{"title":"Clinical evaluation and investigation of vestibular schwannoma in NF2-related schwannomatosis.","authors":"Rupert Obholzer","doi":"10.1016/B978-0-12-824534-7.00029-9","DOIUrl":null,"url":null,"abstract":"<p><p>The management of vestibular schwannoma (VS) in NF2-related schwannomatosis (NF2-SWN) is more complicated than that for sporadic VS because of the almost invariable presence of bilateral VS, as well as the presence of other types of schwannomas in the cerebellopontine angle, elsewhere intracranially, and within the spine. This requires a much more holistic approach to management. The key goal of treatment is to preserve function and quality of life. This can be very challenging given the bilateral nature of the disease in most patients and means that there is likely to be greater accumulated morbidity with treatment over time than for sporadic VS. It is, therefore, critical to assess all aspects related to bilateral cranial nerve function, particularly focusing on audiovestibular function. Hearing assessment using pure-tone audiometry, speech testing, and occasionally electrophysiologic assessment is critical to quantify the degree of hearing loss and to help make decisions around optimal hearing rehabilitation. Vestibular assessment is often helpful in determining the source of any balance disturbance and aids in tailoring vestibular rehabilitation where required. Prospective psychologic and quality of life assessment are very important in order to direct decision-making and ensure that the patient's quality of life is optimized. VS and other tumors are monitored using serial magnetic resonance imaging (MRI), usually on an annual basis. MRI may present particular challenges in NF2-SWN, given that a significant proportion of patients will have auditory implants, which cause significant signal distortion and have issues around magnet displacement. However, there are a number of strategies to limit the implications of scanning in the presence of auditory implants.</p>","PeriodicalId":12907,"journal":{"name":"Handbook of clinical neurology","volume":"212 ","pages":"93-96"},"PeriodicalIF":0.0000,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Handbook of clinical neurology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1016/B978-0-12-824534-7.00029-9","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"Medicine","Score":null,"Total":0}
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Abstract
The management of vestibular schwannoma (VS) in NF2-related schwannomatosis (NF2-SWN) is more complicated than that for sporadic VS because of the almost invariable presence of bilateral VS, as well as the presence of other types of schwannomas in the cerebellopontine angle, elsewhere intracranially, and within the spine. This requires a much more holistic approach to management. The key goal of treatment is to preserve function and quality of life. This can be very challenging given the bilateral nature of the disease in most patients and means that there is likely to be greater accumulated morbidity with treatment over time than for sporadic VS. It is, therefore, critical to assess all aspects related to bilateral cranial nerve function, particularly focusing on audiovestibular function. Hearing assessment using pure-tone audiometry, speech testing, and occasionally electrophysiologic assessment is critical to quantify the degree of hearing loss and to help make decisions around optimal hearing rehabilitation. Vestibular assessment is often helpful in determining the source of any balance disturbance and aids in tailoring vestibular rehabilitation where required. Prospective psychologic and quality of life assessment are very important in order to direct decision-making and ensure that the patient's quality of life is optimized. VS and other tumors are monitored using serial magnetic resonance imaging (MRI), usually on an annual basis. MRI may present particular challenges in NF2-SWN, given that a significant proportion of patients will have auditory implants, which cause significant signal distortion and have issues around magnet displacement. However, there are a number of strategies to limit the implications of scanning in the presence of auditory implants.
期刊介绍：
The Handbook of Clinical Neurology (HCN) was originally conceived and edited by Pierre Vinken and George Bruyn as a prestigious, multivolume reference work that would cover all the disorders encountered by clinicians and researchers engaged in neurology and allied fields. The first series of the Handbook (Volumes 1-44) was published between 1968 and 1982 and was followed by a second series (Volumes 45-78), guided by the same editors, which concluded in 2002. By that time, the Handbook had come to represent one of the largest scientific works ever published. In 2002, Professors Michael J. Aminoff, François Boller, and Dick F. Swaab took on the responsibility of supervising the third (current) series, the first volumes of which published in 2003. They have designed this series to encompass both clinical neurology and also the basic and clinical neurosciences that are its underpinning. Given the enormity and complexity of the accumulating literature, it is almost impossible to keep abreast of developments in the field, thus providing the raison d''être for the series. The series will thus appeal to clinicians and investigators alike, providing to each an added dimension. Now, more than 140 volumes after it began, the Handbook of Clinical Neurology series has an unparalleled reputation for providing the latest information on fundamental research on the operation of the nervous system in health and disease, comprehensive clinical information on neurological and related disorders, and up-to-date treatment protocols.
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