Nctid:
NCT00001668
Payload:
{"FullStudy"=>{"Rank"=>497759, "Study"=>{"DerivedSection"=>{"MiscInfoModule"=>{"VersionHolder"=>"June 24, 2024"}, "ConditionBrowseModule"=>{"ConditionMeshList"=>{"ConditionMesh"=>[{"ConditionMeshId"=>"D000009369", "ConditionMeshTerm"=>"Neoplasms"}, {"ConditionMeshId"=>"D000007680", "ConditionMeshTerm"=>"Kidney Neoplasms"}, {"ConditionMeshId"=>"D000003638", "ConditionMeshTerm"=>"Deafness"}, {"ConditionMeshId"=>"D000034381", "ConditionMeshTerm"=>"Hearing Loss"}, {"ConditionMeshId"=>"D000006623", "ConditionMeshTerm"=>"Von Hippel-Lindau Disease"}, {"ConditionMeshId"=>"D000012164", "ConditionMeshTerm"=>"Retinal Diseases"}, {"ConditionMeshId"=>"D000007674", "ConditionMeshTerm"=>"Kidney Diseases"}]}, "ConditionAncestorList"=>{"ConditionAncestor"=>[{"ConditionAncestorId"=>"D000014570", "ConditionAncestorTerm"=>"Urologic Diseases"}, {"ConditionAncestorId"=>"D000052776", "ConditionAncestorTerm"=>"Female Urogenital Diseases"}, {"ConditionAncestorId"=>"D000005261", "ConditionAncestorTerm"=>"Female Urogenital Diseases and Pregnancy Complications"}, {"ConditionAncestorId"=>"D000091642", "ConditionAncestorTerm"=>"Urogenital Diseases"}, {"ConditionAncestorId"=>"D000052801", "ConditionAncestorTerm"=>"Male Urogenital Diseases"}, {"ConditionAncestorId"=>"D000006311", "ConditionAncestorTerm"=>"Hearing Disorders"}, {"ConditionAncestorId"=>"D000004427", "ConditionAncestorTerm"=>"Ear Diseases"}, {"ConditionAncestorId"=>"D000010038", "ConditionAncestorTerm"=>"Otorhinolaryngologic Diseases"}, {"ConditionAncestorId"=>"D000012678", "ConditionAncestorTerm"=>"Sensation Disorders"}, {"ConditionAncestorId"=>"D000009461", "ConditionAncestorTerm"=>"Neurologic Manifestations"}, {"ConditionAncestorId"=>"D000009422", "ConditionAncestorTerm"=>"Nervous System Diseases"}, {"ConditionAncestorId"=>"D000005128", "ConditionAncestorTerm"=>"Eye Diseases"}, {"ConditionAncestorId"=>"D000014571", "ConditionAncestorTerm"=>"Urologic Neoplasms"}, {"ConditionAncestorId"=>"D000014565", "ConditionAncestorTerm"=>"Urogenital Neoplasms"}, {"ConditionAncestorId"=>"D000009371", "ConditionAncestorTerm"=>"Neoplasms by Site"}, {"ConditionAncestorId"=>"D000020752", "ConditionAncestorTerm"=>"Neurocutaneous Syndromes"}, {"ConditionAncestorId"=>"D000000798", "ConditionAncestorTerm"=>"Angiomatosis"}, {"ConditionAncestorId"=>"D000014652", "ConditionAncestorTerm"=>"Vascular Diseases"}, {"ConditionAncestorId"=>"D000002318", "ConditionAncestorTerm"=>"Cardiovascular Diseases"}, {"ConditionAncestorId"=>"D000072661", "ConditionAncestorTerm"=>"Ciliopathies"}, {"ConditionAncestorId"=>"D000000015", "ConditionAncestorTerm"=>"Abnormalities, Multiple"}, {"ConditionAncestorId"=>"D000000013", "ConditionAncestorTerm"=>"Congenital Abnormalities"}, {"ConditionAncestorId"=>"D000030342", "ConditionAncestorTerm"=>"Genetic Diseases, Inborn"}]}, "ConditionBrowseLeafList"=>{"ConditionBrowseLeaf"=>[{"ConditionBrowseLeafId"=>"M5534", "ConditionBrowseLeafName"=>"Carcinoma", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M10698", "ConditionBrowseLeafName"=>"Kidney Diseases", "ConditionBrowseLeafAsFound"=>"Kidney Disease", "ConditionBrowseLeafRelevance"=>"high"}, {"ConditionBrowseLeafId"=>"M24420", "ConditionBrowseLeafName"=>"Hearing Loss", "ConditionBrowseLeafAsFound"=>"Deafness", "ConditionBrowseLeafRelevance"=>"high"}, {"ConditionBrowseLeafId"=>"M6840", "ConditionBrowseLeafName"=>"Deafness", "ConditionBrowseLeafAsFound"=>"Deafness", "ConditionBrowseLeafRelevance"=>"high"}, {"ConditionBrowseLeafId"=>"M14999", "ConditionBrowseLeafName"=>"Retinal Diseases", "ConditionBrowseLeafAsFound"=>"Retinal Disease", "ConditionBrowseLeafRelevance"=>"high"}, {"ConditionBrowseLeafId"=>"M10703", "ConditionBrowseLeafName"=>"Kidney Neoplasms", "ConditionBrowseLeafAsFound"=>"Kidney Neoplasms", "ConditionBrowseLeafRelevance"=>"high"}, {"ConditionBrowseLeafId"=>"M5547", "ConditionBrowseLeafName"=>"Carcinoma, Papillary", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M9699", "ConditionBrowseLeafName"=>"Von Hippel-Lindau Disease", "ConditionBrowseLeafAsFound"=>"Von Hippel-Lindau Disease", "ConditionBrowseLeafRelevance"=>"high"}, {"ConditionBrowseLeafId"=>"M20469", "ConditionBrowseLeafName"=>"Hemangioblastoma", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M17319", "ConditionBrowseLeafName"=>"Urologic Diseases", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M2875", "ConditionBrowseLeafName"=>"Urogenital Diseases", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M27093", "ConditionBrowseLeafName"=>"Female Urogenital Diseases", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M14127", "ConditionBrowseLeafName"=>"Pregnancy Complications", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M8399", "ConditionBrowseLeafName"=>"Female Urogenital Diseases and Pregnancy Complications", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M27095", "ConditionBrowseLeafName"=>"Male Urogenital Diseases", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M9400", "ConditionBrowseLeafName"=>"Hearing Disorders", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M7601", "ConditionBrowseLeafName"=>"Ear Diseases", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M12961", "ConditionBrowseLeafName"=>"Otorhinolaryngologic Diseases", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M15490", "ConditionBrowseLeafName"=>"Sensation Disorders", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M12404", "ConditionBrowseLeafName"=>"Neurologic Manifestations", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M8271", "ConditionBrowseLeafName"=>"Eye Diseases", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M17320", "ConditionBrowseLeafName"=>"Urologic Neoplasms", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M17315", "ConditionBrowseLeafName"=>"Urogenital Neoplasms", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M16355", "ConditionBrowseLeafName"=>"Syndrome", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M22509", "ConditionBrowseLeafName"=>"Neurocutaneous Syndromes", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M4126", "ConditionBrowseLeafName"=>"Angiomatosis", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M17400", "ConditionBrowseLeafName"=>"Vascular Diseases", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M1076", "ConditionBrowseLeafName"=>"Ciliopathies", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M12", "ConditionBrowseLeafName"=>"Congenital Abnormalities", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M14", "ConditionBrowseLeafName"=>"Abnormalities, Multiple", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"M23686", "ConditionBrowseLeafName"=>"Genetic Diseases, Inborn", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"T5876", "ConditionBrowseLeafName"=>"Von Hippel-Lindau Disease", "ConditionBrowseLeafAsFound"=>"Von Hippel-Lindau Disease", "ConditionBrowseLeafRelevance"=>"high"}, {"ConditionBrowseLeafId"=>"T2685", "ConditionBrowseLeafName"=>"Hemangioblastoma", "ConditionBrowseLeafRelevance"=>"low"}, {"ConditionBrowseLeafId"=>"T2072", "ConditionBrowseLeafName"=>"Endolymphatic Sac Tumor", "ConditionBrowseLeafAsFound"=>"Endolymphatic Sac Tumors", "ConditionBrowseLeafRelevance"=>"high"}]}, "ConditionBrowseBranchList"=>{"ConditionBrowseBranch"=>[{"ConditionBrowseBranchName"=>"Neoplasms", "ConditionBrowseBranchAbbrev"=>"BC04"}, {"ConditionBrowseBranchName"=>"All Conditions", "ConditionBrowseBranchAbbrev"=>"All"}, {"ConditionBrowseBranchName"=>"Urinary Tract, Sexual Organs, and Pregnancy Conditions", "ConditionBrowseBranchAbbrev"=>"BXS"}, {"ConditionBrowseBranchName"=>"Ear, Nose, and Throat Diseases", "ConditionBrowseBranchAbbrev"=>"BC09"}, {"ConditionBrowseBranchName"=>"Nervous System Diseases", "ConditionBrowseBranchAbbrev"=>"BC10"}, {"ConditionBrowseBranchName"=>"Symptoms and General Pathology", "ConditionBrowseBranchAbbrev"=>"BC23"}, {"ConditionBrowseBranchName"=>"Eye Diseases", "ConditionBrowseBranchAbbrev"=>"BC11"}, {"ConditionBrowseBranchName"=>"Heart and Blood Diseases", "ConditionBrowseBranchAbbrev"=>"BC14"}, {"ConditionBrowseBranchName"=>"Diseases and Abnormalities at or Before Birth", "ConditionBrowseBranchAbbrev"=>"BC16"}, {"ConditionBrowseBranchName"=>"Skin and Connective Tissue Diseases", "ConditionBrowseBranchAbbrev"=>"BC17"}, {"ConditionBrowseBranchName"=>"Rare Diseases", "ConditionBrowseBranchAbbrev"=>"Rare"}]}}}, "ProtocolSection"=>{"DesignModule"=>{"StudyType"=>"Observational", "EnrollmentInfo"=>{"EnrollmentCount"=>"75"}}, "StatusModule"=>{"OverallStatus"=>"Completed", "StartDateStruct"=>{"StartDate"=>"April 1997"}, "ExpandedAccessInfo"=>{"HasExpandedAccess"=>"No"}, "StatusVerifiedDate"=>"May 1999", "CompletionDateStruct"=>{"CompletionDate"=>"April 2000"}, "LastUpdateSubmitDate"=>"March 3, 2008", "StudyFirstSubmitDate"=>"November 3, 1999", "StudyFirstSubmitQCDate"=>"December 9, 2002", "LastUpdatePostDateStruct"=>{"LastUpdatePostDate"=>"March 4, 2008", "LastUpdatePostDateType"=>"Estimate"}, "StudyFirstPostDateStruct"=>{"StudyFirstPostDate"=>"December 10, 2002", "StudyFirstPostDateType"=>"Estimate"}}, "OversightModule"=>{}, "ConditionsModule"=>{"KeywordList"=>{"Keyword"=>["Deafness", "Hemangioblastoma", "Papillary Carcinoma", "VHL Gene", "Vestibular Function", "von Hippel-Lindau Disease"]}, "ConditionList"=>{"Condition"=>["Deafness", "Kidney Diseases", "Kidney Neoplasms", "Neoplasms", "Retinal Diseases"]}}, "DescriptionModule"=>{"BriefSummary"=>"The von Hippel Lindau (VHL) gene has recently been identified as the genetic defect resulting in a syndrome of multiple neoplasias. Patients with VHL disease develop retinal angiomata, renal cysts and/or carcinomas, CNS hemangioblastomas as well as pancreatic cysts and pheochromocytomas. Investigators have shown the gene to be a tumor suppressor type proto-oncogene located at chromosomal locus 3p26. The gene includes three exons whose gene product targets a cellular transcription factor Elongin SIII. Binding of the VHL proteins to two subunits of this elongation factor inhibits transcription and may play a crucial role in the clinical development of the von Hippel Lindau phenotype.", "DetailedDescription"=>"The von Hippel Lindau (VHL) gene has recently been identified as the genetic defect resulting in a syndrome of multiple neoplasias. Patients with VHL disease develop retinal angiomata, renal cysts and/or carcinomas, CNS hemangioblastomas as well as pancreatic cysts and pheochromocytomas. Investigators have shown the gene to be a tumor suppressor type proto-oncogene located at chromosomal locus 3p26. The gene includes three exons whose gene product targets a cellular transcription factor Elongin SIII. Binding of the VHL proteins to two subunits of this elongation factor inhibits transcription and may play a crucial role in the clinical development of the von Hippel Lindau phenotype."}, "EligibilityModule"=>{"Gender"=>"All", "StdAgeList"=>{"StdAge"=>["Child", "Adult", "Older Adult"]}, "HealthyVolunteers"=>"No", "EligibilityCriteria"=>"Patients meeting the diagnostic criteria for von Hippel-Lindau (VHL) disease.\n\nNo persons who are pregnant or lactating are eligible for the surgical arm of this protocol until the pregnancy and/or nursing period has reached completion.\n\nNo patients with disorders associated with multiple abnormalities of the middle ear and inner ear. Specific laboratory abnormalities such as anti-HIV-1, FTA-Abs, serum ANA, and ANCA have been associated with AIDS, Syphilis, Systemic Lupus Erythematosus, and Wegener's Granulomatosis, respectively.\n\nNo patients currently undergoing chemotherapeutic regimen with ototoxic agents (e.g., cisplatin). Other agents will be reviewed on a case-by-case basis for their potential to cause ototoxicity and thereby interfere with audiologic data interpretation.\n\nPatients with an ELST in an only hearing ear will be excluded from the protocol for surgical treatment of ELST's (except in cases where other medical indications necessitate intervention for the welfare of the patient).\n\nPatients with only unilateral vestibular function on the side affected by the ELST, as documented by caloric ENG testing, will be excluded from the surgical treatment group in most cases.\n\nNo patients with the inability to understand all of the requirements of the study or inability to give informed consent and/or comply with all aspects of the evaluation."}, "IdentificationModule"=>{"NCTId"=>"NCT00001668", "BriefTitle"=>"Endolymphatic Sac Tumors in a Population of Patients With Von Hippel-Lindau Disease:The Natural History and Pathobiology, and a Prospective Non-Randomized Clinical Trial of Hearing Preservation Surgery in Patients With Early Stage Endolymphatic Sac Tumors", "Organization"=>{"OrgClass"=>"NIH", "OrgFullName"=>"National Institutes of Health Clinical Center (CC)"}, "OfficialTitle"=>"Endolymphatic Sac Tumors in a Population of Patients With Von Hippel-Lindau Disease:The Natural History and Pathobiology, and a Prospective Non-Randomized Clinical Trial of Hearing Preservation Surgery in Patients With Early Stage Endolymphatic Sac Tumors", "OrgStudyIdInfo"=>{"OrgStudyId"=>"970102"}, "SecondaryIdInfoList"=>{"SecondaryIdInfo"=>[{"SecondaryId"=>"97-N-0102"}]}}, "ContactsLocationsModule"=>{"LocationList"=>{"Location"=>[{"LocationZip"=>"20892", "LocationCity"=>"Bethesda", "LocationState"=>"Maryland", "LocationCountry"=>"United States", "LocationFacility"=>"National Institute of Neurological Disorders and Stroke (NINDS)"}]}}, "SponsorCollaboratorsModule"=>{"LeadSponsor"=>{"LeadSponsorName"=>"National Institute of Neurological Disorders and Stroke (NINDS)", "LeadSponsorClass"=>"NIH"}}}}}}