Facing facial weakness: psychosocial outcomes of facial weakness and reduced facial function in facioscapulohumeral muscular dystrophy.

PURPOSE: To assess the psychosocial outcomes of facial weakness in facioscapulohumeral muscular dystrophy (FSHD). MATERIALS AND METHODS: A cross-sectional survey study. The severity of facial weakness was assessed by patients (self-reported degree of facial weakness) and by physicians (part I FSHD clinical score). Questionnaires on facial function, psychosocial well-being, functioning, pain, and fatigue were completed. Regression analyses were performed to explain variance in psychosocial outcomes by demographic and disease variables. RESULTS: One hundred and thirty-eight patients participated. They reported mild to moderate psychological distress, no to mild fear of negative evaluation, and moderate to good social functioning. However, patients with severe self-reported facial weakness scored lower in social functioning. Patients with more facial dysfunction experienced more fear of negative evaluation and lower social functioning. Furthermore, younger age, presence of pain, fatigue, walking difficulty, and current or previous psychological support were associated with lower psychosocial outcomes. Overall, patients report moderate to good psychosocial functioning in this study. The factors contributing to lower psychosocial functioning are diverse. CONCLUSIONS: A multidisciplinary, personalized approach, focusing on coping with physical, emotional, and social consequences of FSHD is supposed to be helpful. Further research is needed to assess the psychosocial outcomes of facial weakness in younger patients.Implications for rehabilitationResearch on the psychosocial consequences of facial weakness in facioscapulohumeral muscular dystrophy (FSHD) is limited.Patients with FSHD experience mild to moderate psychosocial distress, partly due to overall disease severity, such as reduced mobility, and partly due to facial weakness and reduced facial function.Self-reported degree of facial weakness and facial dysfunction were related to lower psychosocial outcomes (social functioning, fear of negative evaluation, and psychological distress).Physician-reported degree of facial weakness was not related to psychosocial outcomes, suggesting an absence of a strong correlation between observed facial weakness and experienced disease burden in this study.This calls for a multidisciplinary, personalized approach with a focus on coping with physical, emotional, and social consequences of FSHD.

Ten-year retrospective evaluation of therapeutic choices and related satisfaction in patients with auricular deformities.

To adequately counsel future patients with auricular abnormalities, this study aimed to analyze the therapeutic choices patients make after consultation concerning their auricle and/or hearing. All patients who visited the auricle consultation between January 2010 and January 2020 were included. This comprises patients with all types of auricular anomalies of both congenital and acquired etiology. Follow-up was performed by telephone to obtain informed consent and to take a short nonvalidated questionnaire regarding their therapeutic choices and satisfaction with these choices. Patient characteristics and information regarding therapeutic choices after consultation were subsequently obtained from the patients' medical files. The main outcome parameters were the patients' therapeutic choices regarding the auricle and hearing. A total of 134 patients (61.2%) were included with a mean follow-up of 3.85 years. For congenital and acquired etiologies, 72.8% and 18.2% declined reconstruction, 14.0% and 18.2% chose autologous, 5.3% and 0% chose alloplastic, 6.1% and 45.4% chose prosthetic, and 1.8% and 18.2% chose another type of reconstruction, respectively. A total of 54.9% declined hearing rehabilitation, while 38.2% chose percutaneous bone conduction device (BCD), 2.0% transcutaneous BCD, and 4.9% BCD on a softband. The overall satisfaction score was 8.7/10 concerning the auricle and 8.3/10 regarding hearing. In conclusion, most patients with a congenital etiology chose to wait or decided against auricular reconstruction, while patients with acquired etiologies mostly opted for reconstruction by a prosthesis. Patients or their caregivers were very satisfied with their choice, regardless of whether this implies reconstruction or no reconstruction, which emphasizes the importance of shared decision-making and thorough counseling.