A systematic review and meta-analysis were conducted to evaluate the diagnostic accuracy of substantia nigra hyper-echogenicity by transcranial sonography (TCS) for the diagnosis of Parkinson's disease (PD). PubMed, Embase and the Cochrane Library were electronically searched from inception to June 2018 for all relevant studies. The methodological quality of each study was evaluated by two independent reviewers, who used the Quality Assessment of Diagnostic Accuracy Studies 2 tool. Articles reporting information sufficient to calculate the sensitivity and specificity of TCS to diagnose PD were included. Statistical analysis included data pooling, heterogeneity testing, sensitivity analyses and forest meta-regression. Thirty-nine studies (3123 participants with PD) were analyzed. The pooled sensitivity and specificity of TCS were 0.84 (95% confidence interval: 0.81 - 0.87) and 0.85 (0.80 - 0.88), respectively, for differentiating PD from normal controls or participants with other parkinsonian syndromes. In the secondary outcome, PD participants exhibited a significant increase in substantia nigra areas than either normal controls (0.14 [0.12 - 0.16], p < 0.0001) or participants with other parkinsonian syndromes (0.11 [0.08 - 0.13], p < 0.0001). This meta-analysis revealed the high diagnostic performance of TCS in differentiating patients with PD from both normal controls and participants with other parkinsonian syndromes. (E-mail: xurenfantjh@163.com) (C) 2018 The Author(s). Published by Elsevier Inc. on behalf of World Federation for Ultrasound in Medicine & Biology.