Intralabyrinthine Schwannomas

Bouchetemble, P.; Heathcote, Kate; Tollard, É.; Choussy, O.; Dehesdin, D.; Marie, Jean‐Paul

doi:10.1097/mao.0b013e31828687f2

Cited by 26 publications

(10 citation statements)

References 32 publications

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“…Intracochlear tumors are thought to cause direct compression of the organ of Corti, cochlear nerve, or blood supply to the cochlea (7,21). Intravestibular tumors are thought to cause hearing loss through compression on vestibular structures leading to endolymphatic hydrops (8). Given that both subtypes produced similar hearing outcomes, potentially a more universal etiology like toxins or other metabolites released by the tumor may alter the delicate metabolic status of the endolymph resulting in significant hearing loss (8).…”

Section: Discussionmentioning

confidence: 99%

“…The location of Scarpa's ganglion in the IAC guards against impact from ILSs, resulting in only a portion of the terminal vestibular neurons being affected by the schwannoma (7). Partial vestibular inhibition can lead to more acute, intense, and potentially intractable dizzy episodes (8)(9)(10). Both tumors cause sensorineural hearing loss, although ILSs mechanistically are thought to precipitate hearing loss secondary to direct compression of the cochlear structures or via endolymphatic hydrops based on respective labyrinth location of the schwannoma (7).…”

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confidence: 99%

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A Case–Case Analysis of Intralabyrinthine Schwannoma Versus Intracanalicular Vestibular Schwannoma

Selleck

Hicks²,

Brown³

2021

Otology &Amp; Neurotology

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Objectives:Determine the differences in presentation of intracanalicular and intralabyrinthine schwannomas.Study Design:Retrospective chart review at a single center.Setting:Tertiary academic referral center.Patients:Eighteen subjects with an intralabyrinthine schwannoma (ILS) and 216 patients with an intracanalicular vestibular schwannoma (VS).Main Outcome Measures:Variables collected included age at diagnosis, gender, presenting signs and symptoms, imaging findings, treatment modality, pure-tone average (PTA), and word recognition scores (WRS) at presentation and at follow-up visits.Results:Incidence of subjective hearing loss was 100% in the ILS group and 80.6% in the intracanalicular VS group. The degree of hearing loss was significantly worse in the ILS group (average PTA of 83.9 dB; average WRS of 46.9%) compared with the VS group (average PTA of 38.7 dB; average WRS of 71.8%) (PTA p = 0.000006; WRS p = 0.007). Other subjective signs and symptoms, including vestibular symptoms, did not differ significantly between the two groups. Approximately one-third of both groups underwent microsurgical resection. 22.2% of ILSs were missed on initial imaging by radiologists.Conclusion:Intralabyrinthine schwannomas differ from intracanalicular schwannomas in the severity of hearing loss at presentation. Otherwise, these two tumors present in a very similar fashion—age at presentation, presenting signs and symptoms—and are treated comparably. Otolaryngologists should maintain a high degree of suspicion for ILS in patients presenting with an asymmetric hearing loss, vertigo, and tinnitus.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

A Case–Case Analysis of Intralabyrinthine Schwannoma Versus Intracanalicular Vestibular Schwannoma

Selleck

Hicks²,

Brown³

2021

Otology &Amp; Neurotology

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“…Despite a few anecdotal reports (29, 30), the safety and efficacy of gamma-knife surgery for ILS remain to be determined. Radiosurgery emerges as the best option for small tumors especially <30 mm (31).…”

Section: Discussionmentioning

confidence: 99%

Intralabyrinthine Schwannoma: Distinct Features for Differential Diagnosis

et al. 2019

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Objectives: The aim of this study was to delineate the clinical and laboratory features suggestive of intralabyrinthine schwannoma (ILS). Methods: We compared the clinical features of 16 patients with ILS, who had been diagnosed at the Seoul National University Bundang Hospital from 2003 to 2018, with those of 18 patients with symptomatic unilateral intracanalicular schwannoma and randomly selected 20 patients with definite or probable unilateral Meniere's disease (MD). Results: Patients with ILS presented with either recurrent spontaneous dizziness/vertigo combined with auditory symptoms ( n = 8), isolated auditory symptoms without dizziness/vertigo ( n = 7), or recurrent spontaneous dizziness/vertigo without auditory symptoms ( n = 1). Most patients reported no improvement ( n = 11) or worsening ( n = 1) of the symptoms despite medical treatments including intratympanic ( n = 5) or intravenous steroids ( n = 2). Conventional brain MRIs failed to detect ILS in about a half of the patients (7/16, 44%). However, ILS showed a filling defect on 3-dimensional (3D) heavily T2-weighted MRIs ( n = 12), and nodular enhancement on 3D contrast-enhanced T1 ( n = 15) or FLAIR MRIs ( n = 13) targeted for the inner ear. Compared to MD or intracanalicular schwannoma, ILS showed mostly abnormal head-impulse tests (HITs, p = 0.001). In contrast, the incidence of canal paresis did not differ among the groups ( p = 0.513). Conclusion: ILS may mimic MD by presenting recurrent dizziness/vertigo and auditory symptoms. ILS should be suspected in patients with recurrent audiovestibulopathy especially when (1) the duration of the dizziness is not typical for MD, (2) the patients do not respond to medical treatments, or (3) HITs are abnormal.

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“…VS are usually unilateral in sporadic disease and bilateral in type-two neurofibromatosis [ 3 ]. Its site of origin is still debated even if there is now general agreement that it is lateral to the Obersteiner-Redlich zone [ 4 , 5 ], where the glial cover of the nerve is replaced by Schwann cells. ILSs are a rare type of VS, with an estimated prevalence of 0.9% in autoptic studies [ 6 ].…”

Section: Literature Reviewmentioning

confidence: 99%

Intralabyrinthine schwannomas: a two-case series and literature review with a focus on hearing rehabilitation

Curatoli

Pontillo

Quaranta

2023

Eur Arch Otorhinolaryngol

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Purpose Intralabyrinthine schwannomas (ILSs) are an uncommon finding. Diagnosis is challenging and no gold standard treatment exists yet. In this article, we present a two-cases series and review the latest available literature to assess the best diagnostic and therapeutic scheme. Methods We reviewed the latest available literature assessing most frequent and relevant sets of symptoms, clinical features of the disease, diagnostic tests and imaging, possible treatments and after-surgery hearing rehabilitation techniques. We then compared literature data to our own series ones. Results ILSs clinical presentation and development may overlap with other, more common otological conditions. Full audiometric battery test, electrophysiological study of VEMPS and MRI with contrast enhancement all appear to be critical to correctly diagnose these tumors. Several treatments exist: radiological follow-up, radiation therapy, full or partial surgical excision. Hearing rehabilitation is mostly accomplished through simultaneous cochlear implantation. Conclusions Our case-series data matches the available literature. ILSs are a rare type of vestibular schwannomas. Diagnosis in challenging and delayed in time as all the diagnostic tests, yet sensitive, are not specific for ILSs. The most suitable treatment seems to be surgical excision of these tumors followed by simultaneous cochlear implantation to restore hearing.

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Intralabyrinthine Schwannomas

Cited by 26 publications

References 32 publications

A Case–Case Analysis of Intralabyrinthine Schwannoma Versus Intracanalicular Vestibular Schwannoma

A Case–Case Analysis of Intralabyrinthine Schwannoma Versus Intracanalicular Vestibular Schwannoma

Intralabyrinthine Schwannoma: Distinct Features for Differential Diagnosis

Intralabyrinthine schwannomas: a two-case series and literature review with a focus on hearing rehabilitation

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