Accurate Screening Tools for Dysphagia in Amyotrophic Lateral Sclerosis (ALS)

The participant will be seated with the nose occluded using a nose clip. After inhaling to total lung capacity, the participant will place his or her lips around the mouthpiece and blow out as forcefully as possible. A flanged rubber mouthpiece will be used to overcome the inability of some individuals to create tight lip seal due to facial muscle weakness. Three trials will be performed and the patients highest maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP) used.

The Iowa Oral Performance Instrument (IOPI) is a device that measures the peak pressure performance of lingual strength and endurance via the action of a bulb placed on the hard palate.

The SFB7 Bioimpedance device measures the function of the tongue by calculating measures of reactance, resistance and phase once a custom electrode array is placed on the midline of the subject's tongue for approximately 2 seconds.

"A capsaicin challenge with three randomized blocks of 0, 50, 100, 200, and 500 μM capsaicin. The capsaicin will be dissolved in a vehicle solution consisting of 80% physiological saline, and 20% ethanol. Participants will be given the instruction cough if you need to prior to capsaicin delivery.The solution will be administered automatically upon detection of an inspired breath and there will be a minimum of one minute between each trial. This is to test the reflexive cough testing for upper airway sensitivity and motor thresholds."

Videofluoroscopic swallowing study will be performed to measure the oropharyngeal swallowing.

Pulmonary Function testing will be performed using conventional methods and will include the following outcomes: forced vital capacity (FVC), sniff nasal inspiratory pressure (SNIP), peak expiratory flow (PEF), forced expiratory volume (FEV1) expressed as a percentage of predicted values.

Swallowing Quality of Life Questionnaire (SWAL-QOL) will be used for participant reporting of swallow-related quality of life.

The Functional Oral Intake Scale (FOIS) will be used for participants to report their food intake habits.

The Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) will be used to track global disease progression in participants.

The Eating Assessment Tool-10 will be used for participant reporting of swallowing system severity.

The Communication Effectiveness Survey (CES) will be used for participant reporting of communication abilities across different speech contexts.

The Center for Neurologic Studies Bulbar Function Scale (CNS-BFS) will be used to report bulbar function in study participants.