Imaging detection of endolymphatic sac tumor–associated hydrops

Clinical article

John A. Butman M.D., Ph.D.1, Edjah Nduom M.D.2,3, H. Jeffrey Kim M.D.4,5, and Russell R. Lonser M.D.2,6
View More View Less
  • 1 Diagnostic Radiology Department, National Institutes of Health Clinical Center;
  • | 2 Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke;
  • | 4 Head and Neck Surgery Branch, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland;
  • | 3 Department of Neurological Surgery, Emory University, Atlanta, Georgia;
  • | 5 Department of Otolaryngology–Head and Neck Surgery, Georgetown University Medical Center, Georgetown University, Washington, DC; and
  • | 6 Department of Neurological Surgery, The Ohio State University, Columbus, Ohio
Restricted access

Purchase Now

USD  $45.00

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $515.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $612.00
Print or Print + Online

Object

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.

Abbreviations used in this paper:

ELST = endolymphatic sac tumor; VHL = von Hippel-Lindau.

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $515.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $612.00
  • 1

    Butman JA, , Kim HJ, , Baggenstos M, , Ammerman JM, , Dambrosia J, & Patsalides A, et al.: Mechanisms of morbid hearing loss associated with tumors of the endolymphatic sac in von Hippel-Lindau disease. JAMA 298:4148, 2007

    • Search Google Scholar
    • Export Citation
  • 2

    Choo DI, , Shotland L, , Mastroianni M, , Glenn GM, , van Waes C, & Linehan WM, et al.: Endolymphatic sac tumors in von Hippel-Lindau disease. J Neurosurg 100:480487, 2004

    • Search Google Scholar
    • Export Citation
  • 3

    Heffner DK: Low-grade adenocarcinoma of probable endolymphatic sac origin. A clinicopathologic study of 20 cases. Cancer 64:22922302, 1989

    • Search Google Scholar
    • Export Citation
  • 4

    Jagannathan J, , Butman JA, , Lonser RR, , Vortmeyer AO, , Zalewski CK, & Brewer C, et al.: Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report. J Neurosurg 107:421425, 2007

    • Search Google Scholar
    • Export Citation
  • 5

    Kim HJ, , Butman JA, , Brewer C, , Zalewski C, , Vortmeyer AO, & Glenn G, et al.: Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease: implications for their natural history, diagnosis, and treatment. J Neurosurg 102:503512, 2005

    • Search Google Scholar
    • Export Citation
  • 6

    Lonser RR, , Baggenstos M, , Kim HJ, , Butman JA, & Vortmeyer AO: The vestibular aqueduct: site of origin of endolymphatic sac tumors. J Neurosurg 108:751756, 2008

    • Search Google Scholar
    • Export Citation
  • 7

    Lonser RR, , Glenn GM, , Walther M, , Chew EY, , Libutti SK, & Linehan WM, et al.: von Hippel-Lindau disease. Lancet 361:20592067, 2003

  • 8

    Lonser RR, , Kim HJ, , Butman JA, , Vortmeyer AO, , Choo DI, & Oldfield EH: Tumors of the endolymphatic sac in von Hippel-Lindau disease. N Engl J Med 350:24812486, 2004

    • Search Google Scholar
    • Export Citation
  • 9

    Manski TJ, , Heffner DK, , Glenn GM, , Patronas NJ, , Pikus AT, & Katz D, et al.: Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel-Lindau disease. JAMA 277:14611466, 1997

    • Search Google Scholar
    • Export Citation
  • 10

    Megerian CA, , Haynes DS, , Poe DS, , Choo DI, , Keriakas TJ, & Glasscock ME III: Hearing preservation surgery for small endolymphatic sac tumors in patients with von Hippel-Lindau syndrome. Otol Neurotol 23:378387, 2002

    • Search Google Scholar
    • Export Citation
  • 11

    Nakashima T, , Naganawa S, , Pyykko I, , Gibson WP, , Sone M, & Nakata S, et al.: Grading of endolymphatic hydrops using magnetic resonance imaging. Acta Otolaryngol Suppl 560 58, 2009

    • Search Google Scholar
    • Export Citation
  • 12

    Nakashima T, , Naganawa S, , Teranishi M, , Tagaya M, , Nakata S, & Sone M, et al.: Endolymphatic hydrops revealed by intravenous gadolinium injection in patients with Ménière's disease. Acta Otolaryngol 130:338343, 2010

    • Search Google Scholar
    • Export Citation
  • 13

    Tagaya M, , Yamazaki M, , Teranishi M, , Naganawa S, , Yoshida T, & Otake H, et al.: Endolymphatic hydrops and blood-labyrinth barrier in Ménière's disease. Acta Otolaryngol 131:474479, 2011

    • Search Google Scholar
    • Export Citation
  • 14

    Vortmeyer AO, , Choo D, , Pack SD, , Oldfield E, & Zhuang Z: von Hippel-Lindau disease gene alterations associated with endolymphatic sac tumor. J Natl Cancer Inst 89:970972, 1997

    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 710 137 9
Full Text Views 189 28 2
PDF Downloads 108 19 2
EPUB Downloads 0 0 0