Cochlear implantation outcomes in patients with auditory neuropathy and internal auditory canal stenosis

Auditory neuropathy, coupled with internal auditory canal stenosis, presents unique challenges for hearing restoration. Advances in cochlear implantation technology have enabled improved auditory and speech perception outcomes in affected patients. This study evaluates the effectiveness of cochlear implantation in enhancing hearing and speech perception in patients diagnosed with auditory neuropathy and internal auditory canal stenosis. Thirteen patients diagnosed with auditory neuropathy and internal auditory canal stenosis underwent cochlear implantation at our institution. Pre- and post-operative assessments included behavioral or pure-tone audiometry to assess hearing thresholds, supplemented by objective measures including auditory steady-state response (ASSR) and 40 Hz objective threshold testing. Mandarin Early Speech Perception (MESP) testing, and IT-MAIS/MAIS scoring. Assessments were conducted preoperatively and at six months, one year, and two years post-implant activation. Statistical comparisons between pre- and post-operative outcomes were performed using paired t-tests. After six months of activation, all patients showed significant improvements in behavioral or pure-tone audiometry and ASSR testing (P < 0.05). MESP testing revealed that all patients passed Level 1, with 53.8 % reaching Level 2, and higher levels achieved progressively over two years. Notably, the proportion of patients passing Level 4 increased dramatically from 7.7 % at six months to 53.8 % at one year and 92.3 % at two years, 53.8 % reached Level 5, and 23 % completed Level 6. IT-MAIS/MAIS scores significantly improved at six months post-activation (P < 0.05) and continued to increase after one and two years, demonstrating enhanced auditory and speech perception capabilities over time. Technology-driven cochlear implantation substantially enhances auditory and speech perception capabilities in patients with auditory neuropathy and internal auditory canal stenosis. These findings establish the substantial therapeutic efficacy of cochlear implantation for this patient population and its potential for broader application in audiological rehabilitation.