{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,4]],"date-time":"2025-10-04T17:47:16Z","timestamp":1759600036975},"reference-count":14,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JNS"],"published-print":{"date-parts":[[2013,8]]},"abstract":"<jats:sec>\n<jats:title>Object<\/jats:title>\n<jats:p>To determine if physiologically based MRI sequences can be used to detect endolymphatic sac tumor (ELST)\u2013associated hydrops, the authors performed contrast-enhanced delayed FLAIR imaging in consecutive ELST patients with clinical findings consistent with hydrops.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>Consecutive patients with von Hippel-Lindau (VHL) disease and clinical findings of endolymphatic hydrops and ELSTs underwent contrast-enhanced delayed FLAIR MRI. Clinical, audiological, operative, and imaging findings were analyzed.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>Three patients (2 male, 1 female) with 4 ELSTs (1 patient had bilateral ELSTs) were identified who had clinical findings consistent with endolymphatic hydrops. Computed tomography and MRI evidence of an ELST was found in all patients. Their mean age at initial evaluation was 39.7 years (range 28\u201351 years). All patients demonstrated progressive sensorineural hearing loss that was associated with episodic vertigo and tinnitus. Contrast-enhanced delayed FLAIR MRI clearly demonstrated dilation of the membranous labyrinth consistent with hydrops in the affected ears but not the unaffected ears. Two patients underwent resection of the associated ELST that resulted in stabilization of progressive hearing loss, as well as amelioration of tinnitus and vertigo.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>Contrast-enhanced delayed FLAIR MRI can be used to detect ELST-associated hydrops. Noninvasive MRI detection of hydrops can permit earlier detection of ELSTs in patients with VHL disease and provides direct insight into a mechanism that underlies ELST-associated audiovestibular morbidity.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2013.2.jns12608","type":"journal-article","created":{"date-parts":[[2013,3,8]],"date-time":"2013-03-08T15:03:29Z","timestamp":1362755009000},"page":"406-411","source":"Crossref","is-referenced-by-count":17,"title":["Imaging detection of endolymphatic sac tumor\u2013associated hydrops"],"prefix":"10.3171","volume":"119","author":[{"given":"John A.","family":"Butman","sequence":"first","affiliation":[{"name":"Diagnostic Radiology Department, National Institutes of Health Clinical Center;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Edjah","family":"Nduom","sequence":"additional","affiliation":[{"name":"Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke;"},{"name":"Department of Neurological Surgery, Emory University, Atlanta, Georgia;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H. Jeffrey","family":"Kim","sequence":"additional","affiliation":[{"name":"Head and Neck Surgery Branch, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland;"},{"name":"Department of Otolaryngology\u2013Head and Neck Surgery, Georgetown University Medical Center, Georgetown University, Washington, DC; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Russell R.","family":"Lonser","sequence":"additional","affiliation":[{"name":"Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke;"},{"name":"Department of Neurological Surgery, The Ohio State University, Columbus, Ohio"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1-jns12608","series-title":"JAMA","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1001\/jama.298.1.41","article-title":"Mechanisms of morbid hearing loss associated with tumors of the endolymphatic sac in von Hippel-Lindau disease","volume":"298","author":"Butman","year":"2007"},{"key":"b2-jns12608","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"480","DOI":"10.3171\/jns.2004.100.3.0480","article-title":"Endolymphatic sac tumors in von Hippel-Lindau disease","volume":"100","author":"Choo","year":"2004"},{"key":"b3-jns12608","series-title":"Cancer","doi-asserted-by":"publisher","first-page":"2292","DOI":"10.1002\/1097-0142(19891201)64:11<2292::AID-CNCR2820641119>3.0.CO;2-#","article-title":"Low-grade adenocarcinoma of probable endolymphatic sac origin. A clinicopathologic study of 20 cases","volume":"64","author":"Heffner","year":"1989"},{"key":"b4-jns12608","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"421","DOI":"10.3171\/JNS-07\/08\/0421","article-title":"Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report","volume":"107","author":"Jagannathan","year":"2007"},{"key":"b5-jns12608","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"503","DOI":"10.3171\/jns.2005.102.3.0503","article-title":"Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease: implications for their natural history, diagnosis, and treatment","volume":"102","author":"Kim","year":"2005"},{"key":"b6-jns12608","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"751","DOI":"10.3171\/JNS\/2008\/108\/4\/0751","article-title":"The vestibular aqueduct: site of origin of endolymphatic sac tumors","volume":"108","author":"Lonser","year":"2008"},{"key":"b7-jns12608","series-title":"Lancet","doi-asserted-by":"publisher","first-page":"2059","DOI":"10.1016\/S0140-6736(03)13643-4","article-title":"von Hippel-Lindau disease","volume":"361","author":"Lonser","year":"2003"},{"key":"b8-jns12608","series-title":"N Engl J Med","doi-asserted-by":"publisher","first-page":"2481","DOI":"10.1056\/NEJMoa040666","article-title":"Tumors of the endolymphatic sac in von Hippel-Lindau disease","volume":"350","author":"Lonser","year":"2004"},{"key":"b9-jns12608","series-title":"JAMA","doi-asserted-by":"publisher","first-page":"1461","DOI":"10.1001\/jama.1997.03540420057030","article-title":"Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel-Lindau disease","volume":"277","author":"Manski","year":"1997"},{"key":"b10-jns12608","series-title":"Otol Neurotol","doi-asserted-by":"publisher","first-page":"378","DOI":"10.1097\/00129492-200205000-00026","article-title":"Hearing preservation surgery for small endolymphatic sac tumors in patients with von Hippel-Lindau syndrome","volume":"23","author":"Megerian","year":"2002"},{"issue":"560","key":"b11-jns12608","series-title":"Acta Otolaryngol Suppl","first-page":"5","article-title":"Grading of endolymphatic hydrops using magnetic resonance imaging","author":"Nakashima","year":"2009"},{"key":"b12-jns12608","series-title":"Acta Otolaryngol","doi-asserted-by":"publisher","first-page":"338","DOI":"10.3109\/00016480903143986","article-title":"Endolymphatic hydrops revealed by intravenous gadolinium injection in patients with M\u00e9ni\u00e8re's disease","volume":"130","author":"Nakashima","year":"2010"},{"key":"b13-jns12608","series-title":"Acta Otolaryngol","doi-asserted-by":"publisher","first-page":"474","DOI":"10.3109\/00016489.2010.534114","article-title":"Endolymphatic hydrops and blood-labyrinth barrier in M\u00e9ni\u00e8re's disease","volume":"131","author":"Tagaya","year":"2011"},{"key":"b14-jns12608","series-title":"J Natl Cancer Inst","doi-asserted-by":"publisher","first-page":"970","DOI":"10.1093\/jnci\/89.13.970-a","article-title":"von Hippel-Lindau disease gene alterations associated with endolymphatic sac tumor","volume":"89","author":"Vortmeyer","year":"1997"}],"container-title":["Journal of Neurosurgery"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg\/119\/2\/article-p406.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg\/119\/2\/article-p406.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg\/119\/2\/article-p406.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,8,14]],"date-time":"2022-08-14T17:00:55Z","timestamp":1660496455000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg\/119\/2\/article-p406.xml"}},"subtitle":["Clinical article"],"short-title":[],"issued":{"date-parts":[[2013,8]]},"references-count":14,"journal-issue":{"issue":"2"},"URL":"https:\/\/doi.org\/10.3171\/2013.2.jns12608","relation":{},"ISSN":["0022-3085","1933-0693"],"issn-type":[{"value":"0022-3085","type":"print"},{"value":"1933-0693","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,8]]}}}