Profound, early-onset deafness is present in 4-11 per 10,000 children, and is attributable to genetic causes in at least 50% of cases. Family history questionnaires were sent to 26,152 families of children with profound, early-onset deafness not known to be related to an environmental cause. The probands were ascertained through the 1988-89 Gallaudet University Annual Survey of Hearing Impaired Children and Youth. The analysis is based on the responses that were received from 8,756 families. Classical segregation analysis was used to analyze the family data, and to estimate the proportions of sporadic, recessive and dominant causes of deafness in the families. These data were consistent with 37.2% of the cases due to sporadic causes, and 62.8% due to genetic causes (47.1% recessive, and 15.7% dominant). An earlier study using the 1969-70 Annual Survey found 49.3% sporadic cases and 50.6% genetic, demonstrating that the proportion of sporadic cases of early-onset deafness has significantly decreased since 1970.

This paper reports the findings of a study that examined the demographic, educational, and audiological characteristics of a sample of children in Texas who had received cochlear implants. Children who had received implants and children who had not were compared. Differences between the two groups are discussed in the context of the criteria that have been published in the literature for selecting implant candidates. Results indicate that although the published selection criteria were predictive of the characteristics of the implanted sample, there was a degree of flexibility in applying the criteria to choose implant recipients. Audiological comparisons revealed that cochlear implant recipients experience greater reductions in average hearing thresholds than do users of conventional aids, although these findings should be viewed as preliminary.

Preliminary findings are provided from the data collected in Puerto Rico through the Annual Survey of Deaf and Hard of Hearing Children and Youth during the 1997-1998 school year. The study was conducted as a part of an initiative to increase participation in the Annual Survey among the deaf and hard of hearing school-age population in Puerto Rico. Demographic, instructional, etiological, audiological, and communication data on 336 deaf and hard of hearing school age children were collected and summarized. The findings suggest the existence of a heterogeneous deaf community rather than the traditionally conceived homogenous community. The discussion emphasizes the description of those attributes that suggest heterogeneity and the urgent need to continue to collect the kind of data gathered in the survey. The authors urge that Puerto Rican educators and researchers be stimulated to address the educational and health-related needs of Puerto Rico's deaf and hard of hearing school-age population.

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