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Oculomotor nerve schwannoma in children not associated with neurofibromatosis is a rare disease, with 26 pediatric cases reported so far. There is no established treatment plan. A 7-year-old girl presented with oculomotor nerve palsy. Surgical reduction of the tumor combined with postoperative gamma knife surgery preserved the oculomotor nerve, improved oculomotor nerve function, and achieved tumor control during the observation period of 20 months. The combination of partial surgical resection and gamma knife surgery as a treatment strategy for oculomotor nerve schwannoma resulted in a good outcome., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Clinical Case: The 59-year-old patient complained of hearing loss on the left, ear murmur for a long time, periodic pain and discomfort in the left ear, dizziness for 6 months. She was found to have concurrent vestibular schwannoma in the internal auditory canal and temporal bone paraganglioma. Both tumors were removed in one operation. The schwannoma was removed by translabirinth access due to preoperative deafness, while the glomus tumor was removed during this access. Postoperative biopsy showed the presence of two unrelated diseases: paraganglioma (ICD-0 code 8690/3) and schwannoma (ICD-0 code 9560/0).

Competing Interests: The authors report no conflicts of interest.

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.)

Facial nerve schwannomas are rare, benign, slow-growing tumors that can occur in any segment of the facial nerve, although 71% of cases are intratemporal. Surgical resection can lead to facial nerve injury. Facial function recovery after reanimation is usually not better than House-Brackmann (HB) grade III. Thus, for cases of intratemporal facial nerve schwannomas (IFNSs) with favorable facial function (HB grade I or II), observation by periodic magnetic resonance imaging is the mainstay of management. Here, we present a case of a large IFNS with normal facial function in which the mass fully occluded the external auditory canal. The occlusion caused squamous debris to accumulate, potentially leading to cholesteatoma. Faced with this therapeutic dilemma, we chose surgical resection with the patient's informed consent. Stripping surgery was achieved with normal postoperative facial function. There was no postoperative facial paralysis or recurrence at 2-year follow-up. We describe the experience of diagnosis and treatment process for this case, and discuss the possibility of total resection of the tumor with preserving the integrity of facial nerve.

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: 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: 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.)

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.

Schwannomas of cranial nerves in the absence of systemic neurofibromatosis are relatively rare. Among these, schwannomas of the trochlear nerve are even less common. They can be found incidentally or when they cause diplopia or other significant neurological deficits. Treatment options include observation only, neuro-ophthalmological intervention, and/or neurosurgical management via resection or sterotactic radiosurgery (SRS). In recent years, the latter has become an attractive therapeutic tool for a number of benign skull base neoplasm including a small number of reports on its successful use for trochlear Schwannomas. However, no treatment algorithm for the management of these tumors has been proposed so far. The goal of this manuscript is to illustrate a case series of this rare entity and to suggest a rational treatment algorithm for trochlear schwannomas, based on our institutional experience of recent cases, and a pertinent review of the literature. Including our series of 5 cases, a total of 85 cases reporting on the management of trochlear schwannomas have been published. Of those reported, less than half (40 %) of patients underwent surgical resection, whereas the remainder were managed conservatively or with SRS. Seventy-six percent (65/85) of the entire cohort presented with diplopia, which was the solitary symptom in over half of the cases (n = 39). All patients who presented with symptoms other than diplopia or headaches as solitary symptoms underwent surgical resection. Patients in the non-surgical group were mostly male (M/F = 3.5:1), presented at an older age and had shorter mean diameter (4.6 vs. 30.4 mm, p < 0.0001) when compared to the surgical group. Twelve patients in the entire cohort were treated with SRS, none of whom had undergone surgical resection before or after radiation treatment. Trochlear schwannoma patients without systemic neurofibromatosis are rare and infrequently reported in the literature. Of those, patients harboring symptomatic trochlear Schwannomas do not form a single homogenous group, but fall into two rather distinct subgroups regarding demographics and clinical characteristics. Among those patients in need of intervention, open microsurgical resection as well as less invasive treatment options exist, which all aim at safe relief of symptoms and prevention of progression. Both open microsurgical removal as well as SRS can achieve good long-term local control. Consequently, a tailored multidisciplinary treatment algorithm, based on the individual presentation and tumor configuration, is proposed.

Background: Secondary trigeminal neuralgia (TN) is uncommon. When a space-occupying lesion with mass effect is identified, the associated TN is often exclusively attributed to the tumor. This report illustrates the importance of considering coexistent actionable pathology when surgically treating secondary TN., Case Description: A 51-year-old woman presented with abrupt-onset TN of the V2 and V3 nerve divisions with hypesthesia. She denied changes in hearing, balance, or facial nerve dysfunction. Magnetic resonance imaging revealed a 1.6-cm contrast-enhancing cerebellopontine angle tumor that effaced the trigeminal nerve, consistent with a vestibular schwannoma. In addition, a branch of the superior cerebellar artery abutted the cisternal segment of the trigeminal nerve on T2-weighted thin-slice magnetic resonance imaging. Intraoperative electrical stimulation of the tumor elicited a response from the facial nerve at low threshold over the entire accessible tumor surface, indicating that the tumor was a facial nerve schwannoma. Considering the patient's lack of facial nerve deficit and that the tumor exhibited no safe entry point for intracapsular debulking, tumor resection was not performed. Working between the tumor and tentorium, a branch of the superior cerebellar artery was identified and decompressed with a Teflon pad. At last follow-up, the patient exhibited resolution of her TN. Her hearing and facial nerve function remained intact., Conclusions: Despite obstruction from a medium-sized tumor, it is still possible to achieve microvascular decompression of the fifth cranial nerve. This emphasizes the importance of considering other actionable pathology during surgical management of presumed tumor-induced TN. Further, TN is relatively uncommon with medium-sized vestibular schwannomas and coexistent causes should be considered., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Introduction: Occipital condyle syndrome consists of the presence of unilateral occipital headache exacerbated by moving the head and is accompanied by paralysis of the ipsilateral hypoglossal nerve. One of its causes is infiltration of the base of the skull by bone metastases, especially those affecting the hypoglossal nerve due to infiltration as it passes through the osseous canal., Case Reports: We report two clinical cases of occipital condyle syndrome secondary to metastatic hepatocarcinoma. The first is that of a 52-year-old male with liver cirrhosis secondary to liver pathology caused by hepatitis C virus with occipital condyle syndrome as the presenting symptom in disseminated hepatocarcinoma. The second case is that of a 56-year-old male after recurrence of hepatocarcinoma following a liver transplant, despite not fulfilling the Milan criteria., Conclusion: Occipital condyle syndrome is an alarm symptom and requires a thorough study by means of imaging tests, since it may be the first symptom of an undetected hepatocarcinoma.

Background: Schwannomas are benign, slow-growing neoplasms of the myelin-producing Schwann cells of peripheral nervous system that most commonly affect sensory nerves. Hypoglossal schwannomas, tumors of purely motor nerves, comprise <5% of all head and neck schwannomas. Since the first description of a hypoglossal schwannoma in 1933, there have been few case reports of extracranial origins. The most common location of an extracranial hypoglossal schwannoma is in the parapharyngeal space and can mimic paragangliomas. We describe the case of a woman presenting with an enlarging neck mass originally thought to be a paraganglioma but ultimately discovered to be a hypoglossal schwannoma at surgery., Case Description: A 63-year-old woman had a well-circumscribed, mobile, nontender, 3-cm firm mass at the mandibular angle. On computed tomography, the mass was at the level of the carotid bifurcation, splaying the branching vessels. It was further evaluated with magnetic resonance imaging, which revealed a homogeneous, T2-hyperintense, T1-isointense mass with homogeneous contrast enhancement and scant flow voids. Biopsy revealed a spindle cell mass with positive S-100 staining. She underwent resection with the mass originating from the hypoglossal nerve., Conclusions: Hypoglossal schwannomas are rare lesions with a variable location along the course of the nerve. An extracranial lesion was described here, which was initially mistaken for a paraganglioma. Surgical resection is the consensus recommendation and is often well tolerated with low risk of long-term recurrence., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Introduction: Vagal paraganglioma are rare tumors that are mostly asymptomatic. We report a case of vagal paraganglioma associated with paraneoplastic polymyalgia rheumatica and review the literature on benign paragangliomas of the head and neck associated with paraneoplastic syndrome., Case Report: A 53-year-old man presented with atypical polymyalgia rheumatica. MRI revealed a tumor that was then surgically excised. Histological examination confirmed the diagnosis of benign vagal paraganglioma. Rapid, complete and permanent resolution of all rheumatological symptoms were observed postoperatively, confirming the diagnosis of paraneoplastic polymyalgia rheumatica., Conclusion: Paraganglioma of the neck associated with paraneoplastic syndrome remains exceptional. A predisposing gene mutation must be systematically investigated. Long-term surveillance must be ensured due to the risk of local recurrence, second tumors or metastasis., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

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.)

The objectives of this study were to identify signs of vestibular nerve suffering through a bedside vestibular examination protocol in case of sudden sensorineural unilateral hearing loss without spontaneous signs of vestibular impairment and to propose a bed-side vestibular examination based protocol for the focused execution of gadolinium-enhanced magnetic resonance imaging (MRI) only if a vestibular schwannoma is suspected. 96 patients, 52 men, 44 women, mean age 57.73 +/- 12.85 years, suffering from sudden sensorineural unilateral hearing loss, which presented neither vertigo nor spontaneous nystagmus, were enrolled. Pure tone audiometry, tympanometry, measurement of acoustic reflexes and Anderson test to detect adaptation, bedside vestibular examination through head shaking test, vibration test, head impulse test, hyperventilation test and detection of nystagmus in supine and lateral decubitus to search for signs of vestibular impairment were performed. Patients with signs of vestibular impairment and pure tone audiometry threshold at high frequencies better than 70 dB nHL were subjected to auditory brainstem responses. Gadolinium enhanced MRI centred on internal acoustic canals was carried out in all patients with sudden sensorineural unilateral hearing loss. Main outcome measures were signs of vestibular impairment at vestibular bedside examination and presence of vestibular schwannoma on MRI. Signs of vestibular impairment were detected in 22/96 cases (22.9%); a vestibular schwannoma was detected by MRI in 5/96 cases (5.2%), always when vestibular impairment was present. In case of sudden sensorineural unilateral hearing loss, vestibular bedside examination seems to be useful to restrict the suspicion of a vestibular schwannoma to cases with signs of vestibular impairment, reducing the number of MRI exams, with considerable economic savings., (© Copyright by Società Italiana di Otorinolaringologia e Chirurgia Cervico-Facciale, Rome, Italy.)

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: Intracranial ganglioneuromas are very rare benign tumors of neural crest origin and generally arise from the peripheral nervous system or adrenal glands. Very few cases of intracranial ganglioneuroma arising from the trigeminal nerve have been reported in the literature, all in East Asia., Case Description: A 52-year-old male presented to his primary care physician for evaluation of right facial and periorbital pain for over 18 months. He also reported a two-week history of diplopia with right lateral gaze, which resolved spontaneously. An MRI brain with and without contrast revealed a 3.0 × 2.6 × 2.4 cm enhancing extra-axial mass involving right Meckel's cave with a posterior 1.0 × 1.4 cm lobular component protruding into the prepontine cistern. The patient underwent a right middle fossa craniotomy for a subtemporal surgical resection of the tumor. Histologic section of the tumor demonstrated mature ganglion cells with surrounding satellite cells in a schwannian rich stroma, consistent with mature type ganglioneuroma., Conclusions: To the best of our knowledge, this is the sixth case of trigeminal ganglioneuroma; however, it is the first case reported in the United States., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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.)

Objective: Here, we examine rates of intracranial tumor diagnoses in patients with and without comorbid psychiatric diagnoses to better understand how psychiatric disease may alter risk profiles for brain tumor diagnosis., Methods: We used a longitudinal version of the California Office of Statewide Health Planning and Development (OSHPD) database, which includes all inpatient admissions in California from 1995 to 2010. We examined patients with confirmed hospital admissions from 1997 to 2004. Patients with an intracranial tumor or psychiatric diagnosis on their first hospital admission were excluded. The primary outcome of interest was the diagnosis of intracranial tumor on any subsequent hospitalization within 5 years. Risk of tumor diagnosis was determined via Cox proportional hazard models adjusted for age, gender, race/ethnicity, and comorbidity burden. Subset analyses were performed for various tumor types., Results: The risk for diagnosis of an intracranial tumor within 5 years, as determined by the hazard ratio, was 1.61 (95% CI, 1.28-2.04) for bipolar, 1.59 (95% CI, 1.41-1.72) for anxious, and 1.34 (95% CI, 1.25-1.43) for depressed cohorts relative to controls. More specifically, the risk for diagnosis of a primary benign neoplasm was elevated in depressed patients, while the risk for diagnosis of a meningioma was elevated in depressed, anxious, and bipolar disorder patients., Conclusions: Patients admitted with certain psychiatric diagnoses appear more likely to be readmitted within 5 years with specific types of intracranial tumor diagnoses. The association between certain psychiatric diagnoses and subsequent brain tumor diagnosis most likely reflects the long-held belief that slow-growing tumors may first present as psychiatric symptoms before being diagnosed. Primary care physicians should consider the possibility of an underlying intracranial tumor in patients with new psychiatric diagnoses., (© Copyright 2016 Physicians Postgraduate Press, Inc.)

Facial nerve palsy causes disfigurement with cosmetic, functional and psychological repercussions. The facial nerve can be affected anywhere along its course. A comprehensive assessment considering all differential diagnoses is critical to optimal management, as prompt, appropriate therapy leads to better outcomes.

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.

We conducted a retrospective review to assess the clinical presentation of patients with tumor-related nonacute complete peripheral facial weakness or an incomplete partial facial paresis and to provide an algorithm for the evaluation and management of these patients. Our study population was made up of 221 patients-131 females and 90 males, aged 14 to 79 years (mean: 49.7)-who had been referred to the Facial Nerve Disorders Clinic at our tertiary care academic medical center over a 23-year period with a documented neoplastic cause of facial paralysis. In addition to demographic data, we compiled information on clinical signs and symptoms, radiologic and pathologic findings, and surgical approaches. All patients exhibited gradual-onset facial weakness or facial twitching. Imaging identified an extratemporal tumor in 128 patients (58%), an intratemporal lesion in 55 patients (25%), and an intradural mass in 38 (17%). Almost all of the extratemporal tumors (99%) were malignant, while 91% of the intratemporal and intradural tumors were benign. A transtemporal surgical approach was used in the 93 intratemporal and intradural tumor resections, while the 128 extratemporal lesions required a parotidectomy with partial temporal bone dissection. The vast majority of patients (97%) underwent facial reanimation. We conclude that gradual-onset facial paralysis or twitching may occur as a result of a neoplastic invasion of the facial nerve along its course from the cerebellopontine angle to the parotid gland. We caution readers to beware of a diagnosis of "atypical Bell's palsy."

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.