Cockayne's syndrom in two siblings, a 11-year-old boy and a 7-year-old girl, is reported.
Both children had the characteristic symptoms of Cockayne's syndrome, dwarfism, senile appearance, mental retardation, retinitis pigmentosa, calcification of bilateral basal ganglia, and sensorineural deafness. The results of auditory brainstem response audiometry demonstrated no response to 95dBHL click stimuli, and behavioral audiometries in different occasions revealed progressive hearing impairment.
Both patients had fitted hearing aids, and showed developmental improvement in auditory behavior, vocalization, and language development. But after four year's follow up study, the boy showed poor activity because of progression of hearing and optic impairment.
Our report suggested that in children with progressive hearing impairment, periodical evaluation and early fitting of hearing aid are important for better communication.
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