Progression of Contralateral Hearing Loss in Patients With Sporadic Vestibular Schwannoma

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Abstract

Background and Introduction: Vestibular schwannomas (VSs) are the most common tumors of the cerebellopontine angle, typically presenting unilaterally with ipsilateral sensorineural hearing loss (SNHL). The mechanism of tumor-induced hearing loss has recently been shown to be related to secreted tumor factors, in addition to mechanical compression of the adjacent auditory nerve, and these factors may percolate through CSF or blood to affect contralateral hearing as well.

Methods: This is a retrospective study of medical records for patients treated for VS at Mass Eye and Ear from January 1994 through October 2018. Included patients had unilateral VS and sequential audiometry allowing for longitudinal assessment of hearing over time. Mass Eye and Ear's audiology database was used to select age- and sex-matched case controls, also with sequential audiometry, from the non-VS population. Subgroup analysis was performed by age, sex, baseline hearing, and tumor size at initial diagnosis. Hearing loss progression was performed using Kaplan-Meier analysis to account for variable follow-up times.

Results: A total of 661 patients were identified with VS and sequential audiometry. The population was predominantly female vs. male (368 vs. 293, p = 0.0035), driven primarily by younger patients with Koos 4 tumors (76 female vs. 49 male, p = 0.016). Patients with normal baseline hearing bilaterally ( N = 241) demonstrated no significant difference in hearing loss progression in VS-contralateral vs. control ears. Patients with abnormal baseline VS-ipsilateral hearing ( N = 190), however, demonstrated significantly higher likelihood of reaching moderate SNHL in VS-contralateral ears. Subgroup analysis by age, sex, and baseline tumor size did not yield any subgroup-specific trends for hearing loss progression.

Discussion and Conclusion: This is the largest study to date tracking long-term bilateral hearing outcomes in patients with VS, and demonstrates that, in patients with abnormal hearing in the VS-ipsilateral ear, there exists a long-term risk of progression to moderate hearing loss in the contralateral ear as well. Combined with the absence of significant changes in word understanding in the affected ears, these findings may provide clues to the nature of tumor-secreted factors involved in VS-associated hearing loss. Female predominance within the VS patient population is confirmed, driven mostly by younger female patients with Koos 4 tumors.

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Most cited references 41

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NLRP3mutation and cochlear autoinflammation cause syndromic and nonsyndromic hearing loss DFNA34 responsive to anakinra therapy

Hiroshi Nakanishi, Yoshiyuki Kawashima, Kiyoto Kurima … (2017)

This study identifies a mutation in the NLRP3 gene that causes sensorineural hearing loss in human patients. NLRP3 encodes a protein important for innate immunity, secretion of the potent cytokine IL-1β, and inflammation. The hearing loss in three affected members of one family improved or completely resolved after treatment with IL-1β blockade therapy. This study shows that the mouse Nlrp3 gene is expressed in immune macrophage-like cells throughout the inner ear, which can be activated to release the potent cytokine IL-1β. These observations suggest that mutations of NLRP3 may cause hearing loss by local autoinflammation within the inner ear. This mechanism could underlie a variety of hearing-loss disorders of unknown etiology that might respond to IL-1β blockade therapy. The NLRP3 inflammasome is an intracellular innate immune sensor that is expressed in immune cells, including monocytes and macrophages. Activation of the NLRP3 inflammasome leads to IL-1β secretion. Gain-of-function mutations of NLRP3 result in abnormal activation of the NLRP3 inflammasome, and cause the autosomal dominant systemic autoinflammatory disease spectrum, termed cryopyrin-associated periodic syndromes (CAPS). Here, we show that a missense mutation, p.Arg918Gln (c.2753G > A), of NLRP3 causes autosomal-dominant sensorineural hearing loss in two unrelated families. In family LMG446, hearing loss is accompanied by autoinflammatory signs and symptoms without serologic evidence of inflammation as part of an atypical CAPS phenotype and was reversed or improved by IL-1β blockade therapy. In family LMG113, hearing loss segregates without any other target-organ manifestations of CAPS. This observation led us to explore the possibility that resident macrophage/monocyte-like cells in the cochlea can mediate local autoinflammation via activation of the NLRP3 inflammasome. The NLRP3 inflammasome can indeed be activated in resident macrophage/monocyte-like cells in the mouse cochlea, resulting in secretion of IL-1β. This pathway could underlie treatable sensorineural hearing loss in DFNA34, CAPS, and possibly in a wide variety of hearing-loss disorders, such as sudden sensorineural hearing loss and Meniere’s disease that are elicited by pathogens and processes that stimulate innate immune responses within the cochlea.

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Management of 1000 vestibular schwannomas (acoustic neuromas): clinical presentation.

Setareh M. Samii, C Matthies (1996)

Despite good knowledge of the key symptoms of vestibular schwannomas and their significance for surgical results, the evolution of symptoms and signs and their relation to tumor extension still need thorough investigation. From 1978 to 1993, operations were performed by the same surgeon (M.S.) on 1000 vestibular schwannomas at the Neurosurgical Department of Nordstadt Hospital. The vestibular schwannomas were diagnosed in 962 patients, including 522 female patients (54%) and 440 male patients (46%); the mean age was significantly higher in female patients (47.6 yr) than in men (45.2 yr). We focused our analysis on the incidence of subjective disturbances versus objective morbidity, on the sequence of symptom onset, and on symptom duration and symptomatology versus tumor size and extension. The most frequent clinical symptoms were disturbances of the acoustic (95%), vestibular (61%), trigeminal (9%), and facial (6%) nerves. Symptom duration was 3.7 years for hearing loss, 1.9 years for facial paresis, and 1.3 years for trigeminal disturbances. Symptom incidence and duration did not strictly correlate with tumor size. Key symptoms of various tumor extension classes precipitated the diagnosis, such as trigeminal disturbances in large tumors with brain stem compression or tinnitus in small neuromas. In cases of trigeminal or facial nerve symptoms, the overall duration of symptomatology was much shorter. According to the subjective perception of the patients, between only one- and two-thirds of nerve disturbances were noticed. Patients with preoperative deafness had become deaf either chronically (23%) or suddenly (3%); even in cases of moderate hearing deficit that lasts a long time, deafness can occur suddenly. The rate of tinnitus was higher in hearing than in deaf patients; however, deafness does not mean relief from tinnitus, because this symptom persists in 46% of preoperatively deaf patients. Vestibular disturbances most often occur as some unsteadiness while walking or as vertigo, and the symptoms frequently are fluctuating, not constant. Differences in tumor biology can be underestimated and are not visible on radiological scans. For example, intrameatal tumors, despite their small size, present with a duration of symptoms that is representative of the larger tumors and are most frequently associated with vestibular symptoms and with tinnitus. Large tumors with brain stem compression present with relatively shorter symptom durations and at a younger age; both factors are suggestive of especially fast tumor growth. The clinical findings presented in this study promote new consideration of the dynamics of tumor growth and of the affected neural tissues.

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Descriptive epidemiology of vestibular schwannomas.

Susan Martin, Paul Davis, Jennifer M Propp … (2005)

Vestibular schwannomas, commonly termed acoustic neuromas, arise from the vestibular branch of the eighth cranial nerve (acoustic nerve) and are benign, slow-growing brain tumors that negatively impact patient quality of life. They are thought to account for the majority of intracranial nerve sheath tumors. To describe incidence rate patterns and trends of primary nerve sheath tumors of the brain/CNS and the subset of vestibular schwannomas in two population-based incidence registries, data were obtained from 11 Central Brain Tumor Registry of the United States (CBTRUS) collaborating state registries and the Los Angeles County Cancer Surveillance Program (LACCSP) (1975-1998). Average annual incidence rates were tabulated by age, gender, race, year, and region and were age-adjusted to the year 2000 U.S. standard population. Multiplicative Poisson regression models were used to compare trends in primary nerve sheath tumors of the brain/CNS overall and in subgroups, including vestibular schwannomas, controlling for age, gender, race, microscopic confirmation, and region. Joinpoint regression analysis was used to identify any sharp changes in incidence over time. The overall incidence of primary nerve sheath tumors of the brain/CNS was 1.1 per 100,000 person-years (CBTRUS, 1995-1999 and LACCSP, 1995-1998). The incidence of vestibular schwannomas was similar for both data sets: 0.6 per 100,000 person-years (CBTRUS, 1995-1999) and 0.8 per 100,000 person-years (LACCSP, 1995-1998). Moreover, the incidence of primary nerve sheath tumors of the brain/CNS overall (CBTRUS, 1985-1999 and LACCSP, 1975-1998) and of vestibular schwannomas (CBTRUS, 1992-1999 and LACCSP, 1992-1998) increased over time. However, the incidence of benign schwannomas in sites other than the acoustic nerve either decreased (CBTRUS, 1992-1999) or experienced no significant change (LACCSP, 1992-1998). While improvements in diagnosis and reporting may explain some of these trends, further consideration of potential etiologic factors may be warranted.

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Author and article information

Contributors

Samuel Early: URI : http://loop.frontiersin.org/people/945796/overview

Charlotte E. Rinnooy Kan: URI : http://loop.frontiersin.org/people/948866/overview

Maura Eggink: URI : http://loop.frontiersin.org/people/1039579/overview

Johan H. M. Frijns: URI : http://loop.frontiersin.org/people/336359/overview

Konstantina M. Stankovic: URI : http://loop.frontiersin.org/people/647976/overview

Journal

Journal ID (nlm-ta): Front Neurol

Journal ID (iso-abbrev): Front Neurol

Journal ID (publisher-id): Front. Neurol.

Title: Frontiers in Neurology

Publisher: Frontiers Media S.A.

ISSN (Electronic): 1664-2295

Publication date (Electronic): 14 August 2020

Publication date Collection: 2020

Volume: 11

Electronic Location Identifier: 796

Affiliations

[1] ¹Eaton-Peabody Laboratories, Department of Otolaryngology—Head and Neck Surgery, Massachusetts Eye and Ear , Boston, MA, United States

[2] ²Department of Otolaryngology—Head and Neck Surgery, Harvard Medical School , Boston, MA, United States

[3] ³San Diego School of Medicine, University of California, San Diego , San Diego, CA, United States

[4] ⁴Leiden University Medical Center , Leiden, Netherlands

[5] ⁵University Medical Center Groningen , Groningen, Netherlands

[6] ⁶Department of Otolaryngology—Head and Neck Surgery, Leiden University Medical Center , Leiden, Netherlands

[7] ⁷Leiden Institute for Brain and Cognition , Leiden, Netherlands

[8] ⁸Program in Speech and Hearing Bioscience and Technology, Harvard Medical School , Boston, MA, United States

[9] ⁹Harvard Program in Therapeutic Science, Harvard Medical School , Boston, MA, United States

Author notes

Edited by: Stefan K. Plontke, Martin Luther University of Halle-Wittenberg, Germany

Reviewed by: Sung Huhn Kim, Yonsei University, South Korea; Maurizio Barbara, Sapienza University of Rome, Italy

*Correspondence: Konstantina M. Stankovic konstantina_stankovic@ 123456meei.harvard.edu

This article was submitted to Neuro-Otology, a section of the journal Frontiers in Neurology

†These authors have contributed equally to this work

Article

DOI: 10.3389/fneur.2020.00796

PMC ID: 7461819

PubMed ID: 33013614

SO-VID: 1063abc5-36c0-4a24-8863-a5848060fba0

License:

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

History

Date received : 08 April 2020

Date accepted : 25 June 2020

Page count

Figures: 3, Tables: 4, Equations: 0, References: 43, Pages: 13, Words: 8470

Funding

Funded by: National Institute on Deafness and Other Communication Disorders 10.13039/100000055

Funded by: Nancy Sayles Day Foundation 10.13039/100010240

Funded by: Lauer Tinnitus Research Center, Massachusetts Eye and Ear 10.13039/100010572

Progression of Contralateral Hearing Loss in Patients With Sporadic Vestibular Schwannoma

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Hearing Loss and Restoration

Most cited references 41

NLRP3mutation and cochlear autoinflammation cause syndromic and nonsyndromic hearing loss DFNA34 responsive to anakinra therapy

Management of 1000 vestibular schwannomas (acoustic neuromas): clinical presentation.

Descriptive epidemiology of vestibular schwannomas.

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