DEAF-BLIND students in schools and classes for the deaf in the United States and Canada.
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Hereditary hearing loss is divided into two groups, syndromic and non-syndromic, the latter being more common and highly heterogeneous. Linkage analyses were performed on a Japanese family showing a dominant form of non-syndromic progressive sensorineural hearing loss. This gene (DFNA11) was localized within the region of chromosome 11q which contains the second gene for a recessive form of non-syndromic sensorineural hearing loss (DFNB2). Since it has been reported that another gene for dominant non-syndromic hearing loss (DFNA3) has been mapped to the same region as the first gene for recessive hearing loss (DFNB1), it is possible that different mutations in the DFNB2 gene may result in either dominant or recessive hearing loss.
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In a survey of 78 hearing impaired children, 33% were found to have ocular abnormalities. The abnormalities were relatively minor, the most frequent being altered retinal pigmentation. Although congenital rubella infection was the most common cause of this finding the cause in others was more obscure. Possible causes for hearing loss associated with retinal pigment and other tissue changes are abnormal cilia, abnormalities in cells with similar embryological origins and genetic defects affecting pigmentation.
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