A case report of fifteen-and-a-half syndrome.

BACKGROUND: Fifteen-and-a-Half Syndrome is an uncommon clinical presentation characterized by the coexistence of one-and-a-half syndrome and bilateral facial palsy. In this study, we provide a comprehensive description of symptom evolution and imaging changes in a patient with Fifteen-and-a-Half Syndrome. CASE PRESENTATION: A 54-year-old male presented with sudden onset of one-and-a-half syndrome, which gradually progressed to fifteen-and-a-half syndrome. The final diagnosis was confirmed to be pontine infarction which occurred at the midline of the pontine tegmentum. CONCLUSION: This case highlights the diverse and progressive early clinical manifestations associated with Fifteen-and-a-half Syndrome. Currently, all reported cases of this syndrome are linked to brainstem infarction; however, early differential diagnosis is crucial to ensure prompt initiation of appropriate treatment for affected patients.

Future treatment options for facial nerve palsy: a review on electrical stimulation devices for the orbicularis oculi muscle.

Facial nerve palsy can cause diminished eyelid closure (lagophthalmos). This occurs due to functional deficits of the orbicularis oculi muscle, potentially leading to sight-threatening complications due to corneal exposure. Current management options range from frequent lubrication with eye drops, to the use of moisture chambers and surgery. However, achieving functional restoration may not always be possible. Recent efforts have been directed towards the support of orbicularis oculi muscle function through electrical stimulation. Electrical stimulation of the orbicularis oculi muscle has been demonstrated as feasible in human subjects. This article offers a comprehensive review of electrical stimulation parameters necessary to achieve full functionality and a natural-looking eye blink in human subjects. At present, readily available portable electrical stimulation devices remain unavailable. This review lays the foundation for advancing knowledge from laboratory research to clinical practice, with the ultimate objective of developing a portable electrical stimulation device. Further research is essential to enhance our understanding of electrical stimulation, establish safety standards, determine optimal current settings, and investigate potential side effects.

Facial nerve palsy after middle meningeal artery embolization for chronic subdural hematoma: a case report.

Middle meningeal artery embolization (MMAE) has emerged as a safe and efficacious alternative to surgery for the treatment of new or recurrent chronic subdural hematoma (CSDH). Several complications such as facial palsy may suddenly occur even in the absence of evident dangerous anastomoses in the angiogram. We herein present a case-report of left facial nerve palsy after MMAE.

Diagnosis and management of facial nerve palsy secondary to granulomatosis with polyangiitis - A systematic review.

OBJECTIVE: Granulomatosis with polyangiitis is associated with otolaryngologic complaints in 70-95 % of cases, with the most common being serous otitis media. In rare cases, patients may experience facial nerve palsy in conjunction with otologic or nasal symptoms; and, often, initially present to an otolaryngologist. It is important for healthcare professionals to be able to recognize the nuisances of facial nerve palsy as a potential presentation of granulomatosis with polyangiitis. STUDY DESIGN: Systematic review. METHODS: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Protocol, PubMed and MED-LINE Databases were queried for articles published from January 2007 to December 2022 describing facial nerve palsy in the context of Granulomatosis with polyangiitis, formerly known as Wegener's Granulomatosis. The keywords included "facial nerve palsy", "facial palsy", "granulomatosis with polyangiitis", "Wegener's granulomatosis", "ANCA positive" in the title/abstract. All full-text articles available in English were screened, including single case presentations. Abstracts, commentaries, and publications deemed outside the scope of our study aims were excluded from review. After removal of duplicate articles, a total of 85 articles were screened. After applying inclusion and exclusion criteria, 14 articles were included in the review. RESULTS: There were a total of 28 reports of facial nerve palsy in the literature in patients who were eventually diagnosed with granulomatosis with polyangiitis. The patients' ages ranged from 14 to 68 years old. None of the patients had been previously diagnosed with GPA, and a majority of them presented initially with other otologic symptoms. Hearing loss was reported in 24 patients (86 %), otalgia was present in 11 patients (39 %), and otorrhea was present in 6 patients (21 %). Bilateral facial paralysis was reported in 10 patients in the literature (36 %). In total, 16 patients underwent surgery for facial paralysis: 6 tympanomastoidectomies, 4 mastoidectomies, 2 explorative tympanotomies. Surgery was generally considered ineffective in resolving facial weakness. All patients ended up receiving some combination of steroids and immunosuppressant, most commonly prednisolone and cyclophosphamide or rituximab, which was eventually transitioned to azathioprine for maintenance. Unlike auditory thresholds, which remained decreased in two patients, all patients recovered facial function following appropriate medical treatment of their vasculitis. CONCLUSIONS: Facial nerve paralysis in patients with granulomatosis with polyangiitis is a rare but treatable phenomenon. In patients with intractable otitis media, unresolving facial palsy, or a combination of otologic issues, it is important to consider GPA as a possible source. The prognosis for facial function appears to be excellent in patients who undergo appropriate treatment for vasculitis, but further studies are needed for confirmation.

The use of basic fibroblast growth factor to treat intractable Bell's palsy administered via transcanal endoscopic ear surgery.

PURPOSE: Facial nerve decompression surgery is an invasive procedure which has hitherto been the main option for patients with severe intractable Bell's palsy which is resistant to drug treatment. We have developed a new salvage treatment for such patients by using minimally invasive transcanal endoscopic ear surgery (TEES) to deliver the biological regenerative agent, basic fibroblast growth factor (bFGF), to the damaged facial nerve. MATERIALS AND METHODS: An endoscopic salvage treatment group was studied prospectively and was made up of severe intractable Bell's palsy patients who did not respond to high dose steroid treatment and had an ENoG value of 5 % or less. This surgery group was retrospectively compared to a similar control group who had received high dose steroid only. RESULTS: Complete recovery to House-Brackmann (HB) Grade I was achieved by 44.8 % of the endoscopic salvage treatment group which was significantly higher than the 21.2 % of the control group at one-year follow up. Patients with an ENoG value of 1 % to 5 % exhibited a significantly higher complete recovery rate of 71.4 % in the endoscopic salvage treatment group than the 28.6 % of the control group. In addition, no complications were observed including hearing loss. CONCLUSIONS: bFGF delivered via TEES shows considerable promise as a new salvage treatment of severe intractable Bell's palsy that is resistant to high dose steroid treatment without the risks presented by facial nerve decompression surgery.

Serum levels of heavy metals in patients with Bell's palsy: a case-control study.

PURPOSE: The exact etiology of Bell's palsy (BP) remains unknown, while its potential etiopathology includes neuritis and inflammation-related demyelination as in optic neuritis. It has been reported that disruption of heavy metal homeostasis may be associated with the inflammatory process of optic neuritis; therefore, heavy metals may be involved in the pathogenesis of facial nerve neuritis. In this study, we aimed to investigate serum levels of heavy metals including essential elements [iron (Fe), zinc (Zn), copper (Cu), cobalt (Co), and manganese (Mn)], and nonessential elements [lead (Pb) and cadmium (Cd)] in patients with BP. METHODS: The study included 25 patients with BP and 31 healthy volunteers. For each participant, serum levels of essential and nonessential elements were measured using the atomic absorption spectrophotometer method. RESULTS: Serum levels of essential elements were significantly lower in the patient group compared to the control group (p < 0.001, for each). Serum levels of Pb increased in the patient group compared to the control group although no significant difference was achieved (p = 0.105). In contrast, serum Cd levels increased significantly in the patient group compared to the control group (p < 0.001). CONCLUSION: Our findings suggest that decreased essential and increased nonessential elements may be associated with BP and thus, serum concentrations of these elements should be taken into account in BP. Studies are warranted to determine the role of these elements in treatment of BP.

Patterns and timing of recovery from facial nerve palsy after nerve-sparing parotid surgery: the role of neuromuscular retraining.

OBJECTIVES: Among the complications of parotid surgery, facial palsy is frequent and burdened by high functional and social impact for the patient. There are few data on the efficacy of facial neuromuscular retraining (FNR) in patients with facial palsy after parotid surgery, and no data exist on its impact in timing and extent of recovery. MATERIAL AND METHODS: A retrospective study was conducted on patients undergoing FN sparing parotid surgery and suffering from postoperative facial palsy. Among 400 patients undergoing surgery between July 2016 and May 2023, those with the preservation of the FN and onset of facial palsy were selected. Nerve function was evaluated during 2 years follow up using the House-Brackman (H&Bs) and Sunnybrook scales (SBs). RESULTS: A total of 46 patients undergoing partial or total parotidectomy were included. At discharge 18 patients (39,1%) had IV to VI grade paralysis according to the H&Bs and the mean SBs value was 54. At 2 and 6 months after surgery, the average value of Sunnybrook increased to 76.5 and 95.4 respectively. After 12 months no patients with IV to VI grade paralysis were represent in our cohort. Two years after surgery, only five patients (10.9%) had persistent grade II paralysis according to HBs. CONCLUSIONS: Our study supports the efficacy of FNR in the rehabilitation of facial paralysis after nerve-sparing parotidectomy. The greater functional improvement is achieved within the first 6 months of rehabilitation. A significant improvement is detected still after 18 months, supporting the importance of long rehabilitation for patients without complete recovery after the first year.

Predictive role of facial nerve palsy improvement in malignant external otitis.

PURPOSE: To evaluate the effects of different factors on facial nerve palsy improvement in patients with malignant external otitis (MEO) and the predictive role of improvement on MEO. METHODS: Data were collected from all MEO patients with facial paralysis who were hospitalized between 2012 and 2017 at a tertiary referral center. We contacted patients at least 6 months after their admission to evaluate their facial nerve function and survival rate. RESULTS: In a study of 19 samples with a mean age of 69.1 years, 9 patients (47.7%) had some or complete improvement, while 10 (52.6%) had no or very minimal improvement. In this study, there was no statistically significant difference between patients with and without facial nerve palsy improvement in terms of age, sex, usage of antifungal treatment alongside antibiotics, duration of hospital stays, HbA1c level, presentation of hearing loss and vertigo, the severity of facial palsy, comorbidity score, mean of fasting blood sugar, leukocytosis, first ESR and ESR drop, CRP and physiotherapy. We found a positive correlation between improving facial palsy and patients' survival rates. CONCLUSION: Considering the possible influence of facial paralysis improvement prognosis on MEO patients' survival, it could affect our approach to the disease.

Severely complicated ear infection in a patient treated with ixekizumab: a case report.

We report a case of a severe ear infection in a 35-year-old man treated with ixekizumab for psoriasis. Ixekizumab is a humanized monoclonal antibody that selectively prevents the interaction between interleukin 17 A and its receptor. Biologicals like ixekizumab are used to achieve symptom relief in autoimmune diseases including psoriasis. Unlike the mild upper respiratory tract infections usually described as side-effects of this treatment, we report a case of a patient who presented with a severe otitis media, complicated with a facial paresis and nasopharyngeal abscess. To the best of our knowledge, this is the first case presenting a severe, complicated ear infection as a possible side effect of ixekizumab. We conclude that when using ixekizumab, vigilance for upper airway infections is needed and if necessary, interruption of therapy should be considered. However, further research is needed to confirm this hypothesis.

Delayed facial nerve palsy after vestibular schwannoma resection: risk factors, extent and prognosis.

INTRODUCTION: Facial nerve palsies may develop during the postoperative period of microsurgical removal of vestibular schwannomas (VSs), even after normal facial function for days or weeks after surgery. The aim of this study was to identify the pathomechanism and predictive factors of delayed palsy. MATERIAL AND METHOD: The clinical data of 193 patients who underwent vestibular schwannoma surgery between 2012 and 2021 were retrospectively analyzed. A total of 134 patients were included. The patients showed intact facial nerve function up to 24 h after surgery. All patients (n = 20) with palsy from postoperative day 4 were included and collectively referred to as delayed facial nerve palsy (DFNP). Various factors were checked using a binomial regression analysis. RESULTS: The mean age of patients with DFNP was 57.8 years (55% female, 45% male). 70% had VS with KOOS ≥ 3, and 60% underwent surgery via a translabyrinthine approach Among the 16 patients with DFNP-related neurotropic pathogens, 25% were seropositive for herpes simplex virus. Most patients (n = 9/20) experienced onset of palsy between postoperative days 6 and 10. Of the four variables included in the significance test, three were significant: KOOS ≥ 3 (p < .04), ipsilateral vestibular organ failure (p < .05), and age group (p < .03). After therapy, 100% of patients recovered almost complete facial nerve function. The parameters mentioned above (KOOS classification and ipsilateral vestibular dysfunction) could be proven risk factors for the occurrence of DFNP.

Neuromuscular diseases associated with COVID-19 vaccines: a systematic review and pooled analysis of 258 patients.

BACKGROUND: Neuromuscular diseases (NMD) emerged as one of the main side effects of the COVID-19 vaccination. We pooled and summarized the evidence on the clinical features and outcomes of NMD associated with COVID-19 vaccination. METHODS: We comprehensively searched three databases, Medline, Embase, and Scopus, using the key terms covering "Neuromuscular disease" AND "COVID-19 vaccine", and pooled the individual patient data extracted from the included studies. RESULTS: A total of 258 NMD cases following COVID-19 have been reported globally, of which 171 cases were Guillain-Barré syndrome (GBS), 40 Parsonage-Turner syndrome (PTS), 22 Myasthenia Gravis (MG), 19 facial nerve palsy (FNP), 5 single fiber neuropathy, and 1 Tolosa-Hunt syndrome. All (100%) SFN patients and 58% of FNP patients were female; in the remaining NMDs, patients were predominantly male, including MG (82%), GBS (63%), and PTS (62.5%). The median time from vaccine to symptom was less than 2 weeks in all groups. Symptoms mainly appeared following the first dose of vector vaccine, but there was no specific pattern for mRNA-based. CONCLUSION: COVID-19 vaccines might induce some NMDs, mainly in adults. The age distribution and gender characteristics of affected patients may differ based on the NMD type. About two-thirds of the cases probably occur less than 2 weeks after vaccination.

Clinical manifestations of Lyme neuroborreliosis in children: a review.

Lyme neuroborreliosis (LNB) is a manifestation of Lyme disease involving the central and peripheral nervous system. It is caused by the spirochete Borrelia burgdorferi, transmitted by tick bites to a human host. Clinical signs of LNB develop after the dissemination of the pathogen to the nervous system. The infection occurs in children and adults, but the clinical manifestations differ. In adults, painful meningoradicultis is the most common manifestation of LNB, while children often present with facial nerve palsy and/or subacute meningitis. Subacute headache can be the only manifestation of LNB in children, especially during the summer months in Lyme disease-endemic regions. Non-specific symptoms, such as loss of appetite, fatigue or mood changes, may also occur, especially in young children. A high level of suspicion and early recognition of the various clinical manifestations presented by children with LNB is essential to minimize delay in diagnosis and optimize management. This review provides an overview of the spectrum of clinical manifestations, and discusses diagnosis, antibiotic treatment, and clinical outcome of LNB in children.

Persistent facial nerve palsy after middle meningeal artery embolization for subdural hematoma: a case report.

OBJECTIVE: To describe a case of persistent facial nerve palsy after middle meningeal artery (MMA) embolization for chronic subdural hematoma (cSDH). METHODS: A literature search was conducted for publications within the last 10 years of facial nerve palsy following cerebral circulation embolization procedures. RESULTS: With inconsistencies between previously believed pathophysiology and clinical features, other mechanisms causing cSDH such as angiogenesis and capillary formation have been proposed. MMA embolization has evolved as a therapeutic approach to reduce recurrence of subdural hematoma; however, postoperative neural complications such as cranial nerve palsies are poorly described in the literature. CONCLUSIONS: cSDH is increasingly more common and is on trajectory to become the most prevalent cranial neurosurgical condition. MMA embolization is described as a safe and minimally invasive procedure; however, as a relatively new procedure further research is needed to elucidate associated complications.

Sutureless transconjunctival insertion of eyelid gold weight.

OBJECTIVE: Surgical intervention for paralytic lagophthalmos has been gold weight implant through supratarsal crease incision for decades. The aim of this study is to propose a modified novel minimally invasive approach that can be described as sutureless and transconjunctival placement of eyelid weights. METHOD: Unilateral eyelid gold weights were implanted in six patients due to paralytic lagophthalmos secondary to peripheral facial nerve palsy. The patients were followed for an average of 6 months. RESULTS: Functional and aesthetically desired results were obtained in all six patients with suture-free transconjunctival placement of the eyelid weight. The patients did not experience any discomfort and avoided the burden of suture removal after the surgery. No complications developed in six patients during the postoperative period. CONCLUSION: Sutureless transconjunctival insertion of eyelid weight without external incision and suturing is practical, relatively easy and fast to perform. It preserves attachment of the levator muscle to the tarsus and presents functional results similar to conventional method. Fixing the implant with sutures to the tarsal plate is not needed. Sutureless of this method avoids external wound care, burden of suture removal for both surgeons and patients, and hence, suture related complications are eliminated.

Treatment of extracapsular fractures of the mandibular condylar process: A retrospective evaluation of 377 cases.

BACKGROUND/AIM: Mandibular condylar fractures represent 25%-35% of all mandibular fractures. Despite profound research, there is still a controverse debate about treating these fractures conservatively or by open reduction and internal fixation (ORIF). The aim of this study is to analyse the outcome after open and closed treatment of extracapsular mandibular condyle fractures regarding general characteristics, post-treatment malocclusion, facial nerve palsy (FNP), maximum mouth opening (MMO) and parotid complications. METHODS: A retrospective cohort of 377 fractures (350 open, 27 closed treatment) was reviewed by reference to clinical and radiological pre- and postoperative documentation. Follow-up period was 12 months. Pearsons' chi-square-test, correlations, Kruskal-Wallis test and t-test were carried out for statistical analysis. RESULTS: The dominant type of fracture was type II in Spiessl and Schroll classification (50.1%). In the open treated fractures, the most common approach was retromandibular transparotid (91.7%). Post-treatment malocclusion occurred in 18.0% and was significantly increased in bilateral fractures (p = .039), in luxation fractures (p = .016) and in patients with full dentition (p = .004). After open reduction and internal fixation (ORIF), temporary FNP was documented in 7.1% whereas a permanent paresis occurred in 1.7%. FNP was significantly associated with high fractures (p = .001), comminution (p = .028) and increased duration of surgery (p = .040). Parotid complications were significantly associated with revision surgery (p = .009). Post-treatment reduction of MMO mainly occurred in female patients (p < .001) as well as in patients with bilateral fractures (p < .001), high fractures (p = .030) and concomitant mandibular (p = .001) and midfacial fractures (p = .009). CONCLUSION: Malocclusion seems to be the most frequent long-term complication after open reduction and osteosynthesis of extracapsular mandibular condyle fractures. We suggest ORIF by a transparotid approach to be an appropriate treatment with a low complication rate regarding especially FNP for extracapsular fractures of the mandibular condyle.

Immune-Nutritional Status as a Novel Prognostic Predictor of Bell's Palsy.

INTRODUCTION: The prognosis of Bell's palsy, idiopathic facial nerve palsy (FNP), is usually predicted by electroneuronography in subacute phase. However, it would be ideal to establish a reliable and objective examination applicable in acute phase to predict the prognosis of FNP. Immune-nutritional status (INS) calculated from peripheral blood examination is recently reported as the prognostic factor in various disease. However, the validity of INS as the prognostic factor in Bell's palsy is not well known. Thus, we conducted a retrospective study to investigate the usefulness of INS as prognostic predictors of Bell's palsy. METHODS: We reviewed the medical records of 79 patients with Bell's palsy and divided into two groups as "complete recovery" and "incomplete recovery" groups. Clinical features such as severity of FNP and INS, including neutrophil-lymphocyte ratio (NLR), lymphocyte-monocyte ratio (LMR), prognostic nutritional index (PNI), and controlling nutrition status (CONUT) score, were assessed. RESULTS: In univariate analysis, statistically significant differences were observed in clinical score of facial movement, NLR, LMR, PNI, and CONUT score at the initial examination between the two groups (p < 0.05). Furthermore, in multivariate analysis, statistically significant differences were also observed in facial movement score and PNI at the initial examination (p < 0.05). CONCLUSION: Immune and nutritional condition play important roles in the pathogenesis of Bell's palsy, suggesting that INS would be one of the useful prognostic factors in Bell's palsy.

Applicability of contemporary quality indicators in vestibular surgery-do they accurately measure tumor inherent postoperative complications of vestibular schwannomas?

BACKGROUND: Due to rising costs in health care delivery, reimbursement decisions have progressively been based on quality measures. Such quality indicators have been developed for neurosurgical procedures, collectively. We aimed to evaluate their applicability in patients that underwent surgery for vestibular schwannoma and to identify potential new disease-specific quality indicators. METHODS: One hundred and three patients operated due to vestibular schwannoma were subject to analysis. The primary outcomes of interest were 30-day and 90-day reoperation, readmission, mortality, nosocomial infection and surgical site infection (SSI) rates, postoperative cerebral spinal fluid (CSF) leak, facial, and hearing function. The secondary aim was the identification of prognostic factors for the mentioned primary outcomes. RESULTS: Thirty-day (90-days) outcomes in terms of reoperation were 10.7% (14.6%), readmission 9.7% (13.6%), mortality 1% (1%), nosocomial infection 5.8%, and SSI 1% (1%). A 30- versus 90-day outcome in terms of CSF leak were 6.8% vs. 10.7%, new facial nerve palsy 16.5% vs. 6.1%. Hearing impairment from serviceable to non-serviceable hearing was 6.8% at both 30- and 90-day outcome. The degree of tumor extension has a significant impact on reoperation (p < 0.001), infection (p = 0.015), postoperative hemorrhage (p < 0.001), and postoperative hearing loss (p = 0.026). CONCLUSIONS: Our data demonstrate the importance of entity-specific quality measurements being applied even after 30 days. We identified the occurrence of a CSF leak within 90 days postoperatively, new persistent facial nerve palsy still present 90 days postoperatively, and persisting postoperative hearing impairment to non-serviceable hearing as potential new quality measurement variables for patients undergoing surgery for vestibular schwannoma.

Discerning a smile - The intricacies of analysis of post-neck dissection asymmetry.

INTRODUCTION: Iatrogenic facial nerve palsy is distressing to the patient and clinician. The deformity is aesthetically displeasing, and can be functionality problematic for oral competence, dental lip trauma and speech. Furthermore such injuries have litigation implications. Marginal mandibular nerve (MMN) palsy causes an obvious asymmetrical smile. MMN is at particular risk during procedures such as rhytidoplasties, mandibular fracture, tumour resection and neck dissections. Cited causes for the high incidence are large anatomical variations, unreliable landmarks, an exposed neural course and tumour grade or nodal involvement dictating requisite nerve sacrifice. An alternative cause for post-operative asymmetry is damage to the cervical branch of the facial nerve or platysmal dysfunction due to its division. The later tends to have a transient course and recovers. Distinction between MMN palsy and palsy of the cervical branch of the facial nerve or platysma division should therefore be made. In 1979 Ellenbogen differentiated between MMN palsy and "Pseudo-paralysis of the mandibular branch of the facial nerve". Despite this, there is paucity in the literature & confusion amongst clinicians in distinguishing between these palsies, and there is little regarding these post-operative sequelae and neck dissections. METHOD: This article reflects on the surgical anatomy of the MMN and cervical nerve in relation to danger zones during lymphadenectomy. The authors review the anatomy of the smile. Finally, case studies are utilised to evaluate the differences between MMN palsy and its pseudo-palsy to allow clinical differentiation. CONCLUSION: Here we present a simple method for clinical differentiation between these two prognostically different injuries, allowing appropriate reassurance, ongoing therapy & management.

Facial nerve paresis in the course of masked mastoiditis as a revelator of GPA.

PURPOSE: The aim of this study was to present a series of 6 patients with facial nerve palsy and masked mastoiditis which constituted as revelators of localized granulomatosis with polyangiitis (GPA) and to evaluate the utility of the ACR/EULAR 2017 provisional classification criteria for GPA in such cases. METHODS: Study group included 58 patients with GPA. Cases with facial nerve palsy and masked mastoiditis were thoroughly analyzed. RESULTS: The mean age of patients was 37 years. All manifested unilateral facial nerve palsy and hearing loss, while only 2 reported aural complaints suggesting inflammatory cause of the disease. All cases were qualified for surgical intervention. Intraoperative findings were similar: granulation tissue in tympanic cavity and/or pneumatic spaces of the mastoid process. Only 50% of histopathological results suggested vasculitis. In all cases, elevated levels of antineutrophil cytoplasmic antibodies (ANCA) against peroxidase 3 (PR3-ANCA) were determined. Two patients presented rapid progression of the disease and died within 1 week and 2 months, respectively. Four other patients manifested gradual improvement of hearing and facial nerve function after treatment. CONCLUSION: GPA should be included into differential diagnosis in all cases of persistent facial nerve palsy especially when otological symptoms coexist. Even localized GPA could be very aggressive, revelating generalized form of the disease. Rapid systemic treatment of GPA can protect hearing and facial nerve from permanent severe dysfunction. The ACR/EULAR 2017 provisional classification criteria for GPA seem to be valuable tool in diagnosing ENT patients with localized otological form of the disease.

Immunoglobulin G4-Related Disease Presenting as Temporal Bone Lesion with Facial Nerve Palsy.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition which could present as local infiltration of skull base structures. We report a case of IgG4-RD with infiltration of the temporal bone and surrounding structures in a patient with systemic vasculitis on systemic steroids. A 31-year-old woman presented with right-sided facial weakness, headache, and right ear hearing loss. On examination, redness and retraction of the right tympanic membrane and facial paresis (House-Brackman IV) were noted. Computed tomography imaging showed mastoiditis, temporal lobe stroke, and brain abscess. Magnetic resonance imaging (MRI) showed infiltration in the infratemporal fossa, nasopharynx, spreading along the Eustachian tube and perineurally along the branches of CN V and CN VII intracranially, forming a dural based mass in the middle cranial fossa. Intracranial mass compressed the temporal lobe of the brain, causing perifocal brain edema. Endoscopic biopsy of the nasopharynx was chosen as the least invasive method. It showed marked fibrosis of the tissue, dense lymphoplasmacytic infiltrates, and an increased number of IGG4-positive plasma cells. Serum IgG4 levels were below the diagnostic criteria of IgG4-RD, but histological characteristics of IgG4-RD were met. The patient was treated with high-dose oral prednisolone. Resolution of symptoms, including facial nerve paresis, was observed and infiltration in the nasopharynx, infratemporal fossa decreased on subsequent MRI tests. No recurrence was noted on the follow-up of 16 months. The case presented itself as a diagnostic challenge for a multidisciplinary team to differentiate pathology caused by either IgG4-RD, systemic vasculitis, or atypic mastoiditis. MRI and histological reports were essential to establish a correct diagnosis.