{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,28]],"date-time":"2026-03-28T08:20:33Z","timestamp":1774686033335,"version":"3.50.1"},"reference-count":10,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2016,5,17]],"date-time":"2016-05-17T00:00:00Z","timestamp":1463443200000},"content-version":"vor","delay-in-days":3304,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["aao-hnsfjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Otolaryngol.--head neck surg."],"published-print":{"date-parts":[[2007,5]]},"abstract":"<jats:sec><jats:title>OBJECTIVES<\/jats:title><jats:p>Ectodermal dysplasias (EDs) are a group of genetic disorders characterized by deficient ectodermal and mesodermal development. Studies examining resultant otolaryngologic issues are few. The objectives of this study were to delineate the head and neck manifestations and quality of life in EDs.<\/jats:p><\/jats:sec><jats:sec><jats:title>STUDY DESIGN AND SETTING<\/jats:title><jats:p>For 75\u00a0individuals, comprehensive histories were taken and otolaryngologic examinations were performed, and subjects rated their otolaryngologic symptom severity. A validated quality of life instrument (SF\u20108) was administered.<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>The majority of subjects had a diagnosis of hypohidrotic ED (72%). Otolaryngologic conditions included otitis media (28%), cerumen impaction (48%), nasal obstruction\/crusting (51%), heat intolerance (76%), and eczema (39%). Physical findings included peg teeth\/hypodontia (76%), alopecia (41%), nasal crusting (41%), and saddle nose deformity (44%). Quality of life scores were generally high. Overall, health was rated \u201cgood to excellent\u201d by 87 percent.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSION<\/jats:title><jats:p>Patients with ED frequently experience significant otolaryngologic symptoms, although most patients report a good quality of life.<\/jats:p><\/jats:sec><jats:sec><jats:title>SIGNIFICANCE<\/jats:title><jats:p>A greater understanding of the otolaryngologic issues in ED should help facilitate diagnosis and improve management.<\/jats:p><\/jats:sec>","DOI":"10.1016\/j.otohns.2006.11.038","type":"journal-article","created":{"date-parts":[[2007,5,1]],"date-time":"2007-05-01T07:10:45Z","timestamp":1178003445000},"page":"843-847","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Head and Neck Manifestations and Quality of Life of Patients with Ectodermal Dysplasia"],"prefix":"10.1002","volume":"136","author":[{"given":"Umang","family":"Mehta","sequence":"first","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Saint Louis University School of Medicine Saint Louis MO"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joseph","family":"Brunworth","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Saint Louis University School of Medicine Saint Louis MO"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Timothy J.","family":"Fete","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Saint Louis University School of Medicine Saint Louis MO"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raj","family":"Sindwani","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Saint Louis University School of Medicine Saint Louis MO"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2016,5,17]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"80","volume-title":"Ectodermal dysplasias: a clinical and genetic study","author":"Freire\u2010Maia N","year":"1984"},{"key":"e_1_2_6_3_1","first-page":"71","article-title":"Two cases in which the skin, hair and teeth were very imperfectly developed","volume":"31","author":"Thurman J","year":"1848","journal-title":"Proc R Med Chir Soc"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1929.01930040075005"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005537-200206000-00005"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0001-6519(05)78595-2"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.130.9.1104"},{"key":"e_1_2_6_8_1","volume-title":"A manual for users of the SF\u20108 Health Survey","author":"Ware JE","year":"2001"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1258\/0022215041615173"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0022215100133687"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.2500\/ajr.2007.21.2989"}],"container-title":["Otolaryngology\u2013Head and Neck Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1016\/j.otohns.2006.11.038","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1016\/j.otohns.2006.11.038","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/aao-hnsfjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1016\/j.otohns.2006.11.038","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T10:30:22Z","timestamp":1760956222000},"score":1,"resource":{"primary":{"URL":"https:\/\/aao-hnsfjournals.onlinelibrary.wiley.com\/doi\/10.1016\/j.otohns.2006.11.038"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,5]]},"references-count":10,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2007,5]]}},"alternative-id":["10.1016\/j.otohns.2006.11.038"],"URL":"https:\/\/doi.org\/10.1016\/j.otohns.2006.11.038","archive":["Portico"],"relation":{},"ISSN":["0194-5998","1097-6817"],"issn-type":[{"value":"0194-5998","type":"print"},{"value":"1097-6817","type":"electronic"}],"subject":[],"published":{"date-parts":[[2007,5]]},"assertion":[{"value":"2016-05-17","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}