Retrospective analysis of gadolinium imaging data of 13 patients (21 ears) with large vestibular aqueduct syndrome (LVAS group) and 23 patients (23 ears) with normal hearing (normal group) diagnosed clinically at the Affiliated Hospital of Jining Medical College from July 2013 to September 2018. First, 3D steady state interference (3D-CISS) and 3D liquid attenuation inversion recovery (3D-FLAIR) sequence MRI scans were performed. Diluted gadolinium contrast agent was injected into the tympanic cavity through the tympanic membrane, and 3D-FLAIR scans were performed after 1, 2, 3, and 6 days, respectively. Score the display of cochlear, vestibular, and semicircular lymphatic spaces between two groups, and compare the scoring results using Wilcoxon rank sum test. Measure the signal intensity of the lymphatic vessel (ED) and lymphatic sac (ES) in the LVAS group before and after injection of contrast agent, calculate the signal intensity ratio (SIR) with the same layer of brainstem, and compare the differences before and after injection using one-way analysis of variance. The results showed that the cochlea, vestibule, and semicircular canals of the LVAS group and the normal group showed low signal intensity on 3D-FLAIR before injection of contrast agent; After injection of contrast agent, the contrast agent was distributed in the cochlea of the inner ear, vestibule, and perilymphatic space of the semicircular canal. There was no statistically significant difference in display scores between the two groups (Z=-0.95, P=0.34). Both ED and ES were not displayed in the normal group, and there was no change in signal intensity after injection of contrast agent; The ED and ES regions of the LVAS group showed complete low signal in 2 ears on 3D-FLAIR, with clear boundaries between low and high signal in 19 ears. After injection of contrast agent, the signal intensity of the low signal area in the ED and ES areas on the affected side increased, and there was a statistically significant difference in SIR values before and after injection of contrast agent (F=111.93, P<0.01). The results of this study suggest that there is no significant lymphatic hydrops in the inner ear (cochlea, vestibule, and semicircular canal) of LVAS patients; The ED and ES of the affected ear may be connected to the anterior extramural lymphatic space; There may be gaps between ED and ES in some affected ears.