Hearing Changes after Diagnosis in Neurofibromatosis Type 2

Masuda, Ann; Fisher, Laurel M.; Oppenheimer, Mark L.; Iqbal, Zarina; Slattery, William H.

doi:10.1097/00129492-200403000-00012

Cited by 56 publications

(48 citation statements)

References 13 publications

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“…However, the study also likely reflects the same bias, because vestibular schwannomas are diagnosed earlier in NF2 patients than in sporadic patients with screening examinations, and an apparent protective effect of NF2 may be observed. 22,23 An audiometric follow-up study in a large group of NF2 patients demonstrated that hearing function deteriorated after 2 years of observation, and that initial normal mean PTA value increased to over 30 decibels after 2 years (this PTA value corresponds to G-R grade 2 or higher). 23 In the current study, the follow-up periods of the tumors with preserved G-R grade 1 hearing were all >2 years (median, 44 months; range, 26-73 months).…”

Section: Discussionmentioning

confidence: 99%

“…22,23 An audiometric follow-up study in a large group of NF2 patients demonstrated that hearing function deteriorated after 2 years of observation, and that initial normal mean PTA value increased to over 30 decibels after 2 years (this PTA value corresponds to G-R grade 2 or higher). 23 In the current study, the follow-up periods of the tumors with preserved G-R grade 1 hearing were all >2 years (median, 44 months; range, 26-73 months). This fact indirectly supports that the results were not severely biased.…”

Section: Discussionmentioning

confidence: 99%

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Radiosurgical treatment of vestibular schwannomas in patients with neurofibromatosis type 2

Phi¹,

Kim²,

Chung³

et al. 2009

Cancer

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BACKGROUND:The radiosurgical treatment of vestibular schwannomas in patients with neurofibromatosis type 2 (NF2) is controversial. The authors investigated the radiologically proven tumor control rate after gamma knife radiosurgery. The factors that affect tumor control and serviceable hearing preservation were analyzed.METHODS:Thirty‐six lesions in 30 patients were included. The median lengths of the clinical and radiologic follow‐ups were 48.5 months and 36.5 months, respectively. The median tumor volume was 3.2 cm3. The mean marginal dose was 12.1 grays (Gy) (range, 8–14 Gy) at an isodose line of 50%±0.6%. The Kaplan‐Meier method and Cox proportional hazards model were used for the statistical analyses.RESULTS:The actuarial tumor control rate was 81%, 74%, and 66%, respectively, in the first, second, and fifth years. Five tumors required a salvage surgery because of tumor control failure. A low marginal dose and a young age at radiosurgery were associated with poor tumor control. Of the 16 tumors with which ipsilateral hearing was serviceable, the actuarial serviceable hearing preservation rates were 50%, 45%, and 33%, respectively, in the first, second, and fifth years. Better ipsilateral hearing (Gardner‐Robertson grade 1, compared with grade 2) at the time of radiosurgery was associated with significantly greater serviceable hearing preservation.CONCLUSIONS:Gamma knife radiosurgery for vestibular schwannomas in NF2 patients provided 5‐year tumor control in approximately two‐thirds of patients and preserved serviceable hearing in approximately one‐third. The rates of other cranial nerve deficits were low, and no secondary malignancy was observed. Radiosurgery should be included in treatment options for NF2 patients. Cancer 2009. © 2009 American Cancer Society.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Radiosurgical treatment of vestibular schwannomas in patients with neurofibromatosis type 2

Phi¹,

Kim²,

Chung³

et al. 2009

Cancer

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“…Histogram of muscle fiber area distribution comparing nontumor bearing mice (black, n = 1,088 muscle fibers) with tumor-bearing control mice (green, n = 1,289 muscle fibers) and B20-treated mice (pink; n = 930 muscle fibers) (C ); and nontumor bearing mice (black) with radiation (blue; n = 1,712 muscle fibers) and combination therapy treated mice (orange; n = 1,249 muscle fibers) (D). causing neurological dysfunction (1,21,22) and any treatment that could relieve these symptoms is highly desirable. Radiation therapy can further cause hearing loss by inducing local damage to mature nerve tissue, which is partly attributable to microvascular injury (23,24).…”

Section: Fig 2 Anti-vegf Treatment Decreases Muscle Atrophy (A) Rementioning

confidence: 99%

Anti-VEGF treatment improves neurological function and augments radiation response in NF2 schwannoma model

Gao

Zhao

Stemmer‐Rachamimov

et al. 2015

Proc. Natl. Acad. Sci. U.S.A.

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Hearing loss is the main limitation of radiation therapy for vestibular schwannoma (VS), and identifying treatment options that minimize hearing loss are urgently needed. Treatment with bevacizumab is associated with tumor control and hearing improvement in neurofibromatosis type 2 (NF2) patients; however, its effect is not durable and its mechanism of action on nerve function is unknown. We modeled the effect anti-VEGF therapy on neurological function in the sciatic nerve model and found that it improves neurological function by alleviating tumor edema, which may further improve results by decreasing muscle atrophy and increasing nerve regeneration. Using a cranial window model, we showed that anti-VEGF treatment may achieve these effects via normalizing the tumor vasculature, improving vessel perfusion, and delivery of oxygenation. It is known that oxygen is a potent radiosensitizer; therefore, we further demonstrated that combining anti-VEGF with radiation therapy can achieve a better tumor control and help lower the radiation dose and, thus, minimize radiation-related neurological toxicity. Our results provide compelling rationale for testing combined therapy in human VS.NF2 schwannoma model | anti-VEGF | neurological function | radiation

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“…3 In NF2 patients with progressive vestibular schwannomas, the median times to hearing loss and volumetric tumor progression are 9 and 9.2 months, respectively, after treatment with erlotinib. 4 Effective treatments are urgently needed for NF2 patients with progressive hearing loss, because hearing loss is associated with impairment in social, emotional, and communication function, and increased depression.…”

Section: Introductionmentioning

confidence: 98%

Bevacizumab for Progressive Vestibular Schwannoma in Neurofibromatosis Type 2

Plotkin¹,

Merker²,

Halpin³

et al. 2012

Otology &Amp; Neurotology

212

150

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Objective: Early studies suggest that bevacizumab treatment can result in tumor shrinkage and hearing improvement for some patients with neurofibromatosis 2 (NF2). The aim of this study was to report extended follow-up in a larger cohort of similarly treated patients.Study Design: Retrospective study. Setting: Tertiary referral centerPatients: 31 consecutive NF2 patients who received bevacizumab for progressive vestibular schwannomas.Main Outcome Measure: Hearing improvement, defined as an improvement in word recognition score above the 95% critical difference compared with baseline, and radiographic response, defined as a ≥20% decrease in tumor volume compared with baseline. Results:The median age was 26 years (range, 12-73). The median volumetric tumor growth rate before treatment was 64% per year. At the time of analysis, the median duration of treatment was 14 months (range, 6-41) with a total of 45 patient-years of follow up. A hearing response occurred in 13/23 (57%) evaluable patients, and a radiographic response in 17/31 (55%) of target vestibular schwannomas. The median time to response was 3 months for both endpoints. The only clinical or radiographic feature at baseline that correlated with change in tumor volume at 3 months was the mean apparent diffusion coefficient (ADC) value, a radiologic marker of edema (p=0.036). 90% of patients had stable or improved hearing after 1 year of treatment and 61% at 3 years. 88% of patients had stable or decreased tumor size after 1 year of treatment and 54% at 3 years. Overall, treatment was well tolerated.Conclusion: Bevacizumab treatment was followed by hearing improvement and tumor shrinkage in over 50% of progressive vestibular schwannomas in NF2 patients. Stable or 2 improved hearing was retained in the majority of patients.3

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Hearing Changes after Diagnosis in Neurofibromatosis Type 2

Abstract: Newly diagnosed neurofibromatosis Type 2 patients who do not require immediate treatment of both vestibular schwannomas are likely to have stable hearing in the unoperated ear(s) for approximately 1 to 2 years.

Cited by 56 publications

References 13 publications

Radiosurgical treatment of vestibular schwannomas in patients with neurofibromatosis type 2

Radiosurgical treatment of vestibular schwannomas in patients with neurofibromatosis type 2

Anti-VEGF treatment improves neurological function and augments radiation response in NF2 schwannoma model

Bevacizumab for Progressive Vestibular Schwannoma in Neurofibromatosis Type 2

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