House Ear Clinic.
Department of Otolaryngology-Head and Neck Surgery.
Otol Neurotol. 2021 Jul 1;42(6):876-882. doi: 10.1097/MAO.0000000000003051.
To describe the clinical presentation of patients with isolated saccular endolymphathic hydrops (EH) detected.
Clinical case series.
University-based tertiary referral center.
All subjects presenting with vertigo or hearing loss who had isolated saccular EH detected.
High-resolution delayed-contrast magnetic resonance imaging (MRI) conducted between November 2015 and November 2016.
Audiovestibular testing results and analysis of clinical histories.
Isolated saccular EH was detected in 18 subjects. Sixteen met criteria for definite Menière's disease (MD, n = 12) or delayed endolymphatic hydrops (DEH, n = 4). One had a history of sudden sensorineural hearing loss (SSNHL) and 3 years after MRI developed recurrent vertigo characteristic of DEH. One patient had a history of atypical DEH (Tumarkin falls without vertigo following SSNHL). Four patients had Tumarkin falls. Most (83%) demonstrated mild-to-severe low-frequency fluctuating loss, and six (33.3%) had a history of ipsilateral sudden profound SNHL. Nine of the 17 (53%) patients tested had an ipsilateral caloric paresis ranging from 26 to 67%. Ipsilateral vestibular-evoked myogenic potentials showed reduced or absent responses in 5 of the 17 tested (29%).
The full spectrum of MD may be associated with saccular hydrops. We propose that MD and DEH often begin in the saccule, and MRI may provide clues to the pathophysiology of MD. Saccular hydrops was present in one patient with SSNHL who did not develop vertigo spells until 3 years after MRI, indicating that saccular hydrops may be the first manifestation of MD or DEH.
描述孤立球囊内淋巴积水（EH）患者的临床表现。
临床病例系列。
以大学为基础的三级转诊中心。
所有出现眩晕或听力损失并检测到孤立球囊 EH 的患者。
2015 年 11 月至 2016 年 11 月进行高分辨率延迟对比磁共振成像（MRI）。
听前庭测试结果和临床病史分析。
在 18 名患者中检测到孤立球囊 EH。16 名符合明确梅尼埃病（MD）或延迟内淋巴积水（DEH）的标准（MD，n=12；DEH，n=4）。1 名患者有突发性感觉神经性听力损失（SSNHL）病史，MRI 后 3 年出现符合 DEH 的复发性眩晕。1 名患者有非典型 DEH（Tumarkin 摔倒，SSNHL 后无眩晕）病史。4 名患者有 Tumarkin 摔倒。大多数（83%）患者表现为轻度至重度低频波动性听力损失，6 名（33.3%）患者有同侧突发性严重 SNHL 病史。17 名测试患者中有 9 名（53%）有同侧冷热刺激试验减弱，范围为 26%至 67%。17 名测试患者中有 5 名（29%）同侧前庭诱发肌源性电位显示反应减弱或缺失。
MD 的全部表现可能与球囊积水有关。我们提出 MD 和 DEH 通常始于球囊，MRI 可能为 MD 的发病机制提供线索。MRI 后 3 年出现眩晕发作的 SSNHL 患者存在球囊积水，表明球囊积水可能是 MD 或 DEH 的首发表现。
