Abstract
The stria vascularis (SV) is a highly vascularized tissue lining the lateral wall of the cochlea. The SV maintains cochlear fluid homeostasis, generating the endocochlear potential that is required for sound transduction. In addition, the SV acts as an important blood-labyrinth barrier, tightly regulating the passage of molecules from the blood into the cochlea. A healthy SV is therefore vital for hearing function. Degeneration of the SV is a leading cause of age-related hearing loss, and has been associated with several hearing disorders, including Norrie disease, Meniere’s disease, Alport syndrome, Waardenburg syndrome, and Cytomegalovirus-induced hearing loss. Despite the SV’s important role in hearing, there is still much that remains to be discovered, including cell-specific function within the SV, mechanisms of SV degeneration, and potential protective or regenerative therapies. In this review, we discuss recent discoveries elucidating the molecular regulatory networks of SV function, mechanisms underlying degeneration of the SV, and otoprotective strategies for preventing drug-induced SV damage. We also highlight recent clinical developments for treating SV-related hearing loss and discuss future research trajectories in the field.
Highlights
The stria vascularis (SV) is a highly vascularized tissue located in the lateral wall of the cochlea that contributes to cochlear homeostasis in two ways (Figure 1)
RNA sequencing of the SV collected from melanocyte inducing transcription factor (Mitf)-M animals indicates that ion transport genes such as transmembrane receptor cation channel M1 (Trpm1), K+ inwardly-rectifying channel J13 (Kcnj13), and solute carrier 45A2 (Slc45a2) are downregulated (Chen et al, 2020)
These results suggest that melanin-producing cells in the SV likely contribute to the ionic regulation of the cochlear fluid, ionic measurements are required to confirm this hypothesis
Summary
The stria vascularis (SV) is a highly vascularized tissue located in the lateral wall of the cochlea that contributes to cochlear homeostasis in two ways (Figure 1). Ion transport proteins in the SV perform active potassium ion (K+) recycling between the endolymph of the scala media and the perilymph of the scala tympani This maintains the endolymph at a high K+ (~157 mM), low sodium (Na+; ~1.3 mM) state, that contrasts with the low K+ (~4.2–6.0 mM), high Na+ (~141–148 mM) state of perilymph (Liu et al, 2017; reviewed in Wangemann 2002, Wangemann 2006). This contrasting ionic composition creates an electric potential difference of +80–100 mV between the cochlear fluids, known as the endocochlear potential (Nin et al, 2016). Summarizes notable discoveries regarding SV function and novel opportunities that have been identified for developing SVassociated hearing loss treatments
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