The cochlear matrisome: Importance in hearing and deafness.

• In the cochlea, sound detection highly relies on the extracellular matrix (ECM) integrity, and hereditary deafness in humans is caused by mutations in numerous ECM-related genes. • Cochlear matrisome plays an active role during the development of the hearing organ and the establishment of its unique architecture. • Cochlear matrisome changes with ageing and in response to cochlear insults, such as noise exposure, ototoxic drug administration and insertion of cochlear implants. • ECM remodeling exerts protective functions or improves tissue repair after injury but may also exacerbate cochlear damage. Deciphering the role of individual ECM components and regulators might provide valuable therapeutic targets for hearing preservation. The extracellular matrix (ECM) consists in a complex meshwork of collagens, glycoproteins, and proteoglycans, which serves a scaffolding function and provides viscoelastic properties to the tissues. ECM acts as a biomechanical support, and actively participates in cell signaling to induce tissular changes in response to environmental forces and soluble cues. Given the remarkable complexity of the inner ear architecture, its exquisite structure-function relationship, and the importance of vibration-induced stimulation of its sensory cells, ECM is instrumental to hearing. Many factors of the matrisome are involved in cochlea development, function and maintenance, as evidenced by the variety of ECM proteins associated with hereditary deafness. This review describes the structural and functional ECM components in the auditory organ and how they are modulated over time and following injury. [ABSTRACT FROM AUTHOR]