Gruppo Otologico Piacenza-Roma, Italy.
Acta Otorhinolaryngol Ital. 2010 Jun;30(3):153-5.
Malformations of bony inner ear are rare anomalies occurring in approximately 20% of patients with congenital sensorineural hearing loss. Conductive hearing loss is usually associated with abnormalities of the external and middle ear. Recent reports of patients with lateral semicircular canal malformations indicate inner ear malformations to be associated with sensorineural or conductive hearing loss. Differential diagnosis of conductive hearing loss should include otosclerosis, isolated ossicular deformities, inner ear anomalies or a combination of these. In this report, a case is described with right vestibule-lateral semicircular canal dysplasia presenting at our centre with bilateral otosclerosis.
内耳骨畸形是一种罕见的异常，约发生于 20%的先天性感觉神经性听力损失患者中。传导性听力损失通常与外耳和中耳的异常有关。最近有报道称，患有水平半规管畸形的患者存在内耳畸形与感音神经性或传导性听力损失相关。传导性听力损失的鉴别诊断应包括耳硬化症、孤立的听小骨畸形、内耳异常或这些病变的组合。本报告描述了一例右侧前庭-水平半规管发育不良患者，在我们中心双侧均表现为耳硬化症。
