MPZL2 (myelin protein zero like 2) is a cell adhesion molecule that mediates homophilic cell-cell interactions 1. Located on chromosome 11, MPZL2 is widely expressed across multiple cell types within the inner ear, including outer hair cells and supporting cells like Deiters cells 12. Mutations in MPZL2 cause autosomal recessive deafness (DFNB111), the second leading genetic cause of mild-to-moderate hereditary hearing loss 1. The disease characteristically presents with progressive sensorineural hearing loss, with onset varying from congenital to young adulthood 3. A founder mutation c.220C>T (p.Gln74*) is prevalent in East Asian populations 43, while c.72delA is common in European populations 3. Clinically, MPZL2-related deafness is moderately progressive and symmetrical, affecting multiple cochlear cell types 13. Gene replacement therapy via AAV vectors successfully restores hearing function and structural integrity in mouse models, maintaining therapeutic effects for extended periods 12. Base editing approaches can correct specific MPZL2 mutations without inducing DNA double-strand breaks, showing promise for treatment 4. Beyond hearing, MPZL2 expression appears relevant to cancer biology as Eva1, a protein transcribed from MPZL2, shows broad tumor expression 5.