内耳畸形儿童和年轻成人单侧与双侧人工耳蜗植入后听觉和口语技能的纵向结局。
Longitudinal outcomes of auditory and spoken language skills following unilateral and bilateral cochlear implantation in children and young adults with inner ear malformations.
文献信息
| PMID | 42804928 |
|---|---|
| 原文 | 在 PubMed 查看原文 ↗ |
| 发表日期 | 2026 |
| 作者 | Carlos Alberto Conceição Santana Júnior |
| 作者单位 | Hospital for Rehabilitation of Craniofacial Anomalies (HRAC), University of São Paulo, Bauru, São Paulo, Brazil; Cochlear Implant Section, Hospital for Rehabilitation of Craniofacial Anomalies (HRAC), University of São Paulo, Bauru, São Paulo, Brazil; Bauru School of Medicine, University of São Paulo, Bauru, São Paulo, Brazil. Electronic address: carlosfono@usp.br. |
| 期刊 | International journal of pediatric otorhinolaryngology |
| SCI 分区 | Q2 |
| IF | 1.7 |
| 研究类型 | 临床研究 · 临床 |
| 所属专科 | 耳科 |
中文摘要
引言： 重度至极重度感音神经性听力损失显著损害口语习得和认知发展。人工耳蜗（CI）绕过受损的内耳结构直接刺激耳蜗神经，是一种有效的康复策略。内耳畸形（IEM）常与先天性听力损失相关；然而，解剖变异性对术后功能结局的影响仍不完全清楚。
目的： 探讨经放射学确诊的IEM患者在接受人工耳蜗植入后听觉技能、口语发展和言语感知的纵向轨迹。
方法： 在一家国家级三级转诊中心进行了一项回顾性纵向观察性研究，纳入19例IEM患者（22只植入耳）。分析了人口学、解剖学、手术和听力学变量。使用线性混合模型（LMM）评估听觉和语言类别的纵向变化，而横断面比较则使用Fisher精确检验、Mann-Whitney U检验和Kruskal-Wallis检验进行。
结果： 在24个月的随访期间，听觉和语言类别均观察到显著的纵向改善（时间效应，p < 0.001）。对于听觉类别，LMM显示显著的时间×EVA交互作用（p = 0.017），表明前庭导水管扩大（EVA）患者的听觉改善速率比其他畸形患者更陡峭。相比之下，语言类别在两组中改善相似，没有显著的时间×EVA交互作用（p = 0.998）。开放式词汇和音素识别在12个月和24个月后也显著改善（p < 0.001）。探索性分析表明，解剖严重程度越高，12个月和24个月时的听觉结局越差。
结论： 在术后头两年，人工耳蜗植入导致听觉技能、口语和言语感知的逐步改善。尽管所有畸形类型的听觉表现均有改善，但EVA患者的纵向听觉恢复更快，而口语发展轨迹与解剖亚型无关，具有可比性。
英文摘要
INTRODUCTION: Severe-to-profound sensorineural hearing loss significantly impairs spoken language acquisition and cognitive development. Cochlear implants (CIs) bypass damaged inner ear structures to directly stimulate the cochlear nerve and represent an effective rehabilitation strategy. Inner ear malformations (IEMs) are frequently associated with congenital hearing loss; however, the influence of anatomical variability on postoperative functional outcomes remains incompletely understood.
OBJECTIVE: To investigate the longitudinal trajectories of auditory skills, spoken language development, and speech perception following cochlear implantation in patients with radiologically confirmed IEMs.
METHODS: A retrospective longitudinal observational study was conducted at a national tertiary referral center including 19 patients (22 implanted ears) with IEMs. Demographic, anatomical, surgical, and audiological variables were analyzed. Longitudinal changes in Auditory and Language Categories were evaluated using Linear Mixed Models (LMMs), whereas cross-sectional comparisons were performed using Fisher's Exact, Mann-Whitney U, and Kruskal-Wallis tests.
RESULTS: Significant longitudinal improvements were observed in both Auditory and Language Categories throughout the 24-month follow-up (Time effect, p < 0.001). For Auditory Category, the LMM demonstrated a significant Time × EVA interaction (p = 0.017), indicating a steeper rate of auditory improvement in patients with enlarged vestibular aqueduct (EVA) than in those with other malformations. In contrast, Language Category improved similarly in both groups, with no significant Time × EVA interaction (p = 0.998). Open-set word and phoneme recognition also improved significantly after 12 and 24 months (p < 0.001). Exploratory analyses demonstrated that greater anatomical severity was associated with poorer auditory outcomes at 12 and 24 months.
CONCLUSION: Cochlear implantation resulted in progressive improvements in auditory skills, spoken language, and speech perception during the first two postoperative years. Although auditory performance improved across all malformation types, patients with EVA exhibited a faster longitudinal auditory recovery, while spoken language development followed comparable trajectories irrespective of anatomical subtype.