Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease: Implications for their natural history, diagnosis, and treatment

H. Jeffrey Kim, John A. Butman, Carmen Brewer, Christopher Zalewski, Alexander Vortmeyer, Gladys Glenn, Edward H. Oldfield, Russell R. Lonser

Research output: Contribution to journalArticle

52 Citations (Scopus)

Abstract

Object. Endolymphatic sac tumors (ELSTs), which often are associated with von Hippel-Lindau (VHL) disease, cause irreversible hearing loss and vestibulopathy. Clinical and imaging surveillance protocols provide new insights into the natural history, mechanisms of symptom formation, and indications for the treatment of ELSTs. To clarify the uncertainties associated with the pathophysiology and treatment of ELSTs, the authors describe a series of patients with VHL disease in whom serial examinations recorded the development of ELSTs. Methods. Patients with VHL disease were included if serial clinical and imaging studies captured the development of ELSTs, and the patients underwent tumor resection. The patients' clinical, audiological, and imaging characteristics as well as their operative results were analyzed. Five consecutive patients (three men and two women) with a mean age at surgery of 34.8 years and a follow-up period of 6 to 18 months were included in this study. Audiovestibular symptoms were present in three patients before a tumor was evident on neuroimaging. Imaging evidence of an intralabyrinthine hemorrhage coincided with a loss of hearing in three patients. Successful resection of the ELSTs was accomplished by performing a retrolabyrinthine posterior petrosectomy (RLPP). Hearing stabilized and vestibular symptoms resolved after surgery in all patients. No patient has experienced a recurrence. Conclusions. Audiovestibular symptoms, including hearing loss, in patients with VHL disease can be the result of microscopic ELSTs. Once an ELST has been detected, it can be completely resected via an RLPP with preservation of hearing and amelioration of vestibular symptoms. Early detection and surgical treatment of small ELSTs, when hearing is still present, should reduce the incidence and severity of hearing loss, tinnitus, vertigo, and cranial nerve dysfunction, which are associated with these tumors.

Original languageEnglish (US)
Pages (from-to)503-512
Number of pages10
JournalJournal of neurosurgery
Volume102
Issue number3
DOIs
StatePublished - Jan 1 2005
Externally publishedYes

Fingerprint

Endolymphatic Sac
von Hippel-Lindau Disease
Natural History
Neoplasms
Hearing Loss
Therapeutics
Hearing
Tinnitus
Cranial Nerves
Vertigo
Neuroimaging
Uncertainty

Keywords

  • Anatomy
  • Endolymphatic sac tumor
  • Neurosurgery
  • Pathophysiology
  • Von Hippel-Lindau disease

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease : Implications for their natural history, diagnosis, and treatment. / Kim, H. Jeffrey; Butman, John A.; Brewer, Carmen; Zalewski, Christopher; Vortmeyer, Alexander; Glenn, Gladys; Oldfield, Edward H.; Lonser, Russell R.

In: Journal of neurosurgery, Vol. 102, No. 3, 01.01.2005, p. 503-512.

Research output: Contribution to journalArticle

Kim, H. Jeffrey ; Butman, John A. ; Brewer, Carmen ; Zalewski, Christopher ; Vortmeyer, Alexander ; Glenn, Gladys ; Oldfield, Edward H. ; Lonser, Russell R. / Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease : Implications for their natural history, diagnosis, and treatment. In: Journal of neurosurgery. 2005 ; Vol. 102, No. 3. pp. 503-512.
@article{3295269aacd74bb4a64c0efb680cb35e,
title = "Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease: Implications for their natural history, diagnosis, and treatment",
abstract = "Object. Endolymphatic sac tumors (ELSTs), which often are associated with von Hippel-Lindau (VHL) disease, cause irreversible hearing loss and vestibulopathy. Clinical and imaging surveillance protocols provide new insights into the natural history, mechanisms of symptom formation, and indications for the treatment of ELSTs. To clarify the uncertainties associated with the pathophysiology and treatment of ELSTs, the authors describe a series of patients with VHL disease in whom serial examinations recorded the development of ELSTs. Methods. Patients with VHL disease were included if serial clinical and imaging studies captured the development of ELSTs, and the patients underwent tumor resection. The patients' clinical, audiological, and imaging characteristics as well as their operative results were analyzed. Five consecutive patients (three men and two women) with a mean age at surgery of 34.8 years and a follow-up period of 6 to 18 months were included in this study. Audiovestibular symptoms were present in three patients before a tumor was evident on neuroimaging. Imaging evidence of an intralabyrinthine hemorrhage coincided with a loss of hearing in three patients. Successful resection of the ELSTs was accomplished by performing a retrolabyrinthine posterior petrosectomy (RLPP). Hearing stabilized and vestibular symptoms resolved after surgery in all patients. No patient has experienced a recurrence. Conclusions. Audiovestibular symptoms, including hearing loss, in patients with VHL disease can be the result of microscopic ELSTs. Once an ELST has been detected, it can be completely resected via an RLPP with preservation of hearing and amelioration of vestibular symptoms. Early detection and surgical treatment of small ELSTs, when hearing is still present, should reduce the incidence and severity of hearing loss, tinnitus, vertigo, and cranial nerve dysfunction, which are associated with these tumors.",
keywords = "Anatomy, Endolymphatic sac tumor, Neurosurgery, Pathophysiology, Von Hippel-Lindau disease",
author = "Kim, {H. Jeffrey} and Butman, {John A.} and Carmen Brewer and Christopher Zalewski and Alexander Vortmeyer and Gladys Glenn and Oldfield, {Edward H.} and Lonser, {Russell R.}",
year = "2005",
month = "1",
day = "1",
doi = "10.3171/jns.2005.102.3.0503",
language = "English (US)",
volume = "102",
pages = "503--512",
journal = "Journal of Neurosurgery",
issn = "0022-3085",
publisher = "American Association of Neurological Surgeons",
number = "3",

}

TY - JOUR

T1 - Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease

T2 - Implications for their natural history, diagnosis, and treatment

AU - Kim, H. Jeffrey

AU - Butman, John A.

AU - Brewer, Carmen

AU - Zalewski, Christopher

AU - Vortmeyer, Alexander

AU - Glenn, Gladys

AU - Oldfield, Edward H.

AU - Lonser, Russell R.

PY - 2005/1/1

Y1 - 2005/1/1

N2 - Object. Endolymphatic sac tumors (ELSTs), which often are associated with von Hippel-Lindau (VHL) disease, cause irreversible hearing loss and vestibulopathy. Clinical and imaging surveillance protocols provide new insights into the natural history, mechanisms of symptom formation, and indications for the treatment of ELSTs. To clarify the uncertainties associated with the pathophysiology and treatment of ELSTs, the authors describe a series of patients with VHL disease in whom serial examinations recorded the development of ELSTs. Methods. Patients with VHL disease were included if serial clinical and imaging studies captured the development of ELSTs, and the patients underwent tumor resection. The patients' clinical, audiological, and imaging characteristics as well as their operative results were analyzed. Five consecutive patients (three men and two women) with a mean age at surgery of 34.8 years and a follow-up period of 6 to 18 months were included in this study. Audiovestibular symptoms were present in three patients before a tumor was evident on neuroimaging. Imaging evidence of an intralabyrinthine hemorrhage coincided with a loss of hearing in three patients. Successful resection of the ELSTs was accomplished by performing a retrolabyrinthine posterior petrosectomy (RLPP). Hearing stabilized and vestibular symptoms resolved after surgery in all patients. No patient has experienced a recurrence. Conclusions. Audiovestibular symptoms, including hearing loss, in patients with VHL disease can be the result of microscopic ELSTs. Once an ELST has been detected, it can be completely resected via an RLPP with preservation of hearing and amelioration of vestibular symptoms. Early detection and surgical treatment of small ELSTs, when hearing is still present, should reduce the incidence and severity of hearing loss, tinnitus, vertigo, and cranial nerve dysfunction, which are associated with these tumors.

AB - Object. Endolymphatic sac tumors (ELSTs), which often are associated with von Hippel-Lindau (VHL) disease, cause irreversible hearing loss and vestibulopathy. Clinical and imaging surveillance protocols provide new insights into the natural history, mechanisms of symptom formation, and indications for the treatment of ELSTs. To clarify the uncertainties associated with the pathophysiology and treatment of ELSTs, the authors describe a series of patients with VHL disease in whom serial examinations recorded the development of ELSTs. Methods. Patients with VHL disease were included if serial clinical and imaging studies captured the development of ELSTs, and the patients underwent tumor resection. The patients' clinical, audiological, and imaging characteristics as well as their operative results were analyzed. Five consecutive patients (three men and two women) with a mean age at surgery of 34.8 years and a follow-up period of 6 to 18 months were included in this study. Audiovestibular symptoms were present in three patients before a tumor was evident on neuroimaging. Imaging evidence of an intralabyrinthine hemorrhage coincided with a loss of hearing in three patients. Successful resection of the ELSTs was accomplished by performing a retrolabyrinthine posterior petrosectomy (RLPP). Hearing stabilized and vestibular symptoms resolved after surgery in all patients. No patient has experienced a recurrence. Conclusions. Audiovestibular symptoms, including hearing loss, in patients with VHL disease can be the result of microscopic ELSTs. Once an ELST has been detected, it can be completely resected via an RLPP with preservation of hearing and amelioration of vestibular symptoms. Early detection and surgical treatment of small ELSTs, when hearing is still present, should reduce the incidence and severity of hearing loss, tinnitus, vertigo, and cranial nerve dysfunction, which are associated with these tumors.

KW - Anatomy

KW - Endolymphatic sac tumor

KW - Neurosurgery

KW - Pathophysiology

KW - Von Hippel-Lindau disease

UR - http://www.scopus.com/inward/record.url?scp=16244373064&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=16244373064&partnerID=8YFLogxK

U2 - 10.3171/jns.2005.102.3.0503

DO - 10.3171/jns.2005.102.3.0503

M3 - Article

C2 - 15796386

AN - SCOPUS:16244373064

VL - 102

SP - 503

EP - 512

JO - Journal of Neurosurgery

JF - Journal of Neurosurgery

SN - 0022-3085

IS - 3

ER -