The Russell H. Morgan Department of Radiology and Radiological Sciences, The Johns Hopkins Medical Institutions, 600 N. Wolfe St., Phipps B100F, Baltimore, MD 21287, USA.
Pediatr Radiol. 2012 Dec;42(12):1449-55. doi: 10.1007/s00247-012-2490-3. Epub 2012 Aug 31.
Although dysplasia of inner ear structures in Down syndrome has been reported in several histopathological studies, the imaging findings have not been widely studied.
To evaluate the prevalence and clinical significance of inner ear anomalies detected on CT images in patients with Down syndrome.
The temporal bone CT images of patients with Down syndrome were assessed for inner ear anomalies; clinical notes and audiograms were reviewed for hearing loss. Logistic regression models were employed to identify which CT findings were associated with sensorineural hearing loss (SNHL).
Inner ear anomalies were observed in 74.5% (38/51) of patients. Malformed bone islands of lateral semicircular canal (LSCC), narrow internal auditory canals (IACs), cochlear nerve canal stenoses, semicircular canal dehiscence (SCCD), and enlarged vestibular aqueducts were detected in 52.5% (53/101), 24.5% (25/102), 21.4% (21/98), 8.8% (9/102) and 2% (2/101) of patients' ears, respectively. IAC stenosis had the highest odds ratio (OR = 5.37, 95% CI: 1.0-28.9, P = 0.05) for SNHL.
Inner ear anomalies occurred in 74.5% of our population, with malformed (<3 mm) bone island of LSCC being the most common (52.5%) anomaly. Narrow IAC was seen in 24.5% of patients with Down syndrome and in 57.1% of ears with SNHL. High-resolution CT is a valuable for assessing the cause of hearing loss in people with Down syndrome.
尽管已有几项组织病理学研究报道了唐氏综合征内耳结构发育不良，但影像学表现尚未得到广泛研究。
评估唐氏综合征患者 CT 图像上内耳异常的发生率及其临床意义。
评估唐氏综合征患者的颞骨 CT 图像是否存在内耳异常，并对听力损失的临床记录和听力图进行回顾。采用逻辑回归模型来确定哪些 CT 表现与感音神经性听力损失（SNHL）相关。
51 例患者中有 74.5%（38/51）存在内耳异常。53 例（101 耳）患者存在外侧半规管骨岛畸形，25 例（102 耳）患者存在内听道狭窄，21 例（98 耳）患者存在蜗神经管狭窄，8.8%（102 耳）患者存在半规管骨裂，2%（101 耳）患者存在前庭导水管扩大。内听道狭窄的感音神经性听力损失比值比（OR）最高（OR=5.37，95%CI：1.0-28.9，P=0.05）。
我们的研究人群中 74.5%存在内耳异常，最常见的异常为（52.5%）LSCC 骨岛畸形（<3mm）。24.5%的唐氏综合征患者存在内听道狭窄，57.1%的存在 SNHL 的患者存在内听道狭窄。高分辨率 CT 是评估唐氏综合征患者听力损失原因的一种有价值的方法。
