Guideline sources The following summarized guidelines for the evaluation and management of vestibular schwannoma are prepared by our editorial team based on guidelines from the American Physical Therapy Association (APTA 2022), the American Cochlear Implant Alliance (ACI Alliance 2022), and the Congress of Neurological Surgeons (CNS 2018). 1 2 3 4 5 6 7 Guidelines 1. Diagnostic investigations Magnetic resonance imaging: obtain high-resolution T2-weighted and contrast-enhanced T1- weighted MRI to detect vestibular schwannomas. A Show 4 more 2. Medical management Expectant management: https://web.pathway.md/diseases/recejfvdCpAJTdRan 1/5 6/29/23, 3:43 AM Vestibular schwannoma Pathway Consider offering expectant management with observation in patients with intracanalicular vestibular schwannomas and small tumors (< 2 cm) if tinnitus is not observed at presentation. B Obtain MRI annually for 5 years, with interval lengthening thereafter with tumor stability. A Consider obtaining more frequent imaging for vestibular schwannomas associated with neurofibromatosis type 2 because of a more variable growth rate for vestibular schwannomas, with annual imaging once the growth rate is established. B Bevacizumab: administer bevacizumab to radiographically reduce the size or prolong tumor stability and improve hearing or prolong time to hearing loss in patients with neurofibromatosis type 2 without surgical options. A Lapatinib: consider administering lapatinib to reduce tumor size and improve hearing in patients with neurofibromatosis type 2. B Aspirin: consider initiating aspirin in patients with vestibular schwannomas undergoing expectant management with observation. B Other agents: do not use erlotinib or everolimus for reducing tumor size or improving hearing in patients with neurofibromatosis type 2. D 3. Nonpharmacologic interventions Vestibular rehabilitation: offer vestibular physical therapy in patients with acute, subacute, or chronic unilateral vestibular hypofunction. A Show 9 more 4. Therapeutic procedures Stereotactic radiosurgery: perform radiosurgery as a treatment option in patients with neurofibromatosis type 2 with enlarging tumor and/or causing hearing loss. A Show 2 more Cochlear implants: discuss and offer a trial of a non-surgical hearing technology where possible before undergoing cochlear implantation in patients with single-sided deafness. E Show 10 more 5. Perioperative care Preoperative vestibular rehabilitation: offer preoperative vestibular rehabilitation to aid in postoperative mobility after vestibular schwannoma surgery. A Perioperative nimodipine: consider administering perioperative nimodipine (with or without hydroxyethyl starch) to improve postoperative facial nerve outcomes and hearing outcomes in patients with vestibular schwannomas. B https://web.pathway.md/diseases/recejfvdCpAJTdRan 2/5 6/29/23, 3:43 AM Vestibular schwannoma Pathway Preoperative gentamicin ablation: consider performing preoperative gentamicin ablation of the vestibular apparatus to improve postoperative mobility after vestibular schwannoma surgery. B 6. Surgical interventions Microsurgical resection: Consider performing hearing preservation surgery via the middle fossa or the retrosigmoid approach in patients with small tumor size (< 1.5 cm) and good preoperative hearing. B Consider performing surgical resection rather than stereotactic radiosurgery for better control of trigeminal neuralgia symptoms. B Consider using endoscopic assistance to aid in visualization. B 7. Patient education General counseling: counsel adult patients with cystic vestibular schwannomas that this type of tumor may be associated with rapid growth, lower rates of complete resection, and facial nerve outcomes that may be inferior in the immediate postoperative period, but similar to noncystic schwannomas over time. A Counseling before expectant management: counsel patients with vestibular schwannomas planned to undergo expectant management that the overall probability of hearing preservation after diagnosis is high (> 75-100%) at 2, moderately high (> 50-75%) at 5 years, and moderately low (> 25%-50%) at 10 years. A Show 2 more Counseling before stereotactic radiosurgery: Inform patients about the minimal risk of malignant transformation of vestibular schwannomas after stereotactic radiosurgery. A Counsel patients with vestibular schwannomas planned to undergo stereotactic radiosurgery that the overall probability of hearing preservation after surgery is moderately high (> 50- 75%) immediately after surgery, at 2 and 5 years, and moderately low (> 25%-50%) at 10 years. A Show 2 more Counseled patients requiring microsurgical resection after stereotactic radiosurgery regarding the increased likelihood of subtotal resection and decreased facial nerve function. A Counseling before microsurgery: Counsel about the greater than average risk of loss of serviceable hearing with surgical resection in patients with larger vestibular schwannoma tumor size. A Counsel patients with small-to-medium-sized sporadic vestibular schwannomas planned to undergo microsurgical resection that the overall probability of hearing preservation after surgery is moderately low (> 25-50%) immediately after surgery, at 2, 5, and 10 years. A Show 2 more https://web.pathway.md/diseases/recejfvdCpAJTdRan 3/5 6/29/23, 3:43 AM Vestibular schwannoma Pathway 8. Follow-up and surveillance Surveillance imaging (post-operative): obtain a postoperative postcontrast 3D T1 magnetization prepared rapid acquisition gradient echo, with nodular enhancement considered suspicious for recurrence. B Show 2 more Surveillance imaging (post-radiosurgery): obtain follow-up imaging at intervals after stereotactic radiosurgery based on clinical indications, a patient's personal circumstances, or institutional protocols. Obtain long-term follow-up with serial MRI to evaluate for recurrence. A Clinical findings Patient demographics Symptoms Age 50 Asymmetric hearing loss Dizziness Past medical history Facial hypoesthesia Facial numbness Neurofibromatosis type 2 Feeling of fullness in the ear Diagnostic maneuvers Headache Hearing loss Unterberger's test Loss of balance Tinnitus Diagnostic procedures Neurological exam Abnormal auditory brainstem response Bruns nystagmus Facial nerve palsy Imaging findings Cerebellopontine angle mass Internal auditory canal mass References 1. Jamie J Van Gompel, Siviero Agazzi, Matthew L Carlson et al. Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on Emerging Therapies for the Treatment of Patients With Vestibular Schwannomas. Neurosurgery. 2018 Feb 1;82 2 E52 E54. Open 2. Dunn IF, Bi WL, Mukundan S et al. Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on the Role of Imaging in the Diagnosis and Management of Patients With Vestibular Schwannomas. Neurosurgery. 2018 Feb 1;82 2 E32 E34. Open 3. Constantinos G Hadjipanayis, Matthew L Carlson, Michael J Link et al. Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on Surgical Resection for the Treatment of Patients With Vestibular Schwannomas. Neurosurgery. 2018 Feb 1;82 2 E40 E43. Open https://web.pathway.md/diseases/recejfvdCpAJTdRan 4/5 6/29/23, 3:43 AM Vestibular schwannoma Pathway 4. Isabelle M Germano, Jason Sheehan, Johnathan Parish et al. Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on the Role of Radiosurgery and Radiation Therapy in the Management of Patients With Vestibular Schwannomas. Neurosurgery. 2018 Feb 1;82 2 E49 E51. Open 5. Matthew L Carlson, Esther X Vivas, D Jay McCracken et al. Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on Hearing Preservation Outcomes in Patients With Sporadic Vestibular Schwannomas. Neurosurgery. 2018 Feb 1;82 2 E35 E39. Open 6. Courtney D Hall, Susan J Herdman, Susan L Whitney et al. Vestibular Rehabilitation for Peripheral Vestibular Hypofunction: An Updated Clinical Practice Guideline From the Academy of Neurologic Physical Therapy of the American Physical Therapy Association. J Neurol Phys Ther. 2022 Apr 1;46 2 118 177. Open 7. Lisa R Park, Amanda M Griffin, Douglas P Sladen et al. American Cochlear Implant Alliance Task Force Guidelines for Clinical Assessment and Management of Cochlear Implantation in Children With Single- Sided Deafness. Ear Hear. 2022 Mar/Apr;43 2 255 267. Open 8. Lustig LR, Rifkin S, Jackler RK et al. Acoustic neuromas presenting with normal or symmetrical hearing: factors associated with diagnosis and outcome. Am J Otol. 1998 Mar;19 2 212 8. Open 9. Wilson DF, Hodgson RS, Gustafson MF et al. The sensitivity of auditory brainstem response testing in small acoustic neuromas. Laryngoscope. 1992 Sep;102 9 961 4. Open https://web.pathway.md/diseases/recejfvdCpAJTdRan 5/5