Background: Currently, 3 Tesla-MRI following intratympanic gadolinium injection has made it possible to assess the existence and the severity of hydrops in each compartment of the endolymphatic spaces in vivo. However, the relationship between vestibular endolymphatic hydrops (EH) visualized by MRI and vestibular functional tests, especially the correlation between caloric test, video-head impulse test, and semicircular canal hydrops, has not been well-investigated.Objective: The purpose of this study is to investigate the relationship between the severity of EH in each compartment of otoliths and semicircular canal and the results of vestibular functional tests.Methods: In this retrospective study, we performed three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) sequences following intratympanic gadolinium injection in 69 unilateral patients with definite Menière's disease. Vestibular and lateral semicircular canal hydrops was graded on MRI using a four grade criterion. All patients underwent pure-tone audiometry, cervical vestibular evoked myogenic potential (cVEMP), ocular vestibular evoked myogenic potential (oVEMP), caloric test and video head impulse test (vHIT). The latency, amplitude and asymmetry ratio of VEMP, canal paresis (CP) and vestibulo-ocular reflex (VOR) gain of lateral semicircular canal of vHIT were collected. The correlation analysis were performed between the parameters of function test and EH.Results: Vestibular EH showed correlations with the duration of disease (r = 0.360) and pure tone average (r = 0.326). AR of cVEMP showed correlations with Vestibular EH (r = 0.407). CP (r = 0.367) and VOR gain of lateral semicircular canal at 60 ms (r = 0.311) showed correlations with lateral semicircular canal hydrops.Conclusion: EH in different compartments is readily visualized by using 3D-FLAIR MRI techniques. The degree of vestibular EH correlated with AR of cVEMP and EH in the semicircular canal ampullar affects the caloric and vHIT response in patients with unilateral Meniere‘s disease.

GJB2 and GJB6 are adjacent genes encoding connexin 26 (Cx26) and connexin 30 (Cx30), respectively, with overlapping expressions in the inner ear. Both genes are associated with the commonest monogenic hearing disorder, recessive isolated deafness DFNB1. Cx26 plays an important role in auditory development, while the role of Cx30 in hearing remains controversial. Previous studies found that Cx30 knockout mice had severe hearing loss along with a 90% reduction in Cx26, while another Cx30 knockout mouse model showed normal hearing with nearly half of Cx26 preserved. In this study, we used CRISPR/Cas9 technology to establish a new Cx30 knockout mouse model (Cx30−/−), which preserves approximately 70% of Cx26. We found that the 1, 3, and 6-month-old Cx30−/− mice showed mild hearing loss at full frequency. Immunofluorescence and HE staining suggested no significant differences in microstructure of the cochlea between Cx30−/− mice and wild-type mice. However, transmission electron microscopy showed slight cavity-like damage in the stria vascularis of Cx30−/− mice. And Cx30 deficiency reduced the production of endocochlear potential (EP) and the release of ATP, which may have induced hearing loss. Taken together, this study showed that lack of Cx30 can lead to hearing loss with an approximately 30% reduction of Cx26 in the present Cx30 knockout model. Hence, Cx30 may play an important rather than redundant role in hearing development.

Background: Although magnetic resonance imaging (MRI) of the membranous labyrinth and electrocochleography (ECochG) have been used to diagnose endolymphatic hydrops (ELH) in patients with Ménière's disease (MD), the relationship between imaging and ECochG is not well-documented.Objectives: This study evaluates the ELH using 3D-FLAIR MRI and extra-tympanic ECochG (ET-ECochG) and correlates the results from 3D-FLAIR MRI to those from ET-ECochG.Materials and Methods: 3D-FLAIR MRI images of 50 patients were assessed using a 2D volume-referencing grading system (VR scores, relative scores according to the known volumes of the cochlea, vestibule, and semicircular canals). Forty healthy subjects were included and compared to 51 definite MD ears of 50 patients while analyzing the ET-ECochG, which used a self-made bronze foil electrode. The amplitude ratio of the summating potential (SP) to the action potential (AP) (SP/AP) and the area ratio of SP to AP (Asp/Aap) were collected. Relative ELH grade scores were then correlated to ET-ECochG (SP/AP, Asp/Aap).Results: The VR scores showed a better correlation (r = 0.88) with the pure tone average (PTA), disease duration, and vertigo frequency of MD than the Bernaerts scores (grading the cochlea and vestibule separately) (r = 0.22). The SP/AP and Asp/Aap of the unilateral MD patients were statistically comparable to those measured in contralateral ears and the results between the definite MD ears with healthy ears were statistically comparable (p < 0.05). In a ROC analysis Asp/Aap (area under curve, AUC 0.98) significantly (p = 0.01) outperformed SP/AP (AUC 0.91). The total score of ELH, vestibular ELH, and cochlear ELH were also correlated with SP/AP and Asp/Aap. The strongest correlation was found between the Asp/Aap and cochlear ELH (r = 0.60).Conclusion: The 2D volume-referencing grading system was more meaningful than the Bernaerts scores. A correlation was found between ELH revealed by 3D-FLAIR MRI and the SP/AP of ET-ECochG in evaluating definite MD patients. The Asp/Aap appeared a more sensitive and reliable parameter than SP/AP for diagnosing the ELH of the membranous labyrinth.