Department of Head and Neck Surgery, Lariboisière University Hospital, Paris, France.
Neuroradiology Unit, Department of Neuroradiology, Lariboisière University Hospital, 75010, Paris, France.
J Neurol. 2021 Feb;268(2):689-700. doi: 10.1007/s00415-020-10220-y. Epub 2020 Sep 9.
Unilateral vestibular atelectasis (UVA), an entity first described by Merchant and Schuknecht in 1988, has rarely been reported in vivo as of yet. We specify here the clinical characteristics of 22 patients diagnosed with UVA.
Patients with a radiological diagnosis of UVA who underwent delayed inner ear MRI were included between April 2017 and January 2020. Full clinical testing including ocular infrared video-oculography, oVEMPs, cVEMPs, vHIT, bithermal caloric testing and auditory testing was performed.
There were 13 men and 9 women, of mean age 58.6 ± 13.7 years. Onset was more frequently sudden (73%) than insidious (27%) though both clinical presentations were reported, and positional vertigo was described in 41% of cases. There were only two (9%) patients reporting Tullio's phenomenon. Vestibular testing showed that in 90% of cases, there was utricular dysfunction on oVEMP, while in 77% of cases, saccular function was preserved on cVEMP. vHIT showed high-velocity canal function impairment in all 22 patients: 8 patients (36%) had one impaired canal, 5 (23%) had two and 9 (41%) had all three canals affected. Caloric tests found complete unilateral areflexia, in 65% of tested cases, and partial deficiency in 35% of cases. Nine patients (40%) displayed asymmetrical hearing.
We described in this study the various clinical presentations of a disease rarely reported in vivo, UVA. Initial clinical presentation can appear similar to an acute vestibular deficit, a recurrent positional vertigo, or fluctuating dizziness.
单侧前庭萎缩(UVA)是 Merchant 和 Schuknecht 于 1988 年首次描述的一种疾病,目前在体内很少有报道。我们在此描述了 22 例经影像学诊断为 UVA 的患者的临床特征。
纳入 2017 年 4 月至 2020 年 1 月期间接受延迟内耳 MRI 检查的 UVA 放射学诊断患者。进行了全面的临床检查,包括眼红外视频眼震图、oVEMP、cVEMP、vHIT、双温冷热试验和听力测试。
共有 13 名男性和 9 名女性,平均年龄为 58.6±13.7 岁。发病时,73%的患者为突发性,27%的患者为隐匿性,但两种临床表现均有报道,41%的患者有位置性眩晕。只有 2 名(9%)患者报告有 Tullio 现象。前庭测试显示,90%的病例 oVEMP 显示前庭上神经功能障碍,77%的病例 cVEMP 显示球囊功能正常。vHIT 显示所有 22 例患者均有高速度管功能障碍:8 例(36%)有 1 个管功能障碍,5 例(23%)有 2 个管功能障碍,9 例(41%)有 3 个管功能障碍。冷热试验发现,65%的受检病例有单侧完全无反射,35%的病例有部分缺失。9 例(40%)患者有听力不对称。
我们在本研究中描述了一种在体内很少有报道的疾病 UVA 的各种临床表现。最初的临床表现可能与急性前庭功能减退、复发性位置性眩晕或波动性头晕相似。