Conservative Management of Bilateral Vestibular Schwannomas in Neurofibromatosis Type 2 Patients

Peyre, Matthieu; Goutagny, Stéphane; Bah, Abdellah; Bernardeschi, Danièle; Larroque, Béatrice; Sterkers, Olivier; Kalamaridès, Michel

doi:10.1227/neu.0b013e31828bae28

Cited by 43 publications

(34 citation statements)

References 24 publications

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“…For trials focused on prevention of hearing decline, we found that patients in our cohort with medium/large tumors (≥ 1 ml) at study enrollment had a shorter median time to hearing decline (38 months) than small tumors (median not reached), and thus represent a more feasible group to study in clinical trials. While rate of tumor growth is not correlated to hearing preservation, 11 our results showed a correlation between larger baseline tumor size and subsequent hearing loss. For this reason, we propose that NF2 patients with VS ≥ 1 ml be stratified as "high risk" for subsequent hearing decline, and that consideration be given to making these patients eligible for trials without the need for documented tumor progression at study enrollment.…”

Section: Trial Design: Time To Hearing Decline or Tumor Progression Acontrasting

confidence: 65%

“…Several single-institution clinical series have been reported from NF2 centers, but these have typically been small and retrospective. [7][8][9][10][11] In addition, many of these studies have reported results primarily as the average growth rates or hearing decline among all subjects. This measure is less useful in designing clinical trials for drug treatment of NF2-related VS. For this purpose, an understanding of individual times to failure for measures such as tumor growth or hearing decline in untreated patients is required.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

Plotkin¹,

Merker²,

Muzikansky³

et al. 2014

Otology &Amp; Neurotology

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Section: Trial Design: Time To Hearing Decline or Tumor Progression Acontrasting

confidence: 65%

Section: Introductionmentioning

confidence: 99%

Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

Plotkin¹,

Merker²,

Muzikansky³

et al. 2014

Otology &Amp; Neurotology

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“…[24][25][26] In large NF2-associated VS natural history studies, the average growth in greatest diameter per year was reported to be between 1.3 and 1.8 mm, corresponding to a 10%-14% increase from the baseline averages of 12.4 and 13.0 mm. 27,28 Another study evaluated the feasibility of volumetric assessment of VS and demonstrated increased sensitivity in detecting disease progression and reduced intrarater measurement variability compared to linear measurements.…”

mentioning

confidence: 99%

Recommendations for imaging tumor response in neurofibromatosis clinical trials

et al. 2013

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Methods: Currently used imaging endpoints, designs of NF clinical trials, and knowledge of the natural history of NF-related tumors, in particular PN and VS, were reviewed. Consensus recommendations for response evaluation for future studies were developed based on this review and the expertise of group members.Results: MRI with volumetric analysis is recommended to sensitively and reproducibly evaluate changes in tumor size in clinical trials. Volumetric analysis requires adherence to specific imaging recommendations. A 20% volume change was chosen to indicate a decrease or increase in tumor size. Use of these criteria in future trials will enable meaningful comparison of results across studies. Conclusions:The proposed imaging response evaluation guidelines, along with validated clinical outcome measures, will maximize the ability to identify potentially active agents for patients with NF and benign tumors. 1,2 Linear measurements are performed for their ease of use and are suitable for most malignant lesions that rapidly change in size. Disease-specific recommendations have been developed for some diseases in which linear measurements are not practical or meaningful, such as the Response Assessment in Neuro-Oncology (RANO) criteria for brain tumors.3,4 In addition, an international effort is under way to develop measurement guidelines for pediatric brain tumors, which will be applicable to neurofibromatosis type 1 (NF1)-related gliomas.

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“…These suggest that hearing deteriorates by approximately 3 dB/year. Studies investigating NF2 patients are fewer in number but suggest the rate of hearing loss is similar with an annual change in pure tone average of around 4 dB and an annual change in speech discrimination scores of around 2 % [6,9,[22][23][24]. Only a recent study by Peyre et al [23] present preservation of hearing serviceability data and they state a figure of 74 % over a 72-month period on average.…”

Section: Discussionmentioning

confidence: 99%

Progress of hearing loss in neurofibromatosis type 2: implications for future management

Kontorinis

Nichani²,

Freeman³

et al. 2014

Eur Arch Otorhinolaryngol

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The objective of this study was to describe changes in hearing over time in patients with neurofibromatosis type 2 (NF2) treated conservatively. A retrospective case review was conducted in a tertiary referral centre. Pure tone audiometry, speech discrimination scores, serviceable hearing (American Academy of Otolaryngology class A or B) and measurement of vestibular schwannoma (VS) size on magnetic resonance imaging were evaluated in 56 patients (89 ears) with NF2 with at least one conservatively managed VS. Over a mean follow-up period of 7 years (range 0.8-21 years) pure tone average thresholds increased gradually with a mean annual rate of 1.3 dB for the right ear (p = 0.0003) and 2 dB for the left ear (p = 0.0009). Speech discrimination scores dropped with an average annual rate of 1.3 and 0.34% in the right and left ear, respectively. Patients maintained serviceable hearing for an average of 7.6 years (range 2.7-19.3 years). The average annual VS growth was 0.4 mm without any correlation with hearing loss. There was a correlation between patients' age and pure tone threshold increase (p < 0.05 for both ears). In this selected population of patients with NF2, hearing threshold increases were very slow. In NF2 patients with indolently behaving tumours, serviceable hearing can be maintained for a significant length of time, making conservative management an attractive option.

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Conservative Management of Bilateral Vestibular Schwannomas in Neurofibromatosis Type 2 Patients

Abstract: : AAO-HNS, American Academy of Otololaryngologists and Head and Neck Surgery classificationMTD, mean tumor diameterNF2, neurofibromatosis type 2PTA, pure-tone averageSDS, speech discrimination scoreVS, vestibular schwannomas.

Cited by 43 publications

References 24 publications

Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

Recommendations for imaging tumor response in neurofibromatosis clinical trials

Progress of hearing loss in neurofibromatosis type 2: implications for future management

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