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Objective: We aimed to explore the application of three‐dimensional (3D) quantitative scoring of endolymphatic hydrops (EH) based on gadolinium‐contrast MRI in both the diagnosis and differential diagnosis of Ménière's disease (MD). Methods: Local threshold method based on signal intensities was used to quantitatively measure volumes, and the degree of EH was scored by 3D quantitative scoring. The receiver‐operating characteristic (ROC) curve was used to compare the diagnostic efficacy of this method against conventional evaluation methods. The scores of MD, sudden sensorineural hearing loss (SSNHL), delayed endolymphatic hydrops (DEH), and vestibular migraine (VM) group were compared and correlated with clinical examination results, including pure tone audiometry (PTA), electrocochleogram, and caloric test. Results: The sensitivity and specificity of 3D quantitative scoring were 94.1% and 92.2%, while conventional evaluation methods were 84.3% and 88.2%, respectively. Cochlear score (CR), vestibular score (VR), semicircular canal score (SR), and total scores (TR) in MD group were significantly higher than those in SSNHL group. The rate of diagnosis of MD was significantly higher in the MD group (92.2%) than VM group (27.3%). In MD, PTA was significantly correlated with CR and SR, the ratio of summating potential to action potential (‐SP/AP) of electrocochleogram was significantly correlated with CR, VR, and SR, also canal paresis (CP) value of caloric test was significantly correlated with SR. Conclusion: 3D quantitative scoring demonstrated better diagnostic efficacy than conventional evaluation methods for Ménière's disease, and it may be an effective clinical tool to distinguish MD from SSNHL and VM. The clinical practicality of inner ear‐enhanced MRI was further confirmed. Level of Evidence: IV. [ABSTRACT FROM AUTHOR]

Vertebral artery compression syndrome (VACS) is an under-recognised condition that may be misdiagnosed as motor neuron disease. We report a case presenting features initially suggestive of primary lateral sclerosis (PLS) but later found to be VACS. A 65-year-old man with hypertension was referred to our neurology department in the suspect of PLS. He presented with a 10-year history of involuntary jerk of the left lower limb, which ascended to the left upper limb 9 years later. He also developed intermittent painful spasms with a tendency to drag his left leg. His symptoms fluctuated with blood pressure. Neurological examination revealed upper motor neuron signs without lower motor neuron or sensory involvement. Electrophysiology studies were unremarkable. Brain MRI disclosed the left side of medulla oblongata was compressed by the tortuous left vertebral artery. Diffusion tensor tractography confirmed the corresponding corticospinal tract disruption. He was diagnosed with VACS and treated with antispasmodic medications and antihypertensive drugs. VACS should be considered into the differential diagnoses of PLS. A thorough clinical assessment and careful interpretation of brain MRI with advanced diffusion neuroimages can help confirm the diagnosis. [ABSTRACT FROM AUTHOR]