Hearing loss has recently been reported in an autophagy-related disease, Vici syndrome.22, 23, 24 This syndrome, which is due to recessive mutations in the EPG5 gene encoding ectopic P granules protein 5 (EPG5), a key autophagy regulator, is a congenital multisystem disorder characterized by corpus callosal agenesis, cataracts, cardiomyopathy, oculocutaneous hypopigmentation, and immunodeficiency. Here, EPG5 is linked to Immunodeficiency.