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Objectives: Tumors involving the vagus nerve are often clinically silent. We offer a case series with different clinical presentations and distinctive post-surgical sequelae that highlight some of the challenges associated with managing cervical vagal nerve tumors., Methods: Single-institution, retrospective review of patients with tumors involving the vagus nerve. We describe clinical presentations and postoperative sequelae of five patients who underwent surgical management of vagal nerve pathology with atypical presentation or subsequent clinical course., Results: Here, we present five patients treated at our institution for vagal tumors. In four of the five patients, the presenting symptoms resolved after surgery. Two patients presented with intractable neurogenic cough, and another two presented with autonomic symptoms, one with syncope/palpitations and the other with intractable sweating. The final patient presented with a rapidly enlarging vagal paraganglioma and developed intractable cough after resection. We present two patients with novel approach to vagal paragangliomas that underwent ligation of feeding blood supply without removing the tumor, resulting in resolution of an intractable cough in one patient and resolution of severe nighttime sweating in the other., Conclusion: Management of tumors associated with the cervical vagus nerve that present with symptoms or rapid growth poses a clinical dilemma. Consideration of the tumor origin with either enucleation of schwannomas or ligation of feeding vessels may preserve function while addressing the presenting symptoms., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: To characterize facial nerve (FN) schwannomas (FNSs) and FN hemangiomas (FNHs) and their clinical features and management strategies, and to describe the results of cable nerve grafting after FN sectioning during tumor removal., Methods: This retrospective study included 84 FNS cases and 42 FNH cases managed between July 1989 and July 2020 at a quaternary referral center for skull base pathology. Clinical details, locations, management, and results of cable nerve grafting at 1 year and during an average period of 3.12 years were evaluated. Sural nerve interpositioning was performed for patients who experienced FN paralysis for less than 1 year and underwent nerve sectioning during tumor removal., Results: FNSs more often involved multiple segments compared with FNHs. The cerebellopontine angle and the mastoid segments were involved in 16 (19.1%) and 34 (40.5%) FNS cases, respectively; however, the cerebellopontine angle and the mastoid segments were involved in 0 and 7 (16.7%) FNH cases, respectively. Sectioned nerves of 99 patients (78.6%) were restored using interposition cable grafting. At the last follow-up evaluation, 56.3% of FNSs and 60.7% of FNHs attained House-Brackmann (HB) grade III. Lower preoperative HB grades were associated with poorer postoperative outcomes. For FNSs, the mean HB grades were 4.13 at 1 year postoperatively and 3.75 at the last follow-up evaluation ( p = 0.001); however, for FNHs, the mean HB grades were 4.04 postoperatively and 3.75 at the last follow-up evaluation. Therefore, extradural coaptation yielded better outcomes., Conclusion: FNSs can occur along any part of the FN along its course, and FNHs are concentrated around the area of geniculate ganglion. The results of cable inter positioning grafts are better in patients with preoperative FN-HB-III or less when compared with higher grades. The outcome of the interpositioning continues to improve even after 1 year in extradural coaptation., Competing Interests: Conflict of interest: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Purpose of Review: Several neoplastic lesions may originate from facial nerves, including facial nerve schwannomas. These neoplastic lesions can cause various symptoms, such as facial nerve paralysis, and decrease the quality of life of patients. Therefore, knowledge of how to manage these diseases is important for otologists. However, the incidence of these diseases is extremely low, and universal management methods have not yet been established. This review summarizes recent advances in knowledge regarding these neoplastic lesions, especially facial nerve schwannomas., Recent Findings: Recent advances and the accumulation of knowledge regarding these benign facial nerve lesions have provided several preferable treatments and management methods, especially for facial nerve schwannomas. However, this still depends on the patient's symptoms and tumor localization., Summary: This review presents the optimal treatment protocol and differential diagnosis of benign facial nerve lesions. This may be useful for pretreatment differentiation and treatment decision-making., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Foster Kennedy syndrome refers to a finding of optic atrophy in one eye from direct compression of the optic nerve by a mass lesion and contralateral papilledema in the nonatrophic optic nerve caused by an increased intracranial pressure. When the fundoscopy finding is not due to a direct compressive mass, the term pseudo-Foster Kennedy syndrome is used; this can be caused by any process or processes that result in optic atrophy in one eye and optic disc edema in the other. Identifying Foster Kennedy syndrome in a patient calls for expedient neuroimaging looking for an intracranial mass lesion. In this article, we present the case of a patient presenting with vision loss and Foster Kennedy syndrome who was found to have a large trigeminal cystic schwannoma. While several other accompanying symptoms were not evident from the patient complaint, a careful history and physical examination revealed additional localizing clues: unilateral sensory changes in the face and pterygoid and masseter atrophy, unilateral cranial nerve VI palsy, and episodes of intense déjà vu sensation, which were presumed to represent temporal lobe-onset focal aware seizures. Trigeminal schwannomas are a rare entity, and they are even more rarely cystic. This case highlights an unusual scenario where a slow expansion of the tumor ultimately resulted in vision loss and presentation of the patient to medical attention., (© 2022 American Academy of Neurology.)

Background: Schwannomas are benign tumors deriving from the sheath of cranial and peripheral nerves. The vagus nerve is comprised of a complex neuro-endocrine-immune network that maintains homeostasis, most tracts of it play a role in parasympathetic activity. We present an example of a rare cervical vagal schwannoma case accompanied by arrhythmia., Case Presentation: A 35-year-old female patient with a left cervical vagus schwannoma and ventricular arrhythmia underwent schwannoma resection in the operating room. The patient's suppressed heart rate increased after tumor removal, and the cardiac rhythm returned to normal postoperatively. Pathological examination demonstrated the diagnosis of schwannoma., Conclusions: This case explains the link between the vagus nerve and the cardiovascular system, proving that a damaged cervical vagus nerve can inhibit the heart rate and lead to arrhythmias, and eventually requiring surgical intervention., (© 2022. The Author(s).)

Oculomotor nerve schwannomas are rare benign cranial nerve tumors. There are only a limited number of reports on this pathology in the literature, and there are currently no established management guidelines that aid providers in deciding on surgical versus nonsurgical management. We assess the published literature on the topic to identify indications for treatment as well as outcome measures (e.g., local control rates, survival rates, and complication rates) that have been reported as associated with the various treatment modalities. We attempt to develop an algorithm for evaluation and treatment of oculomotor nerve schwannomas in order to establish consensus on how these tumors should be treated., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest., (Copyright © 2021. Published by Elsevier Inc.)

Background and Objective: Cranial nerve schwannomas almost always arise from sensory or mixed nerves. Motor cranial nerves, such as the trochlear nerve, are rarely associated with schwannomas. No consensus has yet been made for surgical intervention because of the low number of reported cases of trochlear nerve schwannomas. This study comprises a systematic review of the literature and our experience for surgically treated trochlear nerve schwannomas., Methods: Three databases (Web of Science, PubMed, and Cochrane Library) were searched without date restrictions. Studies were included if they were published in the English literature and presented patients of any age who underwent surgical treatment for trochlear schwannoma. Data extracted from the included studies were combined with our experience., Results: Forty-one studies, presenting 43 patients, met the inclusion criteria. The total number of patients was 45 after our experience was added. The most common symptoms were diplopia (62.2%), headache (46.7%), and motor weakness (37.8%). Mean age during the diagnosis was 45.1 years. Although the subtemporal transtentorial approach (n = 14) is the most preferred method, its application has decreased in recent years. In the last decade, the lateral suboccipital approach (n = 11) has gained popularity. Residual postoperative trochlear nerve deficit was detected in 81% of patients. The probability of neurologic deficit was not statistically associated with tumor volume (P = 0.914), location (P = 0.669), or resection rate (P = 0.554)., Conclusions: Although trochlear schwannomas are rare and their treatment involves challenges, total resection with the proper approach provides the most desirable results., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Trigeminal schwannomas are complex lesions that may be related to many critical neurovascular structures. We present the case of a 59-year-old male presenting a history of left-sided trigeminal neuralgia. Preoperative imaging demonstrated a mass highly suggestive of a trigeminal schwannoma, and microsurgical resection was indicated considering the progressive symptomatology and important mass effect (Video 1). A middle fossa route including an anterior petrosectomy was chosen. The patient was placed supine with the head rotated to the contralateral side, and an arcuate incision was performed. A V-shaped zygomatic osteotomy was done to mobilize the temporalis muscle more inferiorly and better expose the middle fossa floor. Following craniotomy, peeling of the dura propria from the lateral wall of cavernous sinus was carried out starting by coagulation of middle meningeal artery. Some tumor was already identified and removed, and then the anterior petrosectomy was performed until we exposed the posterior fossa dura. The middle fossa dural incision was connected with the other one at the posterior fossa dura, by coagulation of the superior petrosal sinus. The tentorium was completely cut toward the incisura. After lesion debulking, the tumor was progressively removed by peeling the arachnoid from the lesion to maintain arachnoid planes and preserve the nerves and their blood supply. Postoperative imaging demonstrated complete tumor resection. The patient's symptoms improved, and there were no neurologic deficits on follow-up. Extensive laboratory training is fundamental to be familiarized with the normal anatomic nuances and prepared to face the anatomy distorted by lesion. Informed consent was obtained from the patient for the procedure and publication of this operative video., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Schwannomas of the trochlear nerve are relatively rare, and most patients present with preoperative diplopia because of trochlear nerve palsy. We describe the case of a 61-year-old male patient with a trochlear nerve schwannoma and no pre- and postoperative diplopia, despite his trochlear nerve being cut during the operation. We aimed to investigate the frequency of postoperative diplopia associated with intraoperative trochlear nerve disturbance by reviewing previous case reports, wherein postoperative diplopia did not occur after the trochlear nerve was cut intraoperatively. We recorded the frequency of diplopia because of intraoperative trochlear nerve disturbance, such as the trochlear nerve being cut, in cases without pre- and postoperative diplopia. We searched the PubMed, Medline, and Google Scholar databases for works published from 1976 to 2020 and followed the preferred reporting items for systematic reviews and meta-analyses guidelines. We reviewed 36 publications and found 92 cases of trochlear nerve schwannoma. Surgical resection was performed for 43 patients, of whom 40 were kept under observation and 9 were treated with radiation therapy. Of the 43 cases, 9 without preoperative diplopia underwent gross total resection. We analyzed ten cases (including ours) without preoperative diplopia to check for postoperative diplopia. In total, four cases, including ours, did not display postoperative diplopia despite the trochlear nerve being cut. This may be attributed to the preoperatively acquired motor and sensory fusion in the patient's vision because of tumor progression. Our findings may benefit neurosurgeons who treat patients with schwannomas and help them predict patients' outcomes.

Background: An isolated oculomotor nerve (CN III) palsy is a diagnostic concern because of the potential for serious morbidity or life-threatening causes. We present 5 unusual causes of oculomotor nerve palsy that escaped initial diagnosis in order to raise awareness of their associated features that will facilitate correct diagnosis., Methods: This study consisted of a retrospective analysis of clinical features and imaging of 5 patients who were referred for neuro-ophthalmologic evaluation with presumed diagnosis of oculomotor nerve palsy of unknown reasons., Results: A complete CN III palsy and an inferior division CN III palsy were diagnosed with a schwannoma in the cavernous sinus and orbital apex portion, respectively; a middle-aged woman with aberrant regeneration was found to have a small meningioma; an adult man with ptosis was diagnosed with cyclic oculomotor paresis with spasms; and a patient after radiation was diagnosed with neuromyotonia., Conclusions: Localizing the lesion of oculomotor nerve palsy and careful examination of the imaging is crucial. Aberrant regeneration, cyclic pupil changes, and past medical history of amblyopia, strabismus, or radiation are also very helpful for diagnosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by North American Neuro-Ophthalmology Society.)

Abstract: A 78 year-old woman has experienced multiple episodes of transient right third nerve palsy over the course of 15 years and has undergone multiple imaging studies as well as investigations for myasthenia gravis and giant cell arteritis in search for the diagnosis. When seen after the most recent episode, MRI with contrast and Fast-Imaging Employing Steady-State Acquisition protocol revealed a subtle enlargement and enhancement of the cisternal and proximal cavernous portions of the right third cranial nerve. An empiric diagnosis of schwannoma intrinsic to third cranial nerve was made. All patients with cyclical third nerve palsies should have appropriate neuroimaging to rule out subtle structural lesions before other investigations are undertaken., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by North American Neuro-Ophthalmology Society.)

Trochlear nerve neurinomas are rare and solitary tumors without neurofibromatosis are extremely rare. We report a case of trochlear nerve neurinoma presenting with pathological laughter and diplopia. A 40-year-old male patient presented with diplopia and pathological laughter 2 months before admission. MRI showed a multicystic enhanced mass in the left tentorial incisura compressing the midbrain and the upper pons. The tumor was excised using the left trans-Sylvian approach with partial uncal resection. After excision of the tumor, the left trochlear nerve was identified on the surface of the lateral midbrain. The nerve was connected to the tumor. Pathological laughter completely resolved after the operation. This is the second reported case of trochlear nerve neurinoma presenting with pathological laughter. The lesion responsible for pathological laughter could be the midbrain, upper pons, diencephalon, or all of these.

Background: Tumors arising from oculomotor nerve are rare, with few cases reported in the literature. Generally, whereas schwannomas are well encapsulated tumors, neurofibromas tend to invade the entire nerve fibers. These differences influence surgical resection and neurological clinical outcome, with neurofibroma often requiring the sacrifice of the nerve. Accordingly, an incorrect preoperative diagnosis can lead to incomplete patient counseling before surgery., Case Description: We report 2 cases: a patient with oculomotor schwannoma and a patient with oculomotor neurofibroma. After tumor resection, the patient with a diagnosis of schwannoma recovered with 3rd nerve palsy, while patient with the neurofibroma developed a complete oculomotor nerve deficit. For each patient, surgical strategy and neurological outcome are elucidated in relation with differences in preoperative magnetic resonance imaging and histology., Conclusions: To the best of our knowledge, this is the first report of an oculomotor neurofibroma. When an oculomotor nerve tumor is suspected, a careful preoperative evaluation of magnetic resonance imaging guides in distinguishing the different histology, in selecting the treatment strategy, and in correctly informing the patient on expected postoperative neurologic outcome., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: This report presents the third case of trochlear schwannoma arising from the pineal region and the first case to be resected using a paramedian infratentorial supracerebellar approach. Schwannomas of cranial nerves have traditionally been thought to arise from the transitional point where the axonal envelopment switches from glial cells to Schwann cells; however, recent temporal bone histopathologic evidence from vestibular schwannomas challenges this view. Of the 38 cases of pathology-confirmed trochlear schwannoma in the literature, there are only 2 cases arising from the pineal region, where the nerve sheath transition zone is located. Here, we discuss an unusual case of trochlear schwannoma arising from this transition zone., Case Description: A 65-year-old man was admitted to our institute after a traffic accident. He complained of headache and dizziness, and a computed tomography scan revealed an isodense mass in the pineal region with obstructive hydrocephalus. Magnetic resonance imaging with contrast showed an enhancing mass in the pineal region. The tumor was subtotally resected using a paramedian infratentorial supracerebellar approach, and pathology confirmed the diagnosis of trochlear schwannoma., Conclusions: Trochlear schwannoma should be considered when a mass is identified in the pineal region. This diagnosis should still be entertained for mass lesions along the free tentorial edge because the tumor may arise distant from the glial-Schwann transition zone located by the dorsal midbrain. We propose a treatment algorithm for this rare tumor that seeks to maximize functional outcome., (Copyright © 2020. Published by Elsevier Inc.)

Background: Hypoglossal schwannomas are rare, benign intracranial neoplasms; they represent 5% of all nonvestibular schwannomas., Case Description: A 22-year-old male patient had presented 4 months before admission with left-sided hearing loss, an increase in the base of support with lateralization to the left, and dysphagia to solids. Physical examination on admission showed hypotrophy of the tongue and deviation to the left. Magnetic resonance imaging showed evidence of an extraaxial lesion compressing the medulla oblongata and pons, which protruded through the hypoglossal canal. The patient was prepared for surgical treatment in 2 stages: in the first surgery, a left retrosigmoidal approach with total resection of the intracranial lesion was performed. The biopsy reported a schwannoma, and correlating the signs, symptoms, and imaging, the diagnosis of a hypoglossal nerve schwannoma was established. In the second surgery, a lateral cervical approach was performed, with subtotal resection of the lesion, leaving a remnant adhered to the nerve in the hypoglossal canal., Conclusions: Hypoglossal schwannomas are a rare entity, in which surgery is the most viable option with high cure rates. However, its complete resection, without leaving permanent neurologic sequels, is a challenge., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objectives: The aim of this study was to evaluate the preoperative and postoperative facial nerve (FN) function in patients with FN schwannoma (FNS) and analyze the duration of preoperative facial palsy according to the preoperative and postoperative facial function., Materials and Methods: We retrospectively reviewed the medical records of 29 patients with FNS who underwent surgery. We evaluated the FN function according to the type of FN manipulation and location of the anastomoses in the cable nerve graft, and we also analyzed the duration of facial palsy according to the facial function before and after surgery., Results: All 4 patients who underwent nerve-stripping surgery had the House-Brackmann (H-B) Grade III, 12 of 21 who underwent a cable nerve graft had the H-B Grade III or better postoperatively, and all 4 who underwent a hypoglossal facial crossover had the H-B Grade IV. Patients who underwent cable nerve grafting were more likely to have better FN function when the proximal anastomosis site was located in the internal auditory canal, geniculate ganglion, tympanic segment of FN, and distal end in the mastoid segment of FN. The duration of preoperative facial palsy was statistically shorter in patients with better postoperative facial function., Conclusion: Surgery can be considered in patients with FNS who have the H-B Grade III or worse. A shorter duration of facial palsy prior to surgery resulted in better postoperative facial function.

Background: Recurrent painful ophthalmoplegic neuropathy (RPON), previously known as ophthalmoplegic migraine (OM), is an uncommon disorder with repeated episodes of ocular cranial nerve neuropathy associated with ipsilateral headache. The age of presentation is most often during childhood or adolescence. MRI has a central role in the assessment of the RPON, especially to distinguish orbital, parasellar, or posterior fossa lesions that mimic symptoms of RPON. Actually, oculomotor nerve tumors may be masquerade as RPON so that MRI follow-ups are required to detect the possibility of tumor etiology., Case Presentation: We report a 16-year-old boy with a 7-year follow-up and multiple brain MRI data, previously diagnosed as OM. The last brain MRI, performed during an acute phase of oculomotor paresis with ipsilateral headache, showed a nodular lesion described as schwannoma of III cranial nerve. Then, we reviewed the literature on OM and RPON in pediatric age with a focus on brain MRI findings., Conclusions: This review highlights the important role of serial brain MRIs in the long-term follow-up of RPON, especially in the cases with childhood onset, in order to not delay the diagnosis of a possible oculomotor nerve schwannoma., Competing Interests: The authors declare they have no conflicts of interest., (Copyright © 2019 Maria Giuseppina Petruzzelli et al.)

A 69-year-old woman presented with an 8-month history of diplopia and examination findings consistent with a right third-nerve palsy. Head MRI identified the presence of a 5.8 mm, nodular, isointense lesion in the suprasellar cistern, which demonstrated enhancement with gadolinium contrast. The lesion did not show any evidence of growth over a 3-month follow-up period. These MRI findings, alongside the clinical features, suggest oculomotor nerve schwannoma. Oculomotor schwannomas are a rare cause of third-nerve palsy. The presenting features and management options for oculomotor schwannomas are discussed to provide a framework for the diagnosis and management of these patients., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Facial nerve schwannomas are rare benign neoplasms. We report a case of a 60-year-old woman who initially presented with vestibular complaints. Magnetic resonance imaging (MRI) revealed a facial nerve schwannoma centered on the right geniculate ganglion extending in the labyrinthine segment. The patient consulted again after 2 months because she developed a sudden and severe right-sided sensorineural hearing loss. MRI showed no progression or pathological enhancement in the membranous labyrinth. A cone beam computed tomography (CT) of the temporal bone was performed and revealed a large erosion at the region of the geniculate ganglion in open communication with the middle turn of the cochlea. This case report demonstrates the importance of CT in facial nerve schwannomas for evaluating the impact on the surrounding structures.

Trochlear schwannomas typically present with diplopia and are extremely rare benign intracranial neoplasms that require the combined expertise of neurotologists and neurosurgeons for surgical excision. A combined petrosal approach offers a surgical route for removal of growing and/or symptomatic tumors. A study of a trochlear nerve schwannoma that demonstrated rapid growth but was otherwise asymptomatic was presented in this study.

Background: Schwannoma originating from the greater superficial petrosal nerve is an extremely rare type of facial nerve schwannoma located in the middle cranial fossa around the midportion of the petrous bone. Here, we provide the first report of greater superficial petrosal nerve schwannoma presenting with contralateral facial palsy and hemiparesis due to compression of the pyramidal tract by associated intradural peritumoral cyst., Case Description: A 69-year-old woman presented with a 6-month history of gradually worsening gait unsteadiness. Magnetic resonance imaging demonstrated a well-defined tumor occupying the right middle cranial fossa and extending into the tympanic cavity. Notably, the tumor accompanied a large cyst in the intradural space, resulting in a leftward midline shift. Extradural exploration through thinned periosteal dura mater revealed the tumor within the interdural space. After debulking of most of the tumor in a pull-out, piecemeal fashion, intradural exploration revealed the peritumoral cyst located between the meningeal dura mater and brain parenchyma. Following the disappearance of the mass effect from the tumor, fenestration of the peritumoral cyst, which had been deeper-seated than the tumor, was easily achieved while avoiding excessive retraction of the temporal lobe. Postoperatively, mild left hemiparesis involving the face resolved completely and no new symptoms such as right facial palsy, hearing disturbance, or xerophthalmia developed as postoperative complications., Conclusion: The combination of extradural and intradural approaches in the appropriate order is essential for fenestration of an intradural peritumoral cyst along with removal of an interdural tumor., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: To describe the clinical presentation of the facial nerve schwannomas according to the anatomical site of origin., Method: A retrospective study in which the clinical presentation, diagnostic protocol and treatment of facial nerve tumors in adults was evaluated., Results: We found 6 cases, 4 cases of tympanic-mastoid location at the spectrum of its possible clinical presentation: from symptomatic cases with facial paralysis, to an asymptomatic case in the tympanic portion found as intraoperative finding; and also found two cases located at the parotid gland, one with complete facial paralysis and one without facial palsy., Conclusions: For the diagnosis of intratemporal and parotid schwannomas of the facial nerve, a high clinical suspicion is required given its heterogeneous presentation; its clinical course depends on the segment of origin and expansion: more frequently asymptomatic at the tympanic horizontal portion and symptomatic at the mastoid vertical portion. These tumors must be assessed with imaging studies, incisional biopsy is not recommended. The treatment is surgical resection in symptomatic patients with facial paralysis greater than grade III of House-Brackmann, with immediate reconstruction of the nerve., (Copyright: © 2019 Permanyer.)

Background: Intrinsic tumors of the facial nerve are a rare entity. Dealing with this subset of tumors is challenging both in terms of decision making and surgical intervention., Objective: To review the outcomes of surgical management of facial nerve tumors and cable nerve graft interpositioning., Methods: A retrospective analysis was performed at a referral center for skull base pathology. One hundred fifteen patients who were surgically treated for facial nerve tumors were included. In case of nerve interruption during surgery, the cable nerve interpositioning technique was employed wherein the facial nerve palsy lasted for less than 1-yr duration. In cases of facial nerve palsy lasting for greater than 1 yr, the nerve was restituted by a hypoglossal facial coaptation., Results: Various degrees of progressive paralysis were seen in 84 (73%) cases. Sixty nine (60%) of the tumors involved multiple segments of the facial nerve. Sixty-two (53.9%) tumors involved the geniculate ganglion. Seventy four (64.3%) of the cases were schwannomas. Hearing preservation surgeries were performed in 60 (52.1%). Ninety one (79.1%) of the nerves that were sectioned in association with tumor removal were restituted primarily by interposition cable grafting. The mean preoperative House-Brackmann grading of the facial nerve was 3.6. The mean immediate postoperative grading was 5.4, which recovered to a mean of 3.4 at the end of 1 yr., Conclusion: In patients with good facial nerve function (House-Brackmann grade I-II), a wait-and-scan approach is recommended. In cases where the facial nerve has been interrupted during surgery, the cable nerve interpositioning technique is a convenient and well-accepted procedure for immediate restitution of the nerve.

Objective: To investigate the clinical and electrophysiological features of facial nerve paralysis (FNP) due to benign temporal bone masses (TBMs) and elucidate its differences as compared with Bell's palsy., Methods: FNP assessed by the House-Brackmann (HB) grading system and by electroneurography (ENoG) were compared retrospectively., Results: We reviewed 914 patient records and identified 31 patients with FNP due to benign TBMs. Moderate FNP (HB Grades II-IV) was dominant for facial nerve schwannoma (FNS) (n=15), whereas severe FNP (Grades V and VI) was dominant for cholesteatomas (n=8) and hemangiomas (n=3). The average ENoG value was 19.8% for FNS, 15.6% for cholesteatoma, and 0% for hemangioma. Analysis of the correlation between HB grade and ENoG value for FNP due to TBMs and Bell's palsy revealed that given the same ENoG value, the corresponding HB grade was better for FNS, followed by cholesteatoma, and worst in Bell's palsy., Conclusions: Facial nerve damage caused by benign TBMs could depend on the underlying pathology. Facial movement and ENoG values did not correlate when comparing TBMs and Bell's palsy. When the HB grade is found to be unexpectedly better than the ENoG value, TBMs should be included in the differential diagnosis., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Schwannomas arising from the cranial nerves controlling extraocular eye movements are very rare and usually present with some degree of diplopia., Case Presentation: We report a 50-year-old woman who presented with isolated left-sided trigeminal neuralgia of 6 months' duration. Imaging demonstrated a homogeneously enhancing mass in the left ambient cistern, and the patient was brought to the operating room for resection. A retrosigmoid approach was used, and the mass was directly visualized arising from the trochlear nerve and compressing the dorsal root entry zone of the trigeminal nerve. A gross total resection of the mass was achieved, and microvascular decompression of the trigeminal nerve was performed. The tumor was pathologically confirmed as a schwannoma. At 3-month follow-up, the patient's facial pain was resolved, and her extraocular eye movements were intact., Conclusions: A total of 32 pathology-confirmed cases of trochlear schwannoma have been previously reported in the English-language literature. Most of these tumors arose from the cisternal segment of the nerve, and most patients presented with frank trochlear nerve palsy on exam. We report the first case of trochlear schwannoma presenting with isolated trigeminal neuralgia., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: To present an unusual case of a temporal bone meningioma with intrafascicular spread throughout the temporal facial nerve from cerebellopontine angle (CPA) to stylomastoid foramen., Patient: Four-year-old female with progressive facial weakness and normal hearing., Main Outcome Measure: Clinical, radiological, and histopathological findings of temporal bone meningiomas., Results: A patient presented with progressive facial weakness and normal hearing. Imaging demonstrated a mass within the left internal auditory canal radiologically consistent with a schwannoma. Asymmetric enlargement with enhancement of the left facial nerve from CPA to the stylomastoid foramen suggested facial schwannoma. At surgery, gross tumor was noted in the internal auditory canal, the fallopian canal seemed expanded and the facial nerve was enlarged and had an irregular contour. Resection of the facial nerve from the CPA to just proximal to its exit at the stylomastoid foramen was necessary to achieve negative margins. Cable grafting was performed. The histopathologic diagnosis was transitional meningioma with intraneural growth throughout the length of the resected facial nerve segment., Conclusion: Meningiomas involving the temporal bone are exceedingly rare. We report a rare case of a child presenting with progressive facial weakness due to a presumed facial schwannoma spreading along the facial nerve throughout its intratemporal course that at surgery was found to be an intrafascicular CN VII meningioma.

Background: Schwannomas of the vagus nerve and cervical sympathetic nerve are rare; hence, only limited information exists regarding their diagnosis and clinical management., Methods: We conducted a retrospective review of the clinical features, imaging studies, and treatment results of patients with schwannoma of the vagus nerve and schwannoma of the sympathetic nerve., Results: Of 91 patients, 91% (n = 83) were preoperatively diagnosed with schwannoma tumors. Using the hyoid bone as an anatomic landmark, the location of the schwannoma of the vagus nerve in the carotid space was significantly different to the location of schwannoma of the sympathetic nerve (p = .003). Although 52 of the 76 patients followed up (68%) had postoperative nerve weaknesses, 13 patients (50%) and 14 patients (53.8%), respectively, fully recovered from schwannoma of the vagus nerve and schwannoma of the sympathetic nerve., Conclusion: In the carotid space, schwannomas of the vagus nerve are usually located below the hyoid bone, whereas schwannomas of the sympathetic nerve more commonly arise from the suprahyoid compartment. Accurate preoperative diagnosis and the intracapsular enucleation surgical approach decreased the incidence of postoperative morbidity. © 2016 Wiley Periodicals, Head Neck 39: 42-47, 2017., (© 2016 Wiley Periodicals, Inc.)

Facial nerve schwannoma is the most common facial nerve tumor, but its therapeutic strategy remains debated. The aim of this study is to analyze the facial nerve function and the hearing outcomes after surgery or wait-and-scan policy in a facial nerve schwannoma series. A monocentric retrospective review of medical charts of patients followed for an intratemporal facial nerve schwannoma between 1988 and 2013 was performed. Twenty-two patients were included. Data were extracted pertaining to the following variables: patient demographics, tumor localization, clinical and imaging features, facial nerve function and hearing levels, and details of surgical intervention. The majority of tumors were located at the geniculate ganglion. Initial symptoms were mainly facial palsy and hearing loss. The average follow-up was 4.8 ± 4.5 years. Nineteen patients underwent surgery, and three patients were observed. After surgery, 11 patients had a stable or improved facial nerve function (57.9 %), and 8 patients had a worsened facial nerve function (42.1 %). Facial nerve function was in the majority of cases a HB grade III, depending on surgical strategy. No patient presented a postoperative HB grade V or VI. Regarding the hearing, it remained stable after surgery in 52.6 % of cases, and improved in 10.5 % of cases. Among monitored patients, facial nerve function and hearing remained stable. Surgery for facial nerve schwannoma is a safe and effective option in the treatment of these tumors.

Trochlear nerve schwannoma is extremely rare, with only 35 pathologically confirmed patients being reported in the literature. Here, the authors report a patient of trochlear nerve schwannoma in the prepontine cistern manifesting as facial pain and double vision and presenting the image characteristics of repeated intratumoral hemorrhage, which has never been reported in the literature. Total tumor along with a portion of the trochlear nerve was removed by using a retrosigmoid approach. Facial pain disappeared after operation, and the diplopia remained. Follow-up studies have shown no tumor recurrence for 2 years and the simultaneous alleviation of diplopia. Information regarding the clinical presentation, radiological features and surgical outcomes of trochlear nerve schwannoma are discussed and reviewed in the paper.

Introduction: Schwannoma of the cervical vagus nerve is rare. Treatment options include intracapsular enucleation and en bloc resection. The purpose of this study was to compare the outcomes of enucleation and resection in terms of postoperative mortality and morbidity, freedom from vocal cord palsy, freedom from local recurrence, quality-adjusted life-year (QALY) and vocal handicap index (VHI)., Methods: Twentytwo consecutive patients were divided into two groups. Patients in group A (n = 9) underwent intracapsular enucleation, whereas patients in Group B (n = 13) underwent en bloc resection. Main endpoints of the study were postoperative mortality and morbidity, freedom from vocal cord palsy, freedom from local recurrence and quality of life. The quality of life after surgery was assessed according to the quality-adjusted life-year (QALY) EQ-5D-5L methodology, and calculation of the voice handicap index (VHI)., Results: Postoperative mortality was nil. Morbidity included 1 wound dehiscence in group A and 2 transitory dysphagias in group B. Freedom from vocal cord palsy was 22% in group A and zero in group B (p = 0.15). Operation-specific local recurrence rate was 33% (3/9 patients) in group A and nil in group B (0/23 patients) (p = 0.05). QALYs was 0.55 in group A and 0.54 in group B (p = 1.0). VHI was 23.77 in group A and 26.15 in group B (p = 1.00)., Conclusion: Resection is superior to enucleation in terms of freedom from local recurrence. Functional results are comparable for both techniques., (Copyright © 2016 IJS Publishing Group Ltd. Published by Elsevier Ltd. All rights reserved.)

Background: Facial nerve schwannomas are rare lesions that constitute only 0.8% of all intrapetrous mass lesions. The least frequent lesions are tumors originating in the greater petrosal nerve (GPN). We present a case of a GPN schwannoma with temporal lobe edema in which the patient was operated on using an extradural and intradural approach to prevent complications., Case Description: A 66-year-old woman with vertigo and abnormal magnetic resonance imaging findings was referred to our department. Computed tomography scan revealed an isodense subtemporal mass with partial rim calcification and petrosal bone apex erosion. Magnetic resonance imaging confirmed a 22-mm left middle fossa lesion with heterogeneous enhancement and edema of the temporal lobe. A left temporal craniotomy to the middle fossa was performed. The initial extradural exploration revealed the tumor to be in the Glasscock triangle, mainly involving the location of the GPN. The tumor was removed through an intradural approach in piecemeal fashion. Finally, using an extradural and intradural middle fossa approach, the tumor was totally removed, leaving the capsule on the middle fossa floor with continuous facial nerve monitoring. The postoperative course was uneventful without complications of xerophthalmia and facial palsy., Conclusions: GPN schwannomas are very rare lesions. The extradural and intradural middle fossa approach was used to preserve the tumor capsule around the GPN. Using this technique, one can safely protect the geniculate ganglion and the GPN., (Copyright © 2016 Elsevier Inc. All rights reserved.)

An isolated schwannoma of the oculomotor nerve is rare in children without an associated neurofibromatosis. A 13-year-old girl, with a previous medical history of migraine, was admitted for disabling ophthalmic migraine with oblique diplopia. The clinical examination showed a right incomplete ophthalmoplegia with reduced ipsilateral visual acuity (8/10). There was no particular skin reaction. The MRI revealed a right (isosignal-T1 and isosignal-T2) nodular schwannoma located within the cisternal segment of the oculomotor nerve. The angio-CT performed later confirmed the absence of any vascular malformation. The treatment consisted of analgesics and corticotherapy, with complete regression of symptoms three weeks later and a normal MRI follow-up. Therefore, radiosurgery was not performed., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Schwannoma of the trigeminal nerve is a rare condition. Even rarer is hearing loss occurring as a result of this lesion. The aim of this study is to highlight this rare cause of hearing impairment. Here we report the clinical features and findings of the imaging and audiological investigations of a case of trigeminal schwannoma diagnosed at our institution. Our patient presented with headache, giddiness, tinnitus, left-sided facial weakness, left-sided hearing loss, right-sided hemiplegia, and unintelligible speech. Radiological studies revealed a large well-defined mass lesion in the left cerebellopontine angle with a significant mass effect on posterior fossa structures, suggestive of trigeminal nerve tumor. Audio-vestibular assessment was done with pure tone audiometry, impedance audiometry, otoacoustic emission, brainstem-evoked response audiometry, and electronystagmography, which pointed toward a retrocochlear pathology for hearing loss and imbalance.

Objective: We report 18 cases of facial nerve schwannomas in which stripping surgery was attempted to preserve facial nerve integrity and favorable facial nerve function., Methods: We attempted stripping surgery on 18 cases of facial nerve schwannomas. Postoperative facial nerve function was evaluated., Results: Stripping surgery was successfully achieved in 11 cases, and facial nerve decompression was performed on the remaining 7 cases in which stripping surgery was impossible. Favorable facial nerve function was successfully maintained in all cases who underwent stripping surgery and 5 of 7 cases who underwent facial nerve decompression., Conclusions: It was possible to accomplish stripping surgery in most cases with favorable facial nerve function, which maintained good facial nerve function after total tumor removal., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Cranial nerve schwannomas are radiologically characterized by nodular cranial nerve enhancement on magnetic resonance imaging (MRI). Schwannomas typically present with gradually progressive symptoms, but isolated reports have suggested that schwannomas may cause fluctuating symptoms as well., Methods: This is a report of ten cases of presumed cranial nerve schwannoma that presented with transient or recurring ocular motor nerve deficits., Results: Schwannomas of the third, fourth, and fifth nerves resulted in fluctuating deficits of all 3 ocular motor nerves. Persistent nodular cranial nerve enhancement was present on sequential MRI studies. Several episodes of transient oculomotor (III) deficts were associated with headaches, mimicking ophthalmoplegic migraine., Conclusions: Cranial nerve schwannomas may result in relapsing and remitting cranial nerve symptoms.

Schwannomas are rare neurogenic tumor originating from Schwann cells of the nerve sheath, most frequently encountered type of posterior mediastinal tumors. In most cases, schwannomas are benign, malignant and multiple schwannomas are rare. Histopathologically, the tumor is composed of fascicles of spindle cells, which are strongly positive for S-100 proteins. Surgical resection is a treatment of choice, and prognosis is excellent. Here, we report a case of posterior mediastinal schwannoma in a 20- years old male patient who complained of right-sided back pain and two episodes of massive hemoptysis of recent onset. Contrast enhanced computed tomography (CECT) and magnetic resonance imaging of the chest showed a well circumscribed, heterogeneous mass in the posterior mediastinum, compressing the right lower lobe with widening of intervertebral foramens. CT-guided trucut biopsy revealed spindle cell neoplasm. On immunohistochemistry, tumor cells expressed strong positivity for S-100 protein. Final diagnosis was schwannoma, probably originating from the right vagus nerve. Surgical resection of the encapsulated tumor resulted in the successful recovery, without any recurrence over next one year follow up.

Objective: To present a series of patients with facial nerve schwannomas (FNSs) presenting as occluding external auditory canal (EAC) masses., Study Design: Retrospective case series., Patients: Four patients were identified with mastoid segment FNSs occluding the EAC. Three patients presented with conductive hearing loss (CHL), and the fourth presented with facial paralysis, later developing CHL., Intervention: One patient underwent conservative debulking, removing the EAC component only. Two patients were managed nonoperatively with periodic cleaning of entrapped keratin. The fourth patient received radiation therapy., Main Outcome Measures: Facial nerve function, canal cholesteatoma formation, and hearing., Results: Among the patients managed with serial cleaning of entrapped keratin, one maintained normal facial function and one worsened to House-Brackmann II/VI. Facial function worsened to House-Brackmann II/VI in the patient who underwent surgical debulking. The fourth patient, who received radiation, developed complete facial paralysis. All patients accumulated keratin medial to the tumor, and all had CHL., Conclusion: When evaluating an EAC tumor, it is important to obtain imaging before biopsy because biopsy of a schwannoma can result in paralysis. EAC occlusion by a schwannoma presents a challenging management issue, particularly when cholesteatoma forms between the tumor and the tympanic membrane. The primary goal is maintaining normal facial function as long as possible and avoiding secondary ear canal complications. The presence of canal occlusion limits the choice of stereotactic radiation because this leads to a month-long period of tumor swelling and cutaneous sloughing. Resection and grafting are indicated when substantial facial weakness or twitch develops.

Vagal nerve schwannomas that compress the trachea are extremely rare. We report a case in which a giant middle mediastinal schwannoma accompanied by severe tracheal stenosis was excised. A 70-year-old woman had a 30-year history of hoarseness and paralysis of the left recurrent laryngeal nerve. She presented with chronic cough and dyspnea, which she had experienced for several months. Computed tomography indicated a middle mediastinal mass (8.3×7.1×4.9 cm) that directly compressed the lower trachea, carina, both bronchi, and the left main pulmonary artery. The trachea was 4 mm in diameter at the narrowest point. Through a median sternotomy, the superior vena cava, ascending aorta, right pulmonary artery, and trachea were dissected, and the tumor was excised by gentle dissection. Histologic examination indicated a benign schwannoma., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A 60-year-old lady presented with intermittent headaches. Examination revealed striking marked unilateral tongue atrophy. Magnetic resonance imaging (MRI) revealed a cystic lesion in the hypoglossal canal and a provisional diagnosis of cystic hypoglossal schwannoma made. Annual surveillance scans showed stable appearances but surprisingly at 3 years they showed a significant reduction in the size of the lesion. Most patients with hypoglossal schwannomas present with ipsilateral hypoglossal nerve palsy; careful cranial nerve examination is vital in diagnosing such rare lesions. Little is known of their natural history, with most lesions undergoing surgery. This case highlights spontaneous regression following non-operative management.

Facial nerve schwannoma (FNS) is an uncommon disease, bilateral FNS are exceptional. We describe a case of a 23-year-old man who presented a bilateral 3rd segment facial nerve schwannoma, without neurofibromatosis disease. We discuss the clinical presentation, radiologic findings and management of this case. Facial nerve schwannoma can present in various ways. The imaging appearance of FNS is more varied than originally described. Our case demonstrates an uncommon presentation of a relatively rare tumor in which the tumor has been respected. During this time, the tumor may be followed by radiologic imaging.

Purpose: Surgical resection is considered the desirable curative treatment for trigeminal schwannomas. However, complete resection without any complications remains challenging. During the last several decades, stereotactic radiosurgery (SRS) has emerged as a minimally invasive treatment modality. Information regarding long-term outcomes of SRS for patients harboring trigeminal schwannomas is limited because of the rarity of this tumor. The aim of this study was to evaluate long-term tumor control and functional outcomes in patients harboring trigeminal schwannomas treated with SRS, specifically with gamma knife surgery (GKS)., Methods and Materials: Fifty-three patients harboring trigeminal schwannomas treated with GKS were evaluated. Of these, 2 patients (4%) had partial irradiation of the tumor, and 34 patients (64%) underwent GKS as the initial treatment. The median tumor volume was 6.0 cm(3). The median maximum and marginal doses were 28 Gy and 14 Gy, respectively., Results: The median follow-up period was 98 months. On the last follow-up image, 7 patients (13%) had tumor enlargement, including the 2 patients who had partial treatment. Excluding the 2 patients who had partial treatment, the actuarial 5- and 10-year progression-free survival (PFS) rates were 90% and 82%, respectively. Patients with tumors compressing the brainstem with deviation of the fourth ventricle had significantly lower PFS rates. If those patients with tumors compressing the brainstem with deviation of the fourth ventricle are excluded, the actuarial 5- and 10-year PFS rates increased to 95% and 90%, respectively. Ten percent of patients had worsened facial numbness or pain in spite of no tumor progression, indicating adverse radiation effect., Conclusions: GKS can be an acceptable alternative to surgical resection in patients with trigeminal schwannomas. However, large tumors that compress the brainstem with deviation of the fourth ventricle should be surgically removed first and then treated with GKS when necessary., (Copyright © 2013 Elsevier Inc. All rights reserved.)

A 77-year-old female in the hospital was found tachycardic and hypothermic by a nurse, and the patient's respiration subsequently ceased. Forensic autopsy revealed an intracranial cystic tumor that would have compressed the brainstem. On microscopic examination, the tumor was diagnosed as an Antoni A schwannoma growth, and recent multiple intratumoral hemorrhages in the intracranial schwannoma were observed, suggesting the sudden enlargement of the intracranial schwannoma due to intratumoral hemorrhaging. Accordingly, we diagnosed the cause of death as brainstem compression induced by the intratumoral hemorrhaging in the intracranial schwannoma. Meanwhile, a rhinopharyngeal tumor was also detected by the autopsy, which was compatible with an antemortem diagnosis of a dumbbell-shaped hypoglossal schwannoma., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)