Natural history of facial paraganglioma with 2 decades of follow-up: A case report and literature review.

OBJECTIVE: To report 20 years of natural history data for a facial paraganglioma and provide a comprehensive review of the existing literature. PATIENT: 81-year-old female with a remote history of cardiac arrest while under anesthesia who elected to observe her facial paraganglioma for 20 years. INTERVENTIONS: Observation, clinical documentation, radiographic surveillance. MAIN OUTCOME MEASURES: Tumor progression, patient symptomatology, and review of management options. RESULTS: The initial presentation of the facial paraganglioma was facial spasm. Over the course of observation, symptoms progressed to include complete facial nerve paralysis, pulsatile tinnitus, and otalgia on the affected side. Radiologic surveillance demonstrated incremental growth and erosion of surrounding structures, including the posterior external auditory canal, stylomastoid foramen, and lateral semicircular canal with near-dehiscence. Twenty-four cases of facial paraganglioma were identified in the extended literature search and are summarized herein. CONCLUSIONS: This unique case contributes to the scarce literature surrounding facial paragangliomas by reporting the extended natural history of this disease.

Clinical and electrophysiological findings of facial palsy in a case of hereditary gelsolin amyloidosis.

Hereditary gelsolin amyloidosis (HGA) is an autosomal dominant systemic amyloidosis, characterized by cranial and sensory peripheral neuropathy, corneal lattice dystrophy, and cutis laxa. We report a case of HGA presenting with bilateral facial palsy. A 70-year-old Japanese man presented with slowly progressive bilateral facial palsy and facial twitching, which had started in his 40s. His mother also had the same symptoms due to an unknown cause but rest of the family did not. He showed incomplete facial palsy with no frontal muscle movement and partial movement of the orbicularis oris and orbicularis oculi muscles. The patient showed no synkinesis. Electroneurography revealed symmetric low compound motor action potential amplitude of the orbicularis oris muscle, and a nerve excitability test showed a symmetric increase in the response threshold. Despite the partial voluntary movement of the orbicularis oculi muscle, bilateral blink reflexes were absent. He also showed facial spasms after contraction of the orbicularis oris muscle. Genetic testing revealed a heterozygous c.640G>A mutation (p. Asp214Asn); therefore, the patient was diagnosed with HGA. HGA related facial palsy showed moderate bilateral, upper blanch-dominant axonal degeneration of the facial nerve without reinnervation, and trigeminal nerve neuropathy.

Síndrome de Moebius y ortotropía en posición primaria: ¿es infrecuente esta asociación? (Colombia) / Moebius syndrome and orthotropia in primary position: is this association uncommon? (Colombia) / Síndrome de Moebius e ortotropia em posição primária: é uma associação incomum? (Colômbia)

El síndrome de Moebius es una enfermedad congénita poco común que se caracteriza por el compromiso unilateral o bilateral del VI y VII par craneal, lo que compromete los músculos que controlan la oculomotricidad, produciendo una parálisis en la abducción del globo ocular y los músculos involucrados en la expresión facial. Su presentación clínica y grados de severidad son variables, puede presentar compromiso simétrico o asimétrico. Adicionalmente, gran parte de los casos se relacionan con trastornos del lenguaje, anomalías musculoesqueléticas y orofaciales. En el presente artículo se presenta el caso de una paciente femenina de 3 años producto de un embarazo trigemelar con diagnóstico clínico de síndrome de Moebius al nacer, confirmado por neuroimagen en la que se evidencia la ausencia bilateral del nervio facial en ángulos pontocerebelosos, adicionalmente con un defecto completo en los movimientos oculares de abducción y aducción lo que impide el estrabismo convergente común en estos pacientes.

Moebius syndrome is a rare congenital disease characterized by unilateral or bilateral involvement of the VI and VII cranial nerves, which compromises the muscles that control ocular motricity with paralysis in the abduction of the eyeball and the muscles involved in the facial expression. Its clinical presentation and degrees of severity are variable, and it can be symmetric or asymmetric. Additionally, most of the cases are related to language disorders, musculoskeletal and orofacial anomalies. This paper presents the case of a 3-year-old female patient, product of a trigemellar pregnancy with a clinical diagnosis of Moebius syndrome at birth, confirmed by neuroimaging, which shows the bilateral absence of the facial nerve in point-lateral angles. Additionally she has a complete defect in abduction and adduction eye movements, which prevents the common convergent strabismus in these patients.

A síndrome de Moebius é uma doença congênita rara caracterizada pelo envolvimento unilateral ou bilateral dos nervos cranianos VI e VII, que compromete os músculos que controlam a oculomotricidade com paralisia na abdução do globo ocular e dos músculos envolvidos na expressão facial. Sua apresentação clínica e graus de gravidade são variáveis, podendo ser um comprometimento simétrico ou assimétrico. Além disso, a maioria dos casos está relacionada a distúrbios de linguagem, anomalias musculoesqueléticas e orofaciais. Este paper apresenta o caso de uma paciente de 3 anos de idade, fruto de uma gravidez trigêmea com diagnóstico clínico de Síndrome de Moebius ao nascimento, confirmado por neuroimagem em que é evidente a ausência bilateral do nervo facial nos ângulos ponto-cerebelares. Além disso, ela tem um defeito completo nos movimentos oculares de abdução e adução, o que impede o estrabismo convergente comum nesses pacientes.

Absent pyramidal eminence and stapedial tendon associated with congenital stapes footplate fixation: Intraoperative and radiographic findings.

OBJECTIVE: Report an association between congenital stapes footplate fixation (CSFF) and radiological absence of the pyramidal eminence and stapedial tendon. PATIENTS: Children and adults with intraoperatively confirmed CSFF and an absent stapedial tendon. INTERVENTIONS: Computed tomography (CT); exploratory tympanotomy with stapedotomy. MAIN OUTCOME MEASURES: Absence of a pyramidal eminence and stapedial tendon aperture identified on preoperative CT that was confirmed intraoperatively. RESULTS: Eight patients with intraoperative confirmation of CSFF and absent stapedial tendon were retrospectively identified. The average preoperative bone conduction and air conduction pure tone averages were 19.6 dB (SD 15.6 dB) and 55.9 dB (SD 23.6 dB), respectively. The average air-bone gap was 36.3 dB (SD 17.9 dB) preoperatively. In the seven patients who underwent preoperative CT, all were consistently identified to have an absent or hypoplastic pyramidal eminence and absent stapedial tendon aperture at the pyramidal eminence. In six cases, the stapedial footplate appeared normal, while in one case the footplate appeared abnormal which correlated with severe facial nerve prolapse observed intraoperatively. All eight cases underwent exploratory tympanotomy and demonstrated intraoperative stapes footplate fixation, absent stapedial tendon and either absent or hypoplastic pyramidal eminence, which correlated with preoperative CT findings. CONCLUSIONS: This study identifies a clinically pragmatic association between an absent pyramidal eminence identified on high-resolution CT and the diagnosis of CSFF. In a condition that otherwise generally lacks distinctive radiological features, the absence of a pyramidal eminence on CT in a patient with nonprogressive, congenital conductive hearing loss may strengthen clinical suspicion for CSFF.

Early Outcomes in an Emerging Facial Nerve Center: The Oregon Health and Science University (OHSU) Experience.

OBJECTIVES: Nerve transfer (NT) and free gracilis muscle transfer (FGMT) are procedures for reanimation of the paralyzed face. Assessing the surgical outcomes of these procedures is imperative when evaluating the effectiveness of these interventions, especially when establishing a new center focused on the treatment of patients with facial paralysis. We desired to discuss the factors to consider when implementing a facial nerve center and the means by which the specialist can assess and analyze outcomes. METHODS: Patients with facial palsy secondary to multiple etiologies, including cerebellopontine angle tumors, head and neck carcinoma, and trauma, who underwent NT or FGMT between 2014 and 2019 were included. Primary outcomes were facial symmetry and smile excursion, calculated using FACE-gram and Emotrics software. Subjective quality of life outcomes, including the Facial Clinimetric Evaluation (FaCE) Scale and Synkinesis Assessment Questionnaire (SAQ), were also assessed. RESULTS: 14/22 NT and 6/6 FGMT patients met inclusion criteria having both pre-and postoperative photo documentation. NT increased oral commissure excursion from 0.4 mm (SD 5.3) to 2.9 mm (SD 6.8) (P = 0.05), and improved symmetry of excursion (P < 0.001) and angle (P < 0.001). FGMT increased oral commissure excursion from -1.4 mm (SD 3.9) to 2.1 mm (SD 3.7), (P = 0.02), and improved symmetry of excursion (P < 0.001). FaCE scores improved in NT patients postoperatively (P < 0.001). CONCLUSIONS: Measuring outcomes, critical analyses, and a multidisciplinary approach are necessary components when building a facial nerve center. At our emerging facial nerve center, we found NT and FGMT procedures improved smile excursion and symmetry, and improved QOL following NT in patients with facial palsy secondary to multiple etiologies.

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.

Orbicularis Oculi Muscle Reinnervation Confers Corneal Protective Advantages over Static Interventions Alone in the Subacute Facial Palsy Patient.

BACKGROUND: Corneal protection is a priority in flaccid facial palsy patients. Denervation of the orbicularis oculi muscle results in weak palpebral closure and predisposes patients to severe corneal sequelae. While periorbital static procedures enhance corneal coverage in repose, voluntary closure is only regained through dynamic reinnervation of the muscle. This study aims to elucidate the added effect of dynamic reinnervation of the orbicularis oculi muscle on long-term corneal integrity as well as on dynamic closure of the palpebral aperture. METHODS: Retrospective review was performed on two groups of complete palsy patients: those who received solely periorbital static procedures and those who underwent concomitant orbicularis oculi muscle reinnervation and static lid procedures. Only patients with complete ophthalmic examinations were included. Corneal punctate epithelial erosions in addition to static and dynamic palpebral measurements were serially assessed preoperatively and postoperatively. RESULTS: Of 272 facial palsy patients, 26 fit the inclusion criteria. Eleven patients underwent combined muscle reinnervation involving facial-to-masseteric nerve coaptation in addition to static eye procedures, and 15 patients underwent solely static interventions. Analysis revealed a 65.3 percent lower mean punctate epithelial erosion score in reinnervation patients as compared with static patients when evaluated at more than 9 months postoperatively (p < 0.01). Reinnervation patients were also found to have 25.3 percent greater palpebral aperture closure (p < 0.05) and 32.8 percent higher closure velocity (p < 0.01) compared with static patients. CONCLUSION: In patients with subacute facial palsy, dynamic reanimation of the orbicularis oculi muscle with concomitant static interventions provides lasting corneal protection not seen in patients who receive solely static interventions. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Tympanic-mastoid and parotid schwannomas of the facial nerve: clinical presentation related to the anatomic site of origin.

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.

Objetivo: Describir la presentación clínica de los schwannomas del nervio facial de acuerdo con el sitio anatómico de origen. Método: Se realizó un estudio retrospectivo en el que se evaluó la presentación clínica, el protocolo diagnóstico y el tratamiento de tumores del nervio facial en adultos. Resultados: Se encontraron seis casos, cuatro de ellos de localización tímpano-mastoidea en los extremos de su posible presentación clínica: desde casos sintomáticos con parálisis facial, hasta un caso asintomático de la porción timpánica encontrado como hallazgo transoperatorio; y se encontraron dos casos de localización parotídea, uno con parálisis facial completa y otro sin parálisis facial. Conclusiones: Para el diagnóstico de tumores intratemporales y parotídeos del nervio facial se requiere una elevada sospecha clínica dado lo heterogéneo de su presentación; su curso clínico depende del segmento de origen y de su extensión: más frecuentemente son asintomáticos los de la porción timpánica y son sintomáticos los de la porción mastoidea. Estos tumores deben evaluarse con estudios de imagen; no se recomienda realizar biopsia incisional. El tratamiento es la resección quirúrgica en los casos sintomáticos con parálisis facial de grado IV o mayor de House-Brackmann, con reconstrucción inmediata del nervio.

Cochlear Erosion due to a Facial Nerve Schwannoma.

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

For Whom the Bell's Toll: Recurrent Facial Nerve Paralysis, A Retrospective Study and Systematic Review of the Literature.

PURPOSE: To examine the etiology, clinical course, and management of recurrent peripheral facial nerve paralysis. METHODS: Retrospective review at a single tertiary academic center and systematic review of the literature. Clinical presentation, laboratory and imaging findings, treatment and outcome for all cases of recurrent ipsilateral, recurrent contralateral, and bilateral simultaneous cases of facial paralysis are reviewed. RESULTS: Between 2000 and 2017, 53 patients [41.5% men, 29 median age of onset (range 2.5 wk-75 yr)] were evaluated for recurrent facial nerve paralysis at the authors' institution. Twenty-two (41.5%) cases presented with ipsilateral recurrences only, while the remaining 31 patients (58.5%) had at least 1 episode of contralateral recurrent paralysis. No cases of bilateral simultaneous facial nerve paralysis were observed. The median number of paretic events for all patients was 3 (range 2-20). The median nadir House-Brackmann score was 4, with a median recovery to House-Brackmann grade 1.5 over a mean recovery time of 61.8 days (range 1-420 d). Diagnostic evaluation confirmed Melkersson-Rosenthal syndrome in four (7.5%) cases, neurosarcoidosis in two (3.7%), traumatic neuroma in one (1.9%), Ramsay Hunt syndrome in one (1.9%), granulomatosis with polyangiitis in one (1.9%), and neoplastic causes in three (5.7%) cases [facial nerve schwannoma (n = 2; 3.7%), metastatic squamous cell carcinoma to the deep lobe of the parotid gland (n = 1; 1.9%)]; ultimately, 77.4% (41) of cases were deemed idiopathic. Facial nerve decompression via a middle cranial fossa approach was performed in three (5.7%) cases without subsequent episodes of paralysis. CONCLUSION: Recurrent facial nerve paralysis is uncommon and few studies have evaluated this unique population. Recurrent ipsilateral and contralateral episodes are most commonly attributed to idiopathic facial nerve paralysis (i.e., Bell's palsy); however, a subset harbor neoplastic causes or local manifestations of underlying systemic disease. A comprehensive diagnostic evaluation is warranted in patients presenting with recurrent facial nerve paralysis and therapeutic considerations including facial nerve decompression can be considered in select cases.

The inflammatory pseudotumor presenting periodic acid-Schiff-positive inclusions with acute unilateral facial nerve palsy.

Although most acute peripheral facial palsies can be attributed to Bell's palsy, other factors, such as infection, trauma, and neoplasm, can cause facial palsy as well. Among these, facial nerve tumors are rare but should be considered in the differential diagnosis of facial palsy. Palsies due to facial nerve tumors usually present with slow onset but occasionally present as acute episodes. In such cases, facial nerve decompression is the treatment of choice to allow the tumor room to grow without compressing the nerve or its blood supply. We describe a case of severe, acute facial palsy presenting with a spindle-shaped bone erosion on the mastoid portion of the facial canal. Although facial neuroma was suspected preoperatively, emergency decompression surgery revealed that an unusual inflammatory pseudotumor was responsible for the finding. Postoperative histological analysis revealed extensive destruction of the nerve fibers, with extensive infiltration of foamy macrophages containing characteristic, diastase-resistant, periodic acid-Schiff (PAS)-positive inclusions, which are hallmark of the uncommon bacterial infections. This was a case of facial palsy with an unusual etiology. The case shows the benefit of decompression surgery not only as treatment for the palsy but also as exploratory surgery in cases of facial nerve tumor.

Facial nerve hemangioma in the middle ear.

Facial nerve hemangioma is a rare and benign vascular tumor, and accounts for 0.7% of intratemporal tumors. We report the second case described in the literature of a facial nerve hemangioma in its tympanic segment. A 14-year-old male patient presented with a history of progressive right ear hearing loss with preserved facial mimicry. Pure tone audiometry showed a right ear moderate conductive hearing loss. Magnetic resonance imaging demonstrated an expansive lesion involving the tympanic segment of the right facial nerve, suggestive of hemangioma. Watchful waiting was chosen as management. In the first case of middle ear facial hemangioma described in the literature, facial palsy was the symptom that led the patient to seek medical care. In the present case, it can be inferred that the first symptom was conductive hearing loss ipsilateral to the lesion. Facial palsy may not be present and the clinical presentation may resemble otosclerosis, ossicular chain disruption, and third window abnormalities, among other differential diagnoses of conductive hearing loss. The second case of tympanic portion facial nerve hemangioma is reported, describing the specificity of conductive hearing loss as its only clinical manifestation.

A hybrid technique to address exposure keratopathy secondary to facial nerve paresis: A combination of a lateral tarsorrhaphy and lateral wedge resection.

PURPOSE: To present the results of treating combined lower eyelid laxity, retraction and midface descent secondary to facial nerve weakness with a hybrid surgical procedure. MATERIALS AND METHODS: A retrospective analysis of patients from January 2015 to January 2017 who underwent a hybrid surgical technique for the treatment of corneal exposure secondary to facial nerve paresis with a single surgeon was performed. Age, gender, and presence of exposure symptoms were recorded pre-operatively. Outcomes assessed included improvement of lower eyelid laxity and position, operative complications, and post-operative symptomatic relief. RESULTS: A total of 11 patients underwent unilateral eyelid surgery. All patients had symptomatic relief and good functional outcomes defined as improvement in eyelid laxity, lower eyelid position, and objective corneal exposure. No cases required reoperation during an average follow up of 174.5 days. CONCLUSIONS: Combining portions of a tarsorrhaphy and lateral wedge resection technique is a simple and effective procedure to improve lower eyelid position and limit corneal exposure secondary to facial nerve paresis.

Outcomes of lower eyelid retractor recession and lateral horn lysis in lower eyelid elevation for facial nerve palsy.

PurposeTo report outcomes and complications of lower eyelid retractor recession and lateral horn lysis (RR) for lower eyelid elevation in patients with facial nerve palsy (FNP).Patients and methodsRetrospective review. Patients with FNP undergoing RR alone (group 1) or with adjunctive procedures (canthal suspension-group 2, tarsorrhaphy-group 3, and full-thickness skin graft-group 4) during a 5-year period were included. Patient demographics, lagophthalmos, occurrence of eyelid malpositions, recurrent retraction, and repeat procedures were noted from medical records. Measures of lower eyelid height (LEH) and lid lag on downgaze were obtained from standard photographs.ResultsForty-two patients (23 females, mean age was 59 years) were included. Mean follow-up was 24 months (range 6-77). Median improvement in LEH following surgery was significant in Group 1 (0.90 mm, IQR: 0.37-0.91, P=0.20) and in Group 2 (0.51 mm, IQR: 0.30-1.37, P<0.001), respectively. Median improvement in blink lagophthalmos was 3.00 mm (IQR: 3.00-3.50, P=0.02) in Group 1 and 3.50 mm (IQR: 1.75-5.00, P<0.001) in Group 2. Median improvement in lagophthalmos on gentle eye closure was 2.00 mm (IQR: 1.50-3.00, P=0.02) in Group 1 and 1.00 mm (IQR: 0-2.13, P=0.006) in Group 2. No new cases of ectropion were noted. 23.5% of patients required a repeat RR at a mean interval of 20 (range 1-70) months and a further 9.8% required a third procedure at a mean interval of 21 (range 18-29) months.ConclusionRR improves lower eyelid retraction and lagophthalmos in FNP either alone or when combined as an adjunctive procedure. It does not aggravate paralytic ectropion although repeated retractor recessions may be required to improve retraction.

Facial nerve hemangioma in the middle ear / Hemangioma de nervo facial em orelha média

ABSTRACT Facial nerve hemangioma is a rare and benign vascular tumor, and accounts for 0.7% of intratemporal tumors. We report the second case described in the literature of a facial nerve hemangioma in its tympanic segment. A 14-year-old male patient presented with a history of progressive right ear hearing loss with preserved facial mimicry. Pure tone audiometry showed a right ear moderate conductive hearing loss. Magnetic resonance imaging demonstrated an expansive lesion involving the tympanic segment of the right facial nerve, suggestive of hemangioma. Watchful waiting was chosen as management. In the first case of middle ear facial hemangioma described in the literature, facial palsy was the symptom that led the patient to seek medical care. In the present case, it can be inferred that the first symptom was conductive hearing loss ipsilateral to the lesion. Facial palsy may not be present and the clinical presentation may resemble otosclerosis, ossicular chain disruption, and third window abnormalities, among other differential diagnoses of conductive hearing loss. The second case of tympanic portion facial nerve hemangioma is reported, describing the specificity of conductive hearing loss as its only clinical manifestation.

RESUMO O hemangioma do nervo facial é um tumor vascular raro e benigno, que corresponde a 0,7% dos tumores intratemporais. Apresentamos o segundo caso descrito na literatura de hemangioma do nervo facial em sua porção timpânica. Paciente de 14 anos, sexo masculino, apresentando hipoacusia progressiva em orelha direita com mímica facial preservada. A audiometria evidenciou perda condutiva moderada à direita. A ressonância magnética demonstrou lesão expansiva, comprometendo o segmento timpânico do nervo facial direito, sugestiva de hemangioma do nervo facial. A conduta foi expectante. No primeiro caso descrito na literatura de hemangioma do facial em orelha média, o sintoma que levou o paciente ao atendimento médico foi paralisia facial. No presente caso, pode-se inferir que o primeiro sintoma foi a perda auditiva condutiva ipsilateral à lesão. A paralisia facial pode não estar presente, e o quadro clínico pode se assemelhar à otosclerose, disjunção de cadeia e síndrome da terceira janela, dentre outros diagnósticos diferenciais de perdas auditivas condutivas. O artigo relata o segundo caso de hemangioma do nervo facial em sua porção timpânica, mostrando a peculiaridade de perda auditiva condutiva como única manifestação clínica.

The Multiscope Technique for Microvascular Decompression.

BACKGROUND: Endoscopic surgery has rapidly become widespread in neurosurgery in recent years. Endoscopy can offer close and panoramic surgical views with fine illumination, even in the deep intracranial area. However, it also has the following serious drawback: an intracranial blind area between the field lens of the endoscope and the site of the dural opening. This blind area cannot be viewed on the endoscopic monitor, and several surgical complications, including accidental intracranial neurovascular structural injury, can occur in this area. In this article, we report a new multiscope surgical technique that can compensate for this serious disadvantage of endoscopic surgery. METHODS: In the multiscope technique, endoscopic and exoscopic systems are used simultaneously with 2 monitors. Microvascular decompression (MVD) is performed fully endoscopically using an exoscope that compensates for the intracranial blind area of the endoscopic view. Two high-definition monitors for the endoscope and exoscope are placed side-by-side in front of the primary surgeon. RESULTS: Two patients with hemifacial spasm were treated by endoscopic MVD with the multiscope technique. In these procedures, fine surgical views were obtained by both the endoscope and exoscope. Two monitors were placed side-by-side in front of the surgeon; as a result, the physician could easily view them simultaneously during the operation. No surgery-related complications occurred. CONCLUSIONS: The multiscope technique can facilitate the performance of safer neuroendoscopic surgery than conventional endoscopic surgery. This technique can also be adopted in other skull base surgeries, in which the importance of endoscopy is growing.

Facial nerve paralysis associated with temporal bone masses.

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.

Cholesterol granuloma presenting with facial nerve paralysis following translabyrinthine surgery: A case report.

BACKGROUND: Cholesterol granuloma (CG) is a foreign body giant cell inflammatory reaction to blood degradation products, and clinically presents as a cyst with a thick fibrous lining filled with brownish-yellow fluid. We report an unusual case of CG with facial nerve paralysis 9 years after translabyrinthine (TL) surgery for vestibular schwannoma (VS) removal. CASE: A 70-year-old woman, who underwent TL surgery previously, presented with left facial palsy (FP). CT findings revealed devastated cochlea and uncovered facial nerve, and progression of FP up to House-Brackmann grade 6 was observed. Therefore, we suspected recurrence of cystic VS or facial schwannoma, although MRI was inconsistent with that of schwannoma. The cyst was completely surgically excised, and histopathology confirmed CG diagnosis. Postoperatively her facial movement improved to grade 4. Recurrence has not been encountered since 7 years. CONCLUSION: During follow-up after TL surgery for VS, care should be taken for possibility of CG occurring.

Facial Nerve Meningioma: A Cause of Pediatric Facial Weakness.

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.