{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T09:38:30Z","timestamp":1792057110776,"version":"4.4.0"},"reference-count":8,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2005,6,7]],"date-time":"2005-06-07T00:00:00Z","timestamp":1118102400000},"content-version":"vor","delay-in-days":4375,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Am. J. Med. Genet."],"published-print":{"date-parts":[[1993,6,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Profound, early\u2010onset deafness is present in 4\u201311 per 10,000 children, and is attributable to genetic causes in at least 50% of cases.<\/jats:p><jats:p>Family history questionnaires were sent to 26,152 families of children with profound, early\u2010onset deafness not known to be related to an environmental cause. The probands were ascertained through the 1988\u201389 Gallaudet University Annual Survey of Hearing Impaired Children and Youth. The analysis is based on the responses that were received from 8,756 families.<\/jats:p><jats:p>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\u201370 Annual Survey found 49.3% sporadic cases and 50.6% genetic, demonstrating that the proportion of sporadic cases of early\u2010onset deafness has significantly decreased since 1970. \u00a9 1993 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.1320460504","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T16:59:31Z","timestamp":1118509171000},"page":"486-491","source":"Crossref","is-referenced-by-count":353,"title":["Genetic epidemiological studies of early\u2010onset deafness in the U.S. school\u2010age population"],"prefix":"10.1002","volume":"46","author":[{"given":"Mary L.","family":"Marazita","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynn M.","family":"Ploughman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brenda","family":"Rawlings","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Remington","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen S.","family":"Arnos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Walter E.","family":"Nance","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2005,6,7]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320440315"},{"key":"e_1_2_1_3_1","first-page":"831","volume-title":"Otologic Medicine and Surgery","author":"Bodurtha JN","year":"1988"},{"key":"e_1_2_1_4_1","volume-title":"Marriages of the Deaf in America","author":"Fay EA","year":"1898"},{"key":"e_1_2_1_5_1","volume-title":"Demographic Aspects of Hearing Impairment: Questions and Answers","author":"Hothkiss D","year":"1989"},{"key":"e_1_2_1_6_1","first-page":"129","volume-title":"Computer Applications in Human Genetics","author":"Morton NE","year":"1969"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1991.tb19572.x"},{"key":"e_1_2_1_8_1","first-page":"307","volume-title":"Genetic Counseling","author":"Nance WE","year":"1977"},{"key":"e_1_2_1_9_1","unstructured":"RoseSP(1975): \u201cGenetic Studies of Profound Prelingual Deafness.\u201d Ph.D. Thesis Department of Medical Genetics Indiana University."}],"container-title":["American Journal of Medical Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajmg.1320460504","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.1320460504","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,26]],"date-time":"2023-10-26T01:47:35Z","timestamp":1698284855000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.1320460504"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1993,6,15]]},"references-count":8,"journal-issue":{"issue":"5","published-print":{"date-parts":[[1993,6,15]]}},"alternative-id":["10.1002\/ajmg.1320460504"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.1320460504","archive":["Portico"],"relation":{},"ISSN":["0148-7299","1096-8628"],"issn-type":[{"value":"0148-7299","type":"print"},{"value":"1096-8628","type":"electronic"}],"subject":[],"published":{"date-parts":[[1993,6,15]]}}}