Outcomes of cochlear implantation in 75 patients with auditory neuropathy

Jie Wu; Jiyue Chen; Zhiwei Ding; Jialin Fan; Qiuquan Wang; Pu Dai; Dongyi Han

doi:10.3389/fnins.2023.1281884

Outcomes of cochlear implantation in 75 patients with auditory neuropathy

Front Neurosci. 2023 Nov 13:17:1281884. doi: 10.3389/fnins.2023.1281884. eCollection 2023.

Authors

Jie Wu^#¹, Jiyue Chen^#¹, Zhiwei Ding¹, Jialin Fan¹, Qiuquan Wang¹, Pu Dai¹, Dongyi Han¹

Affiliation

¹ Key Lab of Hearing Impairment Science of Ministry of Education, Key Lab of Hearing Impairment Prevention and Treatment of Beijing, National Clinical Research Center for Otolaryngologic Diseases, College of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China.

^# Contributed equally.

Abstract

Background: Cochlear implantation (CI) outcomes in patients with auditory neuropathy (AN) are variable, which hampers patients' decisions on CI.

Objective: This study aims to assess the outcomes of CI in individuals diagnosed with AN and to examine the various factors that may influence the effectiveness of this intervention.

Methods: A total of 75 patients diagnosed with AN were included in the study. The hearing threshold, the score of categories of auditory performance (CAP), speech intelligibility rating (SIR), and speech audiometry were tested. Genetic testing was conducted by medical exome sequencing in 46 patients.

Results: After CI, the average aided hearing threshold for patients with prelingual and post-lingual onset was 38.25 ± 6.63 dB and 32.58 ± 9.26 dB, respectively; CAP score improved to 5.52 ± 1.64 (p < 0.001) and 6.00 ± 0.96 (p < 0.001), respectively; SIR score increased to 3.57 ± 1.22 (p < 0.001) and 4.15 ± 0.95 (p < 0.001), respectively. Maximum speech recognition ranged from 58 to 93% for prelingual onset patients and 43 to 98% for those with post-lingual onset. Speech outcomes of CI in cases with cochlear nerve (CN) deficiency were significantly poorer (p = 0.008). Molecular etiologies, including TWIST1, ACTG1, m.A7445G, and a copy-number variant (CNV) carrying ACTB, were related to AN here.

Conclusion: CI is a viable therapy option for patients with AN; CN deficiency might impact outcomes of CI.

Keywords: auditory neuropathy; cochlear implantation; cochlear nerve; genetic testing; impact factor.

Grants and funding

The author(s) declare financial support was received for the research, authorship, and/or publication of this article. National Natural Science Foundation of China (No. 81770991) to DH; Nature Science Foundation of Beijing (No. 7191011) to PD; Postdoctoral Research Foundation of China (No. 2018M633731) to JW.