Alahmadi Asma, Abdelsamad Yassin, AlAmari Nouf A, Alyousef Mohammed Y, Al-Momani Murad, Altamimi Fahad N, Alhabib Salman F, Hagr Abdulrahman
King Abdullah Ear Specialist Center (KAESC), College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Research Department, MED-EL GmbH, Riyadh, Saudi Arabia.
Eur Arch Otorhinolaryngol. 2025 May;282(5):2203-2215. doi: 10.1007/s00405-024-09087-w. Epub 2024 Dec 10.
Cochlear nerve deficiency (CND) accounts for about one-third of congenital hearing loss cases and presents several challenges during management. Currently, cochlear implant (CI) and auditory brainstem implants (ABI) represent the primary management options for CND. However, robust evidence on the outcomes of different CND management approaches remains limited. Therefore, this systematic review seeks to update and assess the effectiveness of various CND management approaches.
PubMed, Scopus, Web of Science, and CENTRAL databases.
We conducted an online bibliographic search across these databases. We included both interventional and observational studies evaluating CND management outcomes.
Of the 25 studies included, there were retrospective chart reviews, prospective observational studies, and one phase I clinical trial. Most studies focused on children with cochlear nerve aplasia or hypoplasia, with one study including patients with CHARGE syndrome. The mean age at the time of operation ranged from 0.5 to 4.5 years. Tools like the categories of auditory performance (CAP), speech intelligibility rating (SIR), and speech perception category (SPC) were commonly employed. CI outcomes were reported in 17 studies, while ABI outcomes were reported in 4 studies. The study found that the outcomes of both interventions were variable across the included studies.
While CI is feasible and an option in children with CND, its overall effectiveness is limited in some cases like cochlear nerve aplasia. Conversely, ABI appears to be a promising alternative with better auditory, speech, and learning outcomes.
蜗神经缺如（CND）约占先天性听力损失病例的三分之一，在治疗过程中存在诸多挑战。目前，人工耳蜗（CI）和听觉脑干植入（ABI）是CND的主要治疗选择。然而，关于不同CND治疗方法疗效的有力证据仍然有限。因此，本系统评价旨在更新和评估各种CND治疗方法的有效性。
PubMed、Scopus、科学网和CENTRAL数据库。
我们在这些数据库中进行了在线文献检索。我们纳入了评估CND治疗效果的干预性和观察性研究。
纳入的25项研究中，有回顾性病历审查、前瞻性观察性研究和1项I期临床试验。大多数研究关注蜗神经发育不全或发育不良的儿童，有1项研究纳入了患有CHARGE综合征的患者。手术时的平均年龄在0.5至4.5岁之间。常用的工具如听觉表现类别（CAP）、言语可懂度评分（SIR）和言语感知类别（SPC）。17项研究报告了CI的结果，4项研究报告了ABI的结果。研究发现，在纳入的研究中，两种干预措施的结果各不相同。
虽然CI对CND儿童可行且是一种选择，但在某些情况下，如蜗神经发育不全，其总体有效性有限。相反，ABI似乎是一种有前景的替代方法，具有更好的听觉、言语和学习效果。
