Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Otol Neurotol. 2024 Oct 1;45(9):971-984. doi: 10.1097/MAO.0000000000004274.
To assess hearing and speech outcomes in pediatric patients with bilateral cochlear nerve deficiency (CND) who underwent cochlear implantation (CI) and to identify factors associated with improved outcomes.
PubMed, Embase, Web of Science, and Cochran databases were searched following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.
Studies that reported hearing and speech outcomes of pediatric patients with bilateral CND who underwent CI were included. Demographics, comorbidities, inner ear abnormalities, CND classification (aplasia or hypoplasia), details of diagnostic workup, and outcomes data were extracted. Outcomes were assessed using the four-level auditory performance level (APL) scale. Meta-analysis, using Cochran-Armitage tests, was performed on patients with individual data to assess factors associated with performance.
A total of 314 papers were screened, and 40 papers with 378 total patients met inclusion criteria. A total of 339 patients had patient-level data and were included in the meta-analysis. Of the 339 patients, 19% (n = 63) of patients had no measurable stimulation, 28% (n = 95) had improved detection, 22% (n = 76) achieved closed-set speech perception, and 31% (n = 105) achieved open-set speech perception. Patients with cochlear nerve aplasia (p = 0.016) and syndromes (p < 0.001) had significantly worse APL scores relative to patients with cochlear nerve hypoplasia and patients without syndromes, respectively.
While most patients with bilateral CND benefit from CI and almost one-third of patients achieved open-set speech perception, outcomes were heterogenous and one-fifth of patients did not experience measurable benefit from CI.
评估双侧耳蜗神经发育不良（CND）行人工耳蜗植入（CI）的儿科患者的听力和言语康复效果，并确定与改善效果相关的因素。
按照系统评价和荟萃分析的首选报告项目指南，检索 PubMed、Embase、Web of Science 和 Cochrane 数据库。
纳入报告双侧 CND 行 CI 的儿科患者听力和言语康复效果的研究。提取患者人口统计学、合并症、内耳异常、CND 分类（发育不全或发育不良）、诊断评估详细信息和结局数据。使用四级听觉绩效水平（APL）量表评估结局。对具有个体数据的患者进行荟萃分析，使用 Cochran-Armitage 检验评估与性能相关的因素。
共筛选出 314 篇论文，40 篇论文（共 378 例患者）符合纳入标准。共有 339 例患者具有患者水平数据，纳入荟萃分析。在 339 例患者中，19%（n=63）患者无可测量刺激，28%（n=95）患者检测能力提高，22%（n=76）患者实现了闭口音组言语感知，31%（n=105）患者实现了开放式言语感知。与耳蜗神经发育不良和无综合征患者相比，耳蜗神经发育不全患者（p=0.016）和综合征患者（p<0.001）的 APL 评分显著更差。
尽管大多数双侧 CND 患者从 CI 中获益，且近三分之一的患者实现了开放式言语感知，但结局存在异质性，五分之一的患者未从 CI 中获得可测量的获益。
