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The symptoms include aural fullness, fluctuating hearing, and\/or episodes of vertigo similar to those observed in Meniere disease and may occur ipsilateral or contralateral to the previously deafened ear. In most reported cases, the unilateral deafness has been a profound sensorineural hearing loss with a sudden onset that has been variously attributed to bacterial or viral labyrinthitis, acoustic or cranial trauma, otosclerosis, and congenital CMV infection. Familial occurrence of the syndrome has not previously been reported in the literature. In this report, we describe two possible familial instances of delayed DEH. These patients raise the possibility that genetic factors may sometimes be the cause of this unusual syndrome. \u00a9 2007 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.a.31741","type":"journal-article","created":{"date-parts":[[2007,5,11]],"date-time":"2007-05-11T22:20:31Z","timestamp":1178922031000},"page":"1661-1665","source":"Crossref","is-referenced-by-count":17,"title":["Familial unilateral deafness and delayed endolymphatic hydrops"],"prefix":"10.1002","volume":"143A","author":[{"given":"Kelley M.","family":"Dodson","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tamio","family":"Kamei","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aristides","family":"Sismanis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Walter E.","family":"Nance","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2007,6,21]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1984.04050150092023"},{"key":"e_1_2_7_3_1","first-page":"75","article-title":"Performance and management of children with unilateral sensorineural hearing loss","volume":"30","author":"Bess FH","year":"1988","journal-title":"Scand Audiol Suppl"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-5876(02)00120-9"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.5541011202"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348940511400414"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/00129492-200303000-00018"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1288\/00005537-198808000-00010"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/0165-5876(96)83899-7"},{"key":"e_1_2_7_10_1","first-page":"137","article-title":"Delayed endolymphatic hydrops as a clinical entity","volume":"10","author":"Kamei T","year":"2004","journal-title":"Int Tinnitus J"},{"key":"e_1_2_7_11_1","first-page":"349","article-title":"Statistical observation of unilateral total deafness and characteristic unilateral total deafness among young children with tendency toward occurrence of dizziness","volume":"43","author":"Kamei T","year":"1971","journal-title":"Otolaryngol (Tokyo)"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1177\/019459988209000517"},{"key":"e_1_2_7_13_1","first-page":"344","article-title":"Hearing loss with delayed onset of vertigo","volume":"6","author":"Kemink JL","year":"1985","journal-title":"Am J Otol"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/PL00007519"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005537-199607000-00021"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1288\/00005537-198512000-00016"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1177\/019459989511200406"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.57.11.1631"},{"key":"e_1_2_7_19_1","first-page":"375","article-title":"Delayed endolymphatic hydrops","volume":"9","author":"LeLiever WC","year":"1980","journal-title":"J Otolaryngol"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1993.tb04451.x"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.130.5.524"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.1989.01860250048024"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348947508400617"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1288\/00005537-198308000-00006"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0012162203000628"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348947808700601"},{"key":"e_1_2_7_27_1","first-page":"1","article-title":"Endolymphatic hydrops. 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