Congenital defects of the middle ear - uncommon cause of pediatric hearing loss,

INTRODUCTION: In children, hypoacusis, or conductive hearing loss, is usually acquired; otitis media with effusion is the most common etiology. However, in some cases this condition is congenital, ranging from deformities of the external and middle ear to isolated ossicular chain malformations. The non-ossicular anomalies of the middle ear, for instance, persistent stapedial artery and anomaly of the facial nerve, are uncommon but may accompany the ossicular defects. OBJECTIVE: This study aimed to describe the clinical presentation, diagnostic tests, and therapeutic options of congenital malformations of the middle ear. METHODS: This was a retrospective study of cases followed in otolaryngologic consultations since 2007 with the diagnosis of congenital malformation of the middle ear according to the Teunissen and Cremers classification. A review of the literature regarding the congenital malformation of the middle ear and its treatment is presented. CONCLUSION: Middle ear malformations are rarely responsible for conductive hearing loss in children. As a result, there is often a late diagnosis and treatment of these anomalies, which can lead to delays in the development of language and learning.引言：儿童听力减退（hypoacusis）即传导性听力损失，多为后天获得性；分泌性中耳炎（otitis media with effusion）是最常见的病因。但部分病例的病变为先天性，病变范围可从外耳、中耳畸形延伸至孤立性听骨链（ossicular chain）畸形。中耳的非听骨异常，例如永存镫骨动脉（persistent stapedial artery）和面神经（facial nerve）畸形，虽较为少见，但可伴随听骨缺损一同出现。研究目的：本研究旨在阐述先天性中耳畸形的临床表现、诊断检查手段及治疗选择。研究方法：本研究为回顾性研究，纳入2007年以来于耳鼻咽喉科门诊随访、依据Teunissen与Cremers分类标准确诊为先天性中耳畸形的病例，并综述有关先天性中耳畸形及其治疗的相关文献。研究结论：儿童传导性听力损失的病因中，中耳畸形较为少见。因此此类畸形常被延误诊断与治疗，进而可能导致语言及学习发育迟缓。