Sequelae Treatment Needs Following Peripheral Facial Palsy: Retrospective Analysis of 525 Patients.

OBJECTIVE: This study uses retrospective longitudinal data from a large unselected cohort of patients with peripheral facial paralysis to determine the prevalence and patient characteristic predictors of sequelae receiving intervention. STUDY DESIGN: Retrospective case review. SETTING: Karolinska University Hospital in Stockholm Sweden serves as the only tertiary facial palsy center in the region. Here, patients are diagnosed, are followed up, and undergo all major interventions. PATIENTS: All adult patients presenting with peripheral facial palsy due to idiopathic, zoster, or Borrelia origin at Karolinska, January 1, 2010 to December 31, 2011 with follow-up until December 2022. INTERVENTIONS: Patient charts were studied to identify patient characteristics, etiology, initial treatment, severity of palsy, and treatments targeting sequelae. MAIN OUTCOME MEASURES: Types of initial and late treatments were noted. Sunnybrook and/or House-Brackmann scales were used for palsy grading. RESULTS: Five hundred twenty-five patients were included. Thirty-three patients (6.3%) received botulinum toxin injections and/or surgical treatment. In this subgroup, 67% received corticosteroids compared to 85% of all patients ( p = 0.005), cardiovascular disease prevalence was higher (23 and 42%, respectively, p = 0.009). For 81 patients (15%), follow-up was discontinued although the last measurement was Sunnybrook less than 70 or House-Brackmann 3 to 6. CONCLUSIONS: Of patients with peripheral facial palsy, 6.3% underwent injections and/or surgical treatment within 12 years. However, due to a rather large proportion not presenting for follow-up, this might be an underestimation. Patients receiving late injections and/or surgical treatment had more comorbidities and received corticosteroid treatment to a significantly lower extent in the acute phase of disease.

Bell's Palsy in Pregnancy and Postpartum: A Retrospective Case-Control Study of 182 Patients.

OBJECTIVE: To assess the incidence of Bell's palsy in pregnant and postpartum women. Additionally, to compare facial outcomes in terms of Sunnybrook score following Bell's palsy with regard to corticosteroid treatment and other confounding factors. STUDY DESIGN: Retrospective case-control study. SETTING: University Hospital, Stockholm, Sweden. METHODS: All women with Bell's palsy in pregnancy or postpartum (6 weeks after birth) with a computerized medical chart in the Stockholm Region 2005 to 2015 were included. The total number of births in the region during this period was retrieved from the Swedish Medical Birth Register. Nonpregnant age-matched women with Bell's palsy served as controls. Characteristics, medication, and Sunnybrook scores were collected. Risk factors for incomplete recovery (Sunnybrook score <96) at 3 months were calculated by logistic regression. RESULTS: In total, 182 pregnant and postpartum women with Bell's palsy were identified. The estimated incidence among pregnant and postpartum women was 60.5/100,000 person-years. The mean Sunnybrook score at 3 months was 74 among pregnant and postpartum women and 83 for controls (p = .002). At 12 months, Sunnybrook score was 81 and 89, respectively (p = .017). Only one-third of the pregnant women received corticosteroid treatment. CONCLUSION: The incidence of Bell's palsy in pregnancy and postpartum was 60.5 per 100,000 women and year in the Stockholm Region. Sunnybrook score was poorer in pregnant women compared with postpartum and nonpregnant women throughout. Corticosteroid treatment had little effect on any patients, however, only one-third of the pregnant women received this treatment.

Voice rest after vocal fold polyp surgery: A Swedish register study of 588 patients.

Objective: To evaluate the impact of voice rest on patient-reported voice outcome 4 months after vocal fold polyp surgery. Methods: Preoperative information was collected about age, sex, and smoking habits and the voice handicap index-10 (VHI-10). Four months postoperatively, voice rest (total voice rest, spoke single words, and spoke normally), and pre and postoperative voice therapy were reported. This was correlated to voice satisfaction from a two-category subjective evaluation "satisfied/ not satisfied" and to VHI-10. Logistic regression models with relative risk for NOT being satisfied with voice after surgery were performed. Results: Data from 588 patients were available. The group "spoke normally" showed the highest degree of patient satisfaction (92%). Younger patients (<59 years) were more satisfied than older (90% vs. 81.5%). High age and low VHI-10 scores before surgery were statistically significant for negative voice outcome. Gender or voice rest type did not significantly affect outcome. The largest improvement in VHI-scores was in the group who spoke normally and least in the group who spoke single words. Conclusion: We found no significant difference in the two-category subjective voice outcome depending on voice rest. VHI-10 showed a statistically significant positive effect on self-evaluated voice outcome, with the largest improvement in the group with no voice rest. However, the clinical relevance of the VHI changes is unclear. The present study does not show any advantage of total voice rest as compared to relative voice rest or speaking freely. High age and low preoperative VHI scores were significant risk factors for worse voice outcome. Level of evidence: 4.

Complications of Gastrostomy Tubes in Patients With Head and Neck Cancer.

OBJECTIVES: Head and neck cancers (HNCs) include various malignant tumors of the upper aerodigestive tract. Due to their anatomical location, HNCs can cause obstruction, odynophagia, or trismus, leading to dysphagia. In addition, this patient group may be vulnerable to treatment side effects both by surgery and oncological treatment, exposing the patients to an even higher risk of malnutrition. The risk of malnourishment is often resolved by applying a feeding gastrostomy tube. The present study aims to identify complication rates after percutaneous endoscopic gastrostomy (PEG) and open gastrostomy (OG) in patients treated for HNC in a high-volume center. STUDY DESIGN: Retrospective cohort study. METHODS: A retrospective cohort study of all patients treated for a new diagnosis of HNC at the Department of Otorhinolaryngology and Head and Neck Surgery at Karolinska University Hospital between January 1, 2000 and December 31, 2018 in whom gastrostomy was performed. RESULTS: Regarding tumor location, 165 (56.7%) were in the pharynx, 68 (23.4%) in the oral cavity, 57 (19.6%) in the larynx, and 1 (0.3%) in the nasal cavity. PEG was performed in 240 (82.5%) and OG in 51 (17.5%) patients. The overall complication rate was 28.2%: 64 (26.7%) among PEG patients and 18 (35.3%) among OG patients. The incidence of major complications was 3.1%. CONCLUSIONS: Our study confirms that enteral feeding via gastrostomy is a safe method, regardless of the technique used (PEG or OG), with a low rate of major complications and no mortality linked to the procedure. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:1778-1784, 2022.

The facial nerve palsy and cortisone evaluation (FACE) study in children: protocol for a randomized, placebo-controlled, multicenter trial, in a Borrelia burgdorferi endemic area.

BACKGROUND: Children with acute peripheral facial nerve palsy cannot yet be recommended corticosteroid treatment based on evidence. Adults with idiopathic facial nerve palsy are treated with corticosteroids, according to guidelines resulting from a meta-analysis comprising two major randomized placebo-controlled trials. Corresponding trials in children are lacking. Furthermore, acute facial nerve palsy in childhood is frequently associated with Lyme neuroborreliosis, caused by the spirochete Borrelia burgdorferi. The efficacy and safety of corticosteroid treatment of acute facial nerve palsy associated with Lyme neuroborreliosis, has not yet been determined in prospective trials in children, nor in adults. METHOD: This randomized double-blind, placebo-controlled study will include a total of 500 Swedish children aged 1-17 years, presenting with acute facial nerve palsy of either idiopathic etiology or associated with Lyme neuroborreliosis. Inclusion is ongoing at 12 pediatric departments, all situated in Borrelia burgdorferi endemic areas. Participants are randomized into active treatment with prednisolone 1 mg/kg/day (maximum 50 mg/day) or placebo for oral intake once daily during 10 days without taper. Cases associated with Lyme neuroborreliosis are treated with antibiotics in addition to the study treatment. The House-Brackmann grading scale and the Sunnybrook facial grading system are used for physician-assessed evaluation of facial impairment at baseline, and at the 1- and 12-month follow-ups. Primary outcome is complete recovery, measured by House-Brackmann grading scale, at the 12-month follow-up. Child/parent-assessed questionnaires are used for evaluation of disease-specific quality of life and facial disability and its correlation to physician-assessed facial impairment will be evaluated. Furthermore, the study will evaluate factors of importance for predicting recovery, as well as the safety profile for short-term prednisolone treatment in children with acute facial nerve palsy. DISCUSSION: This article presents the rationale, design and content of a protocol for a study that will determine the efficacy of corticosteroid treatment in children with acute facial nerve palsy of idiopathic etiology, or associated with Lyme neuroborreliosis. Future results will attribute to evidence-based treatment guidelines applicable also in Borrelia burgdorferi endemic areas. TRIAL REGISTRATION: The study protocol was approved by the Swedish Medical Product Agency (EudraCT nr 2017-004187-35) and published at ClinicalTrials.gov ( NCT03781700 , initial release 12/14/2018).

Quality of Life in Bell's Palsy: Correlation with Sunnybrook and House-Brackmann Over Time.

OBJECTIVES: To compare patient-graded facial and social/well-being function with physician-graded facial function in Bell's palsy over time. STUDY DESIGN: A prospective follow-up study at two tertiary otorhinolaryngological centers. METHODS: A total of 96 patients, 36 women and 60 men, aged 18-77 years, were included. Facial Clinimetric Evaluation (FaCE) scale and Facial Disability Index (FDI) scores were compared with Sunnybrook and House-Brackmann scores. RESULTS: Inclusion was on mean day 7 (96 patients) and follow-up on days 53 (81 patients) and 137 (32 patients). Initially, correlations between FaCE total score, FaCE domains, FDI physical function, FDI social/well-being function and Sunnybrook and House-Brackmann scores were low to fair, except for FaCE facial movement (r = 0.55). Correlations between FaCE total score and Sunnybrook score were very good to excellent at visits 2 (r = 0.83) and 3 (r = 0.81). Women scored FaCE social and FDI social/well-being function lower than men, despite similar Sunnybrook scores. CONCLUSION: In early stages of Bell's palsy, there were low to fair correlations between FaCE/FDI (except for facial movement) and Sunnybrook score. This implies that the design of the quality of life (QoL) instruments is less suited for the acute phase. The high correlations at follow-ups suggest that the questionnaires can be used for evaluation of QoL over time. Our results indicate that women experience more facial palsy-related psychosocial dysfunction. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E612-E618, 2021.

Gain-of-function mutation of microRNA-140 in human skeletal dysplasia.

MicroRNAs (miRNAs) are post-transcriptional regulators of gene expression. Heterozygous loss-of-function point mutations of miRNA genes are associated with several human congenital disorders1-5, but neomorphic (gain-of-new-function) mutations in miRNAs due to nucleotide substitutions have not been reported. Here we describe a neomorphic seed region mutation in the chondrocyte-specific, super-enhancer-associated MIR140 gene encoding microRNA-140 (miR-140) in a novel autosomal dominant human skeletal dysplasia. Mice with the corresponding single nucleotide substitution show skeletal abnormalities similar to those of the patients but distinct from those of miR-140-null mice6. This mutant miRNA gene yields abundant mutant miR-140-5p expression without miRNA-processing defects. In chondrocytes, the mutation causes widespread derepression of wild-type miR-140-5p targets and repression of mutant miR-140-5p targets, indicating that the mutation produces both loss-of-function and gain-of-function effects. Furthermore, the mutant miR-140-5p seed competes with the conserved RNA-binding protein Ybx1 for overlapping binding sites. This finding may explain the potent target repression and robust in vivo effect by this mutant miRNA even in the absence of evolutionary selection of miRNA-target RNA interactions, which contributes to the strong regulatory effects of conserved miRNAs7,8. Our study presents the first case of a pathogenic gain-of-function miRNA mutation and provides molecular insight into neomorphic actions of emerging and/or mutant miRNAs.

Validation of a Swedish version of the Facial Disability Index (FDI) and the Facial Clinimetric Evaluation (FaCE) scale.

CONCLUSION: Swedish versions of the Facial Disability Index (FDI) and Facial Clinimetric Evaluation (FaCE) scale are psychometrically valid. Both questionnaires can be used for clinical studies on peripheral facial palsy patients, and provide important information on quality of life. OBJECTIVES: To translate and validate Swedish versions of the FDI and FaCE scale in patients with peripheral facial palsy. METHODS: Translation of the original questionnaires followed international guidelines. Internal consistency and test-retest stability were assessed in adult patients with stable peripheral facial palsy. Facial function was examined with the Sunnybrook and House-Brackmann scales. Subjects answered the questionnaires twice with a 2-week interval. Validity was assessed by comparing FDI and FaCE scale scores to SF-36 and Sunnybrook/House-Brackmann scores. RESULTS: Ninety-three patients were included, 53% women and 47% men, mean age 56.9 years and mean duration of palsy 51.9 months. The questionnaires showed good/excellent psychometric properties with Cronbach's α scores between 0.76 and 0.92. In the test-retest analysis, intra-class correlation coefficients were very good for both questionnaires with scores of 0.83-0.97. Both questionnaires showed good sensitivity to discriminate between patients with varying degrees of facial dysfunction. Moderate to strong correlation was found between the social domains in the questionnaires when compared with the equivalent domains in SF-36.

The effect of prednisolone on sequelae in Bell's palsy.

OBJECTIVE: To study whether prednisolone reduces sequelae in Bell's palsy. DESIGN: Prospective, randomized, double-blind, placebo-controlled, multicenter trial with 12 months of follow-up. SETTING: Seventeen referral centers. PATIENTS: In all, 829 patients aged 18 to 75 years. INTERVENTIONS: Randomization within 72 hours in a factorial fashion to placebo plus placebo (n = 206); prednisolone, 60 mg/d for 5 days, with the dosage then tapered for 5 days, plus placebo (n = 210); valacyclovir hydrochloride, 1000 mg 3 times daily for 7 days, plus placebo (n = 207); or prednisolone plus valacyclovir (n = 206). MAIN OUTCOME MEASURES: Facial function at 12 months assessed with the Sunnybrook and House-Brackmann grading systems. RESULTS: In 184 of the 829 patients, the Sunnybrook score was less than 90 at 12 months; 71 had been treated with prednisolone and 113 had not (P < .001). In 98 patients, the Sunnybrook score was less than 70; 33 had received prednisolone and 65 had not (P < .001). The difference between patients who received prednisolone and who did not in House-Brackmann gradings higher than I and higher than II was also significant (P < .001 and P = .01, respectively). No significant difference was found between patients who received prednisolone and those who did not in Sunnybrook scores less than 50 (P = .10) or House-Brackmann grades higher than III (P = .80). Synkinesis was assessed with the Sunnybrook score in 743 patients. Ninety-six patients had a synkinesis score more than 2, of whom 33 had received prednisolone and 63 had not (P = .001). Sixty patients had a synkinesis score more than 4, of whom 22 had received prednisolone and 38 had not (P = .005). CONCLUSION: Prednisolone significantly reduces mild and moderate sequelae in Bell's palsy. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00510263.

Prediction of nonrecovery in Bell's palsy using Sunnybrook grading.

OBJECTIVES/HYPOTHESIS: To develop a clinical prognostic model to identify Bell's palsy patients with risk for nonrecovery at 12 months. STUDY DESIGN: Data from a prospective, randomized, double-blind, placebo-controlled, multicenter study. METHODS: There were 829 patients with Bell's palsy randomized in a factorial fashion to treatment with prednisolone or no prednisolone. Facial function was assessed with the Sunnybrook grading scale. Univariate and multivariate logistic regression analyses at different time points were used to identify factors predicting nonrecovery, defined as Sunnybrook <70 at 12 months. Variables studied were age, gender, time to inclusion, prednisolone treatment, side of palsy, pain at inclusion, and Sunnybrook scores. Factors of predictable significance were used to construct prognostic models at baseline, days 11 to 17, and at 1 month. Receiver operating characteristics curves were created to test the predictive capacity of the models. RESULTS: At baseline, treatment with prednisolone or no prednisolone (P = .0005), age (P = .04) and the Sunnybrook score (P = .0002) were significant factors for predicting nonrecovery. The receiver operating characteristics area under the curve at baseline for these three variables was 0.74 (sensitivity 0.83, specificity 0.57). At days 11 to 17 and at 1 month, the Sunnybrook score was the only significant predictive variable. The respective areas under the curves for the Sunnybrook score at these time points were 0.83 (sensitivity 0.81, specificity 0.75) and 0.94 (sensitivity 0.91, specificity 0.85). CONCLUSIONS: Sunnybrook grading at 1 month most accurately predicts nonrecovery at 12 months in Bell's palsy.

Early deterioration in Bell's palsy: prognosis and effect of prednisolone.

OBJECTIVE: To assess if early deterioration is a negative prognostic factor in Bell's palsy and if prednisolone treatment reduces early progression and enhances recovery. STUDY DESIGN: Data extracted from the randomized, double-blind, placebo-controlled multicenter, Scandinavian Bell's palsy study. SETTING: Sixteen tertiary referral centers in Sweden and one in Finland. PATIENTS: A total of 829 patients aged 18 to 75 years with Bell's palsy. INTERVENTION: The study design was factorial; 416 patients were given prednisolone, whereas 413 did not receive the drug. Data were analyzed with a modified intention-to-treat principle and the last-observation-carried-forward method. MAIN OUTCOME MEASURES: Facial function was assessed within 72 hours before treatment start, at Days 11 to 17, and at 12 months. Sunnybrook was used as the main facial grading system with complete recovery defined as Sunnybrook 100. RESULTS: In 236 (28%) of 829 patients, the palsy deteriorated from baseline to the first follow-up at Days 11 to 17. Complete recovery at 12 months was 45% among subjects with early deterioration compared with 73% in patients with no initial deterioration (p < 0.0001). In the early deterioration group, complete recovery at 12 months was 62% in patients treated with prednisolone and 31% in those not treated (p < 0.0001). CONCLUSION: Early deterioration in Bell's palsy is a negative prognostic factor for complete recovery at 12 months. Prednisolone given within 72 hours may reduce early progression and improve the outcome of palsy.

The effect of study design and analysis methods on recovery rates in Bell's palsy.

OBJECTIVES/HYPOTHESIS: We investigated how study design affects the rate of recovery in Bell's palsy. STUDY DESIGN: Prospective, randomized, double-blind, placebo-controlled, multicenter trial. METHODS: Data were extracted from the Scandinavian Bell's palsy study, which included 829 patients. The study design was factorial; 416 patients given prednisolone, 413 not given prednisolone, 413 patients given valacyclovir, 416 not given valacyclovir. Data were analyzed with intention-to-treat principle and complete-case analysis methods and recovery was defined as Sunnybrook score 100, House-Brackmann grade I or < or =grade II at 12 months. RESULTS: With the intention-to-treat principle and last-observation-carried-forward method (n = 829) and recovery defined as Sunnybrook 100, 300 of the 416 patients (72%) receiving prednisolone had recovered compared with 237 of the 413 (57%) who did not receive prednisolone (P < .0001). With recovery defined as House-Brackmann grade I, the corresponding recovery rates were 324 of 416 (78%) and 266 of 413 (64%) (P < .0001). With complete-case analysis and recovery defined House-Brackmann grade I (n = 782), 335 of 389 patients (86%) given prednisolone recovered compared with 277 of 393 (70%) in the group not given prednisolone (P < .0001). With recovery defined as House-Brackmann < or =grade II (n = 797), the corresponding recovery rates were 380 of 396 (96%) and 353 of 401 (88%) (P < .0001). The analysis method affected the recovery rates in the valacyclovir and no-valacyclovir groups in a similar way as in the prednisolone and no-prednisolone groups. CONCLUSIONS: Recovery rates in a Bell's palsy study are substantially affected by the choice of analysis method and definition of recovery.