Sensorineural hearing loss in patients with vestibular schwannoma correlates with the presence of utricular hydrops as diagnosed on heavily T2-weighted MRI

Eliezer, Michaël; Poillon, Guillaume; Gillibert, André; Horion, Julien; Marie, Jean‐Paul; Guichard, J.-P.; Magné, Nicolas; Attyé, Arnaud

doi:10.1016/j.diii.2019.01.006

Cited by 25 publications

(22 citation statements)

References 30 publications

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“…Eliezer et al, using a 3D non-contrast T2 heavily weighted sequence at 3T, showed that the utricle volume was correlated with the patient's hearing loss in a series of 23 VSs. As most subjects with VS benefit from a wait-and-scan strategy, based on these MRI results, they suggest that a treatment leading to decreased EH could be administrated to achieve better hearing outcomes (44). In a recent paper (45), a saccular dilatation on the ipsilateral side was demonstrated in 30% of VSs (53 out of 183 patients with typical VS).…”

Section: Biological Markersmentioning

confidence: 99%

Biomarkers in Vestibular Schwannoma–Associated Hearing Loss

et al. 2019

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Vestibular schwannomas (VSs) are benign tumors composed of differentiated neoplastic Schwann cells. They can be classified into two groups: sporadic VS and those associated with neurofibromatosis type 2 (NF2). VSs usually grow slowly, initially causing unilateral sensorineural hearing loss (HL) and tinnitus. These tumors cause HL both due to compression of the auditory nerve or the labyrinthine artery and due to the secretion of different substances potentially toxic to the inner ear or the cochlear nerve. As more and more patients are diagnosed and need to be managed, we are more than ever in need of searching for biomarkers associated with these tumors. Owing to an unknown toxic substance generated by the tumor, HL in VS may be linked to a high protein amount of perilymph. Previous studies have identified perilymph proteins correlated with tumor-associated HL, including μ-Crystallin (CRYM), low density lipoprotein receptor-related protein 2 (LRP2), immunoglobulin (Ig) γ-4 chain C region, Ig κ-chain C region, complement C3, and immunoglobulin heavy constant γ 3. Besides, the presence of specific subtypes of heat shock protein 70 has been suggested to be associated with preservation of residual hearing. It has been recently demonstrated that chemokine receptor-4 (CXCR4) is overexpressed in sporadic VS as well as in NF2 tumors and that hearing disability and CXCR4 expression may be correlated. Further, the genetic profile of VS and its relationship with poor hearing has also been studied, including DNA methylation, deregulated genes, growth factors, and NF2 gene mutations. The knowledge of biomarkers associated with VS would be of significant value to maximize outcomes of hearing preservation in these patients.

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Section: Biological Markersmentioning

confidence: 99%

Biomarkers in Vestibular Schwannoma–Associated Hearing Loss

et al. 2019

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“…(24)(25)(26)79); auditory symptoms (85,86); disease duration (24,79); but not for the glycerol test (25)]. Furthermore, correlations were found for 3-point ordinal vestibular scale [cVEMP-side difference (SD) (24); PTA (11,25,87); oVEMP-amplitudes (88), but not with VOG during caloric stimulation SD (24,89) or the glycerol test ( 25 90) investigated 19 MD patients following doubledose iMRI and found that the signal intensity ratio of the cochlear basal turns in the affected ear was significantly higher than in the unaffected ear and that there was a positive correlation between the signal intensity ratio of the cochlear basal turn and the grades of cochlear and vestibular hydrops in the affected ear. The SNR was assessed and calculated manually according to (91) using the signal in perilymph of both cochlear basal turns and noise in coplanar circular 50 mm 2 ROIs in the cerebellum.…”

Section: Els Patterns In MD Hc (Ii)mentioning

confidence: 99%

Intravenous Delayed Gadolinium-Enhanced MR Imaging of the Endolymphatic Space: A Methodological Comparative Study

et al. 2021

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In-vivo non-invasive verification of endolymphatic hydrops (ELH) by means of intravenous delayed gadolinium (Gd) enhanced magnetic resonance imaging of the inner ear (iMRI) is rapidly developing into a standard clinical tool to investigate peripheral vestibulo-cochlear syndromes. In this context, methodological comparative studies providing standardization and comparability between labs seem even more important, but so far very few are available. One hundred eight participants [75 patients with Meniere's disease (MD; 55.2 ± 14.9 years) and 33 vestibular healthy controls (HC; 46.4 ± 15.6 years)] were examined. The aim was to understand (i) how variations in acquisition protocols influence endolymphatic space (ELS) MR-signals; (ii) how ELS quantification methods correlate to each other or clinical data; and finally, (iii) how ELS extent influences MR-signals. Diagnostics included neuro-otological assessment, video-oculography during caloric stimulation, head-impulse test, audiometry, and iMRI. Data analysis provided semi-quantitative (SQ) visual grading and automatic algorithmic quantitative segmentation of ELS area [2D, mm2] and volume [3D, mm3] using deep learning-based segmentation and volumetric local thresholding. Within the range of 0.1–0.2 mmol/kg Gd dosage and a 4 h ± 30 min time delay, SQ grading and 2D- or 3D-quantifications were independent of signal intensity (SI) and signal-to-noise ratio (SNR; FWE corrected, p < 0.05). The ELS quantification methods used were highly reproducible across raters or thresholds and correlated strongly (0.3–0.8). However, 3D-quantifications showed the least variability. Asymmetry indices and normalized ELH proved the most useful for predicting quantitative clinical data. ELH size influenced SI (cochlear basal turn p < 0.001), but not SNR. SI could not predict the presence of ELH. In conclusion, (1) Gd dosage of 0.1–0.2 mmol/kg after 4 h ± 30 min time delay suffices for ELS quantification. (2) A consensus is needed on a clinical SQ grading classification including a standardized level of evaluation reconstructed to anatomical fixpoints. (3) 3D-quantification methods of the ELS are best suited for correlations with clinical variables and should include both ears and ELS values reported relative or normalized to size. (4) The presence of ELH increases signal intensity in the basal cochlear turn weakly, but cannot predict the presence of ELH.

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“…The cochlea is a subtle auditory organ located in the core of temporal bone. In this review, we found ten studies investigating this topic, including four reports on the structural abnormality of the cochlea (13,(37)(38)(39), three reports on the pathological changes in perilymph (40)(41)(42) and three reports regarding the functional evaluation of cochlea (43-45) (Table 4).…”

Section: Cochlear Dysfunctionmentioning

confidence: 99%

“…Recently, endolymphatic hydrops, which could be detected in MRI scans, was correlated with poor hearing in SVS (60). Eliezer et al analyzed T2-weighted MRI scans in 23 patients with obstructive SVS, which revealed a moderate correlation between hearing loss and the volume of the vestibular endolymphatic space and utricle (38). Using the same MRI sequence, another research group performed the study in 183 SVS patients and noticed that saccular dilation was common in SVS (28% unilaterally and 15.7% bilaterally), and a significant association was found between saccular dilation and progressive sensorineural hearing loss (39).…”

Section: Cochlear Dysfunctionmentioning

confidence: 99%

Current Understanding of Hearing Loss in Sporadic Vestibular Schwannomas: A Systematic Review

Gan

Zhang

et al. 2021

Front. Oncol.

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ObjectiveHearing loss is the most common initial symptom in patients with sporadic vestibular schwannomas (SVS). Hearing preservation is an important goal of both conservative and surgical therapy. However, the mechanism of SVS-associated hearing loss remains unclear. Thus, we performed this systematic review to summarize the current understanding of hearing loss in the SVS and distill a testable hypothesis to further illuminate its underlying mechanism.MethodsA systematic review querying four databases (PubMed, Medline, Embase, and Web of Science) was performed to identify studies evaluating hearing loss in patients with SVS and exploring the potential mechanisms of hearing impairment.ResultsA total of 50 articles were eligible and included in this review. After analysis, the retrieved studies could be categorized into four types: (1) 29 studies explore the relationship between hearing loss and the growth pattern of the tumor (e.g., tumor size/volume, growth rate, tumor location, etc.); (2) ten studies investigate the potential role of cochlear dysfunction in hearing deterioration, including structural abnormality, protein elevation in perilymph, and cochlear malfunctioning; (3) two studies looked into SVS-induced impairment of auditory pathway and cortex; (4) in the rest nine studies, researchers explored the molecular mechanism underlying hearing loss in SVS, which involves molecular and genetic alterations, inflammatory response, growth factors, and other tumor-associated secretions.ConclusionsMultiple factors may contribute to the hearing impairment in SVS, including the growth pattern of tumor, cochlear dysfunction, impairment of auditory pathway and cortex, genetic and molecular changes. However, our current understanding is still limited, and future studies are needed to explore this multifactorial hypothesis and dig deeper into its underlying mechanism.

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Sensorineural hearing loss in patients with vestibular schwannoma correlates with the presence of utricular hydrops as diagnosed on heavily T2-weighted MRI

Cited by 25 publications

References 30 publications

Biomarkers in Vestibular Schwannoma–Associated Hearing Loss

Biomarkers in Vestibular Schwannoma–Associated Hearing Loss

Intravenous Delayed Gadolinium-Enhanced MR Imaging of the Endolymphatic Space: A Methodological Comparative Study

Current Understanding of Hearing Loss in Sporadic Vestibular Schwannomas: A Systematic Review

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