Halle Tyler R, Soares Bruno P, Todd N Wendell
Department of Otolaryngology-Head & Neck Surgery, Emory University School of Medicine, 1365 Clifton Road, Atlanta, GA, 30322, USA.
Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, 1800 Orleans Street, Baltimore, MD, 21287, USA.
Int J Pediatr Otorhinolaryngol. 2017 Apr;95:5-8. doi: 10.1016/j.ijporl.2017.01.028. Epub 2017 Jan 29.
OBJECTIVES/HYPOTHESIS: We aim to define the frequencies of anomalies of the inner ear, oval window, and round window ipsilateral to isolated non-syndromic unilateral aural atresia.
Retrospective case series. We reviewed high resolution computed tomography scans of the temporal bones of 70 children with isolated non-syndromic unilateral congenital aural atresia. Scans were reviewed according to the Jahrsdoerfer criteria and further evaluated for anomalies of the vestibule, semi-circular canals, cochlea, internal auditory canal and vestibulocochlear nerve.
Inner ear dysplasia was seen in two of 70 atretic ears: one with a dysmorphic lateral semicircular canal and another with a large vestibule assimilating the lateral semicircular canal. Abnormalities of the oval window and round window ipsilateral to the atresia were identified in 21% (15) and 7% (5), respectively, of the atretic ears. Oval window and round window abnormalities were associated with disproportionately lower Jahrsdoerfer scores compared to aural atresia patients without these abnormalities (P < 0.001 and P = 0.04, respectively).
Compared to studies that included syndromic or bilateral atresia cases, we found inner ear and oval window abnormalities less common in children with isolated non-syndromic unilateral aural atresia. However, round window anomalies seem to occur at about the same frequency.
目的/假设：我们旨在确定孤立性非综合征性单侧耳道闭锁同侧内耳、卵圆窗和圆窗异常的发生率。
回顾性病例系列研究。我们回顾了70例孤立性非综合征性单侧先天性耳道闭锁患儿颞骨的高分辨率计算机断层扫描。扫描结果根据雅尔施多费尔标准进行评估，并进一步评估前庭、半规管、耳蜗、内耳道和前庭蜗神经的异常情况。
70只闭锁耳中有2只出现内耳发育异常：1只伴有形态异常的外侧半规管，另1只伴有吸收外侧半规管的大前庭。闭锁同侧卵圆窗和圆窗异常分别在21%（15只）和7%（5只）的闭锁耳中被发现。与没有这些异常的耳道闭锁患者相比，卵圆窗和圆窗异常与明显更低的雅尔施多费尔评分相关（分别为P < 0.001和P = 0.04）。
与纳入综合征性或双侧闭锁病例的研究相比，我们发现孤立性非综合征性单侧耳道闭锁患儿内耳和卵圆窗异常较少见。然而，圆窗异常的发生率似乎大致相同。
