PENDREDS SYNDROME

Although 5% of all cases of congenital deafness are caused by Pendred' s syndrome, there are few reports in the literature. Seven patients wi th Pendred's syndrome in three families living in the same village wer e detected. For that reason, the syndrome is reviewed in light of the literature. The sex distribution of the patients with Pendred's syndro me and their families was recorded. We tested for thyroxine, triiodoth yronine, thyroid-stimulating hormone, triiodothyronine resin uptake, a nd perchlorate, and performed caloric testing. In one patient, subtota l thyroidectomy was performed. In the histopathologic study, a thyroid nodule filled with colloid was found. Chromosome studies showed no an omalies in any patient. Five of the patients were deaf-mutes. We obser ved that the parents were cousins in all three families. These familie s also had healthy children, and the existence of the syndrome in both sexes points to an autosomal recessive trait.