Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report

Jay Jagannathan, John A. Butman, Russell R. Lonser, Alexander Vortmeyer, Christopher K. Zalewski, Carmen Brewer, Edward H. Oldfield, H. Jeffrey Kim

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

√Endolymphatic sac tumors (ELSTs) are locally invasive neoplasms that arise in the posterior petrous bone and are associated with von Hippel-Lindau (VHL) disease. These tumors cause symptoms even when microscopic in size (below the threshold for detectability on imaging studies) and can lead to symptoms such as hearing loss, tinnitus, vertigo, and facial nerve dysfunction. While the mechanisms of audiovestibular dysfunction in patients harboring ELSTs are incompletely understood, they have critical implications for management. The authors present the case of a 33-year-old man with VHL disease and a 10-year history of progressive tinnitus, vertigo, and left-sided hearing loss. Serial T1-weighted magnetic resonance (MR) imaging and computed tomography scans revealed no evidence of tumor, but fluid attenuated inversion recovery (FLAIR) MR imaging sequences obtained after hearing loss demonstrated evidence of left intralabyrinthine hemorrhage. On the basis of progressive disabling audiovestibular dysfunction (tinnitus and vertigo), FLAIR imaging findings, and VHL disease status, the patient underwent surgical exploration of the posterior petrous region, and a small (2-mm) ELST was identified and completely resected. Postoperatively, the patient had improvement of the tinnitus and vertigo. Intralabyrinthine hemorrhage may be an early and the only neuroimaging sign of an ELST in patients with VHL disease and audiovestibular dysfunction. These findings support tumor-associated hemorrhage as a mechanism underlying the audiovestibular dysfunction associated with ELSTs.

Original languageEnglish (US)
Pages (from-to)421-425
Number of pages5
JournalJournal of neurosurgery
Volume107
Issue number2
DOIs
StatePublished - Aug 1 2007
Externally publishedYes

Fingerprint

Endolymphatic Sac
Hemorrhage
von Hippel-Lindau Disease
Tinnitus
Vertigo
Neoplasms
Hearing Loss
Magnetic Resonance Imaging
Petrous Bone
Facial Nerve
Neuroimaging
Tomography

Keywords

  • Endolymphatic sac tumor
  • Hemorrhage
  • Mechanism
  • Von hippel-lindau disease

ASJC Scopus subject areas

  • Surgery
  • Medicine(all)
  • Clinical Neurology

Cite this

Jagannathan, J., Butman, J. A., Lonser, R. R., Vortmeyer, A., Zalewski, C. K., Brewer, C., ... Kim, H. J. (2007). Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report. Journal of neurosurgery, 107(2), 421-425. https://doi.org/10.3171/JNS-07/08/0421

Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report. / Jagannathan, Jay; Butman, John A.; Lonser, Russell R.; Vortmeyer, Alexander; Zalewski, Christopher K.; Brewer, Carmen; Oldfield, Edward H.; Kim, H. Jeffrey.

In: Journal of neurosurgery, Vol. 107, No. 2, 01.08.2007, p. 421-425.

Research output: Contribution to journalArticle

Jagannathan, J, Butman, JA, Lonser, RR, Vortmeyer, A, Zalewski, CK, Brewer, C, Oldfield, EH & Kim, HJ 2007, 'Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report', Journal of neurosurgery, vol. 107, no. 2, pp. 421-425. https://doi.org/10.3171/JNS-07/08/0421
Jagannathan, Jay ; Butman, John A. ; Lonser, Russell R. ; Vortmeyer, Alexander ; Zalewski, Christopher K. ; Brewer, Carmen ; Oldfield, Edward H. ; Kim, H. Jeffrey. / Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report. In: Journal of neurosurgery. 2007 ; Vol. 107, No. 2. pp. 421-425.
@article{0f95bfd9d2e6436f89ed5f0d695d04ca,
title = "Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report",
abstract = "√Endolymphatic sac tumors (ELSTs) are locally invasive neoplasms that arise in the posterior petrous bone and are associated with von Hippel-Lindau (VHL) disease. These tumors cause symptoms even when microscopic in size (below the threshold for detectability on imaging studies) and can lead to symptoms such as hearing loss, tinnitus, vertigo, and facial nerve dysfunction. While the mechanisms of audiovestibular dysfunction in patients harboring ELSTs are incompletely understood, they have critical implications for management. The authors present the case of a 33-year-old man with VHL disease and a 10-year history of progressive tinnitus, vertigo, and left-sided hearing loss. Serial T1-weighted magnetic resonance (MR) imaging and computed tomography scans revealed no evidence of tumor, but fluid attenuated inversion recovery (FLAIR) MR imaging sequences obtained after hearing loss demonstrated evidence of left intralabyrinthine hemorrhage. On the basis of progressive disabling audiovestibular dysfunction (tinnitus and vertigo), FLAIR imaging findings, and VHL disease status, the patient underwent surgical exploration of the posterior petrous region, and a small (2-mm) ELST was identified and completely resected. Postoperatively, the patient had improvement of the tinnitus and vertigo. Intralabyrinthine hemorrhage may be an early and the only neuroimaging sign of an ELST in patients with VHL disease and audiovestibular dysfunction. These findings support tumor-associated hemorrhage as a mechanism underlying the audiovestibular dysfunction associated with ELSTs.",
keywords = "Endolymphatic sac tumor, Hemorrhage, Mechanism, Von hippel-lindau disease",
author = "Jay Jagannathan and Butman, {John A.} and Lonser, {Russell R.} and Alexander Vortmeyer and Zalewski, {Christopher K.} and Carmen Brewer and Oldfield, {Edward H.} and Kim, {H. Jeffrey}",
year = "2007",
month = "8",
day = "1",
doi = "10.3171/JNS-07/08/0421",
language = "English (US)",
volume = "107",
pages = "421--425",
journal = "Journal of Neurosurgery",
issn = "0022-3085",
publisher = "American Association of Neurological Surgeons",
number = "2",

}

TY - JOUR

T1 - Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report

AU - Jagannathan, Jay

AU - Butman, John A.

AU - Lonser, Russell R.

AU - Vortmeyer, Alexander

AU - Zalewski, Christopher K.

AU - Brewer, Carmen

AU - Oldfield, Edward H.

AU - Kim, H. Jeffrey

PY - 2007/8/1

Y1 - 2007/8/1

N2 - √Endolymphatic sac tumors (ELSTs) are locally invasive neoplasms that arise in the posterior petrous bone and are associated with von Hippel-Lindau (VHL) disease. These tumors cause symptoms even when microscopic in size (below the threshold for detectability on imaging studies) and can lead to symptoms such as hearing loss, tinnitus, vertigo, and facial nerve dysfunction. While the mechanisms of audiovestibular dysfunction in patients harboring ELSTs are incompletely understood, they have critical implications for management. The authors present the case of a 33-year-old man with VHL disease and a 10-year history of progressive tinnitus, vertigo, and left-sided hearing loss. Serial T1-weighted magnetic resonance (MR) imaging and computed tomography scans revealed no evidence of tumor, but fluid attenuated inversion recovery (FLAIR) MR imaging sequences obtained after hearing loss demonstrated evidence of left intralabyrinthine hemorrhage. On the basis of progressive disabling audiovestibular dysfunction (tinnitus and vertigo), FLAIR imaging findings, and VHL disease status, the patient underwent surgical exploration of the posterior petrous region, and a small (2-mm) ELST was identified and completely resected. Postoperatively, the patient had improvement of the tinnitus and vertigo. Intralabyrinthine hemorrhage may be an early and the only neuroimaging sign of an ELST in patients with VHL disease and audiovestibular dysfunction. These findings support tumor-associated hemorrhage as a mechanism underlying the audiovestibular dysfunction associated with ELSTs.

AB - √Endolymphatic sac tumors (ELSTs) are locally invasive neoplasms that arise in the posterior petrous bone and are associated with von Hippel-Lindau (VHL) disease. These tumors cause symptoms even when microscopic in size (below the threshold for detectability on imaging studies) and can lead to symptoms such as hearing loss, tinnitus, vertigo, and facial nerve dysfunction. While the mechanisms of audiovestibular dysfunction in patients harboring ELSTs are incompletely understood, they have critical implications for management. The authors present the case of a 33-year-old man with VHL disease and a 10-year history of progressive tinnitus, vertigo, and left-sided hearing loss. Serial T1-weighted magnetic resonance (MR) imaging and computed tomography scans revealed no evidence of tumor, but fluid attenuated inversion recovery (FLAIR) MR imaging sequences obtained after hearing loss demonstrated evidence of left intralabyrinthine hemorrhage. On the basis of progressive disabling audiovestibular dysfunction (tinnitus and vertigo), FLAIR imaging findings, and VHL disease status, the patient underwent surgical exploration of the posterior petrous region, and a small (2-mm) ELST was identified and completely resected. Postoperatively, the patient had improvement of the tinnitus and vertigo. Intralabyrinthine hemorrhage may be an early and the only neuroimaging sign of an ELST in patients with VHL disease and audiovestibular dysfunction. These findings support tumor-associated hemorrhage as a mechanism underlying the audiovestibular dysfunction associated with ELSTs.

KW - Endolymphatic sac tumor

KW - Hemorrhage

KW - Mechanism

KW - Von hippel-lindau disease

UR - http://www.scopus.com/inward/record.url?scp=34547687827&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=34547687827&partnerID=8YFLogxK

U2 - 10.3171/JNS-07/08/0421

DO - 10.3171/JNS-07/08/0421

M3 - Article

C2 - 17695400

AN - SCOPUS:34547687827

VL - 107

SP - 421

EP - 425

JO - Journal of Neurosurgery

JF - Journal of Neurosurgery

SN - 0022-3085

IS - 2

ER -