Deafness with Sporadic Goiter: Pendred's Syndrome

Abstract

Deafness is encountered in association with 3 types of thyroid disorders: adult myxedema, endemic cretinism, and Pendred's syndrome. Impairment of hearing occurs in approximately one-half of the patients with adult myxedema; hearing loss improves in about one-half of these patients after treatment with thyroid extract.^1-3 Otologic examination shows the auditory impairment to be of perceptive, conductive, or mixed type. The aural anatomical changes and the type of hearing deficit present may vary with the degree of severity of the thyroid disease. Thus, the deafness may be conductive if edema of the middle ear or eustachian tube is present; or perceptive, due to an intralabyrinthine hypertension based upon myxedematous infiltration affecting the endolymphatic circulation. If the myxedema is severe and prolonged, chromatolysis of central nervous system nerve cells may occur and serve as a basis for perceptive deafness.⁴ In endemic cretinism and in Pendred's syndrome, all patients suffer from