Correlation between dysphonia and dysphagia evolution in amyotrophic lateral sclerosis patients

Purpose: Amyotrophic lateral sclerosis (ALS) is a non-demyelinating neurodegenerative disease in adults with motor disorders. It is the most prevalent neuromuscular disease worldwide. Two forms exist: a sporadic form (90% of cases) and a family form due to mutations in more than 20 genes. Patient clinical characteristics are muscle weakness, dysphagia, dysphonia and respiratory failure. The cause of ALS is unknown as we do not know why it affects some people and not others. The aim of this pilot study is to evaluate the possible relationship between dysphonia and dysphagia in ALS patients and if a deterioration of speech ability is linked with a worsening of dysphagia. Materials and methods: Between March 2017 and February 2018, 27 newly diagnoses patients (about 2 months) were examined in the ENT Clinic of our University Hospital. The bulbar-onset of ALS was diagnosed by neurologists. Results: According to the endoscopic and clinical results, patients were classified in 4 classes of dysphagia. Laryngology and speech pathology assessment with spectroacuostic analysis of speech using Praat software are fundamental for the proper evaluation of dysphonia of these patients. This study was the first attempt to find a vocal indicator of advancement in swallowing dysfunction in the patient population of ALS. Preliminary findings indicate that the reduction in the maximum fundamental frequency is associated with advancement of dysphagia. Conclusions: Monitoring the vocal parameters could be useful in order to give an early rehabilitation aid to these patients improving their quality of life and reducing aspiration risks. © 2020 Informa UK Limited, trading as Taylor & Francis Group

Correlation between dysphagia and malocclusion in rett syndrome a preliminary study

Objectives: Rett syndrome (RS) is a severe neurological developmental disorder characterised by stereotypical hand movements, epileptic seizures, craniofacial dysmorphism and digestive dysfunction. This study aimed to examine the correlation between the severity of malocclusion and dysphagia in patients with RS. Methods: This preliminary study was conducted at the Ear, Nose & Throat Clinic of the University Hospital of Siena, Siena, Italy, from January 2014 to December 2017. A total of 56 patients with RS were examined and grouped according to the severity of dysphagia (absent, mild, moderate or severe) and malocclusion (<2 mm, 2–3 mm, 3–4 mm or >4 mm). Results: All of the patients were female and the mean age was 11.3 years. Eight (14.3%) patients had mild, 18 (32.1%) had moderate and 30 (53.6%) had severe dysphagia. Four (7.1%) patients had <2 mm occlusion, 10 (17.9%) had 2–3 mm occlusion, 26 (46.4%) had 3–4 mm occlusion and 16 (28.6%) had >4 mm occlusion. Mild dysphagia was observed in 100% and 40% of patients with <2 and 2–3 mm malocclusion, respectively, while moderate dysphagia was present in 60% and 38.5% of patients with 2–3 and 3–4 mm malocclusion, respectively. Severe dysphagia was observed in 28.6% and 87.5% of patients with 3–4 and >4 mm malocclusion, respectively. There was a significant correlation between dysphagia and malocclusion severity (P <0.001). Conclusion: A higher degree of malocclusion was associated with more severe dysphagia among a cohort of patients with RS