Endolymphatic Sac Tumors

Wick, Cameron C.; Manzoor, Nauman F.; Semaan, Maroun T.; Megerian, Cliff A.

doi:10.1016/j.otc.2014.12.006

Cited by 44 publications

(61 citation statements)

References 46 publications

(62 reference statements)

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“…Of note, several human diseases of the endolymphatic duct and sac are known. These include Meniere's disease, characterized by vertigo and tinnitus and linked to changes in the endolymph content and pressure (Nakashima et al, 2016), endolymphatic sac epithelial tumors, frequently associated to the Von-Hippel-Lindau syndrome (Wick et al, 2015), and the Enlarged Vestibular Aqueduct, linked to dilated endolymphatic sac and duct epithelia (Griffith and Wangemann, 2011). Given the well-established involvement of senescence in many diseases and its physiological occurrence in endolymphatic sac morphogenesis, it would be interesting to investigate the potential role of senescence in endolymphatic sac pathologies.…”

Section: Developmental Senescencementioning

confidence: 99%

Complementary and distinct roles of autophagy, apoptosis and senescence during early inner ear development

2019

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Auditory and vestibular organs form the adult vertebrate inner ear, both contain highly differentiated and specialized sensory epithelia that receive sound and position stimuli, respectively, and convey information to the brain (Magariños et al., 2012). Sensory patches of the inner ear contain sensory cells of two types, outer and inner hair cells, several types of supporting cells and neurons (Magariños et al., 2012; Burns et al., 2015). These cells do not regenerate in adult mammals, despite it has been described that there are small populations of resident stem cells in the adult inner ear (Oshima et al., 2007). Both sensory organs have a common developmental origin in the sensory otic placode, a thickening of the ectoderm that invaginates towards the neural tube forming the otic cup, which later closes up and forms the otic vesicle, also named otocyst (Adam et al., 1998; Magariños et al., 2012). The otic vesicle is an embryonic transitory organ that contains the information required to generate most cells of the adult inner ear (Magariños et al., 2014). The otic vesicle can be explanted and the whole organ cultured in Review Article Complementary and distinct roles of autophagy, apoptosis and senescence during early inner ear development

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Section: Developmental Senescencementioning

confidence: 99%

Complementary and distinct roles of autophagy, apoptosis and senescence during early inner ear development

2019

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“…The jugular bulb is skeletonized, as is the tegmental dura and sinodural angle. Once the posterior semicircular canal is skeletonized, the outline of a bulging tumor involving the endolymphatic sac is seen [10]. When the posterior fossa dura is healthy, the sac and duct are removed en bloc.…”

Section: Surgical Treatmentmentioning

confidence: 99%

“…ELSTs are rare, with fewer than 300 cases reported in the literature [10][11][12][13]. The aim of this article is to review the presentation, diagnosis, treatment, and outcomes for this entity.…”

Section: Introductionmentioning

confidence: 99%

Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone

et al. 2018

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An endolymphatic sac tumor (ELST) is a rare, indolent but locally aggressive tumor arising in the posterior petrous ridge. Patients present with sensorineural hearing loss and tinnitus. As the tumor progresses, patients may experience vertigo, ataxia, facial nerve paresis, pain and otorrhea. Most patients present in their 4th or 5th decade with a wide age range. Patients with von Hippel-Lindau disease have an increased likelihood of developing ELST. Histologically, ELST is a low-grade adenocarcinoma. As it progresses, it destroys bone and extends into adjacent tissues. The likelihood of regional or distant metastases is remote. The optimal treatment is resection with negative margins. Patients with positive margins, gross residual disease, or unresectable tumor are treated with radiotherapy or radiosurgery. Late recurrences are common, so long follow-up is necessary to assess efficacy. The likelihood of cure depends on tumor extent and is probably in the range of 50-75%.

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“…Eine sehr seltene Differenzialdiagnose ist ein Tumor des Saccus endolymphaticus, dieser tritt z. B. beim von-Hippel-Lindau-Syndrom [70].…”

Section: Syndrom Des Erweiterten Aquaeductus Vestibuliunclassified

Diagnostik und Therapie des Hörsturzes

Plontke

2017

Laryngo-Rhino-Otol

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This article reviews recent aspects of diagnostics, differential diagnostics and evidence in systemic and local therapy of idiopathic, sudden, sensorineural hearing loss (ISSHL). Since a number of disorders can be accompanied by sudden hearing loss a meaningful and targeted diagnostic strategy is of utmost importance. An important differential diagnosis of sudden hearing loss are intralabyrinthine schwannomas (ILS). The incidence of ILS is probably significantly underestimated. This may be due to the lack of awareness or lack of explicit search for an intralabyrinthine tumor on MRI or an inappropriate MRI technique ('head-MRI' instead of 'temporal bone-MRI' with too high slice thickness) for the evaluation of sudden hearing loss. Therefore, the request to the radiologist should specifically include the question for (or exclusion of) an ILS. With special MRI techniques, it is today possible to visualize an endolymphatic hydrops. The evidence in the therapy of ISSHL is - with respect to quality not quantity of studies - unsatisfying. The value of systemically (low dose) or intratympanically applied corticosteroids in the primary treatment of ISSHL is still unclear. In order to investigate the efficacy and safety of high dose corticosteroids in primary treatment for ISSHL a national, multicenter, three-armed, randomized, triple-blind controlled clinical trial is currently performed in Germany (http://hodokort-studie.hno.org/). After insufficient recovery of threshold with systemic therapy of ISSHL, intratympanic corticosteroid therapy appears to be associated with a significantly higher chance of improvement of hearing threshold than no therapy or placebo. Both, hearing gain and final hearing thresholds, however, appear to be independent of the start of secondary therapy. Based on the currently available data from clinical studies, no recommendation can be made with respect to type of corticosteroid and specifics of the intratympanic application protocol.

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Endolymphatic Sac Tumors

Cited by 44 publications

References 46 publications

Complementary and distinct roles of autophagy, apoptosis and senescence during early inner ear development

Complementary and distinct roles of autophagy, apoptosis and senescence during early inner ear development

Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone

Diagnostik und Therapie des Hörsturzes

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