The Journal of International
Advanced Otology
Case Report

Sporadic Endolymphatic Sac Tumor-A Very Rare Cause of Hearing Loss, Tinnitus, and Dizziness


Department of Otorhinolaryngology, Head and Neck Surgery & Audiology, Rigshospitalet, Copenhagen, Denmark


Department of Pathology, Teilumbygningen, Rigshospitalet, Copenhagen, Denmark


Department of Otorhinolaryngology, Tokyo Women’s University East Medical Center, Tokyo, Japan


Nordsjællands Hospital, Audiology department, Hillerød, Denmark

J Int Adv Otol 2017; 13: 289-291
DOI: 10.5152/iao.2017.2237
Read: 2073 Downloads: 851 Published: 03 September 2019


Sporadic endolymphatic sac tumor is a very rare neoplasm. It is low malignant, locally destructive and expansive, but non-metastasizing. The tumor is very rare in the sporadic form, but more often associated with Von Hippel-Lindau disease. A 65-year old man with left sided tinnitus and hearing loss for several months. Audiometry showed an asymmetrical sensory neural hearing loss on the left side up to 60 dB. The speech discrimination score was 46% and stapedial reflexes were absent. Several years earlier, he had suffered from periods of dizziness. Magnetic resonance imaging (MRI) showed a destructive and locally invasive tumor in the peripheral vestibular system expanding into the cerebellopontine angle. Paraganglioma and von Hippel-Lindau`s disease were excluded. Vestibular examination showed no function of vestibular organ left side. The tumor was resected radically by translabyrintine approach. Per-operative freeze-microscopy showed inflammation tissue, whereas subsequent microscopy showed papillary-cystic endolymphatic sac tumor. Endolymphatic sac tumor is a rare neoplasm. The tumor may present with asymmetrically sensory neural hearing loss with or without tinnitus, dizziness and facial nerve paresis. An MRI scan is the appropriate diagnostic tool final dianosis is made by the post-operative histo-pathology. Dizziness can be the first sign of a tumor in this area.



Cite this article as: Schnack DT, Kiss K, Hansen S, Miyazaki H, Bech B, Caye-Thomasen P. Sporadic Endolymphatic Sac Tumor–A Very Rare Cause of Hearing Loss, Tinnitus, and Dizziness. J Int Adv Otol 2017; 13: 289-91.

EISSN 2148-3817