Ribeiro Fernando, Marotta Liciere, Eckley Claudia A
Department of Otorhinolaryngology, Santa Casa School of Medicine and Hospitals of São Paulo, São Paulo, Brazil.
Ear Nose Throat J. 2009 Dec;88(12):E18-20.
Modern magnetic resonance imaging has significantly improved the diagnosis of cochlear nerve deficiencies. A careful assessment of all clinical, imaging, and auditory data is of utmost importance in such cases in order to properly establish the site of the abnormality. We report the case of a 3-year-old girl with unilateral cochlear nerve aplasia, normal middle and inner ear anatomy, and an absence of otoacoustic emissions, all of which erroneously suggested cochlear damage. We also briefly review the embryogenesis of the inner ear and auditory pathway. A patient with similar findings in the setting of a bilateral hearing loss and insufficient imaging would be at risk of inappropriate cochlear implantation.
现代磁共振成像显著改善了对蜗神经缺损的诊断。在此类病例中，仔细评估所有临床、影像学和听觉数据对于正确确定异常部位至关重要。我们报告了一例3岁女孩，其单侧蜗神经发育不全，中耳和内耳解剖结构正常，且无耳声发射，所有这些均错误地提示存在耳蜗损伤。我们还简要回顾了内耳和听觉通路的胚胎发生。在双侧听力损失且影像学检查不足的情况下有类似表现的患者，存在不适当进行人工耳蜗植入的风险。
