The median time to rate slowing in 5 poor performers was 29 months after implant activation. Three of 5 patients achieved closed-set speech perception scores higher than 60% after 6 to 16 months of implant use at the slower rates. At last follow-up (median, 42 mo), no poor performer had yet achieved open-set speech perception abilities. Of all CI recipients with ANSD included in analysis, open-set speech perception abilities developed in 46% (6/13).Conclusion
In CI recipients with ANSD who demonstrate limited auditory skills development despite prolonged implant use, lowering the stimulation rate may facilitate acquisition of closed-set speech perception abilities. Further efforts on the study of programming parameters in ANSD patients with CIs are necessary to maximize auditory development in this patient population.