Cochlin is the major protein component of inner ear extracellular matrix. It contains one LCCL/FCH and two VWF-A domains, any of which may be deleted by alternate splicing. Cochlin is a target of IFN- producing T cells in autoimmune sensorineural hearing loss (ASNHL). Cochlin is upregulated in glaucoma and promotes the aggregation of trabecular meshwork cells in the eye. Point mutations in Cochlin are associated with glaucoma and the autosomal hearing loss disorder DFNA9.