Cochlear Implantation Outcomes in Patients with Auditory Neuropathy Spectrum Disorder of Genetic and Non-Genetic Etiologies: A Multicenter Study
Pei-Hsuan Lin,
Hung-Pin Wu,
Che-Ming Wu,
Yu-Ting Chiang,
Jacob Shujui Hsu,
Cheng-Yu Tsai,
Han Wang,
Li-Hui Tseng,
Pey-Yu Chen,
Ting-Hua Yang,
Chuan-Jen Hsu,
Pei-Lung Chen,
Chen-Chi Wu,
Tien-Chen Liu
Affiliations
Pei-Hsuan Lin
Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei 10002, Taiwan
Hung-Pin Wu
Department of Otolaryngology, Taichung Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Taichung 42743, Taiwan
Che-Ming Wu
Department of Otolaryngology & Head and Neck Surgery, New Taipei Municipal TuCheng Hospital (Built and Operated by Chang Gung Medical Foundation), New Taipei City 23652, Taiwan
Yu-Ting Chiang
Department of Otolaryngology, National Taiwan University Hospital, Taipei 10002, Taiwan
Jacob Shujui Hsu
Graduate Institute of Medical Genomics and Proteomics, College of Medicine, National Taiwan University, Taipei 10051, Taiwan
Cheng-Yu Tsai
Department of Otolaryngology, National Taiwan University Hospital, Taipei 10002, Taiwan
Han Wang
Department of Otolaryngology, National Taiwan University Hospital, Taipei 10002, Taiwan
Li-Hui Tseng
Department of Otolaryngology, National Taiwan University Hospital, Taipei 10002, Taiwan
Pey-Yu Chen
Department of Otolaryngology, MacKay Memorial Hospital, Taipei 10449, Taiwan
Ting-Hua Yang
Department of Otolaryngology, National Taiwan University Hospital, Taipei 10002, Taiwan
Chuan-Jen Hsu
Department of Otolaryngology, National Taiwan University Hospital, Taipei 10002, Taiwan
Pei-Lung Chen
Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei 10002, Taiwan
Chen-Chi Wu
Department of Otolaryngology, National Taiwan University Hospital, Taipei 10002, Taiwan
Tien-Chen Liu
Department of Otolaryngology, National Taiwan University Hospital, Taipei 10002, Taiwan
With diverse etiologies and clinical features, the management of pediatric auditory neuropathy spectrum disorder (ANSD) is often challenging, and the outcomes of cochlear implants (CIs) are variable. This study aimed to investigate CI outcomes in pediatric patients with ANSD of different etiologies. Thirty-six children with ANSD who underwent cochlear implantation between 2001 and 2021 were included. Comprehensive etiological analyses were conducted, including a history review, next-generation sequencing-based genetic examinations, and imaging studies using high-resolution computed tomography and magnetic resonance imaging. Serial behavioral and speech audiometry were performed before and after surgery, and the outcomes with CI were evaluated using the Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores. By etiology, 18, 1, 1, and 10 patients had OTOF-related, WFS1-related, OPA1-related, and cochlear nerve deficiency (CND)-related ANSD, respectively. Six patients had no definite etiology. The average CI-aided behavioral threshold was 28.3 ± 7.8 dBHL, and those with CND-related ANSD were significantly worse than OTOF-related ANSD. The patients’ median CAP and SIR scores were 6 and 4, respectively. Favorable CI outcomes were observed in patients with certain etiologies of ANSD, particularly those with OTOF (CAP/SIR scores 5–7/2–5), WFS1 (CAP/SIR score 6/5), and OPA1 variants (CAP/SIR score 7/5). Patients with CND had suboptimal CI outcomes (CAP/SIR scores 2–6/1–3). Identifying the etiologies in ANSD patients is crucial before surgery and can aid in predicting prognoses.
