Correlation between dysphonia and dysphagia evolution in amyotrophic lateral sclerosis patients

Abstract

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.

Keywords:

• Sclerosis lateral amyotrophic
• dysphagia
• dysphonia
• speech production measurement

Disclosure statement

The authors report no conflict of interest.

Additional information

Notes on contributors

Chiara Mezzedimi

Chiara Mezzedimi obtained title of Otolaryngologist at University of Siena and subsequently she attended Audiology and Phoniatry School at Florence University. Actually, she works at University Hospital of Siena where coordinates the Speech and Swallawing Service. She teaches to Speech Therapist students at Siena University and collaborates with Childhood Neuropsichiatry and Neurology Clinic.

Enza Vinci

Enza Vinci, in 2016, she obtained the title of Speech and Language Therapist graduating with full marks at the University of Siena. Subsequently she obtained a 1st level Master at the Lumsa University in Neuropsychology of developmental age. She currently works as a freelancer.

Fabio Giannini

Fabio Giannini obtained degree with honors in Medicine and Surgery in 1976. He graduated in Neurology, Neurophysiopathology and Psychiatry. University Researcher from 1981 at Department of Neuroscience. Since 1998 Head of the Clinic for Neuromuscular Diseases. Actually associate Professor at University of Siena, he is a highly experienced Neurologist in neurodegenerative diseases.

Serena Cocca

Serena Cocca, after attending Medicine study, she enrolled in Ear Nose Throat School of University of Pisa. She obtained the title of Otolaryngologist graduating with honors. She is also author of numerous publications in international journals with particular interest to dysphagia in Rett's disease, oral oncology, Rhinosinusitis.