Pendred's Syndrome: Report of a Case

Abstract

A case with sensorineural hearing loss and simple goiter (Pendred's syndrome) was reported. The case, whose mother had congenital deaf-mutism with a simple goiter, was a thirteen years old girl who suffered from congenital hearing loss and simle goiter. The thyroid function tests revealed euthyroidism except for ¹³¹I up take which showed 46%. Neither hyper- nor hypothyroidism was seen clinically and mental or physical development seemed normal.
Pure tone audiogram revealed a high tone hearing loss in her both ears. After a battery of the audiological examinations, the inner ear was suspected as a lesion of the hearing loss. The hearing threshold of this patient showed fluctuation during her clinical course.
For past three years, sudden hearing loss ocurred three times, and the first one was idiopathic, the second after viral infection and the third after swimming. In each occasion her hearing threshold was recovered from complete deafness 40dB to in the right ear and from 80dB to 55dB in the left ear within a week.