Posterior petrous face meningiomas presenting with Ménière’s-like syndrome: a case series and review of the literature

2021 ◽  
pp. 1-8
Author(s):  
Ramin A. Morshed ◽  
Nicole T. Jiam ◽  
Elaina J. Wang ◽  
Stephen T. Magill ◽  
Renata M. Knoll ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Early Recognition ◽  
Preoperative Evaluation ◽  
Case Series ◽  
Tumor Location ◽  
Endolymphatic Sac ◽  
Episodic Vertigo ◽  
Cranial Nerve Viii ◽  
Fluctuating Hearing Loss

OBJECTIVE Ménière’s disease is an inner ear disorder classically characterized by fluctuating hearing loss, tinnitus, and aural fullness accompanied by episodic vertigo. While the pathogenesis of Ménière’s remains under debate, histopathological analyses implicate endolymphatic sac dysfunction with inner ear fluid homeostatic dysregulation. Little is known about whether external impingement of the endolymphatic sac by tumors may present with Ménière’s-like symptoms. The authors present a case series of 7 patients with posterior fossa meningiomas that involved the endolymphatic sac and new onset of Ménière’s-like symptoms and review the literature on this rare clinical entity. METHODS A retrospective review of patients undergoing resection of a posterior petrous meningioma was performed at the authors’ institution. Inclusion criteria were age older than 18 years; patients presenting with Ménière’s-like symptoms, including episodic vertigo, aural fullness, tinnitus, and/or hearing loss; and tumor location overlying the endolymphatic sac. RESULTS There were 7 cases of posterior petrous face meningiomas involving the vestibular aperture presenting with Ménière’s-like symptoms. Imaging and intraoperative examination confirmed no cranial nerve VIII compression or labyrinthine artery involvement accounting for audiovestibular symptoms. Of the 7 patients in the series, 6 experienced significant improvement or resolution of their vertigo, and all 7 had improvement or resolution of their tinnitus after resection. Of the 5 patients who had preoperative hearing loss, 2 experienced improvement or resolution of their ipsilateral preoperative hearing deficit, whereas the other 3 had unchanged hearing loss compared to preoperative evaluation. CONCLUSIONS Petrous face meningiomas overlying the endolymphatic sac can present with a Ménière’s syndrome. Early recognition and microsurgical excision of these tumors is critical for resolution of most symptoms and stabilization of hearing loss.

Fluctuating Hearing Loss following Immune Reaction in the Endolymphatic Sac of Guinea Pigs

ORL ◽  
1995 ◽  
Vol 57 (3) ◽  
pp. 122-128 ◽  
Author(s):  
Shunichi Tomiyama ◽  
Toshiyuki Kinoshita ◽  
Ken Jinnouchi ◽  
Tetsuo Ikezono ◽  
Yuichi Gotoh ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Guinea Pigs ◽  
Immune Reaction ◽  
Endolymphatic Sac ◽  
Fluctuating Hearing Loss

Adult aqueductal stenosis presenting with fluctuating hearing loss and vertigo

1983 ◽  
Vol 59 (4) ◽  
pp. 703-705 ◽  
Author(s):  
Orhan Barlas ◽  
Hüsameddin Gökay ◽  
M. İnan Turantan ◽  
Nermin Başerer
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Positive Contrast ◽  
Aqueductal Stenosis ◽  
The Other ◽  
Content Type ◽  
Contrast Ventriculography ◽  
Inner Ear Dysfunction ◽  
Fluctuating Hearing Loss ◽  
Fine Print

✓ Two cases of aqueductal stenosis presenting with fluctuating hearing loss, tinnitus, and vertigo are presented. Audiovestibulometric assesment of both cases disclosed the characteristic pattern of disorder of the membranous inner ear. Non-tumoral aqueductal stenosis was demonstrated by computerized tomography in one case and by positive contrast ventriculography in the other. Shunting procedures of the cerebrospinal fluid resulted in resolution of inner ear dysfunction in both patients.

scholarly journals The Natural History of Facial Schwannomas: A Meta-Analysis of Case Series

2018 ◽  
Vol 80 (05) ◽  
pp. 458-468 ◽  
Author(s):  
Matthew Bartindale ◽  
Jeffrey Heiferman ◽  
Cara Joyce ◽  
Neelam Balasubramanian ◽  
Douglas Anderson ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Facial Nerve ◽  
Meta Analysis ◽  
Case Series ◽  
Tumor Location ◽  
Tumor Diameter ◽  
Facial Weakness ◽  
Nerve Segment ◽  
Level Data ◽  
History Of

Abstract Objective This study is to establish predictors of facial paralysis and auditory morbidity secondary to facial schwannomas by assimilating individualized patient data from the literature. Design A systematic review of the literature was conducted for studies regarding facial schwannomas. Studies were only included if they presented patient level data, House–Brackmann grades, and tumor location by facial nerve segment. Odds ratios (OR) were estimated using generalized linear mixed models. Main Outcome Measures Facial weakness and hearing loss. Results Data from 504 patients were collected from 32 studies. The geniculate ganglion was the most common facial nerve segment involved (39.3%). A greater number of facial nerve segments involved was positively associated with both facial weakness and hearing loss, whereas tumor diameter did not correlate with either morbidity. Intratemporal involvement was associated with higher odds of facial weakness (OR = 4.78, p < 0.001), intradural involvement was negatively associated with facial weakness (OR = 0.56, p = 0.004), and extratemporal involvement was not a predictor of facial weakness (OR = 0.68, p = 0.27). The odds of hearing loss increased with more proximal location of the tumor (intradural: OR = 3.26, p < 0.001; intratemporal: OR = 0.60, p = 0.14; extratemporal: OR = 0.27, p = 0.01). Conclusion The most important factors associated with facial weakness and hearing loss are tumor location and the number of facial nerve segments involved. An understanding of the factors that contribute most heavily to the natural morbidity can help guide the appropriate timing and type of intervention in future cases of facial schwannoma.

Endolymphatic sac tumors in von Hippel—Lindau disease

2004 ◽  
Vol 100 (3) ◽  
pp. 480-487 ◽  
Author(s):  
Daniel Choo ◽  
Lawrence Shotland ◽  
Maryann Mastroianni ◽  
Gladys Glenn ◽  
Carter van Waes ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Family Members ◽  
Magnetic Resonance Images ◽  
Endolymphatic Sac ◽  
Specific Gene ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Vhl Disease ◽  
Fine Print

Object. Von Hippel—Lindau (VHL) disease is a hereditary multiple-neoplasia syndrome mapping to chromosome 3p25–26. Endolymphatic sac (ELS) tumors have been identified as a neoplastic manifestation of VHL disease. The purpose of this study was to evaluate comprehensively the natural history of inner ear disease in a large population of patients with confirmed or suspected VHL disease and to correlate the clinical features with the VHL genotype. Methods. The authors collated and analyzed clinical and genotypic data obtained in patients enrolled in an Institutional Review Board—approved protocol in which families and individuals affected by VHL disease were studied. These data included results from multidisciplinary history workups and physical examinations, imaging studies, and a battery of audiological tests. One hundred seventy-five patients were enrolled in the study, 129 with confirmed VHL disease and 46 of their family members in whom test results for VHL disease were negative and who served as controls. Twenty-one patients had ELS tumors that were evident on magnetic resonance images; three of them had bilateral ELS lesions. Hearing loss, often sudden in onset and severe to profound in nature, vestibulopathy, aural fullness, and tinnitus represented the primary symptoms of ELS tumor. Distinct patterns of auditory and vestibular dysfunction occurred at different stages of the disease. Phenotypic data showed that 17 of 21 patients with ELS tumors did not have pheochromocytomas, whereas all had VHL disease affecting the kidney, all but two had VHL disease affecting the central nervous system, and all but one had disease affecting the pancreas. Genotyping revealed 10 rearrangements (partial deletions), eight single bp substitutions, and one 3-bp insertion. Although there was no difference in the incidence of hearing loss between populations, symptoms of imbalance and aural fullness were more common in patients with VHL disease but without imaging evidence of ELS tumor than they were in family members who did not have VHL disease (p < 0.01). Conclusions. Endolymphatic sac tumors are frequently associated with VHL disease. Symptoms of disequilibrium or aural fullness in patients with VHL disease may be an early indication of endolymphatic dysfunction. Patients with VHL disease provide a unique opportunity to examine the effects of specific gene mutations and a discrete neoplastic process on the human inner ear. The study of ELS tumors in this group also provides a pathological model of ELS function and supplies evidence for a role of the ELS in clinical Ménière-like disease(s).

Quantitative Study of Scarpa's Ganglion and Vestibular Sense Organs in Endolymphatic Hydrops

1981 ◽  
Vol 90 (2) ◽  
pp. 121-125 ◽  
Author(s):  
Ernst Richter
Keyword(s):  
Hearing Loss ◽  
Ganglion Cell ◽  
Endolymphatic Hydrops ◽  
Sense Organs ◽  
Episodic Vertigo ◽  
Cell Counts ◽  
Scarpa's Ganglion ◽  
Caloric Response ◽  
Temporal Bones ◽  
Fluctuating Hearing Loss

The density of vestibular hair cells and the number of neurons in Scarpa's ganglion were estimated for 11 temporal bones with endolymphatic hydrops. The ten subjects from which these bones were taken all exhibited decreased caloric response (when tested), fluctuating hearing loss, and episodic vertigo. The degeneration of vestibular sense organs was found within the “normal” range for all except one case, which showed total degeneration of the posterior crista ampullaris. Ganglion cell counts in all cases were low. In three of the ten subjects, counts fell below the lowest values seen in a sample of “normal” ears. These three subjects exhibited fluctuating hearing loss and episodic vertigo for more than six years prior to death. In cases of unilateral endolymphatic hydrops there was no significant difference between counts in the affected and unaffected ear. Thus, the apparent ganglion cell degeneration may be due to ear disease other than endolymphatic hydrops.

scholarly journals Syphilis of the inner ear causing fluctuating hearing loss responsive to steroid therapy.

1989 ◽  
Vol 82 (2) ◽  
pp. 207-211
Author(s):  
Tadahito Saito ◽  
Mituo Mukudai ◽  
Kyoko Tamaki ◽  
Tosihide Tabata
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Steroid Therapy ◽  
Fluctuating Hearing Loss

Delayed Endolymphatic Hydrops

1978 ◽  
Vol 87 (6) ◽  
pp. 743-748 ◽  
Author(s):  
Harold F. Schuknecht
Keyword(s):  
Hearing Loss ◽  
Endolymphatic Hydrops ◽  
Meniere’S Disease ◽  
Ménière’S Disease ◽  
Disease Entity ◽  
Profound Hearing Loss ◽  
Episodic Vertigo ◽  
Delayed Endolymphatic Hydrops ◽  
Ménière's Disease ◽  
Fluctuating Hearing Loss

Delayed endolymphatic hydrops is a disease entity that can be differentiated from Meniere's disease. Typically it occurs in patients who have sustained a profound hearing loss in one ear, usually from infection or trauma, and then after a prolonged period of time develop either episodic vertigo from the same ear (ipsilateral delayed endolymphatic hydrops) or fluctuating hearing loss, also sometimes with episodic vertigo, in the opposite ear (contralateral delayed endolymphatic hydrops). The ipsilateral form of the disease may be treated by labyrinthectomy but no satisfactory therapy is available for the contralateral form of the disease.

Fluctuating Hearing Loss in the Only Hearing Ear: Cochlear Implantation in the Contralateral Deaf Side

2018 ◽  
Vol 158 (6) ◽  
pp. 1101-1106 ◽  
Author(s):  
Francesca Yoshie Russo ◽  
Daniele De Seta ◽  
Ghizlene Lahlou ◽  
Stéphanie Borel ◽  
Yann Nguyen ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Signal To Noise Ratio ◽  
Case Series ◽  
Pure Tone Audiometry ◽  
Noisy Environments ◽  
Tertiary Referral Center ◽  
Unilateral Deafness ◽  
Monosyllabic Words ◽  
Fluctuating Hearing Loss

Objective To investigate the hearing performance of adult patients presenting unilateral deafness with contralateral fluctuating hearing loss who received a cochlear implant on the deaf side. Study Design Case series with chart review. Setting University tertiary referral center. Subjects and Methods Preoperatively and at 6 and 12 months postoperatively, 23 patients underwent pure tone audiometry and speech audiometry with disyllabic and monosyllabic words in a quiet environment and sentences in quiet and noisy (signal-to-noise ratio +10 dB SPL) environments under best-aided conditions. The Abbreviated Profile of Hearing Aid Benefit (APHAB) inventory was evaluated preoperatively and at 6 and 12 months postoperatively. Results No difference was found between pre- and postoperative tests for disyllabic and monosyllabic words. For sentences in quiet and noisy environments, a difference between pre- and postoperative performance was present at 1 year ( P = .002 and P = .02, respectively). In a noisy environment, a difference was present at 6 and 12 months postoperatively as compared with the preoperative value (mean ± SD: 6 months: 42% ± 7.1% vs 61% ± 6.5%, P = .016). A significant improvement in the APHAB score was found at 6 and 12 months postimplantation (Friedman’s 2-way analysis of variance by ranks, P < .001). The number of years of hearing deprivation of the deaf ear was not correlated with performance. Conclusion When incapacitating fluctuating hearing loss occurs in patients presenting a contralateral deaf ear, a cochlear implant is indicated in the latter ear, significantly improving performance in noisy conditions and allowing a better quality of communication to be achieved.

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