Hearing Preservation With the Middle Cranial Fossa Approach for Neurofibromatosis Type 2

Objective Bevacizumab for hearing preservation in patients with neurofibromatosis type 2 (NF2) is an emerging practice. We set out to characterize the effectiveness and toxicity of bevacizumab in our patient group. Study Design Case series with chart review. Setting Tertiary referral center. Subjects and Methods Seventeen consecutive patients with NF2 received bevacizumab treatment for vestibular schwannomas, including 2 patients treated to maintain cochlear implant performance. Volumetric analysis of serial magnetic resonance imaging scans was used to evaluate radiographic response, and hearing response was evaluated with serial audiograms. Patient-reported outcomes were also assessed, including subjective hearing improvement, changes in tinnitus, vertigo, headaches, ear pain, and improvement in ability to communicate via telephone. Results A positive radiographic response occurred in 8 of 17 (47%) patients and the median tumor volume change was a tumor decrease of 19%. A positive hearing response was recorded in 5 of 9 (56%) patients. Two patients had a word recognition score improvement over 40%. There was an approximately 40% improvement in patient-reported outcomes. Primary toxicities included hypertension, proteinuria, dysgeusia, and amenorrhea. Conclusion Bevacizumab treatment was followed by hearing improvement in 56% of patients, while decreased tumor volume was noted in 47%. These outcomes agree favorably with prior reported series. There were significant improvements in patient-reported outcomes that have not been described previously.

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Hearing Preservation and Facial Nerve Outcomes in Vestibular Schwannoma Surgery: Results Using the Middle Cranial Fossa Approach

Otology & Neurotology ◽

10.1097/01.mao.0000185153.54457.16 ◽

2006 ◽

Vol 27 (2) ◽

pp. 234-241 ◽

Cited By ~ 75

Author(s):

H Alexander Arts ◽

Steven A Telian ◽

Hussam El-Kashlan ◽

B Gregory Thompson

Keyword(s):

Facial Nerve ◽

Vestibular Schwannoma ◽

Hearing Preservation ◽

Middle Cranial Fossa ◽

Middle Cranial Fossa Approach ◽

Cranial Fossa

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Tumor control and hearing preservation after Gamma Knife radiosurgery for vestibular schwannomas in neurofibromatosis type 2

Journal of Neuro-Oncology ◽

10.1007/s11060-010-0181-1 ◽

2010 ◽

Vol 98 (2) ◽

pp. 265-270 ◽

Cited By ~ 21

Author(s):

Manish Singh Sharma ◽

Rakesh Singh ◽

Shashank S. Kale ◽

Deepak Agrawal ◽

Bhawani Shankar Sharma ◽

...

Keyword(s):

Gamma Knife ◽

Gamma Knife Radiosurgery ◽

Neurofibromatosis Type ◽

Hearing Preservation ◽

Neurofibromatosis Type 2 ◽

Vestibular Schwannomas ◽

Tumor Control

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STEREOTACTIC RADIOSURGERY FOR VESTIBULAR SCHWANNOMAS IN PATIENTS WITH NEUROFIBROMATOSIS TYPE 2

Neurosurgery ◽

10.1227/01.neu.0000255340.26027.53 ◽

2007 ◽

Vol 60 (3) ◽

pp. 460-470 ◽

Cited By ~ 102

Author(s):

David Mathieu ◽

Douglas Kondziolka ◽

John C. Flickinger ◽

Ajay Niranjan ◽

Richard Williamson ◽

...

Keyword(s):

Stereotactic Radiosurgery ◽

Tumor Volume ◽

Gamma Knife Radiosurgery ◽

Neurofibromatosis Type ◽

Hearing Preservation ◽

Neurofibromatosis Type 2 ◽

Vestibular Schwannomas ◽

Factors Affecting

Abstract OBJECTIVE Vestibular schwannomas present significant management challenges in patients with neurofibromatosis Type 2 (NF2). We evaluated the results of gamma knife radiosurgery for the management of these tumors, focusing on tumor response, hearing preservation, and other factors affecting outcomes. METHODS Stereotactic radiosurgery was performed to manage 74 schwannomas in 62 patients. Ipsilateral serviceable hearing was present in 35% of tumors before the procedure. The mean tumor volume was 5.7 cm3. The mean margin and maximum dose used were 14 and 27.5 Gy, respectively. Cox regression analyses were performed to identify factors affecting outcomes. RESULTS The median follow-up period was 53 months, and two patients were lost to follow-up. Actuarial local control rates at were 85, 81, and 81% at 5, 10, and 15 years, respectively. Tumor volume was significant as a predictor of local control. Since 1992, using current radiosurgery techniques (magnetic resonance imaging scan targeting and reduced margin dose to 14 Gy or less), the actuarial serviceable hearing preservation rate is 73% at 1 year, 59% at 2 years, and 48% at 5 years after radiosurgery. Facial neuropathy occurred in 8% of tumors, trigeminal neuropathy occurred in 4%, and vestibular dysfunction occurred in 4%. Radiation dose and tumor volume were predictive of development of new deficits. No radiosurgery-associated secondary tumors or atypical or malignant changes were noted. CONCLUSION Stereotactic radiosurgery is a safe and effective management modality for neurofibromatosis Type 2 vestibular schwannomas. Although results do not seem to be as good as for patients with sporadic unilateral tumors, gamma knife radiosurgery results seem favorable and indicate that radiosurgery should be strongly considered for primary tumor management in selected patients.

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Early Proactive Management of Vestibular Schwannomas in Neurofibromatosis Type 2

Neurosurgery ◽

10.1097/00006123-200108000-00007 ◽

2001 ◽

Vol 49 (2) ◽

pp. 274-283 ◽

Cited By ~ 8

Author(s):

Derald E. Brackmann ◽

Jose N. Fayad ◽

William H. Slattery ◽

Rick A. Friedman ◽

John Diaz Day ◽

...

Keyword(s):

Facial Nerve ◽

Neurofibromatosis Type ◽

Hearing Preservation ◽

Neck Surgery ◽

Neurofibromatosis Type 2 ◽

Nerve Function ◽

Speech Discrimination ◽

Facial Nerve Function ◽

Proactive Management

Abstract OBJECTIVE The treatment of patients with neurofibromatosis Type 2 has always been challenging for neurosurgeons and neurotologists. Guidelines for appropriate management of this devastating disease are controversial. METHODS A retrospective study of 28 patients with neurofibromatosis Type 2 who underwent 40 middle fossa craniotomies for excision of their acoustic tumors is reported. Eleven patients underwent bilateral procedures. The study focused on hearing preservation and facial nerve results for this group of patients. The 16 male patients and 12 female patients ranged in age (at the time of surgery) from 10 to 70 years, with a mean age of 22.6 years. The mean tumor size was 1.1 cm (range, 0.5–3.2 cm), and the majority of tumors were less than 1.5 cm. RESULTS Measurable hearing was preserved in 28 ears (70%), with 42.5% being within 15 dB pure-tone average and 15% speech discrimination score of preoperative levels. In 55% of cases there was no change in the hearing class, as defined by the American Academy of Otolaryngology-Head and Neck Surgery. Of the 11 patients who underwent bilateral operations, 9 (82%) retained some hearing bilaterally. After 1-year follow-up periods (mean, 12.8 mo), 87.5% of patients exhibited normal facial nerve function (House-Brackmann Grade I). CONCLUSION Early surgical intervention to treat acoustic tumors among patients with neurofibromatosis Type 2 is a feasible treatment strategy, with high rates of hearing and facial nerve function preservation.

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Stereotactic Radiosurgery for Vestibular Schwannomas in Patients with Neurofibromatosis Type 2: An Analysis of Tumor Control, Complications, and Hearing Preservation Rates

Yearbook of Otolaryngology-Head and Neck Surgery ◽

10.1016/s1041-892x(08)79191-1 ◽

2008 ◽

Vol 2008 ◽

pp. 17-18 ◽

Cited By ~ 1

Author(s):

B.J. Balough

Keyword(s):

Stereotactic Radiosurgery ◽

Neurofibromatosis Type ◽

Hearing Preservation ◽

Neurofibromatosis Type 2 ◽

Vestibular Schwannomas ◽

Tumor Control

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Impact of Surgery on Long-Term Results of Hearing in Neurofibromatosis Type-2 Associated Vestibular Schwannomas

Cancers ◽

10.3390/cancers11091376 ◽

2019 ◽

Vol 11 (9) ◽

pp. 1376 ◽

Cited By ~ 7

Author(s):

Gugel ◽

Grimm ◽

Liebsch ◽

Zipfel ◽

Teuber ◽

...

Keyword(s):

Neurofibromatosis Type ◽

Hearing Preservation ◽

Neurofibromatosis Type 2 ◽

Auditory Evoked Potential ◽

Long Term Results ◽

Data Sets ◽

Guided Surgery

Hearing preservation is a major goal in the treatment of neurofibromatosis type 2 (NF2) associated vestibular schwannoma (VS), particularly in children and adolescents. In this study, we retrospectively reviewed hearing and volumetry data sets of 39 operated tumors (ears) in 23 patients under the age of 25 and in a follow-up period of 21 to 167 months. Hearing data over a compatible period on 20 other tumors, which did not receive surgery due to their less aggressive nature, were included for comparison. Surgery was carried out via a retrosigmoid approach with the brainstem auditory evoked potential (BAEP) guide. Immediately after surgery, functional hearing was maintained in 82% of ears. Average hearing scores were better in the non-surgery ears. However, the hearing scores in both groups worsened gradually with a similar dynamic during the 42-month postoperative follow-up period. No accelerated impairment of hearing was evident for the operated cases. Rather, the gap between the two hearing deterioration lines tended to close at the end of the follow-up period. Our result suggested that the BAEP-guided surgery did not cause additional hearing deterioration in the long-term and seemed to slow down hearing deterioration of those tumors that were initially more aggressive.

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