Abstract：
Hearing impairments and dental anomalies are found in many genetic syndromes. Otodental syndrome is a rare combination of hearing loss and the presence of a pathognomonic dental phenotype known as globodontia, in which the tooth exhibits an abnormal globe shape. There is no histologic evidence of structural anomalies in the enamel, dentin, or pulp. This report describes the case of a 12-year-old boy who had hearing loss and 2 supernumerary globe-shaped teeth in the sites of the permanent maxillary central incisors. The diagnosis of otodental syndrome was established based on the clinical, radiographic, and histologic features, but other conditions, including dens evaginatus, talon cusp, dens invaginatus, and compound odontoma, should be included in the differential diagnosis. Dental treatment consisted of the extraction of both anomalous teeth, allowing spontaneous eruption of the impacted permanent central incisors. Early diagnosis of otodental syndrome permits a multidisciplinary approach to prevent other pathologic conditions, reduce functional damage, and avoid social problems.
摘要：
在许多遗传综合征中发现听力障碍和牙齿异常。耳牙综合征是一种罕见的组合，听力损失和存在称为globodontia的病理性牙齿表型，牙齿表现出异常的球形。没有组织学证据表明牙釉质结构异常，牙本质,或纸浆。本报告描述了一个12岁男孩的案例，该男孩在永久性上颌中切牙的部位有听力损失和2颗多余的球形牙齿。根据临床建立耳神经综合征的诊断，射线照相,和组织学特征，但是其他条件，包括evaginatus,塔伦尖点,凹窝，和复合牙瘤，应包括在鉴别诊断中。牙科治疗包括拔除两颗异常牙齿，允许受影响的永久性中切牙自发喷发。耳综合征的早期诊断允许多学科方法来预防其他病理状况。减少功能损伤，避免社会问题。
