Abstract

• 1) Pendred's syndrome is very rare disease in western countries, although 5% of all case of congenital deafness by Pendred's syndrome, and until not reported in Korea.
  2) Pendred's syndrome, believed to be one of the more frequent cause of autosomal recessive deafness, has been considered until rescently to present a consistant clinical picture of sensorineural hearing loss and nodular goiter.
  3) Goiter generally appears in these patients at puberty or later, but may be in early childhood.
  4) It is a deficiency of thyroxine synthesis occurs, thyroid stimulating hormone increase, and goiterous enlargement is seen in the thyroid tissue, most affected persons are clinically euthyroid, but hypothyriodism.
  5) Two patients was performed subtotal thyroidectomy, and one is right lobectomy with combined isthmus.