Refining the predictive value of preoperative apparent diffusion coefficient (ADC) by whole-tumor analysis for facial nerve outcomes in vestibular schwannomas.

BACKGROUND: Apparent diffusion coefficient (ADC) in MRI has been shown to correlate with postoperative House-Brackmann (HB) scores in patients with vestibular schwannoma despite limited methodology. To rectify limitations of single region of interest (ROI) sampling, we hypothesize that whole-tumor ADC histogram analysis will refine the predictive value of this preoperative biomarker related to postoperative facial nerve function. METHODS: Of 155 patients who underwent resection of vestibular schwannoma (2014-2020), 125 patients were included with requisite clinical and radiographic data. After volumetric analysis and whole-tumor ADC histogram, regression tree analysis identified ADC cutoff for significant differences in HB grade. Outcomes were extent of resection, facial nerve function, hospital length of stay (LOS), and complications. RESULTS: Regression tree analysis defined three quantitative ADC groups (× 10-6 mm2/s) as high (> 2248.77; HB 1.7), mid (1468.44-2248.77; HB 3.1), and low (< 1468.44; HB 2.3) range (p 0.04). The mid-range ADC group had significantly worse postoperative HB scores and longer hospital LOS. Large tumor volume was independently predictive of lower rates of gross total resection (p <0.0001), higher postoperative HB score (p 0.002), higher rate of complications (p 0.04), and longer LOS (p 0.003). CONCLUSIONS: Whole-tumor histogram yielded a robust regression tree analysis that defined three ADC groups with significantly different facial nerve outcomes. This likely reflects tumor heterogeneity better than solid-tumor ROI sampling. Whole-tumor ADC warrants further study as a useful radiographic biomarker in patients with vestibular schwannoma who are considering surgical resection.

Dengue-associated longitudinally extensive transverse myelitis.

We diagnosed a patient with dengue fever who developed acute onset of sensorimotor quadriparesis with bladder involvement, and facial nerve involvement. Despite initial negative results in routine investigations and cerebrospinal fluid analysis, spinal MRI confirmed longitudinally extensive transverse myelitis. The aetiological workup was negative, prompting an investigation into the presence of dengue in the cerebrospinal fluid, which returned positive. This case underscores the importance of considering rare neurological complications in dengue, the value of advanced diagnostic techniques and the potential effectiveness of tailored interventions in challenging cases.

Bionic blink improves real-time eye closure in unilateral facial paralysis.

Facial paralysis is the inability to move facial muscles thereby impairing the ability to blink and make facial expressions. Depending on the localization of the nerve malfunction it is subcategorised into central or peripheral and is usually unilateral. This leads to health deficits stemming from corneal dryness and social ostracization.Objective: Electrical stimulation shows promise as a method through which to restore the blink function and as a result improve eye health. However, it is unknown whether a real-time, myoelectrically controlled, neurostimulating device can be used as assistance to this pathological condition.Approach: We developed NEURO-BLINK, a wearable robotic system, that can detect the volitional healthy contralateral blink through electromyography and electrically stimulate the impaired subcutaneous facial nerve and orbicularis oculi muscle to compensate for lost blink function. Alongside the system, we developed a method to evaluate optimal electrode placement through the relationship between blink amplitude and injected charge.Main results: Ten patients with unilateral facial palsy were enrolled in the NEURO-BLINK study, with eight completing testing under two conditions. (1) where the stimulation was cued with an auditory signal (i.e. paced controlled) and (2) synchronized with the natural blink (i.e. myoelectrically controlled). In both scenarios, overall eye closure (distance between eyelids) and cornea coverage measured with high FPS video were found to significantly improve when measured in real-time, while no significant clinical changes were found immediately after use.Significance: This work takes steps towards the development of a portable medical device for blink restoration and facial stimulation which has the potential to improve long-term ocular health.

Extracranial transport of brain lymphatics via cranial nerve in human.

Extracranial waste transport from the brain interstitial fluid to the deep cervical lymph node (dCLN) is not extensively understood. The present study aims to show the cranial nerves that have a role in the transport of brain lymphatics vessels (LVs), their localization, diameter, and number using podoplanin (PDPN) and CD31 immunohistochemistry (IHC) and Western blotting. Cranial nerve samples from 6 human cases (3 cadavers, and 3 autopsies) were evaluated for IHC and 3 autopsies for Western blotting. The IHC staining showed LVs along the optic, olfactory, oculomotor, trigeminal, facial, glossopharyngeal, accessory, and vagus nerves. However, no LVs present along the trochlear, abducens, vestibulocochlear, and hypoglossal nerves. The LVs were predominantly localized at the endoneurium of the cranial nerve that has motor components, and LVs in the cranial nerves that had sensory components were present in all 3 layers. The number of LVs accompanying the olfactory, optic, and trigeminal nerves was classified as numerous; oculomotor, glossopharyngeal, vagus, and accessory was moderate; and facial nerves was few. The largest diameter of LVs was in the epineurium and the smallest one was in the endoneurium. The majority of Western blotting results correlated with the IHC. The present findings suggest that specific cranial nerves with variable quantities provide a pathway for the transport of wastes from the brain to dCLN. Thus, the knowledge of the transport of brain lymphatics along cranial nerves may help understand the pathophysiology of various neurological diseases.

Diagnostic Challenges and Imaging Considerations for Intraparotid Facial Nerve Schwannoma: A Case Report and Literature Review.

BACKGROUND A mass in the parotid gland usually indicates parotid gland neoplasia. Warthin tumors or pleomorphic adenomas are common differential diagnoses. Less frequently, other differential diagnoses and sites of origin are considered. Schwannomas are rare, benign tumors in the head and neck region. Even more rarely, these tumors occur in the intraparotid course of the facial nerve. In the following, we report about 2 patients in whom a mass in the right parotid gland was found incidentally during magnetic resonance imaging (MRI). CASE REPORT We reviewed data from the literature on intraparotid facial nerve schwannomas (IPFNS) and compared them with those from our cases. The focus was on data such as clinical history, clinical symptoms, electroneurography, and various imaging modalities, such as ultrasonography and MRI combined with diffusion-weighted imaging. CONCLUSIONS It is challenging to distinguish facial nerve schwannomas from other neoplasms. Patient's history, clinical symptoms, MRI examination with diffusion-weighted imaging, and high-resolution ultrasound imaging are decisive factors for diagnosis and should be performed when IPFNS is suspected. Diagnosis and therapy for IPFNS remain challenging. A wait-and-scan approach could be an option for patients with small tumors and good facial nerve function. On the other hand, patients with advanced tumors associated with limited facial nerve function can benefit from surgical approaches or stereotactic radiosurgery.

[Analysis of the outcome of 12 cases of facial nerve tumors].

Objective:This study aims to provide a comprehensive summary of the pathogenesis, screening modalities, treatment strategies, repair modalities and preliminary results associated with facial nerve tumors. Methods:A retrospective analysis was conducted on the clinical data of 12 patients with facial nerve tumors who were admitted to our department between May 2018 and February 2023. The study population consisted of 5 males and 7 females, with ages ranging from 35 to 90 years. Clinical symptoms observed in these patients included facial nerve palsy, hearing loss, tinnitus, headache, and otalgia, etc. The severity of facial nerve dysfunction was assessed using the House-Brackmann（H-B） facial nerve function classification, with 3 cases classified as grade Ⅰ, 4 cases as grade Ⅲ, 2 cases as grade Ⅳ, and 3 cases as grade Ⅴ. There was a total of 11 patients who presented with hearing loss. Among these patients, 7 cases were diagnosed with conductive hearing loss, 2 cases with sensorineural hearing loss, and 2 cases with mixed hearing loss. The selection of the observation or surgical route for tumor localization was based on clinical symptoms, facial nerve function grading, and imaging examination results including temporal bone CT and enhanced MRI. Specifically, the location of the tumor was selected for observation or the best surgical route: 2 cases were followed up for observation, 1 case underwent biopsy, and 9 cases underwent tumor resection（7 cases of trans-mastoid approach, 2 cases of combined parotid-mastoidal approach）, concurrent repair of the facial nerve（4 cases of auricular nerve grafting, 3 cases of facial nerve diversion anastomosis, 2 cases of peroneal nerve grafting）. （4 cases of auricular nerve graft, 3 cases of facial nerve diversion anastomosis and 2 cases of peroneal nerve grafting）. Periodic postoperative evaluation of facial nerve function was conducted. Results:1-year follow-up was available. Intraoperatively, it was observed that 66.7%（6 out of 9） of the facial nerve tumors were present in multiple segments. Among these segments, the vertical segment had the highest proportion, accounting for 77.8%（7 out of 9）, followed by the labyrinthine segment/geniculate ganglion with 66.7%（6 out of 9） and the horizontal segment with 55.6%（5 out of 9）. Postoperative pathology confirmed 8 cases with nerve sheath meningioma, Ⅰ with seminal fibroma and 1 with hemangioma. Postoperative facial nerve function was graded as H-B grade I in one patient）, grade Ⅲ in three, grade Ⅳ in four, grade Ⅴ in 2, and grade Ⅵ in 2 patients. The auditory outcomes following surgery are as follows: 8 individuals experienced postoperative hearing loss, while 2 individuals demonstrated postoperative hearing preservation. Conclusion:In the case of patients presenting with facial nerve palsy as their initial symptom, it is imperative to consider the potential presence of a facial nerve tumor. To determine the appropriate course of action, it is necessary to ascertain the size and location of the tumors through imaging examinations. This information will aid in the decision making process regarding whether surgical intervention is warranted, and so, the most suitable approach. Additionally, the choice of repair method during the operation should be guided by the extent of facial nerve defect.

Comparison of the Reliability of the House- Brackmann, Facial Nerve Grading System 2.0, and Sunnybrook Facial Grading System for the Evaluation of Patients with Peripheral Facial Paralysis.

BACKGROUND: To compare the reliability of the House-Brackmann (HB), Facial Nerve Grading System 2.0 (FNGS 2.0), and Sunnybrook Facial Grading System (SB) which are widely used in the evaluation of peripheral facial paralysis (PFP) patients. METHODS: Thirty-five video-recorded adult PFP patients were included in the study. The evaluators comprised 6 physicians. Evaluations were conducted twice independently, utilizing video recordings. Simultaneously, the evaluators were asked to keep time during the evaluation. For the analysis of reliability, Fleiss' kappa coefficient was used for the HB, and the intraclass correlation coefficient (ICC) was used for the FNGS 2.0 and SB. RESULTS: The mean evaluation time of 1 patient was found to be 1.06 ± 0.24, 1.47 ± 0.23, and 2.32 ± 0.41 minutes for the HB, FNGS 2.0, and SB, respectively. For interrater reliability, Fleiss' kappa for the HB was 0.495 and 0.403; ICC for the FNGS 2.0 was 0.966 and 0.958; ICC for the SB was 0.960 and 0.967 for the first and second measurements, respectively. For intrarater reliability, Fleiss' kappa for the HB was 0.391, 0.446, 0.564, 0.502, 0.626, and 0.455; ICC for the FNGS 2.0 was 0.87, 0.982, 0.966, 0.929, 0.933, and 0.948; ICC for the SB was 0.935, 0.96, 0.895, 0.941, 0.96, and 0.94 for the 6 raters, respectively. CONCLUSION: In the present study, statistically high intra- and interrater correlations were found for the FNGS 2.0 and SB, while a moderate correlation was found for the HB. Although the HB seems to be more practical, it has been concluded that the FNGS 2.0 and SB are more reliable.

Localization of the Vertical Part of the Facial Nerve in the High-Resolution Computed Tomography During Pediatric Cochlear Implantation.

BACKGROUND: This study proposed a classification of the vertical portion of the facial nerve (VPFN) location, incorporating the previous classifications regarding the posterior-to-anterior and medial-to-lateral dimensions. We also evaluated the implication of this proposed classification on the round window visibility during pediatric cochlear implantation (CI). METHODS: It was a retrospective multicenter observational cohort study. This study included 334 cases that underwent CI between 2015 and 2022 at multiple referral institutes. Two physicians evaluated the preoperative computed tomography images of 334 patients and determined the radiological type of the VPFN. These types were matched with intraoperative round window accessibility. RESULTS: The Spearman's correlation coefficient showed a strong correlation between the proposed VPFN type and the intraoperative round window visibility, as the P-value was <.001. CONCLUSION: This classification could provide the surgeon preoperatively with the precise location of the VPFN in the lateral-to-medial and posterior-to-anterior dimensions. Furthermore, this location classification of the VPFN was significantly correlated with intraoperative round window accessibility, with an accuracy of 90.42%. Therefore, types C and D were expected to have difficult accessibility into the round window, and more surgical interventions were needed to modify the posterior tympanotomy or use other approaches.

[Rehabilitation of patients with facial nerve neuropathy in cases of increased muscle tone on the healthy side of the face]. / Reabilitatsiya patsientov s neiropatiei litsevogo nerva pri povyshenii tonusa myshts na zdorovoi storone litsa.

OBJECTIVE: To evaluate the effectiveness of physical rehabilitation (PR) and injections of botulinum toxin type A (BTA) in the correction of muscles hypertonicity ty of the healthy side of the face or its prevention in patients with facial nerve neuropathy (FNN). MATERIAL AND METHODS: One hundred and forty-six patients with FNN of various etiologies with muscles hypertonicity of the healthy side or the risk of its development were studied. The term of treatment was 33 [10; 99] days. There are 88 women and 58 men aged 42 [34; 56] years. Diagnosis included clinical examination and stimulation electroneuromyography (ENMG, n=113; 77.4%) with assessment of M-wave amplitude asymmetry. All patients were prescribed a standard PR complex, in addition - relaxation of the muscles of the healthy side by injections of BTA (Incobotulinumtoxin A) - BTA group (n=38) or by special PR techniques - physiotherapy group (n=108). RESULTS: In both groups, patients were comparable in age, sex and severity of FNN at clinical assessment. But objectively, in the BTA group there were more symptomatic forms (63.2% vs 43.5%; p=0.038), cases of M-wave amplitude asymmetry over 90% in ≥2 branches (52.6% vs 18.5%; p=0.032). Displacement of the face midline was a factor limiting recovery (68.4% and 62%; χ2=0.495; p=0.482) in two groups. With amplitude asymmetry of more than 90% in ≥2 branches (a predictor of the risk of unfavorable outcome), a favorable outcome occurred more often in the BTA group (80% versus 45%; χ2=5.227; p=0.023). In case of amplitude asymmetry of less than 90% in all branches, a favorable outcome was observed in two groups. CONCLUSION: Indications for botulinum therapy in patients with FNN are deep prosoparesis or prosoplegia, muscles hypertonicity of the healthy side, displacement of the face midline, ENMG predictors of the risk of unfavorable outcome. PR is an effective method of prevention in patients with a favorable prognosis of outcome or treatment of mild forms.

How I do it: hearing preservation in large vestibular schwannomas using vestibular nerve fiber preservation technique.

BACKGROUND: To improve hearing function after resection of large vestibular schwannomas, we describe a strategy of vestibular-nerve-fiber preservation. Anatomical considerations and stepwise dissection are described. METHOD: Steps include locating the vestibular nerve at the brainstem and identifying a dissection plane between nerve fibers and tumor capsule. Using this plane to mobilize and resect tumor reduced manipulation and maintained vascularity of underlying cochlear and facial nerves. CONCLUSION: Preservation of hearing function is feasible in large vestibular schwannomas with vestibular-nerve-fiber preservation. Reducing manipulation and ischemic injury of underlying cochlear and facial nerves thereby helped facilitate hearing preservation, even in large tumors.

Hypoxic Bone Mesenchymal Stem Cell-Derived Exosomes Direct Schwann Cells Proliferation, Migration, and Paracrine to Accelerate Facial Nerve Regeneration via circRNA_Nkd2/miR-214-3p/MED19 Axis.

Background: Facial nerves have the potential for regeneration following injury, but this process is often challenging and slow. Schwann cells (SCs) are pivotal in this process. Bone mesenchymal stem cells (BMSC)-derived exosomes promote tissue repair through paracrine action, with hypoxic preconditioning enhancing their effects. The main purpose of this study was to determine whether hypoxia-preconditioned BMSC-derived exosomes (Hypo-Exos) exhibit a greater therapeutic effect on facial nerve repair/regeneration and reveal the mechanism. Methods: CCK-8, EdU, Transwell, and ELISA assays were used to evaluate the functions of Hypo-Exos in SCs. Histological analysis and Vibrissae Movements (VMs) recovery were used to evaluate the therapeutic effects of Hypo-Exos in rat model. circRNA array was used to identify the significantly differentially expressed exosomal circRNAs between normoxia-preconditioned BMSC-derived exosomes (Nor-Exos) and Hypo-Exos. miRDB, TargetScan, double luciferase assay, qRT-PCR and WB were used to predict and identify potential exosomal cirRNA_Nkd2-complementary miRNAs and its target gene. The function of exosomal circRNA_Nkd2 in facial nerve repair/regeneration was evaluated by cell and animal experiments. Results: This study confirmed that Hypo-Exos more effectively promote SCs proliferation, migration, and paracrine function, accelerating facial nerve repair following facial nerve injury (FNI) compared with Nor-Exos. Furthermore, circRNA analysis identified significant enrichment of circRNA_Nkd2 in Hypo-Exos compared with Nor-Exos. Exosomal circRNA_Nkd2 positively regulates mediator complex subunit 19 (MED19) expression by sponging rno-miR-214-3p. Conclusion: Our results demonstrated a mechanism by which Hypo-Exos enhanced SCs proliferation, migration, and paracrine function and facial nerve repair and regeneration following FNI through the circRNA_Nkd2/miR-214-3p/Med19 axis. Hypoxic preconditioning is an effective and promising method for optimizing the therapeutic action of BMSC-derived exosomes in FNI.

Post-parotidectomy facial nerve rehabilitation outcomes: Comparison between benign and malignant neoplasms.

BACKGROUND: Facial nerve dysfunction can be a devastating trouble for post-parotidectomy patients. OBJECTIVE: To assess rehabilitation outcomes concerning patients with post-parotidectomy facial nerve dysfunction, comparing benign versus malignant neoplasms. METHODS: Prospective study enrolling adults who underwent parotidectomy with facial nerve sparing between 2016 and 2020. The Modified Sunnybrook System (mS-FGS) was used for facial assessments. Physiotherapy began on the first post-operative day with a tailored program of facial exercises based on Neuromuscular Retraining, to be performed at home 3 times/day. From the first outpatient consultation, Proprioceptive Neuromuscular Facilitation was added to the treatment of cases with moderate or severe facial dysfunctions. RESULTS: Benign and malignant groups had a statistically significant improvement in mS-FGS (p < 0.001 and p = 0.005, respectively). There was no significant difference between groups regarding treatment duration or number of physiotherapy sessions performed. The history of previous parotidectomy resulted in more severe initial dysfunctions and worse outcome. Age over 60 years and initially more severe dysfunctions impacted the outcome. CONCLUSION: Patients with benign and malignant parotid neoplasms had significant and equivalent improvement in postoperative facial dysfunction following an early tailored physiotherapy program, with no significant difference in the final facial score, treatment duration, or number of sessions required.

Evaluating magnetic resonance imaging characteristics and risk factors for hemifacial spasm.

PURPOSE: The specific neurovascular compression (NVC) event responsible for the symptomatic manifestation of hemifacial spasm (HFS) remains difficult to assess accurately using magnetic resonance imaging (MRI). We aim to evaluate the MRI characteristics of HFS. METHOD: We retrospectively included patients with HFS and divided them into a test group (n = 186) and a validation group (n = 28). The presence, severity, and offending vessel type of NVC in each portion, and the orientation of the offending vessel around the facial nerve, were recorded. Conditional logistic regression analyses were performed to evaluate correlations using test group. The validation group was used to verify whether our findings improved diagnostic performance. RESULTS: Deformity in the proximal cisternal segment was significantly correlated with HFS occurrence (odds ratio [OR]: 256.58, p = .002), whereas contact was not (p = .233). Both contact and deformity in the root detachment point (OR: 19.98 and 37.22, p < .001 and p = .013, respectively) or attached segment (OR: 4.99 and 252.52, p = .001 and p < .001, respectively) were significantly correlated with HFS occurrence. Our findings improved specificity, positive predictive value, and accuracy of diagnosis than conventional diagnostic methods. The vertebral artery predominantly compress the facial nerve in the inferior-anterior position, the anterior inferior cerebellar artery predominantly in the inferior position, the posterior inferior cerebellar artery predominantly in the inferior position, vein predominantly in the posterior-superior position. CONCLUSIONS: This study further demonstrates that within the susceptible portion of facial nerve, different portions of the nerve respond differently to NVC. Each offending vessel has its own preferred conflict orientation. Our study offers reference for neurosurgeons in diagnosis and treatment.

Appropriate dosage, timing, and site of intramuscular injections of brain-derived neurotrophic factor (BDNF) promote motor recovery after facial nerve injury in rats.

INTRODUCTION/AIMS: Daily intramuscular injections of fibroblast growth factor 2 (FGF2) but not of brain-derived neurotrophic factor (BDNF) significantly improve whisking behavior and mono-innervation of the rat levator labii superioris (LLS) muscle 56 days after buccal nerve transection and suture (buccal-buccal anastomosis, BBA). We explored the dose-response of BDNF, FGF2, and insulin growth factor 2 (IGF2) on the same parameters, asking whether higher doses of BDNF would promote recovery. METHODS: After BBA, growth factors were injected (30 µL volume) daily into the LLS muscle over 14, 28, or 56 days. At 56 days, video-based motion analysis of vibrissal whisking was performed and the extent of mono- and poly-reinnervation of the reinnervated neuromuscular junctions (NMJs) of the muscle determined with immunostaining of the nerve with ß-tubulin and histochemical staining of the endplates with Alexa Fluor 488-conjugated α-bungarotoxin. RESULTS: The dose-response curve demonstrated significantly higher whisking amplitudes and corresponding increased mono-innervation of the NMJ in the reinnervated LLS muscle at concentrations of 20-30 µg/mL BDNF administered daily for 14-28 days after BBA surgery. In contrast, high doses of IGF2 and FGF2, or doses of 20 and 40 µg/mL of BDNF administered for 14-56 days had no effect on either whisking behavior or in reducing poly-reinnervation of endplates in the muscle. DISCUSSION: These data suggest that the re-establishment of mono-innervation of whiskerpad muscles and the improved motor function by injections of BDNF into the paralyzed vibrissal musculature after facial nerve injury have translation potential and promote clinical application.

[A sampling survey of intraoperative facial nerve monitoring in China].

Objective: This study aims to investigate the current application and the level of knowledge of intraoperative facial nerve monitoring among medical staff in China. Methods: A comprehensive online questionnaire was conducted among medical professionals across different regions in China from October 2022 to February 2023. The survey exclusively targeted departments specializing in otolaryngology, head and neck surgery, neurosurgery, and oral and maxillofacial surgery. The questionnaire covered various aspects including general information, intraoperative facial nerve monitoring practices, training history, indications for monitoring, parameters used during monitoring procedures, as well as factors influencing its implementation. Results: A total of 417 participants from 31 provincial, municipal, and autonomous regions were included. Intraoperative facial nerve monitoring was found to be implemented in 227 (54.4%,227/417) repondents of 53 institutions (24.9%, 53/213). The top three indications for implementing this technique were acoustic neuroma, parotid gland surgery, and modified middle ear surgery (mastoidectomy). Herein 81.1%(184/227) medical staff involved in intraoperative facial nerve monitoring had received relevant training, 57.3%(130/227)-92.1%(209/227) reported a lack of clear description regarding recording thresholds, stimulation currents/frequencies/wave widths. Conclusion: The majority of the institutions surveyed have not yet adopted intraoperative facial nerve monitoring. Furthermore, significant gaps concerning the procedure exist. It is imperative to establish standards or guidelines to promote its better development and application.

Physiopathologic Bases of Moebius Syndrome: Combining Genetic, Vascular, and Teratogenic Theories.

Moebius syndrome (MBS) is a congenital cranial dysinnervation disorder (CCDD) characterized by a bilateral palsy of abducens and facial cranial nerves, which may coexist with other cranial nerves palsies, mostly those found in the dorsal pons and medulla oblongata. MBS is considered a "rare" disease, occurring in only 1:50,000 to 1:500,000 live births, with no gender predominance. Three independent theories have been described to define its etiology: the vascular theory, which talks about a transient blood flow disruption; the genetic theory, which takes place due to mutations related to the facial motor nucleus neurodevelopment; and last, the teratogenic theory, associated with the consumption of agents such as misoprostol during the first trimester of pregnancy. Since the literature has suggested the existence of these theories independently, this review proposes establishing a theory by matching the MBS molecular bases. This review aims to associate the three etiopathogenic theories at a molecular level, thus submitting a combined postulation. MBS is most likely an underdiagnosed disease due to its low prevalence and challenging diagnosis. Researching other elements that may play a key role in the pathogenesis is essential. It is common to assume the difficulty that patients with MBS have in leading an everyday social life. Research by means of PubMed and Google Scholar databases was carried out, same in which 94 articles were collected by using keywords with the likes of "Moebius syndrome," "PLXND1 mutations," "REV3L mutations," "vascular disruption AND teratogens," and "congenital facial nerve palsy." No exclusion criteria were applied.

Surgical management of nervus intermedius neuralgia: A report of 4 cases and literature review.

BACKGROUND: Nervus intermedius neuralgia (NIN) is characterized by paroxysmal episodes of sharp, lancinating pain in the deep ear. Unfortunately, only a few studies exist in the literature on this pain syndrome, its pathology and postoperative outcomes. METHOD: We conducted a retrospective review of four cases diagnosed with NIN who underwent a neurosurgical intervention at our center from January 2015 to January 2023. Detailed information on their MRI examinations, intraoperative findings and other clinical presentations were obtained, and the glossopharyngeal and vagus nerves were isolated for immunohistochemistry examination. RESULTS: A total of 4 NIN patients who underwent a microsurgical intervention at our institution were included in this report. The NI was sectioned in all patients and 3 of them underwent a microvascular decompression. Of these 4 patients, 1 had a concomitant trigeminal neuralgia (TN), and 1 a concomitant glossopharyngeal neuralgia (GPN). Three patients underwent treatment for TN and 2 for GPN. Follow-up assessments ranged from 8 to 99 months. Three patients reported complete pain relief immediately after the surgery until last follow-up, while in the remaining patient the preoperative pain gradually resolved over the 3 month period. Immunohistochemistry revealed that a greater amount of CD4+ and CD8+ T cells had infiltrated the glossopharyngeal versus vagus nerve. CONCLUSIONS: NIN is an extremely rare condition showing a high degree of overlap with TN/GPN. An in depth neurosurgical intervention is effective to completely relieve NIN pain, without any serious complications. It appears that T cells may play regulatory role in the pathophysiology of CN neuralgia.