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

Abstract

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.

Middle Ear Neuroendocrine Adenoma: A Case Report and Literature Review

“…In most cases, such as in ours, cholesteatoma is the first suspect. e differential diagnoses of a neuroendocrine adenoma include jugulotympanic paraganglioma, vascular malformations, acoustic neurinoma, meningioma, endolymphatic sac papillary tumor, rhabdomyosarcoma, teratoid tumor, and adenocarcinoma [8][9][10]. However, histologic, imaging, and immunohistochemical investigations allow for an accurate diagnosis [2].…”

Seltene Erkrankungen des vestibulären Labyrinths: von Zebras, Chamäleons und Wölfen im Schafspelz

“…9 ) 271 272 273 . They are characterized by a locally destructive and infiltrating growth pattern, whereas metastases are very rare (only three cases with spinal or cerebellar metastases reported so far) 274 275 . Currently, less than 200 cases of ELSTs have been described in the medical literature 276 .…”

“…Die Tumoren wachsen lokal destruktiv und infiltrierend, metastasieren aber äusserst selten (insgesamt drei Fallbeschreibungen mit zerebellären bzw. spinalen Metastasen in der Literatur) 274 275 . Aktuell sind in der Literatur weniger als 200 Fälle von Tumoren des endolymphatischen Sacks (ELST) beschrieben 276 .…”

Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone

“…Patients with ELST often present with the gradual onset of sensorineural hearing loss and tinnitus [14][15][16][17][18][19][20][21][22]. This may slowly progress to include earache, vertigo, ataxia, facial nerve paresis, and otorrhea (Table 1) [14].…”

“…Thus, particularly in patients with clinical manifestations simulating Ménière's disease, the endolymphatic sac region must be carefully examined in order not to miss an ELST [25]. ELSTs may be sporadic or associated with von Hippel-Lindau (VHL) disease which is attributable to inactivation of a tumor suppressor gene on the short arm of chromosome 3, and inherited as an autosomal dominant trait with variable expression [1,14,15,20,[26][27][28][29][30][31][32][33][34][35][36][37]. VHL predisposes patients to multiple hemangioblastomas of the retina or central nervous system as well as tumors and cysts in various organs, including renal cell carcinoma, pheochromocytoma, paraganglioma, ELST, epididymal cystadenoma, and pancreatic serous cystadenoma or neuroendocrine tumors [1].…”