{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T14:22:09Z","timestamp":1788186129033,"version":"build-2803163510"},"reference-count":25,"publisher":"Georg Thieme Verlag KG","issue":"S 02","funder":[{"DOI":"10.13039\/100000053","name":"National Eye Institute","doi-asserted-by":"crossref","award":["NIH\/NEI 5K08EY027464\u201302 [ABD]"],"award-info":[{"award-number":["NIH\/NEI 5K08EY027464\u201302 [ABD]"]}],"id":[{"id":"10.13039\/100000053","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Neurol Surg B Skull Base"],"published-print":{"date-parts":[[2022,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>\n          Objective\u2003Endolymphatic sac tumors (ELSTs) are a frequent cause of hearing loss and other audiovestibular dysfunction in patients with von Hippel-Lindau disease (VHL). Unified screening recommendations for VHL patients have not been established. To develop consensus guidelines, the VHL Alliance formed an expert committee to define evidence-based clinical screening recommendations.<\/jats:p><jats:p>\n          Patients and Methods\u2003Recommendations were formulated by using the Grading of Recommendations, Assessment, Development, and Evaluation framework after a comprehensive literature review.<\/jats:p><jats:p>\n          Results\u2003Diagnosis of ELSTs in VHL requires a combination of clinical evaluation and imaging and audiometric findings. Audiovestibular signs\/symptoms are often an early feature of small ELSTs, including those that are not visible on imaging. Diagnostic audiograms have the greatest sensitivity for the detection of ELST-associated sensorineural hearing loss and can help confirm clinically relevant lesions, including those that may not be radiographically evident. Magnetic resonance imaging (MRI) can be a more specific test for ELSTs in VHL particularly when supplemented with computed tomography imaging for the identification of small tumors. VHL patients between the ages 10 and 60 years carry high preponderance for ELST presentation.<\/jats:p><jats:p>\n          Conclusion\u2003We recommend that clinical evaluation (yearly) and diagnostic audiograms (every other year) be the primary screening tools for ELSTs in VHL. We suggest that screening be performed between the ages 11 and 65 years or with the onset of audiovestibular signs\/symptoms for synchronicity with other testing regimens in VHL. We recommend that baseline imaging (MRI of the internal auditory canals) can be performed between the ages of 15 and 20 years or after positive screening.<\/jats:p>","DOI":"10.1055\/s-0041-1725033","type":"journal-article","created":{"date-parts":[[2021,4,8]],"date-time":"2021-04-08T23:02:09Z","timestamp":1617922929000},"page":"e225-e231","source":"Crossref","is-referenced-by-count":11,"special_numbering":"S","title":["Endolymphatic Sac Tumor Screening and Diagnosis in von Hippel-Lindau Disease: A Consensus Statement"],"prefix":"10.1055","volume":"83","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8009-6430","authenticated-orcid":false,"given":"Gautam U.","family":"Mehta","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, House Institute, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. Jeffery","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3706-8781","authenticated-orcid":false,"given":"Paul W.","family":"Gidley","sequence":"additional","affiliation":[{"name":"Department of Head and Neck Surgery, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony B.","family":"Daniels","sequence":"additional","affiliation":[{"name":"Division of Ocular Oncology and Pathology, Department of Ophthalmology and Visual Sciences, Vanderbilt University Medical Center, Nashville, Tennessee, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mia E.","family":"Miller","sequence":"additional","affiliation":[{"name":"Division of Neurotology, House Institute, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory P.","family":"Lekovic","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, House Institute, Los Angeles, California, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John A.","family":"Butman","sequence":"additional","affiliation":[{"name":"Neuroradiology Section, Radiology and Imaging Sciences Department, The Clinical Center, National Institutes of Health, Bethesda, Maryland, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russell R.","family":"Lonser","sequence":"additional","affiliation":[{"name":"Department of Neurological Surgery, The Ohio State University Wexner Medical Center, Ohio State University, Columbus, Ohio, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2021,4,8]]},"reference":[{"issue":"04","key":"ref1","doi-asserted-by":"crossref","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":"R R Lonser","year":"2008","journal-title":"J Neurosurg"},{"issue":"11","key":"ref2","doi-asserted-by":"crossref","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":"D K Heffner","year":"1989","journal-title":"Cancer"},{"issue":"12","key":"ref3","doi-asserted-by":"crossref","first-page":"1342","DOI":"10.1288\/00005537-199312000-00004","article-title":"Reclassification of aggressive adenomatous mastoid neoplasms as endolymphatic sac tumors","volume":"103","author":"J C Li","year":"1993","journal-title":"Laryngoscope"},{"issue":"08","key":"ref4","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1177\/000348949510400805","article-title":"Are papillary adenomas endolymphatic sac tumors?","volume":"104","author":"A Pollak","year":"1995","journal-title":"Ann Otol Rhinol Laryngol"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1288\/00005537-199508000-00006","article-title":"Endolymphatic sac tumors: histopathologic confirmation, clinical characterization, and implication in von Hippel-Lindau disease","volume":"105","author":"C A Megerian","year":"1995","journal-title":"Laryngoscope"},{"issue":"18","key":"ref6","doi-asserted-by":"crossref","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":"T J Manski","year":"1997","journal-title":"JAMA"},{"issue":"03","key":"ref7","doi-asserted-by":"crossref","first-page":"480","DOI":"10.3171\/jns.2004.100.3.0480","article-title":"Endolymphatic sac tumors in von Hippel-Lindau disease","volume":"100","author":"D Choo","year":"2004","journal-title":"J Neurosurg"},{"issue":"12","key":"ref8","doi-asserted-by":"crossref","first-page":"1032","DOI":"10.1097\/GIM.0b013e31822beab1","article-title":"von Hippel-Lindau disease: surveillance strategy for endolymphatic sac tumors","volume":"13","author":"MLM Poulsen","year":"2011","journal-title":"Genet Med"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"E673","DOI":"10.1002\/hed.24067","article-title":"International Endolymphatic Sac Tumor (ELST) Consortium. Characterization of endolymphatic sac tumors and von Hippel-Lindau disease in the International Endolymphatic Sac Tumor Registry","volume":"38","author":"B Bausch","year":"2016","journal-title":"Head Neck"},{"issue":"01","key":"ref10","doi-asserted-by":"crossref","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":"J A Butman","year":"2007","journal-title":"JAMA"},{"issue":"03","key":"ref11","doi-asserted-by":"crossref","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":"C A Megerian","year":"2002","journal-title":"Otol Neurotol"},{"issue":"02","key":"ref12","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1002\/lary.23646","article-title":"Surgical resection of 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Atkins","year":"2004","journal-title":"BMJ"},{"issue":"11","key":"ref19","doi-asserted-by":"crossref","first-page":"771","DOI":"10.3109\/14992027.2013.824117","article-title":"von Hippel-Lindau disease: deafness due to a non-MRI-visible endolymphatic sac tumor despite targeted screening","volume":"52","author":"M LM Binderup","year":"2013","journal-title":"Int J Audiol"},{"issue":"02","key":"ref20","doi-asserted-by":"crossref","first-page":"406","DOI":"10.3171\/2013.2.JNS12608","article-title":"Imaging detection of endolymphatic sac tumor-associated hydrops","volume":"119","author":"J A Butman","year":"2013","journal-title":"J Neurosurg"},{"issue":"04","key":"ref21","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1016\/j.carj.2018.04.002","article-title":"Gadolinium deposition in the brain: a systematic review of existing guidelines and policy statement issued by the Canadian Association of Radiologists","volume":"69","author":"A F Costa","year":"2018","journal-title":"Can Assoc Radiol J"},{"issue":"24","key":"ref22","doi-asserted-by":"crossref","first-page":"2481","DOI":"10.1056\/NEJMoa040666","article-title":"Tumors of the endolymphatic sac in von Hippel-Lindau disease","volume":"350","author":"R R Lonser","year":"2004","journal-title":"N Engl J Med"},{"issue":"761","key":"ref23","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1259\/0007-1285-64-761-447","article-title":"Patient dose and associated risk due to radiological investigation of the internal auditory meatus","volume":"64","author":"J J Tweed","year":"1991","journal-title":"Br J Radiol"},{"issue":"02","key":"ref24","doi-asserted-by":"crossref","first-page":"226","DOI":"10.3174\/ajnr.A1807","article-title":"Radiation dose to the lens using different temporal bone CT scanning protocols","volume":"31","author":"Y Niu","year":"2010","journal-title":"AJNR Am J Neuroradiol"},{"issue":"03","key":"ref25","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1016\/j.jacr.2013.10.008","article-title":"Patient perspectives on radiation dose","volume":"11","author":"J Graff","year":"2014","journal-title":"J Am Coll Radiol"},{"issue":"05","key":"ref26","doi-asserted-by":"crossref","first-page":"782","DOI":"10.1097\/00129492-200409000-00022","article-title":"Endolymphatic sac tumor in a 4-year-old boy","volume":"25","author":"M E Kupferman","year":"2004","journal-title":"Otol Neurotol"},{"key":"ref28","first-page":"0123456789","article-title":"Evaluation, diagnosis and surveillance of renal masses in the setting of VHL disease","author":"J Chahoud","year":"2020","journal-title":"World J Urol"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"2S","DOI":"10.1378\/chest.95.2_Supplement.2S","article-title":"Rules of evidence and clinical recommendations on the use of antithrombotic agents","volume":"95","author":"D L 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