The Syndrome
Pendred syndrome is a disorder that is characterized by earlyThyroid Rescue 911 Review hearing loss in children. It is a genetic syndrome which also affects the thyroid gland and the balance of the patient. Children with this disorder may lose their hearing at birth or until they are up to three years of age. Hearing loss is sudden and happens in stages. It is also progressive, which means that the child will hear less as time passes and some of them may go totally deaf.Almost all children with the syndrome become bilaterally deaf or has some degree of deafness in both ears. It is also common for one ear to be deafer than the other.
The Thyroidal Involvement
The thyroid is a small organ located in front of the trachea immediately below the larynx. This gland produces the two hormones necessary to regulate the body's metabolic rate. In children, it also plays an important role in growth although children with the syndrome do not usually have growth problems even with involvement of the thyroid.The disorder is genetic and is caused by mutations in the SLC26A4 gene. This gene is also found in the cochlea, the thyroid, and the kidney. In the ear, it causes the early hearing loss as well as loss of balance to some degree. In the thyroid, it causes less organification of iodine, hence, lesser thyroid hormone is produced.
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