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OBJECTIVE To determine whether cervical vestibular evoked myogenic potentials (cVEMPs) are predictive of hearing preservation in patients undergoing vestibular schwannoma removal through middle fossa craniotomy approach. STUDY DESIGN Retrospective case study. SETTING Tertiary referral center. PATIENTS Eighteen patients who underwent a middle fossa craniotomy for vestibular schwannoma (stage I or II of Koos classification) with attempted hearing preservation from January 2008 to February 2016 were retrospectively reviewed. INTERVENTION Pre-surgical cVEMPs test, videonystagmography (caloric test), and magnetic resonance imaging (MRI) as well as a pre- and post-surgical audiometry test. MAIN OUTCOME MEASURES cVEMPs parameters including amplitude asymmetry ratio (AR), P13, and N23 latencies and peak-to-peak amplitude between P13 and N23 waves were calculated. Hearing data were classified according to the AAO-HNS hearing classes. The nerve of origin of the tumor was specified during surgery and the largest tumor diameter was measure on MRI axial plane on T2-CISS weighed images. RESULTS Preoperative amplitude asymmetry ratio was lower (n = 15, Mann-Whitney U test, p

Neurofibromatosis 2 (NF2) is a genetically inherited tumor predisposition syndrome. It predisposes to the development of multiple tumors of the central nervous system including schwannomas, meningiomas and ependymomas. Bilateral vestibular schwannomas (VS) are pathognomonic for the disease. In childhood, non-auditory symptoms often mark the onset of the NF2, such as facial palsy, peripheral neuropathy, and neurosurgical emergencies. In this article, we describe the case of a 6-year-old child presenting with an ischemic brain-stem stroke, who was later diagnosed with NF2. We report the clinical and genetic findings and review the previous literature on vasculopathy reported in association with NF2, with a focus on the symptoms presenting at disease onset. For our case patient, an unpublished germline mutation resulting in frameshift (c.876_877insT) was identified on blood screening. We report our full multidisciplinary assessment with vascular angiography, volumetric MRI and audiometry. Vasculopathy is not currently included in the criteria traditionally used for diagnosis of NF2. We suggest that vascular stroke in childhood may be considered a presenting symptom for NF2.

Objective: Document the use of transmastoid labyrinthectomy to treat disabling vertigo secondary to a lesion in the internal auditory canal. Patient: A 69-year-old man with nonserviceable left hearing experienced disabling attacks of vertigo refractory to medical measures. Magnetic resonance imaging revealed a small left intracanalicular lesion with an irregular configuration and modest enhancement, suggesting either an unusual acoustic neuroma or a cavernoma. Tumor size remained stable on serial imaging, and the patient declined microsurgical resection, stereotactic radiation, or intratympanic gentamicin therapy. Intervention: Transmastoid labyrinthectomy followed by a customized vestibular rehabilitation program. Main Outcome Measure: Comparison of patient symptoms preoperatively and at 5 and 8 months after surgery. Results: Complete relief of vertigo was achieved, but the patient has moderate imbalance postoperatively. Conclusion: Transmastoid labyrinthectomy alone may be a viable treatment option in patients with an internal auditory canal neoplasm causing disabling attacks of vertigo.