SLC26A4 and autosomal recessive nonsyndromic hearing loss 4: Recessive SLC26A4 mutations contribute to both Pendred syndrome (PS; MIM #274600) [9] and nonsyndromic hearing loss (DFNB4; MIM #600791) [10], which share the phenotypes of sensorineural hearing impairment (SNHI) accompanied by an enlarged vestibular aqueduct (EVA; MIM 603545) and/or incomplete partition of the cochlea (i.e., Mondini dysplasia), although the phenotype of PS also includes goiter.