Congenital ossicular chain malformation (COCM) is a congenital developmental disorder that impairs hearing function. It most commonly presents unilaterally, either as an isolated anomaly or in association with a variety of systemic syndromes; in severe cases, it may coexist with malformations of the auricle and external auditory canal. Owing to its insidious onset and the presence of an intact tympanic membrane, affected children are frequently misdiagnosed with secretory otitis media. Bilateral COCM, if left untreated in a timely manner, can hinder speech and cognitive development, while specific anatomical variants may elevate the risk of cholesteatoma formation. Diagnosis is predicated on the presence of persistent conductive hearing loss from early childhood, with preoperative high-resolution temporal bone CT scanning serving as a pivotal tool for anatomical evaluation. The management of COCM is primarily guided by patient and family preferences, with therapeutic options encompassing hearing aid fitting and surgical intervention. To standardize the diagnosis and treatment of COCM, optimize the selection of surgical strategies, and enhance clinical outcomes, domestic otology experts have jointly developed the "Expert consensus on a novel classification system, surgical management strategies, and prognostic evaluation for congenital ossicular chain malformations based on stapes footplate mobility and suprastructural integrity (2026 edition)". This consensus is grounded in the latest research evidence and accumulated clinical experience, systematically summarizing the epidemiology, pathogenesis, clinical manifestations, auxiliary examinations, treatment principles, and complications of non-syndromic COCM. Building upon existing classification systems, the consensus introduces a novel clinical classification framework centered on stapes footplate mobility and the integrity of suprastapes structures, which directly informs surgical decision-making. This new classification prioritizes guiding surgical strategy formulation and predicting postoperative outcomes, thereby providing a robust reference for the standardized diagnosis and clinical management of COCM.
先天性听骨链畸形是影响听觉的先天发育异常，多单侧发病，可独立存在，也可为多种综合征的表型之一，严重时合并耳廓及外耳道畸形。因病程隐匿、鼓膜完整，儿童易误诊为分泌性中耳炎，双侧受累未及时干预会影响言语与认知发育，部分解剖变异还会增加胆脂瘤风险。诊断依据为自幼稳定的传导性听力下降，术前高分辨率颞骨CT具有重要的诊断价值。先天性听骨链畸形的治疗主要根据患者或其家属意愿，以佩戴助听器和手术为主。为了规范先天性听骨链畸形的诊断与治疗，优化手术策略选择并提高临床疗效，由国内耳科学专家在现有研究证据与临床实践经验基础上共同制订了《基于镫骨底板及底板上结构的先天性听骨链畸形新分型及其手术策略和预后评估专家共识（2026版）》。该共识系统梳理了先天性听骨链畸形（非综合征性）的流行病学、发病机制、临床表现、辅助检查、治疗原则及并发症，并在原有分型的基础上提出以镫骨底板活动性与镫骨板上结构评估为核心，用于指导手术方式选择的新临床分型。新分型旨在精准指导手术方案的选择并有效预测手术预后，从而为先天性听骨链畸形的规范化诊疗提供重要参考。.