scholarly journals Identification of Potential Meniere's Disease Targets in the Adult Stria Vascularis

2021 ◽  
Vol 12 ◽  
Author(s):  
Shoujun Gu ◽  
Rafal Olszewski ◽  
Lacey Nelson ◽  
Alvaro Gallego-Martinez ◽  
Jose Antonio Lopez-Escamez ◽  
...  

The stria vascularis generates the endocochlear potential and is involved in processes that underlie ionic homeostasis in the cochlear endolymph, both which play essential roles in hearing. The histological hallmark of Meniere's disease (MD) is endolymphatic hydrops, which refers to the bulging or expansion of the scala media, which is the endolymph-containing compartment of the cochlea. This histologic hallmark suggests that processes that disrupt ion homeostasis or potentially endocochlear potential may underlie MD. While treatments exist for vestibular symptoms related to MD, effective therapies for hearing fluctuation and hearing loss seen in MD remain elusive. Understanding the potential cell types involved in MD may inform the creation of disease mouse models and provide insight into underlying mechanisms and potential therapeutic targets. For these reasons, we compare published datasets related to MD in humans with our previously published adult mouse stria vascularis single-cell and single-nucleus RNA-Seq datasets to implicate potentially involved stria vascularis (SV) cell types in MD. Finally, we provide support for these implicated cell types by demonstrating co-expression of select candidate genes for MD within SV cell types.

1982 ◽  
Vol 90 (4) ◽  
pp. 470-481 ◽  
Author(s):  
Masaaki Kitahara ◽  
Taizo Takeda ◽  
Yoshiro Yazawa ◽  
Hideharu Matsubara ◽  
Hitoshi Kitano

Experimental hydrops caused by underabsorption of endolymphatic fluid is a model of remissional stage of Meniere's disease. In this study, another type of model, ie, hydrops caused by overproduction of endolymphatic fluid, was accomplished by applying various pressures into scala media through a micropipette via stria vascularis. This type of hydrops could be a model of attacks of Meniere's disease. By using two types of the model, effects of glycerol administration and of opening the endolymphatic sac were discussed.


2009 ◽  
Vol 30 (7) ◽  
pp. 1006-1012 ◽  
Author(s):  
Shin Kariya ◽  
Sebahattin Cureoglu ◽  
Hisaki Fukushima ◽  
Shigenobu Nomiya ◽  
Rie Nomiya ◽  
...  

2016 ◽  
Vol 274 (2) ◽  
pp. 757-763 ◽  
Author(s):  
Roberto Teggi ◽  
Laura Zagato ◽  
Simona Delli Carpini ◽  
Lorena Citterio ◽  
Claudia Cassandro ◽  
...  

1995 ◽  
Vol 104 (11) ◽  
pp. 858-863 ◽  
Author(s):  
Elizabeth M. Keithley ◽  
Steve Horowitz ◽  
Michael J. Ruckenstein

Meniere's disease has traditionally been thought to arise from a disruption in longitudinal endolymphatic flow. This view has been brought into question by recent experimental studies that have focused attention on derangements of cochlear fluid and electrolyte homeostatic mechanisms in Meniere's disease, including abnormalities in Na,K-ATPase enzymes found in the cochlear lateral wall. The current study examined the immunohistochemical labeling pattern of the major ion-transporting enzyme of the stria vascularis, Na,K-ATPase, in archival sections of hydropic and nonhydropic human temporal bones for increased density of label that could indicate overproduction of fluid. The results showed good labeling of the stria vascularis in the celloidin sections. The hydropic ears tended to have darker label, but the difference was not statistically significant. The findings are consistent with normal functioning of the stria vascularis in cases of Meniere's disease.


F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 1295 ◽  
Author(s):  
Jose Antonio Lopez-Escamez ◽  
Angel Batuecas-Caletrio ◽  
Alexandre Bisdorff

Ménière’s disease (MD) represents a heterogeneous group of relatively rare disorders with three core symptoms: episodic vertigo, tinnitus, and sensorineural hearing loss involving 125 to 2,000 Hz frequencies. The majority of cases are considered sporadic, although familial aggregation has been recognized in European and Korean populations, and the search for familial MD genes has been elusive until the last few years. Detailed phenotyping and cluster analyses have found several clinical predictors for different subgroups of patients, which may indicate different mechanisms, including genetic and immune factors. The genes associated with familial MD areCOCH,FAM136A,DTNA,PRKCB,SEMA3D, andDPT. At least two mechanisms have been involved in MD: (a) a pro-inflammatory immune response mediated by interleukin-1 beta (IL-1β), tumor necrosis factor alpha (TNFα), and IL-6, and (b) a nuclear factor-kappa B (NF-κB)-mediated inflammation in the carriers of the single-nucleotide variant rs4947296. It is conceivable that microbial antigens trigger inflammation with release of pro-inflammatory cytokines at different sites within the cochlea, such as the endolymphatic sac, the stria vascularis, or the spiral ligament, leading to fluid imbalance with an accumulation of endolymph. Computational integration of clinical and “omics” data eventually should transform the management of MD from “one pill fits all” to precise patient stratification and a personalized approach. This article lays out a proposal for an algorithm for the genetic diagnosis of MD. This approach will facilitate the identification of new molecular targets for individualized treatment, including immunosuppressant and gene therapy, in the near future.


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