色素性乾皮症の聴覚と言語機能検査成績

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  • Hearing and Speech Assessment in A Case of Xeroderma Pigmentosum.

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Xeroderma pigmentosum is a skin disorder characterized by excessive sensitivity to ultraviolet light resulting in atrophy of exposed skin in childhood with the development of pigmentaion and malignant change. Especially the patient in group A has other neurological defects; progressive mental retardation, motor disturbance, and hearing impairment. In this paper the hearing impairment process and speech and language process are described in a 8-year-old patient who had xeroderma pigmentosum group A. By the age of 7 years 2 months, threshold in his right ear was within normal range. However, in his left ear hearing loss was already 63dB. At the age of 7 years 5 months he had sudden decrease of hearing on the right ear. His hearing impairment progressed independently in each ear. He began to use a hearing aid at the 7 years 5 months. The effect of hearing aid has comparatively satisfactory. Routine audiometric assessment suggested that the hearing loss was of a sensorineural type suggestive of a cochlear and retrocochlear lesions. His IQ was less than 45 at the age of 7 years 2 months, when threshold in his right ear was nearly in the normal range. Therefore it was suggested that his mental deterioration preceded his hearing impairment.

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