Facial nerve hemangioma in the middle ear

ABSTRACT Facial nerve hemangioma is a rare and benign vascular tumor, and accounts for 0.7% of intratemporal tumors. We report the second case described in the literature of a facial nerve hemangioma in its tympanic segment. A 14-year-old male patient presented with a history of progressive right ear hearing loss with preserved facial mimicry. Pure tone audiometry showed a right ear moderate conductive hearing loss. Magnetic resonance imaging demonstrated an expansive lesion involving the tympanic segment of the right facial nerve, suggestive of hemangioma. Watchful waiting was chosen as management. In the first case of middle ear facial hemangioma described in the literature, facial palsy was the symptom that led the patient to seek medical care. In the present case, it can be inferred that the first symptom was conductive hearing loss ipsilateral to the lesion. Facial palsy may not be present and the clinical presentation may resemble otosclerosis, ossicular chain disruption, and third window abnormalities, among other differential diagnoses of conductive hearing loss. The second case of tympanic portion facial nerve hemangioma is reported, describing the specificity of conductive hearing loss as its only clinical manifestation.摘要：面神经血管瘤（facial nerve hemangioma）是一种罕见的良性血管性肿瘤，占颞骨内肿瘤（intratemporal tumors）的0.7%。本文报告文献记载的第2例发生于鼓室段（tympanic segment）的面神经血管瘤。患者为14岁男性，表现为渐进性右侧听力下降，面部表情肌功能完好。纯音测听（pure tone audiometry）显示右侧中度传导性听力损失（conductive hearing loss）。磁共振成像（magnetic resonance imaging）可见右侧面神经鼓室段存在膨胀性病变，提示为血管瘤。最终选择予以观察随访作为治疗方案。文献中首例中耳面神经血管瘤病例以面瘫（facial palsy）为首发就诊症状，而本次病例可推断首发症状为病变同侧的传导性听力损失。本病可无面瘫表现，临床表现易与耳硬化症（otosclerosis）、听骨链中断（ossicular chain disruption）、第三窗异常（third window abnormalities）等可引发传导性听力损失的鉴别诊断疾病相混淆。本次报告的第2例面神经鼓室段血管瘤病例，其唯一临床表现为具有特征性的传导性听力损失。