The historical cornerstone of Pendred's syndrome is derved from Vaughan Pendred's description of two sisters with goiter and congenital deafness in 1986. Patietns with this syndrome has variable degrees of congenital sensoryneural deafness,
goiter,
hypothyroidism and positive perchlorate discharge test.
Iodine organification defect in the synthesis of thyroid hormone has been thought to be the major cause of hypothyroidism and goiter in Pendred's syndrome. The real biochemical abnormality responsible for the iodine organificantion is still
unknown
and
the etiology of sensoryneural hearing loss is unclear, too.
For the first time in the Korean Surgical Society, authors presented 5 cases of Pendred's syndrome experienced at the department of Surgery, Presbyterian Medial Center, Korea from January 1990 to February, 1992.
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