S umma r y: The Pendred syndrome is defined as strumaconnected with perceptual partial hearing loss.More recent definition of the syndrome includes partial perceptual hearing loss connected with the finding of extended vestibular aquaduct. The literature data indicate that Pendred syndrome participates in 5 - 10% of all hereditary cases of hearing loss. The gene coding chloride anion transporter, whose mutations are directly related to Pendred syndrome was detected on chromosome 7, was detected at the National Human Genome Research Institute (U.S.A.) in 1997 was accordingly named PDS gene (Pendred syndrome gene). The present paper reviews the knowledge about Pendred syndrome, summarized diagnostic as well as clinical criteria of Pendred syndrome.

        Key words: Pendred Syndroma, diagnostic, characterization of gene.