title: Imaging detection of endolymphatic sac tumor-associated hydrops.
abstract: OBJECTIVE
  To determine if physiologically based MRI sequences can be used to detect endolymphatic sac tumor (ELST)-associated hydrops, the authors performed contrast-enhanced delayed FLAIR imaging in consecutive ELST patients with clinical findings consistent with hydrops.
  
  
  METHODS
  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.
  
  
  RESULTS
  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 28-51 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Diagnostic Radiology Department, National Institutes of Health Clinical Center, National Institutes of Health, Bethesda, MD, USA.
authors.forename: John A
authors.initials: JA
authors.lastname: Butman
authors.forename: Edjah
authors.initials: E
authors.lastname: Nduom
authors.forename: H Jeffrey
authors.initials: HJ
authors.lastname: Kim
authors.forename: Russell R
authors.initials: RR
authors.lastname: Lonser
mesh_terms: D000328:Adult; D004428:Ear Neoplasms / Q000150:complications* / Q000473:pathology; D018159:Endolymphatic Hydrops / Q000209:etiology* / Q000473:pathology; D004712:Endolymphatic Sac / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000473:pathology