Review Article

The Role of Autoimmunity in the Pathogenesis of Sudden Sensorineural Hearing Loss

Table 1

The main autoimmune target antigens in the inner ear.

Inner ear antigenDistributionReferences

Collagen type IIIn the subepithelial layer of the endolymphatic duct and spiral ligament.[26, 6468]
HSP-70Hair cells and supporting cells.[16, 17, 6973]
β-TubulinHair cells, supporting cells, the spiral ligament of the stria vascularis, and the spiral ganglion.[54, 7478]
CochlinIn the regions of the fibrocytes of the spiral limbus and the spiral ligament and in the cochlear and vestibular labyrinth.[52, 53, 7982]
Beta-tectorinHair cells in the basal region of the hair bundle lying over the apical surface of the auditory epithelium, in the basilar papilla, in the clear cells and the cuboidal cells, and in the striolar region of the lagena macula.[52, 53, 77, 83, 84]
Kresge Hearing Research Institute-3 (KHRI-3)This is a protein specific to the inner ear and is expressed in the saccular wall cells and transitional epithelial cells in the utricle and ampules, by cells in the endolymphatic sac, and by supporting cells.[85, 86]