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Updated: May 26, 2026
04:34
Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
[Multidisciplinary approach to the endolymphatic sac tumour]
L G González-Bonet1, L I Amoros-Sebastiá, J Piquer-Belloch
1Servicios de Neurocirugía, Hospital de La Ribera, Alzira, Valencia. gonzalez_luibon@gva.es
Neurocirugia (Asturias, Spain)
|December 15, 2011
Summary
Endolymphatic sac tumors, associated with Von Hippel-Lindau disease, can cause sudden hearing loss and vertigo. Early detection and surgical removal are crucial for preserving hearing function.
Area of Science:
- Otolaryngology
- Neurosurgery
- Oncology
Background:
- Papillary adenocarcinoma of the endolymphatic sac (ELS) is a rare tumor, often linked to Von Hippel-Lindau disease (VHL).
- ELS tumors exhibit slow growth but high local aggressiveness, typically without metastasis.
- Symptoms include Meniere's syndrome due to endolymphatic duct compression, often presenting as sudden, irreversible hearing loss.
Observation:
- A case study of a 17-year-old male with unilateral deafness and vertigo is presented.
- The patient had a family history of VHL disease.
- Imaging revealed a mass at the vestibular aqueduct, confirmed as an ELS tumor post-petrosectomy.
Findings:
- ELS tumors can present with sudden, irreversible hearing loss, even with inconclusive imaging.
- Surgical resection via a presigmoidal approach, preserving the inner ear, is the elective treatment.
- Preoperative embolization or stereotactic radiation can mitigate perioperative bleeding risks.
Implications:
- Suspect ELS tumors in patients with VHL history and symptoms like vertigo and hearing changes.
- Prompt diagnosis and intervention are vital for hearing preservation.
- This case highlights the importance of considering ELS tumors in the differential diagnosis of audiovestibular symptoms.
