{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,7,17]],"date-time":"2024-07-17T23:17:10Z","timestamp":1721258230752},"reference-count":22,"publisher":"Cambridge University Press (CUP)","issue":"8","license":[{"start":{"date-parts":[[2017,3,8]],"date-time":"2017-03-08T00:00:00Z","timestamp":1488931200000},"content-version":"unspecified","delay-in-days":2776,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Laryngol. Otol."],"published-print":{"date-parts":[[2009,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S0022215108004088_sec1\"><jats:title>Objective:<\/jats:title><jats:p>To present the theory that large vestibular aqueduct syndrome (i.e. the recognised existence of an enlarged vestibular aqueduct with progressive sensorineural hearing loss) and endolymphatic hydrops are due to a common primary dysfunction of inner-ear fluid homeostasis.<\/jats:p><\/jats:sec><jats:sec id=\"S0022215108004088_sec2\"><jats:title>Method:<\/jats:title><jats:p>Case report and review of the world literature concerning large vestibular aqueduct syndrome and endolymphatic hydrops.<\/jats:p><\/jats:sec><jats:sec id=\"S0022215108004088_sec3\"><jats:title>Results:<\/jats:title><jats:p>We report a family in which one sibling suffered from large vestibular aqueduct syndrome while the other had classic M\u00e9ni\u00e8re's disease. This suggests that large vestibular aqueduct syndrome and endolymphatic hydrops, in some cases, may be due to a common primary dysfunction of inner-ear fluid homeostasis.<\/jats:p><\/jats:sec><jats:sec id=\"S0022215108004088_sec4\"><jats:title>Conclusion:<\/jats:title><jats:p>To our knowledge, this is the first report in the world literature to postulate that variation in the relative compliance of inner-ear membranes could be the factor that determines the manifestation of the disorder as either endolymphatic hydrops or large vestibular aqueduct syndrome.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s0022215108004088","type":"journal-article","created":{"date-parts":[[2008,11,12]],"date-time":"2008-11-12T11:01:40Z","timestamp":1226487700000},"page":"919-921","source":"Crossref","is-referenced-by-count":10,"title":["Large vestibular aqueduct syndrome and endolymphatic hydrops: two presentations of a common primary inner-ear dysfunction?"],"prefix":"10.1017","volume":"123","author":[{"given":"J H","family":"Spiegel","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A K","family":"Lalwani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2017,3,8]]},"reference":[{"key":"S0022215108004088_ref18","first-page":"3","article-title":"Hydrostatic pressure in the inner ear fluid compartments and its effects on inner ear function","volume":"507","author":"Bohmer","year":"1993","journal-title":"Acta Otolaryngol Suppl (Stockh)"},{"key":"S0022215108004088_ref5","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.1995.01890010011003"},{"key":"S0022215108004088_ref20","doi-asserted-by":"publisher","DOI":"10.1017\/S0022215100128695"},{"key":"S0022215108004088_ref12","first-page":"746","article-title":"The enlarged vestibular aqueduct syndrome (EVA syndrome)","volume":"72","author":"Belenky","year":"1993","journal-title":"ENT J"},{"key":"S0022215108004088_ref16","doi-asserted-by":"publisher","DOI":"10.1288\/00005537-199503000-00012"},{"key":"S0022215108004088_ref10","first-page":"303","article-title":"The classification and treatment of Meniere's disease","volume":"47","author":"Shea","year":"1993","journal-title":"Acta Otol Rhinol Laryngol (Belg)"},{"key":"S0022215108004088_ref17","doi-asserted-by":"publisher","DOI":"10.1016\/S0196-0709(83)80071-4"},{"key":"S0022215108004088_ref6","doi-asserted-by":"publisher","DOI":"10.1288\/00005537-198912000-00006"},{"key":"S0022215108004088_ref7","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.33.369"},{"key":"S0022215108004088_ref2","first-page":"337","article-title":"The vestibular aqueduct and paravestibular canal: an anatomic and radiologic investigation","volume":"15","author":"Wibrand","year":"1974","journal-title":"Acta Radiol (Stockh)"},{"key":"S0022215108004088_ref1","first-page":"3","article-title":"Postnatal development of the vestibular aqueduct and endolymphatic sac","volume":"91","author":"Kodama","year":"1982","journal-title":"Ann Otol Rhinol Laryngol"},{"key":"S0022215108004088_ref21","first-page":"64","article-title":"X-linked mixed deafness with congenital fixation of the stapedial footplate and perilymphatic gusher","volume":"7","author":"Nance","year":"1971","journal-title":"Birth Defects"},{"key":"S0022215108004088_ref14","doi-asserted-by":"publisher","DOI":"10.3109\/00016489009122564"},{"key":"S0022215108004088_ref9","doi-asserted-by":"publisher","DOI":"10.1288\/00005537-199105000-00006"},{"key":"S0022215108004088_ref4","first-page":"387","article-title":"The large vestibular aqueduct syndrome","volume":"6","author":"Emmett","year":"1985","journal-title":"Am J Otol"},{"key":"S0022215108004088_ref15","doi-asserted-by":"publisher","DOI":"10.3109\/00016489409127317"},{"key":"S0022215108004088_ref22","doi-asserted-by":"publisher","DOI":"10.1097\/00005537-199608000-00009"},{"key":"S0022215108004088_ref3","doi-asserted-by":"publisher","DOI":"10.1002\/lary.1978.88.5.723"},{"key":"S0022215108004088_ref13","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.1989.01860250056026"},{"key":"S0022215108004088_ref11","first-page":"177","article-title":"Computed tomographic diagnosis of X-linked congenital mixed deafness, fixation of the stapedia footplate, and perilymphatic gusher","volume":"15","author":"Talbot","year":"1994","journal-title":"Am J Otol"},{"key":"S0022215108004088_ref8","doi-asserted-by":"publisher","DOI":"10.1007\/BF00464410"},{"key":"S0022215108004088_ref19","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1016\/S0030-6665(20)33277-1","article-title":"Correlation of pathology with symptoms of Meniere's disease","volume":"1","author":"Schuknecht","year":"1968","journal-title":"Otolaryngol Clin North Am"}],"container-title":["The Journal of Laryngology &amp; 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