Loss or reduction of function of pendrin consequent to gene mutation causes autosomal recessive forms of non‐syndromic as well as syndromic sensorineural hearing loss, ie, DFNB4 and Pendred syndrome, of which a malformation of the inner ear called enlarged vestibular aqueduct (EVA) with or without cochlear incomplete partition type 2 is the main radiological finding. Here, SLC26A4 is linked to hearing loss disorder.