Infiltrative fibrous lesion of the facial nerve mimicking a facial nerve tumor.

PURPOSE: To report three cases of facial nerve lesions that were clinically expected to be facial nerve tumors but showed fibrotic infiltration without any apparent signs of a specific tumor on histopathological findings. We also aimed to investigate the clinical characteristics of these cases. METHODS: Medical records of patients who underwent surgery for facial nerve lesions were reviewed. RESULTS: All three cases initially had House-Brackmann (HB) grade IV-V facial nerve palsy. On radiological imaging, schwannoma or glomus tumor originating from the facial nerve was suspected. All patients underwent complete surgical removal of the neoplasm followed by facial nerve reconstruction using the sural nerve. The lesions were histologically confirmed as infiltrative fibrous lesions without tumor cells. In two cases, facial nerve palsy improved to HB grade III by nine months post-surgery, and there were no signs of recurrence on follow-up MRI. The other case, after 1 year of follow-up, showed persistence of HB grade V facial nerve palsy without any evidence of recurrence. CONCLUSION: Fibrotic lesions of the facial nerve could mimic primary facial nerve tumors. Clinicians should consider this condition even when a facial nerve tumor is suspected.

Intratemporal facial nerve neurofibroma causing facial paralysis in an infant: Case report and review of the literature.

This article describes the first recorded case of intratemporal neurofibroma in an infant. A literature review of all other existing cases of intratemporal neurofibroma is performed, finding that the majority of cases involve multiple segments and can be found in the mastoid segment most often. Most common symptoms described included facial paralysis, otalgia, and conductive hearing loss, respectively.

Facial Nerve Tumors in Children: Two Clinical Cases and a Review of the Literature.

We provide an extensive review of clinical features, diagnosis, and treatment of primitive facial nerve tumors in children, and report 2 recent personal observations. We conducted a comprehensive literature search through PubMed, Medline, and ScienceDirect and collected information on patients' age, symptoms, tumor types and sites, diagnostic procedures, surgical approaches, and outcomes. Overall, we reviewed 26 pediatric cases from 20 papers. About 69.2% of children presented with some degree of facial palsy. Other symptoms included hearing loss, dizziness, and tinnitus. 84.6% of tumors were schwannomas, followed by meningiomas, epithelioid hemangioendothelioma, and germ cell tumors. The geniculate ganglion was the most commonly affected segment of the facial nerve. A total of 92.3% of children received surgery as complete or partial tumor resection. Facial nerve function improved in 26.9% of children. No tumor recurrence was reported. Facial nerve tumors are extremely rare in children but should be considered in the differential diagnosis of facial palsy, even in newborns. Audiometric and radiologic examinations are necessary; radiologic imaging allows to determine tumor localization, and the correct surgical approach surgery is suggested in almost all cases.

Extensive Intratemporal Tumor-An Unusual Diagnosis.

A 21-year-old man presented with left-sided facial paralysis and sensorineural hearing loss; physical examination was otherwise normal. What is your diagnosis?

[Differential diagnosis and management of hemangioma at geniculate ganglion].

Objective: To investigate the clinical characteristics, differential diagnosis, treatments and prognosis of facial nerve hemangioma and schwannoma at genicular ganglion, so as to provide reference for clinical diagnosis and treatments of facial nerve tumor at genicular ganglion. Methods: Clinical data of 13 patients with facial nerve tumors at genicular ganglion confirmed by postoperative pathology in the Ninth People's Hospital affiliated to Shanghai Jiaotong University School of Medicine from March 2018 to April 2020 were retrospectively analyzed, including seven cases of hemangioma and six cases of schwannoma. There were eight males and five females. Their ages ranged from 20 to 65, with an average age of 40. The course of disease ranged from 3 to 118 months, with an average of 52 months. All the patients underwent preoperative HRCT of the temporal bone and facial nerve dynamic contrast-enhanced(DCE) MRI examinations. All the patients had detailed surgical procedures and at least one-year postoperative follow-up. Results: On HRCT of the temporal bone, (4/7) hemangioma at geniculate ganglion showed characteristic honeycomb appearance, while 6/6 schwannoma and 3/7 hemangiomas showed expansive bone changes. On DCE-MRI, geniculate ganglion hemangioma (7/7) showed characteristic "point-to-surface" enhancement, and schwannoma (6/6) showed characteristic "face-to-surface" enhancement. For five hemangioma-patients with HB-Ⅱ-Ⅳ before surgery, the facial nerve anatomy was completely preserved through transcanal endoscopic approach(TEA), and the facial nerve function improved one year after surgery (two cases of HB-I, two cases of HB-Ⅱ, and one case of HB-Ⅲ). For two patients, with preoperative facial nerve function HB-Ⅴ-Ⅵ, since their tumors was inseparable from the nerves, they were performed with facial nerve anastomosis during the surgery, and the facial nerve function was improved to HB-Ⅳ level one year after surgery. For six patients with meningioma whose facial nerve function was greater than or equal to HB-Ⅲ, based on the preoperative hearing level, the involved segments, and duration of facial paralysis, three of them were conducted surgeries through middle cranial fossa approach, one by translabyrinthine approach, and one via mastoid approach. Two patients among them with complete facial paralysis over three years preoperatively were not performed facial nerve anastomosis after total resections of the tumors, and there was no improvement in facial nerve function one year after surgery. Three patients underwent facial nerve anastomosis after total tumor resections, and their facial nerve function was HB-Ⅲ in one patient, HB-Ⅳ in two patients one year after surgery. One patient (preoperative HB-Ⅲ) had a normal hearing level preoperatively, and the tumor involved the labyrinth segment. To protect the hearing, partial tumor was resected through the middle cranial fossa approach, and facial nerve function improved to HB-Ⅱ one year after surgery. Conclusions: Temporal bone HRCT combined with DCE-MRI are useful for the differential diagnosis of hemangioma and schwannoma at geniculate ganglion and provide references for preoperative clinical decision makings. It is extremely necessary to select the appropriate surgical approach based on the patient's hearing and involved segments. For geniculate ganglion hemangioma, early surgery can improve the possibilities of anatomical integrity of facial nerve, thereby improving facial nerve function postoperatively.TEA is a kind of surgical method worth consideration, with the characteristics of minimally invasive, favorable postoperative features, and so on. For schwannoma, one-stage functional reconstruction of the facial nerve is recommended during the resection of the tumors because of the inevitable damage to the anatomical integrity of the facial nerve.

Aspiration cytology of facial nerve schwannoma of parotid gland: A rare diagnosis.

Schwannoma is a benign nerve sheath tumour rarely found in the head and neck region and much less commonly found in the intraparotid facial nerve. It is a slow-growing encapsulated tumour arising from differentiated Schwann cells or axonal nerve sheath. It can occur anywhere along the course of the facial nerve. Patients most commonly present with an asymptomatic swelling, in the absence of any signs of facial nerve palsy. Accordingly, diagnosis is usually difficult before surgical removal and histopathological examination. Here, we report a rare case of facial nerve schwannoma (FNS), diagnosed on fine needle aspiration cytology, in a 35-year-old male who presented with a painless, gradually increasing swelling in the right infra-auricular region for the last 2 years. His general examination revealed no signs of facial muscle weakness. The cytodiagnosis of intraparotid FNS was further confirmed by immunohistochemistry on cell block.

Psychometric Validation of the FACE-Q Craniofacial Module for Facial Nerve Paralysis.

Background: Systematic reviews have identified the need for a patient-reported outcome measure for facial nerve paralysis (FNP). The aim of this study was to determine the psychometric properties of FACE-Q Craniofacial module scales when used in a combined sample of children and older adults with FNP. Methods: Data were collected between December 2016 and December 2019. We conducted qualitative interviews with children and adults with FNP. FACE-Q data were collected from patients aged 8 years and older with FNP. Rasch measurement theory analysis was used to examine the reliability and validity of the relevant scales in the FNP sample. Results: Twenty-five patients provided 2052 qualitative codes related to appearance, physical, psychological, and social function. Many patient concerns were common across age. The field-test sample included 235 patients aged 8-81 years. Of the 13 scales examined, all 122 items had ordered thresholds and good item fit to the Rasch model. For 12 scales, person separation index values were ≥0.79 and Cronbach's alpha values were ≥0.82. The 13th scale's reliability values were ≥0.71. Conclusion: The FACE-Q Craniofacial module scales described in this study can be used to collect and compare evidence-based outcome data from children and adults with FNP.

Post-Irradiation Facial Neuromyotonia/Myokymia: A Hemifacial Spasm Mimic.

Background: Hemifacial spasm is diagnosed on a clinical base, with certain atypical features alerting the physician for mimics. Phenomenology shown: Hemifacial neuromyotonia/myokymia characterized by tonic hemifacial contraction followed by multifocal undulating hemifacial twitches. Educational value: These features are a red flag for (post-irradiation) facial neuromyotonia/myokymia which generally responds well to low dose carbamazepine.

Internal auditory canal hypoplasia associated with bilateral vestibulocochlear nerve aplasia and deviant facial nerve course: A case report and MRI findings.

The evaluation of internal auditory canals and cochlea has gained significant importance due to the increasing number of cochlear implantations worldwide. This region's anatomical study is essential for cochlear implant surgery using magnetic resonance imaging as the method of choice. We report a case of a 6-year-old male patient diagnosed with a rare bilateral malformation of the internal auditory canals associated with an aberrant course of the facial nerve and vestibulocochlear nerve aplasia. This report raises the importance of identifying this rare malformation for appropriate management and reinforces awareness of possible complications.

Magnetic resonance imaging of the caudal portion of the digastric muscle in canine idiopathic facial neuropathy.

Idiopathic is the most common etiology for acute onset of facial neuropathy in dogs with limited number of studies describing MRI characteristics. A retrospective, observational study was performed using archived records, aiming to describe the MRI features of the caudal portion of the digastric muscle in dogs diagnosed with idiopathic facial neuropathy and to determine correlation with prognosis. Client-owned dogs presented to a referral hospital between 2009 and 2019, diagnosed with unilateral idiopathic facial neuropathy and having undergone MRI, with images including the caudal portion of the digastric muscle, were included (n = 19). MRI appearance of the affected muscle, including degree of muscle atrophy, signal intensity, enhancement post-contrast, and enhancement characteristics of the affected facial nerve, was described and compared to the contralateral, clinically unaffected caudal portion of the digastric muscle. Correlation between MRI appearance and outcome at 1-month and 3-months following onset of clinical signs was investigated. The majority of patients demonstrated some degree of muscle atrophy (n = 17, 89%), hyperintensity in T2W (n = 17, 89%), and pre-contrast T1W (n = 15, 79%) images, as well as contrast enhancement of the affected muscle (n = 14, 74%) and affected facial nerve (n = 9, 47%). There was no statistically significant correlation between atrophy or enhancement of the affected caudal portion of the digastric muscle nor between enhancement of the affected facial nerve and outcome. Hyperintensity both in T2W images and pre-contrast T1W images was significantly correlated with a worse prognosis. Ensuring inclusion and evaluation of this muscle in MRI may therefore be indicated in canine idiopathic facial neuropathy.

Surgical management of intraoperatively diagnosed facial nerve schwannoma located at internal auditory canal and cerebellopontine angle - our experiences of 14 cases.

BACKGROUND: Facial nerve schwannomas located at internal auditory canal and cerebellopontine angle (IAC/CPA FNS) were diagnosed intraoperatively, it poses a therapeutic dilemma to the surgeon. OBJECTIVE: To report our experience in managing IAC/CPA FNS and to propose a treatment strategy. METHODS: A total of 14 patients with IAC/CPA FNS who were diagnosed intraoperatively and treated by operation between 2015 and 2019 were retrospectively studied. RESULTS: Unilateral hearing loss was the most common symptom and all these patients had normal facial nerve function preoperatively. Surgical approaches used in these patients including translabyrinthine (2 cases), retrosigmoid (RS) (11 cases), and middle cranial fossa (MCF) approach (1 case). Eight patients underwent partial resection, three patients underwent subtotal resection and three patients had complete tumor removal with facial nerve reconstruction. All partial resection patients and two patients underwent subtotal resection achieved a long-term HB grade I facial nerve function. The long-term facial nerve function of patients underwent complete resection and nerve grafting was no better than HB grade III.1 of the eight patients underwent partial resection experienced tumor regrowth during the follow-up. CONCLUSIONS: Partial or subtotal resection for IAC/CPA FNS may provide an opportunity of retaining excellent facial nerve function. Regular postoperative imaging is helpful to monitor the recurrence.

[A case of facial-onset sensory and motor neuronopathy (FOSMN) with cerebellar ataxia and abnormal decrement in repetitive nerve stimulation test].

A 59-year-old woman presented with a 7-year history of facial numbness on the left side, and gradual worsening of symptoms. Over several years, facial muscle weakness, dysarthria, tongue atrophy and fasciculation had progressed. Then, she developed cerebellar ataxia affecting the left extremities, in addition to earlier symptoms. Brain MRI revealed cerebellar atrophy, and 99mTc-SPECT depicted cerebellar hypoperfusion. A repetitive nerve stimulation test (RNS) indicated abnormal decrement in the nasalis and trapezius muscles on the left side. Facial-onset sensory and motor neuronopathy (FOSMN) was diagnosed. Administration of intravenous immunoglobulin resulted in improvement of some symptoms. Although cerebellar ataxia is not a common symptom of FOSMN, a case showing TDP-43-positive glial cytoplasmic inclusions in cerebellar white matter has been reported. Therefore, it is possible that FOSMN may cause cerebellum impairment in some patients. Furthermore, RNS positive rate in the trapezius muscle is known to be high in amyotrophic lateral sclerosis (ALS) patients. It is speculated that RNS of the affected muscles in FOSMN may show abnormal decrement by the same mechanisms as ALS.

Extratemporal intraparotid facial nerve schwannoma.

Schwannomas of the eighth nerve are common, usually found in syndromic association with neurofibromatosis-2. The occurrence of seventh nerve schwannoma, especially in its extratemporal course, is very rare. Here, we present a case report of an extratemporal facial nerve schwannoma diagnosed preoperatively with cytopathology and postoperative histopathologic confirmation. Histopathology provides the confirmatory diagnosis in such cases. An atypical diagnosis of neural schwannomas should be kept in mind when facial palsy is clinically encountered in the absence of any other aetiological factors.

Fibrovascular Tumor-Like Lesions of the Facial Nerve.

BACKGROUND: Facial nerve tumors (FNTs) are relatively rare benign lesions that arise from any segment of the facial nerve (FN). About half of all patients present with FN dysfunction, mainly long-standing or progressive facial paralysis. Diagnosis of an FNT is usually based on radiological imaging and confirmed by histological study. Most reported cases of FNTs are schwannomas and hemangiomas. OBJECTIVES: The aim of this study was to review 4 cases of lesions with clinical, radiological, and surgical findings that suggested an FNT, the pathology revealing a fibrovascular proliferation with no clear signs of a specific tumor. METHOD: Medical records of patients who had surgery due to an FN lesion were reviewed. Cases with known tumoral lesions were excluded. Four patients with tumor-like lesions were identified. Their imaging studies were re-evaluated. The pathological study included hematoxylin-eosin, Masson's trichrome, and immunohistochemistry for S100 protein, neurofilaments, CD31, Wilms' tumor 1 (WT1), and D240. RESULTS: The 4 cases revealed tumor-like fibrovascular lesions that could not be classified as typical pathological entities. All cases had a complete facial palsy preoperatively. Computed tomography and magnetic resonance imaging (MRI) suggested schwannoma or hemangioma. A complete excision was achieved, and a facial reconstruction was performed immediately after interruption. Postoperative FN function was improved in all cases. The histological study showed nervous tracts of normal morphology, with fibrous and vascular tissue interspersed in variable proportions. All cases showed areas of fibrosis with Masson's stain. In all cases, nervous tissue and Schwann cells tested positively for neurofilaments and S100, respectively. In vascular areas, endothelial cells stained positively for CD31, and negatively for D240 and WT1. CONCLUSIONS: Fibrovascular lesions of the FN may mimic primary FNTs, especially schwannomas and hemangiomas. Surgical excision with grafting or nerve transfer is the procedure of choice if a complete facial paralysis is found. This unusual condition should be considered when counseling patients with FN lesions. The lack of hyperintensity on MRI T2-weighted images may suggest the presence of fibrous tissue.

Idiopathic Intracranial Hypertension Triggering Hemifacial Spasm.

ABSTRACT: Idiopathic intracranial hypertension (IIH) is a syndrome associated with increased intracranial pressure without a clear underlying cause that is classically seen in young women. Patients typically present with headache and ocular findings, including disc edema and, less frequently, an abduction deficit. To make a diagnosis of IIH, other than cranial nerve 6 or 7 dysfunction, patients must have a normal neurologic examination. When cranial nerve 7 is affected patients can present with hemifacial spasm. We present the case of a young woman with IIH who had hemifacial spasm as one of the presenting symptoms. Her symptoms resolved once she was treated for IIH with acetazolamide.

Facial Nerve Involvement Accompanying Optic Neuritis Secondary to Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disorder.

ABSTRACT: We present the first reported case of facial nerve involvement accompanying an optic neuritis in myelin oligodendrocyte glycoprotein antibody-associated disorder.

Primary facial nerve paraganglioma: report and review of the literature.

This report describes the diagnosis and treatment of a patient with a rare primary facial nerve paraganglioma as well as a review of the current literature. A 60-year-old male patient presented to our clinic with a 4-month history of left-sided progressive facial paralysis House-Brackmann V. Biopsy taken during facial nerve (FN) decompression confirmed the diagnosis of paraganglioma. The left FN was sacrificed during resection of the mass and a 12-7 jump graft, using the left greater auricular nerve, was performed with acceptable outcomes. The rarity of these tumours does not discount their clinical importance or the necessity to include them in the differential when presented with unilateral FN paralysis. Investigation should begin with CT and MRI imaging to identify and localise the potential mass. Histologic confirmation requires tissue. While surveillance imaging is occasionally an option, often complete surgical resection of the mass and sacrifice of the nerve is necessary.

Case Report: Ocular Tilt Reaction with Internuclear Ophthalmoplegia and Multiple Cranial Nerve Palsies.

SIGNIFICANCE: Ocular tilt reaction (OTR) is an abnormal eye-head postural reaction that consists of skew deviation, head tilt, and bilateral ocular torsion. Understanding of the pathway of the vestibulo-ocular reflex (VOR) is essential because this will help to localize the pathology. PURPOSE: The aim of this study was to report a case of OTR with contralateral internuclear ophthalmoplegia (INO) and fifth and seventh cranial nerve palsies. CASE REPORT: A 51-year-old gentleman with underlying diabetes mellitus presented with sudden onset of diplopia for 3 days. On examination, his visual acuity was 20/30 bilaterally without a relative afferent pupillary defect. He had a right OTR consisting of a right head tilt, a skew deviation with a left eye hypertropia, and bilateral ocular torsion (right excyclotorsion and left incyclotorsion) with nystagmus. He also had a left adduction deficit and right abduction nystagmus consistent with a left INO. Ocular examination revealed evidence of proliferative diabetic retinopathy bilaterally. Two days after the initial presentation, the patient developed left seventh and fifth cranial nerve palsies. MRI showed left pontine infarction and multiple chronic lacunar infarctions. There was an incidental finding of a vascular loop compression on cisternal portions of the left trigeminal, facial, and vestibulocochlear nerves. Antiplatelet treatment was started on top of a better diabetic control. The diplopia was gradually resolved with improved clinical signs. In this case, the left pontine infarction had likely affected the terminal decussated part of the vestibulocochlear nerve from the right VOR pathway, medial longitudinal fasciculus, and cranial nerve nuclei in the left pons. CONCLUSIONS: The OTR can be ipsilateral to the lesion if the lesion is before the decussation of the VOR pathway in the pons, or it can be contralateral to the lesion if the lesion is after the decussation. In case of an OTR that is associated with contralateral INO and other contralateral cranial nerves palsy, a pathology in the pons that is contralateral to the OTR should be considered. Neuroimaging study can hence be targeted to identify the possible cause.