Pendred syndrome is a genetic condition that causes hearing loss in both ears either from birth or early childhood, and often causes a goiter. The hearing loss may be identified on a newborn screening test. Imaging (an MRI of the internal auditory canals or a CT of the temporal bone) shows inner ear malformations. Genetic testing can confirm the diagnosis. The hearing loss is often treated with cochlear implantation. The goiter may be treated with thyroid hormone supplementation. If a diagnosis of Pendred syndrome is suspected or confirmed, then it is appropriate to consult with an otolaryngologist and genetic counselor.