MR imaging and clinical characteristics of Lermoyez syndrome

Abstract

Background: Lermoyez syndrome (LS) is a rare auditory disorder, the details of LS remain unclear.

Objectives: To investigate the grades of endolymphatic hydrops (ELH) and clinical characteristics of clinically diagnosed LS.

Material and methods: Nine patients with clinically diagnosed LS were included. three-dimensional-real-infra red imaging characteristics were analyzed. ELH grades and detailed clinical characteristics were evaluated.

Results: The group consisted of two women and seven men with an age at presentation of 48.4 ± 10.9 (28–61) years, an onset age of 40.9 ± 12.0 (23–58) years, and a disease duration of 7.6 ± 7.5 years. Of the nine patients, ELH was observed in 100% of the patients on the clinically affected side. In addition, 22.2% of vestibular ELH and 77.8% of cochlear ELH were classified as mild or moderate in grade.

Conclusions and significance: The patients with LS had Meniere’s triad, fluctuation of hearing that occurred in a reverse relationship to that of the vertiginous attack, and vestibular and cochlear ELH, which may suggest that LS is a variant of MD. However, the sex difference and milder cochlear ELH in LS compared with those in typical MD may indicate an underlying intrinsic difference in the mechanism of LS.

Chinese abstract

背景：Lermoyez综合征（LS）是一种罕见的听觉障碍。该病细节尚不清楚。

目的：探讨临床诊断为LS的内淋巴积水的等级和临床特点。

材料和方法：包括9例经临床诊断为LS的患者。分析了三维实红外成像特性。 ELH等级和详细的临床特征被评估。

结果：研究对象由两名女性和七名男性组成。论文提交时, 他们的年龄为48.4±10.9（28-61）岁。他们的发病年龄为40.9±12.0（23-58）岁, 病程为7.6±7.5年。在这9例患者中, 在临床上100％的患者中观察到ELH患侧。此外, 将22.2％的前庭ELH和77.8％的耳蜗ELH归为轻度或中度。

结论和意义：LS患者患有美尼尔氏三联征、听力波动（与眩晕症发作呈反向关系） 以及前庭和耳蜗ELH, 这可能表明LS是MD的变体。但是, 性别差异和与典型MD相比LS的耳蜗ELH较轻, 可能表明LS机理存在潜在的内在差异。

Keywords:

• Lermoyez syndrome
• endolymphatic hydrops
• MRI

Disclosure statement

No potential conflict of interest was reported by the author(s).

Additional information

Funding

This work was supported by the National Natural Science Foundation of China (No. 81670933).