oa South African Medical Journal - Deaf-mutism in children : with special reference to the congenital type after maternal rubella
In the vast majority of cases of deaf-mutism, the aetiology is genetic in origin. The mutism is secondary to the deafness. In a minority of cases, the aetiological factor is an early postnatal acquired infection. Maternal rubella in the first four months of gestation may be followed by congenital deafness in the child, apparently due to defective development in the organ of Corti. Maternal rubella however, plays a relatively insignificant role in the causation of deaf-mutism in general. The diagnosis is difficult to make in early life, and deafness often simulates mental deficiency or behaviour disorder. Aphasias should be differentiated from deafness, as hearing aids are useless in these cases. True mental deficiency or microcephaly is not infrequently associated with post-rubella congenital deafness. Post-rubella deaf-mutism is usually bilateral, and may be complete or partial, sometimes being associated with other congenital defects, notably cardiac lesions. Unilateral or bilateral cataract or ocular defects may be associated with deafness, particularly if the mother had contracted rubella during the first two months of pregnancy, but fortunately the association occurs not too commonly. Many post-rubella deaf-mutes are undersized and dystrophic. Instruction in lip-reading, individual tuition, and the use of hearing aids should be commenced as early as possible in deaf-mutes. Social adjustment within the family should be aimed at.
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