Meibomian Gland Dysfunction in Cranial Nerve VII Palsy.

PURPOSE: To examine clinical findings on meibomian gland dysfunction (MGD) in patients with unilateral cranial nerve VII (CN VII) palsy. METHODS: This prospective observational study included 35 patients with unilateral CN VII palsy. The following features were compared between the affected and the unaffected sides: incidence of eyelid abnormalities (irregular eyelid margin, vascular engorgement, and plugged meibomian gland orifices), Marx line score, meibom expression score, area and density scores for corneal fluorescein staining, Schirmer test I (without topical anesthesia), and tear break-up time. RESULTS: The affected side demonstrated significantly higher incidences of vascular engorgement (p = 0.004) and plugged meibomian gland orifices (p < 0.001) than the unaffected side. The incidence of an irregular eyelid margin was similar for both sides (p = 0.168). The scores for the Marx line (p < 0.001), meibom expression (p < 0.001), area (p = 0.034), and density (p = 0.026) were significantly higher for the affected side than for the unaffected side. The affected side showed a significantly higher Schirmer value than the unaffected side (p = 0.030). Tear break-up time was significantly shorter on the affected side than on the unaffected side (p = 0.023). CONCLUSIONS: MGD was more markedly demonstrated on the affected side in patients with unilateral CN VII palsy.

Clinical and radiological guidance in managing facial nerve schwannomas.

OBJECTIVE: To present a review of all patients diagnosed with a facial nerve schwannoma (FNS) managed in our center over almost two decades, and suggest guidelines for their classification and management. STUDY DESIGN: Retrospective case review SETTING: Tertiary referral center PATIENTS: Twenty-eight patients with a facial nerve schwannoma INTERVENTION: Conservative or surgical management depending on clinical and radiological features MAIN OUTCOME MEASURE: Patient demographics, site of tumor, and clinical symptoms, including facial nerve function (House-Brackmann score) at baseline and follow-up. In those managed surgically, operative approach and surgical outcomes were also recorded. RESULTS: Of 28 patients, 16 were male. Mean age at presentation was 46 years. The majority presented with either facial weakness or hearing loss. The internal auditory canal segment of the facial nerve was the most commonly affected (19/28, 68%). Multi-segmental lesions were found in almost half (46%) of patients. Facial weakness was most commonly associated with involvement of the labyrinthine segment (89%). Overall, 16 (57%) patients were managed surgically. CONCLUSION: FNS may be difficult to distinguish on both clinical and imaging grounds from other cerebellopontine pathologies on the basis of audiovestibular symptoms alone. The presence of facial weakness in combination with imaging findings suggestive of FNS is highly suggestive for FNS. In patients with brainstem compression, rapid tumor growth, or House-Brackmann greater than 4, we suggest a surgical approach based on preoperative audiovestibular status, helping optimize long-term facial function and minimize morbidity. Facial nerve reanimation at the time of primary surgery is preferred.

Subjective and objective outcome measures in the treatment of facial nerve synkinesis with onabotulinumtoxinA (Botox).

PURPOSE: To evaluate the Sunnybrook Facial Grading System (SFGS) and Facial Clinimetric Evaluation Scale (FaCE Scale) instrument outcome measures pre- and 30-day posttreatment of facial nerve synkinesis with botulinum toxin with attempts to correlate the 2 scales. METHODS: An IRB approved retrospective review of 22 patients with facial nerve synkinesis where the surgeon completed the SFGS and the patient completed the FaCE prior to receiving onabotulinumtoxinA therapy, the SFGS, and FaCE scales were completed again 1 month later. RESULTS: Of the 22 patients, 9 complete datasets were analyzed. Mean patient age was 59.8; 8 (89%) women and 1 (11%) men. Overall SFGS composite score decreased from 57.6 ± 20.9 to 45.2 ± 13.5, (p = 0.001). SFGS subdomain synkinesis significantly improved (p < 0.001), while voluntary movement significantly decreased (p = 0.002). A difference in the resting symmetry was not statistically significant (p = 0.08). The FaCE scale composite score significantly improved from 40.9 ± 9.5 to 47.6 ± 11.9, (p = 0.03). FaCE subdomains facial comfort (p = 0.005) and social function (p = 0.009) significantly improved, while oral function, eye comfort, facial movement, and lacrimal control did not. The Δ pre/post-SFGS composite score did not correlate with the Δ pre/post-FaCE composite score (rs= -0.318). Subdomain analysis demonstrated significant negative correlation between Δ pre/post-SFGS synkinesis score and Δ pre/post-FaCE eye comfort score (rs = -0.826, p < 0.01). CONCLUSIONS: Significant improvement was seen in objectively reported synkinesis following botulinum toxin therapy. An improvement was noted in the overall subjective facial nerve functioning following therapy along with improvement in social functioning and facial comfort. A meaningful negative correlation was noted when comparing the SFGS "synkinesis" subdomain with the FaCE scale subdomain "eye comfort", implying improvement in eye comfort with control of synkinesis.

Outcomes of surgical treatment for hemifacial spasm associated with the vertebral artery: severity of compression, indentation, and color change.

OBJECT: The object of surgical treatment for hemifacial spasm (HFS) is the exclusion of pulsatile neurovascular compression of the root exit zone (REZ). However, spasm persists transiently or permanently in some cases even after complete decompression. In particular, we mainly experience these results when the vertebral artery (VA) is the offender. Hence, we verified color changes of the nerve and indentations from within the operative field in HFS patients with the VA as the offender. So, we reviewed retrospectively the records of those patients who were treated with microvascular decompression (MVD) in order to assess the relationship between operative findings and clinical results. METHODS: A total of 232 patients with HFS associated with the VA were treated with MVD between January 1994 and January 2009 at our institution. The patients were classified into one of the following three categories based on compression severity: Group I, mild; Group II, moderate; Group III, severe. The patients were also classified into one of the following four categories based on the existence of indentation and discoloration of nerve VII: Group A (-/-), B (+/-), C (-/+), or D (+/+). RESULTS: A total of 94.2% and 96.6% of the patients in Groups I and II, respectively, had improved to grades I-II at the last follow-up. The surgical outcomes of Group III were slightly poorer than those of Groups I and II. Group A showed the poorest outcomes with 60% of the patients classified as grades III-IV. In Group B, 98.4% of the patients showed a marked improvement and Groups C and D showed relatively poor outcomes compared with those of Group B. CONCLUSIONS: Severe deviations and color changes of the facial nerves may be the risk factors for poor surgical outcomes. Future studies with larger sample sizes and investigations of the pathophysiology underlying these findings are needed.

Classification of chronic orofacial pain: applicability of chronic headache criteria.

OBJECTIVE: The aim of this study was to test the temporal definitions of chronic daily headache (CDH) in an orofacial pain population and examine the features of the so-defined chronic orofacial pain (COFP). STUDY DESIGN: Patients with orofacial pain presenting for >3 months were prospectively collected for 2 years. Temporal patterns were applied based on the headache literature, and patients were subdivided into: COFP: ≥15 days monthly, lasting >4 hours (long-lasting CDH); daily orofacial pain (DOFP): ≥15 days monthly, but mean attack duration ≤4 hours (short-lasting CDH); and episodic orofacial pain (EOFP): <15 days monthly (episodic headaches). RESULTS: A total of 328 patients were examined. The temporal criteria defined 149 patients as COFP, 116 as DOFP, and 41 as EOFP. COFP was not distinctive in the parameters used in the diagnostic process (laterality, severity, muscle tenderness, waking) or demographic features. These were, however, significantly associated with specific diagnosis. CONCLUSION: This study provides a base for the clear definition of COFP that has the distinctive advantage of being identical to that used in headache. Additionally, our results clearly show that COFP should be regarded as a temporal definition and not a diagnosis.

Facial nerve hemangiomas: vascular tumors or malformations?

OBJECTIVE: To reclassify facial nerve hemangiomas in the context of presently accepted vascular lesion nomenclature by examining histology and immunohistochemical markers. STUDY DESIGN: Cohort analysis of patients diagnosed with a facial nerve hemangioma between 1990 and 2008. SETTING: Collaborative analysis at a specialty hospital and a major academic hospital. SUBJECTS AND METHODS: Seven subjects were identified on composite review of office charts, a pathology database spanning both institutions, and an encrypted patient registry. Clinical data were compiled, and hematoxylin-eosin-stained specimens were reviewed. For six patients, archived pathological tissue was available for immunohistochemical evaluation of markers specific for infantile hemangioma (glucose transporter protein isoform 1 [GLUT1] and Lewis Y antigen) and for lymphatic endothelial cells (podoplanin). RESULTS: All patients clinically presented with slowly progressive facial weakness at a mean age of 45 years without prior symptomatology. Hemotoxylin-eosin-stained histopathological slides showed irregularly shaped, dilated lesional vessels with flattened endothelial cells, scant smooth muscle, and no internal elastic lamina. Both podoplanin staining for lymphatic endothelial cells and GLUT1 and LewisY antigen staining for infantile hemangioma endothelial cells were negative in lesional vessels in all specimens for which immunohistochemical analysis was performed. CONCLUSION: Lesions of the geniculate ganglion historically referred to as "hemangiomas" do not demonstrate clinical, histopathological, or immunohistochemical features consistent with a benign vascular tumor, but instead are consistent with venous malformation. We propose that these lesions be classified as "venous vascular malformations of the facial nerve." This nomenclature should more accurately predict clinical behavior and guide therapeutic interventions.

New classification of rating facial nerve dysfunction.

In consideration of the complexity of the physiopathology of the facial nerve and to get a standard model of classification of his dysfunction, it appears evident the necessity in the clinical practice to have a rapid, simple and easy system. Grading of facial function is necessary for evaluating and communicating the spontaneous course and the results of medical and surgical treatment. Different grading scales have been proposed but none has been universally accepted; the author revises the principal systems of classification and proposes a new and easy model.

Facial nerve grading systems (1985-2002): beyond the House-Brackmann scale.

OBJECTIVE To assess methods of evaluating the function of the facial nerve that have been introduced over the past 15 years, particularly in comparison with the House-Brackmann scale. DATA SOURCES A Medline search was undertaken of the English-language medical literature between 1983 and 2000 to identify proposed methods of evaluating facial nerve function. STUDY SELECTION Although all grading systems were considered, attention was focused on the systems that provided improvements in either precision or ease of use. CONCLUSIONS Because of the limitations and subjectivity of the House-Brackmann scale, several new scales of various degrees of objectivity and ease of use have been introduced. The Nottingham system offers a more objective but easy-to-use facial nerve grading system that has been demonstrated to be valid and that would be easy for the average practitioner to adopt. The authors propose a more systematic evaluation of this system to determine whether its widespread application is appropriate.

Reliability of the Sunnybrook Facial Grading System by novice users.

OBJECTIVES: To evaluate the intrarater and inter-rater reliability of the Sunnybrook Facial Grading System (SFGS) by novice users. DESIGN: Prospective interval study using one measurement instrument. SETTING: Tertiary teaching hospital (Sunnybrook & Women's College Health Science Centre). METHODS: Twenty-two patients with a wide spectrum of facial dysfunction recorded on videotapes were rated using the SFGS by eight novice observers independently in two different sittings separated by 3 weeks. The order of patients was randomized for the second sitting. Intraclass correlation coefficients were calculated for component scores and for total scores within and between raters. RESULTS: The intrarater reliability coefficients for the eight raters ranged from .838 to .929. This largely overlaps with the data obtained in previous studies with expert raters. The inter-rater reliability for all eight raters at time 1 was .982 and for time 2 was .970. This is higher than what was previously obtained with expert raters. CONCLUSION: The SFGS is as reliable when applied by novice users as by expert users.

Toronto Facial Grading System: interobserver reliability.

The Toronto Facial Grading System (TFGS) is an observer scale for rating facial nerve dysfunction. The TFGS scores aspects of resting symmetry, symmetry of voluntary movement, and synkinesis for each division of the face (subscores) and then provides calculated total scores and an overall composite score of facial function. The developers of the scale have validated its sensitivity for identifying small changes in facial dysfunction and the independence of the different components measured. Herein we report our results in a study of interobserver reliability using the TFGS. Twenty-five patients from the Massachusetts Eye and Ear Infirmary Facial Nerve Center with varying degrees of facial paresis, paralysis, and synkinesis were videotaped, and the video recordings were scored by 5 independent observers using the TFGS. Intraclass correlation coefficients (kappa) and 95% confidence intervals were calculated for subscores and for each total and composite score. Intraclass correlation coefficients ranged from 0.59 to 0.85, all considered substantial to near-perfect agreement between observers. We believe the TFGS is superior to other scales by virtue of its sensitivity, comprehensiveness, ease of use, and interobserver reliability. The TFGS presently appears to be the best option in those situations in which accurate and precise documentation of facial function is required.

Specific impairment of smiling increases the severity of depressive symptoms in patients with facial neuromuscular disorders.

Depressive symptoms and related emotional distress are prevalent among patients with facial neuromuscular disorders, and the psychological distress impacts the functional disabilities associated with the facial impairment. A specific impairment in the ability to smile may elevate the risk for depression, with patients experiencing a reduced physiological feedback associated with smiling as well as the social consequences of the inability to communicate positive emotion. We tested the hypothesis that specific impairments in the ability to smile increase the severity of depressive symptoms in patients with facial neuromuscular disorders. Twenty-nine consecutive patients (mean age, 50.2 years; SD, 17.0 years; range, 18-81 years) with a facial neuromuscular disorder, who volunteered and completed all of the assessment measures participated. Facial neuromuscular impairments were assessed using multiple measures of facial motility and dysfunction, and emotional functioning was assessed using self-report measures of depression, anxiety, and positive and negative affect. Severity of global facial impairment was statistically controlled in evaluating the association between specific impairment in smiling and the degree of depressive symptoms. Separate hierarchical linear regression analyses indicated the specific impairment of smiling contributed to the prediction of depression (R(2) =.41, df = 3,25, p =.00) and anxiety (R(2) =.35, df = 3,25, p =.00), controlling first for the contribution of global impairment and facial physical disability. The specific impairment of smiling did not contribute to the prediction of positive emotional experience. Specific impairment of smiling and physical disability, but not global impairment of facial motion, were key predictors of depression in patients with facial neuromuscular disorders. The results emphasize the need to assess and treat depression and anxiety in patients with a facial neuromuscular disorder.

Validation of a treatment-based classification system for individuals with facial neuromotor disorders.

BACKGROUND AND PURPOSE: A method for linking treatments to signs and symptoms of facial neuromotor disorders is needed. We describe the construct validation of a treatment-based classification system for facial neuromotor disorders. SUBJECTS AND METHODS: Based on physical signs and symptoms, 148 patients (mean age = 48.9 years, SD = 16.1, range = 20-93) were assigned to treatment-based categories. The pattern of impairment and disability was compared with clinic expectations. RESULTS: The distribution of impairment and disability scores demonstrated the expected signs and symptoms of the treatment-based categories. Confirmatory principal-components factor analysis indicated 4 factors, corresponding to the treatment-based categories; the factor loadings confirmed the presence of the key sign or symptom characteristic of the categories. CONCLUSION AND DISCUSSION: Classifying facial neuromotor disorders into treatment-based categories appears to be a valid method for categorizing patients with specific impairments or disabilities and may be useful in linking treatments to outcomes.

Impairment and disability in patients with facial neuromuscular dysfunction.

Integration of impairment measures and disability measures may provide clinicians with an accurate and comprehensive picture of the patient's dysfunction. The purpose of this study was to indicate the usefulness of two new scales of measuring facial impairment and disability in describing characteristics of individuals with facial neuromuscular dysfunction. Fifty-one individuals with unilateral facial neuromuscular dysfunction and a House-Brackmann grade of III or higher were included in the study. The subjects' movement impairments were assessed using the Facial Grading System (FGS). The subjects reported their physical and social function on the Facial Disability Index (FDI). Nine variables were subjected to a confirmatory principal-components factor analysis to indicate important factors in describing patients with facial nerve disorders. The confirmatory principal-components factor analysis identified three factors, impairment, disability, and temporal characteristics of disease, accounting for 72% of the variance in describing individuals with facial neuromuscular dysfunction. Integration of these measures may provide clinicians with an accurate and comprehensive picture of the patient's dysfunction, thus aiding in the determination of intervention and the measurement of clinical outcomes.