impact of the pre-phonatory inspiratory volume on the speech quality of neuromuscular patients dependent on non-invasive ventilation

Conditions: Neuromuscular Diseases; Non Invasive Ventilation

Summary: we want to demonstrate that modifications of the ventilation parameters are liable to improve the different characteristics of phonation (duration, intensity, prosody..) in neuromuscular patients who are dependent on non invasive ventilation.

speech and communication quality depend on respiration efficiency. the respiratory involvement observed in neuromuscular disorders can impair speech quality in patients, while the underlying disease may also contribute to alter phonation. nowadays, the first line treatment of neuromuscular chronic neuromuscular respiratory failure is noninvasive ventilation (niv). with disease progression, it is used with increasingly duration during daytime. in that situation, mouthpiece ventilation is preferred as it allows efficient ventilation while being more comfortable for patients who can chose when they want to be ventilated. however, in that situation, patients do not receive ventilatory support while they are speaking which puts them in a less favorable situation for speech. we think that pre-phonation inspiratory volume is an essential part of speech quality. without mechanical ventilation, this volume is reduced as a consequence of respiratory failure but it is liable to increase significantly if the patient used the volume delivered by the ventilator . we believe that phonation is improved by niv by applying specific ventilation parameters in patients dependant on mechanical ventilation. the modification could be used by neuromuscular patients to improve speech quality; the patients would then be able to use their usual ventilatory support to improve phonation and modulation of their speech. in this crossover open labelled, randomised study, done in a single center (home ventilation unit of the referral center of hospital raymond poincaré hupifo (university hospital of western paris and ile de france) (garches, france)), phonation characteristics will be studied in 3 situations (during spontaneous breathing without ventilatory support, with the usual niv parameters, with speech-specific niv parameters) during which speech trials will be performed.

Eligibility criteria:
inclusion criteria: * adult patients (age \> or equal to 18) * chronic restrictive respiratory failure due to neuromuscular disease * spontaneous breathing autonomy of at least one hour during the days * stable clinical state * patient with middle school education level (able to read) * patients using life support ventilator ((astral 150 (resmed®), elysée (resmed®), trilogy (philips respironics®), ventilogicls (weinman®), vivo 40 (breas®), pb560 ou légendair (covidien®), monal t50 (air liquide system®)) exclusion criteria: * refusal to participate * unability to cooperate * illiterate patients * tracheostomised patients * spontaneous breathing autonomy \< 1h * cardiovascular instability * not registered with the social security system

Keywords: mechanical ventilation noninvasive ventilation neuromuscular disorder phonation mouthpiece ventilation