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Review
. 2023 Feb:428:108682.
doi: 10.1016/j.heares.2022.108682. Epub 2022 Dec 24.

The role of the stria vascularis in neglected otologic disease

J Dixon Johns  1 , Samuel M Adadey  2 , Michael Hoa  3
Affiliations
Review

The role of the stria vascularis in neglected otologic disease

J Dixon Johns et al. Hear Res. 2023 Feb.

Abstract

The stria vascularis (SV) has been shown to play a critical role in the pathogenesis of many diseases associated with sensorineural hearing loss (SNHL), including age-related hearing loss (ARHL), noise-induced hearing loss (NIHL), hereditary hearing loss (HHL), and drug-induced hearing loss (DIHL), among others. There are a number of other disorders of hearing loss that may be relatively neglected due to being underrecognized, poorly understood, lacking robust diagnostic criteria or effective treatments. A few examples of these diseases include autoimmune inner ear disease (AIED) and/or autoinflammatory inner ear disease (AID), Meniere's disease (MD), sudden sensorineural hearing loss (SSNHL), and cytomegalovirus (CMV)-related hearing loss (CRHL). Although these diseases may often differ in etiology, there have been recent studies that support the involvement of the SV in the pathogenesis of many of these disorders. We strive to highlight a few prominent examples of these frequently neglected otologic diseases and illustrate the relevance of understanding SV composition, structure and function with regards to these disease processes. In this study, we review the physiology of the SV, lay out the importance of these neglected otologic diseases, highlight the current literature regarding the role of the SV in these disorders, and discuss the current strategies, both approved and investigational, for management of these disorders.

Keywords: Autoimmune inner ear disease (AIED); Autoinflammatory inner ear disease (AID); Cytomegalovirus-related hearing loss (CRHL); Meniere's disease (MD); Stria vascularis (SV); Sudden sensorineural hearing loss (SSNHL).

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Figures

Figure 1:
Figure 1:
Schematic of Stria Vascularis (SV) A, The mammalian cochlea consists of 3 fluid-filled chambers with two chambers (scala tympani and scala vestibuli) containing perilymph and one chamber (scala media) containing endolymph. The SV is housed in the lateral wall of the cochlea and its 3 main cell layers are composed of marginal cells (MC), intermediate cells (IC), and basal cells (BC), respectively, with the marginal cells facing the endolymph. The SV generates the highly positive endocochlear potential (+80 mV or greater) and is responsible for the high potassium concentration of the endolymph which distinguishes it from the perilymph in the other two fluid-filled chambers. Fibrocyte populations in the spiral ligament (Types I-V) are also depicted. B, Magnified view of the layers of the stria vascularis with major ion channels implicated in its function. Marginal cells excrete potassium into the endolymph and accomplish this process through their expression of a group of ion channels, including KCNE1/KCNQ1, SLC12A2, and NKA. Marginal cells are connected by tight junctions apically. Marginal cells extend processes basally toward the intermediate cells which also extend processes apically toward the marginal cells and basally towards the basal cells. Macrophages, pericytes, and endothelial cells form the basis for the blood-labyrinth barrier (BLB). The space between the marginal and intermediate cells forms the intrastrial space which has a low potassium and the inward-rectifier K+ channel, KCNJ10, concentrates potassium in the intermediate cells and is critical to the ability of the stria vascularis to both generate the endocochlear potential (EP) and regulate cochlear ionic homeostasis in the endolymph. Both basal and intermediate cells expression gap junctions. Basal cells along with intermediate cells and fibrocytes function together as a syncytium and are connected through gap junctions and maintain a barrier through a network of tight junctions. C, Rare spindle and root cells are located at the superior and inferior periphery of the stria vascularis and the region adjacent to the spindle cells, respectively. Inferiorly, the spindle cells overlie the spiral prominence and extend superiorly toward the edge of the stria vascularis, contacting marginal cells. Spindle cells and root cells both are known to express SLC26A4 (otherwise known as pendrin) and KCNJ16 (Korrapati, Taukulis et al., 2019; Gu et al., 2020). Abbreviations: Na+-K+-ATPase (NKA), Na+/K+/Cl− symporter 1 (SLC12A2), potassium voltage-gated channel subfamily E regulatory subunit 1 (KCNE1), potassium voltage-gated channel subfamily Q regulatory subunit 1 (KCNQ1), ATP-sensitive inward-rectifier potassium channel 10 (KCNJ10), solute carrier family 26 member 4 (SLC26A4) otherwise known as pendrin, potassium inwardly rectifying channel subfamily J member 16 (KCNJ16).
Figure 2.
Figure 2.
Spectrum of Autoinflammatory and Autoimmune Disorders The figure depicts a few examples of self-directed inflammation disorders and the genes that have been associated with them. Self-directed inflammation disorders lie on a spectrum between autoinflammatory disorders, which involve proteins that are expressed in innate immune cell pathways, or autoimmune disorders, which involve proteins that are associated with adaptive immune pathways. There are disorders that may involve both innate and adaptive immune responses but present with prominent components of either autoimmune or autoinflammatory pathways as depicted. (Adapted from McGonagle et al. (2006)(McGonagle and McDermott, 2006); AIRE, autoimmune regulator protein; ALPS, autoimmune lymphoproliferative syndrome; APS-1, autoimmune polyendocrine syndrome-1; CIAS, cold-induced autoinflammatory syndrome 1; TNFRSF, TNF super family receptor; CINCA, chronic infantile neurologic, cutaneous and articular syndrome; CMRO, chronic multifocal recurrent osteomyelitis; CTLA4, cytotoxic T-lymphocyte antigen 4; FCAS, familial cold autoinflammatory syndrome; FMF, familial Mediterranean fever; HIDS, hyper-IgD syndrome; IPEX, immune dysregulation, polyendocrinopathy, enteropathy, X-linked; FOXP3, forkhead box P3; FEFV, Mediterranean fever protein; MWS, Muckle-Wells syndrome; NALP, Nacht, LRR, and PYD domains; PSTPIP1, proline serine threonine phosphatase-interacting protein; NOD, nucleotide-binding oligomerization domain; PAPA, pyogenic sterile arthritis, pyoderma gangrenosum, and acne; SLE, systemic lupus erythematosus; T1D, type 1 diabetes; TLR4, Toll receptor-4; TRAPS, TNF-receptor-associated periodic syndrome.)
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