From the Departments of Radiology (R.H., Y.P.).
Otolaryngology-Head and Neck Surgery (Q.D.), Eye and ENT Hospital of Shanghai Medical School, Fudan University, Shanghai, China.
AJNR Am J Neuroradiol. 2020 Jun;41(6):1076-1080. doi: 10.3174/ajnr.A6576. Epub 2020 May 28.
Incomplete partition type III, also referred to as X-linked deafness, is a rare genetic inner ear malformation. Its characteristic CT findings, including bulbous dilation of the internal auditory canal and absence of the modiolus with the interscalar septa present, have been well-recognized. In this series of 19 cases, we report the abnormalities of the vestibule and semicircular canals and provide a comprehensive description of their CT and MR imaging findings. The inner ear malformations in incomplete partition type III were bilateral and basically symmetric, with involvement of the internal auditory canal, nerve canals in the fundus, cochlea, vestibule, semicircular canals, vestibular aqueduct, otic capsule, round window, oval window, and stapes. An irregular vestibule with a cystic appearance is also a distinctive imaging feature, which could be seen in about 90% of our patients, with a cystic appearance of the semicircular canals present in nearly half of the cases.
不完全分隔型 III 型，也称 X 连锁型耳聋，是一种罕见的内耳遗传性畸形。其典型 CT 表现为：内听道呈球状扩张，耳蜗中轴缺失，骨嵴间分隔存在。在这一系列 19 例患者中，我们报告了前庭和半规管的异常，并对其 CT 和 MRI 影像学表现进行了全面描述。不完全分隔型 III 型的内耳畸形为双侧且基本对称，病变累及内听道、颅底神经管、耳蜗、前庭、半规管、前庭水管、骨迷路、圆窗、卵圆窗和镫骨。不规则的囊性前庭也是一个独特的影像学特征，约 90%的患者可见，近一半的患者可见半规管囊性改变。
