Pendred's Syndrome

Ok Young Kim

Copyright ⓒ 1994. Ewha Womans University School of Medicine. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Published Online: Mar 30, 1994

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.