Middle Ear Tophi: A Case Series of Two Unusual Lesions and a Report of Facial Weakness and Review of the Literature.

OBJECTIVE: Tophaceous lesions of the middle ear from calcium pyrophosphate deposition disease (CPPD, or pseudogout) and gout are infrequently reported. Recognizing its characteristic findings will allow clinicians to accurately narrow the differential diagnosis of bony-appearing middle ear lesions and improve management. PATIENTS: Two consecutive cases of tophaceous middle ear lesions presenting to a tertiary care center between January 2021 and December 2021. Neither with previous rheumatologic history. INTERVENTIONS: Surgical excision of tophaceous middle ear lesions. MAIN OUTCOME MEASURE: Improvements in facial weakness and conductive hearing loss. RESULTS: The first case was a 66-year-old gentleman with progressive conductive loss, ipsilateral progressive facial weakness over years, and an opaque, irregular-appearing tympanic membrane anterior to the malleus found to have CPPD on surgical pathology, with immediate postoperative improvement of facial function. The second was a 75-year-old gentleman with progressive conductive loss and similar appearing tympanic membrane as case 1, previously diagnosed with tympanosclerosis, found to have gout on surgical pathology. In both cases, the CT showed a heterogenous, bony-appearing lesion in the middle ear, and both tophaceous lesions were a of gritty, chalky consistency intraoperatively. CONCLUSION: Tophaceous lesions of the middle ear are rare but have similar findings. Notably, the tympanic membrane can appear opaque and irregular, and the CT demonstrates a radiopaque, heterogeneous appearance. Facial weakness is an unusual finding. Specimens of suspected tophi must be sent to pathology without formalin for accurate diagnosis.

The Ansa Hypoglossi: Quantifying Axonal Density of a Donor Nerve for Facial Reinnervation.

Background: There are a number of nerve grafting options for facial reanimation and the ansa hypoglossi (AH) may be considered in select situations. Objective: To compare axonal density, area, and diameter of AH with other nerves more usually used for facial reanimation. Methods: AH specimens from patients undergoing neck dissections were submitted in formalin. Proximal to distal cross sections, nerve diameters, and the number of axons per nerve, proximally and distally, were measured and counted. Results: Eighteen nerve specimens were analyzed. The average manual axon count for the distal and proximal nerve sections was 1378 ± 333 and 1506 ± 306, respectively. The average QuPath counts for the proximal and distal nerve sections were 1381 ± 325 and 1470 ± 334, respectively. The mean nerve area of the proximal and distal nerve sections was 0.206 ± 0.01 and 0.22 ± 0.064 mm2, respectively. The mean nerve diameter for the proximal and distal nerve sections were 0.498 ± 0.121 and 0.526 ± 0.75 mm, respectively. Conclusion: The histological characteristics of the AH support clinical examination of outcomes as a promising option in facial reanimation.

The clinical availability of facial nerve enhancement in temporal bone MRI for the patients of idiopathic acute peripheral facial palsy.

PURPOSE: This study is to evaluate the duration of facial nerve enhancement in gadolinium-enhanced temporal bone MRI after the onset of acute facial palsy. METHODS: Gd-enhanced MRI imagines were examined in 13 patients with idiopathic acute facial palsy within 14 days after the onset. The degree of facial nerve function was measured according to the House-Brackmann (H-B) grading system at their first visit at outpatient clinic. The follow-up MRI was taken about 16.5 months (7-24 months) after onset of disease. The degree of facial nerve enhancement was measured with signal intensity (SI) which was quantitatively analyzed using the region-of-interest (ROI) measurements for each segment of the facial nerve. SI was statistically analyzed by comparing SI values of contralateral site and ipsilateral site using the paired t test with SPSS program. RESULTS: The gadolinium enhancement was statistically increased at labyrinthine segment and geniculate ganglion area of facial nerve at initial temporal bone MRI. The gadolinium enhancement was statistically decreased at all the segments of facial nerve except tympanic segment (p < 0.05) at follow-up MRI. CONCLUSIONS: The facial nerve enhancement in Gd-enhanced MRI images prolonged more than 21 months of the onset. The newly developed pathologic lesions of acute facial palsy especially occur at the site of labyrinthine and geniculate ganglion.

Facial nerve in skullbase tumors: imaging and clinical relevance.

Facial nerve, the 7th cranial nerve, is a mixed nerve composed of sensory and motor fibers, and its main branch is situated in the cerebellopontine angle. Facial nerve dysfunction is a debilitating phenomenon that can occur in skullbase tumors and Bell's pals. Recovery of the facial nerve dysfunction after surgery for skullbase tumors can be disappointing, but is usually favorable in Bell's palsy. Advances in magnetic resonance imaging (MRI) allow to visualize the facial nerve and its course in the cerebellopontine angle, also when a large tumor is present and compresses the nerve. Here, we describe the anatomical, neurochemical and clinical aspects of the facial nerve and highlight the recent progress in visualizing the facial nerve with MRI.

Clinico-histopathological review of 255 patients who underwent parotidectomy for pleomorphic adenoma: a 10-year retrospective study-a proposal for an optimal diagnostic and therapeutic algorithm for patients with recurrent pleomorphic adenoma.

PURPOSE: Pleomorphic adenoma (mixed tumor) is the most common neoplasm of the parotid gland and one of the most frequent types of salivary gland tumor, generally with benign behavior and relatively slow growing. The adenomas could arise from the superficial, deep or from both superficial and deep parotid's lobes. METHODS: The aim of this review is to retrospectively analyze the surgical management of patients with pleomorphic adenoma of the parotid gland performed at the Department of Otorhinolaryngology (Department of Sense Organs of "Azienda Policlinico Umberto I" in Rome), from 2010 to 2020, with a focus on the percentage of recurrence and on the complication related to surgery to suggest an optimal diagnostic and therapeutic algorithm for patients with recurrent pleomorphic adenoma. The analysis of the complications observed in case of different surgical approaches was performed using the X2 test. RESULTS: The choice of a surgical approach (superficial parotidectomy-SP, total parotidectomy-TP, extracapsular dissection-ECD) depends on several elements, such as the location and the size of the adenoma, the availability of existing technical facilities and the professional experience of the surgeon. A transient facial palsy was present in 37.6%, 2.7% reported a permanent facial nerve palsy, 1.6% developed a salivary fistula, 1.6% a post-operative bleeding and 2.3% showed Frey Syndrome. CONCLUSION: The surgical management of this benign lesion is required, even in asymptomatic cases, to prevent the progressive growing and to reduce the risk of malignant transformation. The goal of surgical excision is to obtain the complete resection to minimize the risk of tumor recurrence and avoiding facial nerve disability. Therefore, an accurate preoperative study of the lesion and the choice of the most appropriate surgical treatment are essential to minimize the rate of recurrence.

Proposal for a novel classification of benign parotid tumors based on localization.

OBJECTIVE: Postoperative facial nerve paralysis is the most problematic complication after surgical treatment of parotid tumors. Localization of tumors is highly relevant for the surgical approach, but existing classification systems do not focus on the association between localization and surgical technique. Therefore, we created a new localization-based classification system for benign parotid tumors and investigated the characteristics of tumors in each localization and the frequency of postoperative facial nerve paralysis by retrospectively applying the classification to previous cases. METHODS: First, we defined 6 portions of the parotid gland (upper, U; lower, L; posterior, P; anterior, A; superficial, S; deep, D) by dividing the transverse plane into an upper and lower portion at the mandibular marginal branch, the longitudinal plane into a posterior and anterior portion at the midline of the parotid anteroposterior diameter, and the sagittal plane into a superficial and deep portion along the course of the facial nerve. Then, we defined 8 locations by combining the 6 portions in all possible ways (i.e., U-P-S, U-P-D, U-A-S, U-A-D, L-P-S, L-P-D, L-A-S, L-A-D). We used this classification to define the tumor localization in 948 patients who had undergone partial superficial parotidectomy for benign parotid tumors and then investigated the incidence, histopathological type, signs/symptoms, diagnosis, surgery, and complications in each area. RESULTS: Pleomorphic adenomas comprised approximately 70% of tumors in the upper portion but only approximately 35% in the lower portion. The rate of postoperative facial nerve paralysis was significantly higher for tumors in deep locations than in superficial locations (33.9% vs 14.9%, respectively), and the odds ratios for postoperative facial nerve paralysis in the U-P-D and U-A-D locations were 7.6 and 4.8 compared to the L-P-S location. When maximum diameter, operation time, bleeding volume, sex (reference: female), and age were added as control variables, the odds ratios were 4.2 and 3.0. CONCLUSION: Determining tumor localization preoperatively with the new localization-based classification of parotid tumors is helpful not only for predicting the histopathological type but also for predicting surgical complications, particularly postoperative facial nerve paralysis.

Cholesterol granuloma in facial nerve canal presenting with facial nerve paralysis.

Cholesterol granulomas (CGs) are frequently found in the temporal bone, but their presence in the facial nerve has not been reported. We report a case of a 58-year-old woman who presented with left facial palsy caused by a CG that appeared to have originated in the facial nerve. Temporal bone computed tomography (CT) revealed soft tissue masses in the left middle ear spaces, and the facial canal was dilated from the genu to the vertical portion. Magnetic resonance imaging (MRI) revealed high signal intensity on T1-weighted images and partially high signal intensity on T2-weighted images, although no significant enhancement was observed on gadolinium-enhanced MRI. Surgical findings revealed CG in the middle ear spaces, but the facial canal remained intact and lacked continuity with the internal mass. Histopathological analysis verified the mass as a CG accompanied by cholesterol crystals. The mass was located within and continuous with the epineurium. These findings indicate that hemorrhage in the facial canal may have triggered the formation of the CG, causing left facial palsy due to increased pressure in the facial canal.

Emotional facial palsy: an unusual and rarely explored neurological sign.

The anatomy of the cortico-bulbar tract that drives voluntary movements of mimic muscles is well described. Some cases of facial palsy with inverse automatic-voluntary dissociation (emotional facial palsy; EFP) are reported in the literature. These cases suggested a completely independent path of the fibers whose lesion results in EFP. We aimed to review the clinical reports of EFP available in the literature to characterize the anatomical aspect of the fibers whose lesion results in the isolated impairment of spontaneous smiling. Cortico-pontine fibers that control spontaneous smiling arise from the medial surface of the prefrontal cortex and descend through the anterior limb of the internal capsule, thalamus, and brain steam, independently from those that control voluntary movement. The mesial temporal lobe, particularly the amygdala, plays a crucial role in the network driving emotionally evoked facial expressions. We would highlight the relevance of an unusual and rarely explored neurological sign that could be added to clinical examination in ruling out focal brain pathology, such as stroke, tumors, or multiple sclerosis.

The otologic approach in the management of posterior petrous surface meningiomas.

PURPOSE: Report our experience in the management of posterior petrous surface meningiomas (PPSMs), and identify features that affect hearing, facial nerve (FN) function, and control of the disease. METHODS: Retrospective case series of 131 patients surgically managed for PPSMs. FN status, hearing and tumour radicality were assessed and compared between patients with tumours of different locations (Desgeorges classification) and internal auditory canal involvement (IAC). RESULTS: At the time of surgery 74.8% of patients had a hearing loss. Hearing was mostly unserviceable in tumors attached to the meatus. Pure tone audiometry did not correlate to IAC extension, while speech discrimination scores were statistically worse when the tumor occupied the IAC (unpaired t test, p = 0.0152). Similarly, extrameatal tumors undergoing removal by otic preserving techniques maintained postoperative hearing, whereas hearing worsened significantly in tumors involving the IAC (paired t test, p = 0.048). The FN was affected preoperatively in 11.4% of cases. Postoperative FN palsy was significantly correlated to the IAC involvement (Fisher's exact test, p = 0.0013), while it was not correlated to tumor size. According to the Desgeorges classification, a postoperative FN palsy complicated the majority of anteriorly extending tumors and, two-fifths of meatus centred tumors. 75% of posterior located tumors had a postoperative FN grade I HB. CONCLUSIONS: Since the involvement of the IAC by the tumor affects both hearing and FN function, the IAC is of primary importance in PPSMs and should be studied and addressed as much as the tumor location in the CPA.

The Alterations and Significance of Intercellular Adhesion Molecule-1 in Mouse Brainstem During Herpes Simplex Virus Type 1-Induced Facial Palsy.

Herpes simplex virus type 1 (HSV-1) results in the development of Bell's pals but still, the pathophysiology of the facial nerve paralysis is still not fully studied. The main objective is to establish an animal model of type 1 herpes simplex virus (HSV-1)-induced face paralysis in the mouse and to investigate the pattern of changes in intercellular adhesion molecule -1(ICAM-1) expression in the facial nucleus of the brain stem in mice with facial paralysis as well as the effects of glucocorticoids on intercellular adhesion molecule -1(ICAM-1) expression. A total of 170 4-week-old Balb/c male mice were randomly divided into the virus inoculation group (n = 135), saline control group (n = 26), and blank control group (n = 9). Mice in the virus inoculation group that showed facial paralysis were divided into A, B, and C subgroups. The A group did not receive any treatments, the B group received methylprednisolone sodium succinate (MPSS) intervention, and the C group received MPSS + RU486 treatment. The mouse model of facial paralysis was established by inoculating HSV-1 to the skin at the back of the ears. The facial nerve function of mice was assessed, and real-time PCR and western blot were used to assess ICAM-1 expression in the facial nucleus of the brain stem in mice with facial paralysis after drug intervention. In the virus inoculation group, 95 mice (55.88%) showed varying degrees of facial paralysis symptoms within 2-5 days after inoculation. The ICAM-1 gene and protein expression levels remained at low levels in the facial nucleus of the brain stem of mice in the saline group, which showed no significant difference compared to the normal control group (P > 0.05). However, for mice of the virus inoculation group, ICAM-1 expression increased at 6 h after the occurrence of facial paralysis and peaked after 2 days, differing significantly from the blank control group (P < 0.01). ICAM-1 expression subsequently decreased gradually. In the HSV-1 + MPSS group, ICAM-1 protein expression decreased significantly on the 2nd day after facial paralysis. In the HSV-1 + MPSS + RU486 group, MPSS inhibition of ICAM-1 protein expression was reduced. The results suggested that ICAM-1 is involved in the pathological processes by which HSV-1 induces facial paralysis in mice, and the treatment effects of MPSS for Bell's palsy can be achieved by the inhibition of MCP-1.

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

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

Management of facial nerve schwannoma: when is the timing for surgery.

PURPOSE: Although the estimated prevalence is extremely low, facial nerve schwannoma (FNS) is the most common primary tumor of the facial nerve (FN). In the present study, the outcome of surgical management in 18 patients with FNS was analyzed and an appropriate time for surgery was proposed. MATERIALS AND METHODS: A total of 18 patients with FNS who underwent surgical management by a single surgeon from 1999 to 2018 were retrospectively analyzed. RESULTS: Among the 18 patients, five had no facial paralysis before surgery. Near-total removal was performed in three cases, and two cases were managed with decompression. In 13 cases with various degree of preoperative facial palsy, nerve continuity was lost during surgery. FN was reconstructed using cable graft in ten cases, direct anastomosis in one case, and facial-hypoglossal nerve transfer in one case. Facial reanimation surgery without FN reconstruction was performed in one case due to a long-standing facial paralysis before surgery. Preoperative House-Brackmann (H-B) grade in all patients was significantly worse as tumor size increased. The correlation was not observed between the duration and severity of preoperative facial palsy. Analysis of 12 patients who underwent FN reconstruction revealed that all patients with good preoperative facial function (H-B grade II-III) recovered to H-B grade III after surgery (7/7, 100%). However, patients with poor preoperative facial function (H-B grade IV or worse) had only a 40% (2/5) chance of improving to grade III after surgery. Preoperative tumor size and duration of facial palsy did not affect postoperative final facial function. CONCLUSION: We suggest that H-B grade III facial palsy is the best time for surgical intervention, regardless of the tumor size or duration of facial palsy.

A new and reliable landmark in parotid surgery: tragomastoid groove (Akil Groove).

OBJECTIVE: Parotid surgery is a frequently performed surgery in otorhinolaryngology practice with many possible complications. Due to the high ratio of facial paralysis during parotid surgery, we defined a new landmark for identifying and protecting the facial nerve as early as possible during surgery. MATERIALS AND METHODS: The study was designed as a prospective anatomical method. The important details and relationship of the tragomastoid groove to the facial nerve truncus were examined during surgery on 30 patients. In addition, the demographics of the patients, the type of surgery and the pathological results of surgeries were evaluated. RESULTS: The mean distance of the tragomastoid groove to the facial nerve truncus was 20.53±1.71 mm, the mean deepness of the tragomastoid groove was 1.91±0.26 mm, and the mean superficial part of the tragomastoid groove was 0.83±0.23 mm. The tragomastoid groove was situated either across from the facial nerve at the place where the facial nerve truncus exits the stylomastoid foramen or just inferior to the truncus in all patients. CONCLUSIONS: The tragomastoid groove was defined for the first time in the literature as a reliable landmark for identifying the facial nerve truncus easily during parotid surgery.

Tips and Pearls for Recovering Symmetry After Facial Palsy Using a Tendon Sling: Our 20-Year Experience.

Background: In facial palsy reconstruction, static techniques with the use of slings can improve the appearance and functionality of the paralyzed face and may be used in patients who cannot undergo complex surgeries or as an ancillary procedure to a dynamic reconstruction. The objective of this study was to assess the improvement in facial symmetry and quality of life among older patients with flaccid facial palsy with the use of a plantaris tendon sling. Methods: A total of 46 patients who had undergone a static reconstruction with the plantaris tendon sling were studied. The surgical technique is detailed emphasizing the tips and pearls. Results: The displacement of the oral commissure was assessed with the FACIAL CLIMA demonstrating a mean elevation of 1.5 ± 0.4 cm and an improvement of 97 ± 7% in the recovery of oral commissure symmetry 2 years after the surgery, whereas the Sunnybrook Facial Grading System showed an improvement of symmetry at rest (-15 ± 5) at 2 years. The Facial Disability Score indicated an improvement of the physical disability (+73.5 ± 14) as well as the social impairment (+21 ± 7) at 1 year postoperatively. Patients were followed for a median of 2 years (range 2-6 years). Conclusions: The use of tendon slings for static facial paralysis reconstruction is a reliable technique with no functional sequelae and good long-term results.

Levator recession and minimal lateral tarsorrhaphy for the management of lagophthalmos and corneal exposure in facial palsy.

PURPOSE: Recession of levator muscle can potentially decrease the severity of corneal exposure by reducing margin-reflex distance 1. The purpose of this study is evaluation of effects of levator recession on lagophthalmos in facial palsy. MATERIALS AND METHODS: In a non-comparative case series of consecutive patients with exposure keratopathy due to facial paralysis between 2012 and 2017, levator recession was performed through lid crease incision. Small-size (⩽3 mm) lateral tarsorrhaphy was performed on a case-by-case basis for those with moderate-to-severe keratopathy. Preoperative and postoperative measurements of margin-reflex distance 1 and lagophthalmos were compared using paired t-test. RESULTS: Thirty-four patients (14 men and 20 women) were enrolled with an average age of 52.3 years. Mean follow-up was 13.3 months (range, 6-36 months). The most common etiology of facial paralysis was Bell's palsy (22 cases), followed by motor vehicle accident (8), parotid surgery (3), and brain tumor surgery (1). No additional procedure was performed for eight patients, while lateral tarsorrhaphy was performed in 26 cases. Mean margin-reflex distance 1 decreased from 5.0 ± 0.4 mm to 4.0 ± 0.5 mm (p < 0.001) and mean lagophthalmos decreased from 3.3 ± 0.9 mm to 1.8 ± 0.9 mm (p < 0.001). Subgroups of patients with additional lateral tarsorrhaphy and without tarsorrhaphy experienced the same amount of reduction in margin-reflex distance 1 and lagophthalmos. CONCLUSION: Recession of levator muscle was effective in reduction of margin-reflex distance 1 and lagophthalmos in facial palsy patients. This procedure can be added to the surgical armamentarium for management of patients with facial palsy and lagophthalmos.

Non-recovery animal model of severe facial paralysis induced by freezing the facial canal.

OBJECTIVE: Some cases of peripheral facial paralysis are resistant to treatment, thus, a non-recovery model of facial paralysis is needed to develop new treatment strategies for this condition. The purpose of the current study was to develop an animal model of which facial palsy was severe and prolonged. METHODS: Ten 8-week-old female Hartley guinea pigs weighing between 400 and 500 g were used for the animal model. The vertical segment of the facial canal was accessed via the otic bulla, without removing the bony wall of the facial canal. The canal was then frozen for 5 s using freeze spray. Facial movements, electroneurography (ENoG), histology, and changes in temperature were evaluated. RESULTS: All animals exhibited complete facial paralysis immediately after the procedure and recovered gradually, however, not all of them had recovered completely 15 weeks after freezing. The ENoG values one week after freezing for all animals (10/10) were 0%. Histological examination one week after freezing revealed that most of the vertically placed myelinated nerve fibers which had been frozen were remarkably affected and denatured. The number of vertically placed myelinated nerve fibers increased 15 weeks after freezing, but the nerve fibers were smaller than normal nerve fibers and were distorted in shape. CONCLUSION: Complete facial paralysis was induced in Hartley guinea pigs by freezing the facial canal. The behavioral, ENoG, and histopathological data suggest that the facial paralysis was severe and prolonged. This model may assist in developing novel treatment for severe facial palsy and facilitate basic research on facial nerve regeneration.

The effect of insulin-like growth factor 1 on the recovery of facial nerve function in a guinea pig model of facial palsy.

The efficacy of insulin-like growth factor 1 (IGF-1) in the treatment of peripheral facial nerve palsy was investigated using an animal model. The facial nerve within the temporal bone was exposed and compressed by clamping. The animals were treated with either IGF-1 or saline which was topically administered by a gelatin-based sustained-release hydrogel via an intratemporal route. The recovery from facial nerve palsy was evaluated at 8 weeks postoperatively based on eyelid closure, complete recovery rate, electroneurography and number of axons found on the facial nerve. IGF-1 treatment resulted in significant improvement in the changes of the degree of eyelid closure over the total time period and complete recovery rate. A separate study showed that IGF-1 receptor mRNA was expressed in facial nerves up to 14 days after the nerve-clamping procedure. IGF-1 was thus found to be effective in the treatment of peripheral facial nerve palsy when topically applied using a sustained-release gelatin-based hydrogel via an intratemporal route.

Using Eye Aspect Ratio to Enhance Fast and Objective Assessment of Facial Paralysis.

A rapid and objective assessment of the severity of facial paralysis allows rehabilitation physicians to choose the optimal rehabilitation treatment regimen for their patients. In this study, patients with facial paralysis were enrolled as study objects, and the eye aspect ratio (EAR) index was proposed for the eye region. The correlation between EAR and the facial nerve grading system 2.0 (FNGS 2.0) score was analyzed to verify the ability of EAR to enhance FNGS 2.0 for the rapid and objective assessment of the severity of the facial paralysis. Firstly, in order to accurately calculate the EAR, we constructed a landmark detection model based on the face images of facial paralysis patients (FP-FLDM). Evaluation results showed that the error rate of facial feature point detection in patients with facial paralysis of FP-FLDM is 17.1%, which was significantly superior to the landmark detection model based on normal face images (NF-FLDM). Secondly, in this study, the Fréchet distance was used to calculate the difference in bilateral EAR of facial paralysis patients and to verify the correlation between this difference and the corresponding FNGS 2.0 score. The results showed that the higher the FNGS 2.0 score , the greater the difference in bilateral EAR. The correlation coefficient between the bilateral EAR difference and the corresponding FNGS 2.0 score was 0.9673, indicating a high correlation. Finally, through a 10-fold crossvalidation, we can know that the accuracy of scoring the eyes of patients with facial paralysis using EAR was 85.7%, which can be used to enhance the objective and rapid assessment of the severity of facial paralysis by FNGS 2.0.