Tracking amyotrophic lateral sclerosis disease progression using passively collected smartphone sensor data.

BACKGROUND: Passively collected smartphone sensor data provide an opportunity to study physical activity and mobility unobtrusively over long periods of time and may enable disease monitoring in people with amyotrophic lateral sclerosis (PALS). METHODS: We enrolled 63 PALS who used Beiwe mobile application that collected their smartphone accelerometer and GPS data and administered the self-entry ALS Functional Rating Scale-Revised (ALSFRS-RSE) survey. We identified individual steps from accelerometer data and used the Activity Index to summarize activity at the minute level. Walking, Activity Index, and GPS outcomes were then aggregated into day-level measures. We used linear mixed effect models (LMMs) to estimate baseline and monthly change for ALSFRS-RSE scores (total score, subscores Q1-3, Q4-6, Q7-9, Q10-12) and smartphone sensor data measures, as well as the associations between them. FINDINGS: The analytic sample (N = 45) was 64.4% male with a mean age of 60.1 years. The mean observation period was 292.3 days. The ALSFRS-RSE total score baseline mean was 35.8 and had a monthly rate of decline of -0.48 (p-value <0.001). We observed statistically significant change over time and association with ALSFRS-RSE total score for four smartphone sensor data-derived measures: walking cadence from top 1 min and log-transformed step count, step count from top 1 min, and Activity Index from top 1 min. INTERPRETATION: Smartphone sensors can unobtrusively track physical changes in PALS, potentially aiding disease monitoring and future research.

Wearable device and smartphone data quantify ALS progression and may provide novel outcome measures.

Amyotrophic lateral sclerosis (ALS) therapeutic development has largely relied on staff-administered functional rating scales to determine treatment efficacy. We sought to determine if mobile applications (apps) and wearable devices can be used to quantify ALS disease progression through active (surveys) and passive (sensors) data collection. Forty ambulatory adults with ALS were followed for 6-months. The Beiwe app was used to administer the self-entry ALS functional rating scale-revised (ALSFRS-RSE) and the Rasch Overall ALS Disability Scale (ROADS) surveys every 2-4 weeks. Each participant used a wrist-worn activity monitor (ActiGraph Insight Watch) or an ankle-worn activity monitor (Modus StepWatch) continuously. Wearable device wear and app survey compliance were adequate. ALSFRS-R highly correlated with ALSFRS-RSE. Several wearable data daily physical activity measures demonstrated statistically significant change over time and associations with ALSFRS-RSE and ROADS. Active and passive digital data collection hold promise for novel ALS trial outcome measure development.

Mother's voice and heartbeat sounds elicit auditory plasticity in the human brain before full gestation.

Brain development is largely shaped by early sensory experience. However, it is currently unknown whether, how early, and to what extent the newborn's brain is shaped by exposure to maternal sounds when the brain is most sensitive to early life programming. The present study examined this question in 40 infants born extremely prematurely (between 25- and 32-wk gestation) in the first month of life. Newborns were randomized to receive auditory enrichment in the form of audio recordings of maternal sounds (including their mother's voice and heartbeat) or routine exposure to hospital environmental noise. The groups were otherwise medically and demographically comparable. Cranial ultrasonography measurements were obtained at 30 ± 3 d of life. Results show that newborns exposed to maternal sounds had a significantly larger auditory cortex (AC) bilaterally compared with control newborns receiving standard care. The magnitude of the right and left AC thickness was significantly correlated with gestational age but not with the duration of sound exposure. Measurements of head circumference and the widths of the frontal horn (FH) and the corpus callosum (CC) were not significantly different between the two groups. This study provides evidence for experience-dependent plasticity in the primary AC before the brain has reached full-term maturation. Our results demonstrate that despite the immaturity of the auditory pathways, the AC is more adaptive to maternal sounds than environmental noise. Further studies are needed to better understand the neural processes underlying this early brain plasticity and its functional implications for future hearing and language development.

Questionable sound exposure outside of the womb: frequency analysis of environmental noise in the neonatal intensive care unit.

AIM: Recent research raises concerns about the adverse effects of noise exposure on the developing preterm infant. However, current guidelines for NICU noise remain focused on loudness levels, leaving the problem of exposure to potentially harmful sound frequencies largely overlooked. This study examined the frequency spectra present in a level-II NICU. METHODS: Noise measurements were taken in two level-II open-bay nurseries. Measurements were taken over 5 days for a period of 24 h each. Spectral analysis was focused on comparing sound frequencies in the range of human speech during daytime (7 AM-7 PM) vs. night-time (7 PM-7 AM). RESULTS: On average, daytime noise levels (Leq = 60.05 dBA) were higher than night-time (Leq = 58.67 dBA). Spectral analysis of frequency bands (>50 dB) revealed that infants were exposed to frequencies <500 Hz 100% of the time and to frequencies >500 Hz 57% of the time. During daytime, infants were exposed to nearly 20% more sounds within the speech frequency range compared with night-time (p = 0.018). CONCLUSION: Measuring the frequency spectra of NICU sounds is necessary to attain a thorough understanding of both the noise levels and the type of sounds that preterm infants are exposed to throughout their hospital stay. The risk of high-frequency noise exposure in the preterm population is still unclear and warrants further investigation.

An acoustic gap between the NICU and womb: a potential risk for compromised neuroplasticity of the auditory system in preterm infants.

The intrauterine environment allows the fetus to begin hearing low-frequency sounds in a protected fashion, ensuring initial optimal development of the peripheral and central auditory system. However, the auditory nursery provided by the womb vanishes once the preterm newborn enters the high-frequency (HF) noisy environment of the neonatal intensive care unit (NICU). The present article draws a concerning line between auditory system development and HF noise in the NICU, which we argue is not necessarily conducive to fostering this development. Overexposure to HF noise during critical periods disrupts the functional organization of auditory cortical circuits. As a result, we theorize that the ability to tune out noise and extract acoustic information in a noisy environment may be impaired, leading to increased risks for a variety of auditory, language, and attention disorders. Additionally, HF noise in the NICU often masks human speech sounds, further limiting quality exposure to linguistic stimuli. Understanding the impact of the sound environment on the developing auditory system is an important first step in meeting the developmental demands of preterm newborns undergoing intensive care.

Maternal sounds elicit lower heart rate in preterm newborns in the first month of life.

BACKGROUND: The preferential response to mother's voice in the fetus and term newborn is well documented. However, the response of preterm neonates is not well understood and more difficult to interpret due to the intensive clinical care and range of medical complications. AIM: This study examined the physiological response to maternal sounds and its sustainability in the first month of life in infants born very pretermaturely. METHODS: Heart rate changes were monitored in 20 hospitalized preterm infants born between 25 and 32 weeks of gestation during 30-minute exposure vs. non-exposure periods of recorded maternal sounds played inside the incubator. A total of 13,680 min of HR data was sampled throughout the first month of life during gavage feeds with and without exposure to maternal sounds. RESULTS: During exposure periods, infants had significantly lower heart rate compared to matched periods of care without exposure on the same day (p<.0001). This effect was observed in all infants, across the first month of life, irrespective of day of life, gestational age at birth, birth weight, age at testing, Apgar score, caffeine therapy, and requirement for respiratory support. No adverse effects were observed. CONCLUSION: Preterm newborns responded to maternal sounds with decreased heart rate throughout the first month of life. It is possible that maternal sounds improve autonomic stability and provide a more relaxing environment for this population of newborns. Further studies are needed to determine the therapeutic implications of maternal sound exposure for optimizing care practices and developmental outcomes.

Stress levels and depressive symptoms in NICU mothers in the early postpartum period.

OBJECTIVE: This study examined whether particular maternal and infant factors can identify mothers at risk for increased stress upon admission to the neonatal intensive care unit (NICU). METHODS: Eighty-five mothers of preterm infants (25-34 weeks gestation) were assessed using the Parental Stressor Scale (PSS:NICU) and the Edinburgh Postnatal Depression Scale (EPDS) within 3.24 ± 1.58 d postpartum. Hierarchical linear regression models were used to determine the extent to which maternal stress is influenced by individual factors. RESULTS: Fifty-two percent of mothers experienced increased stress (PSS:NICU score ≥3) and 38% had significant depressive symptoms (EPDS score ≥10). Stress related to alterations in parental role was the most significant source of stress among NICU mothers. Distance from the hospital and married marital status were significant predictors for stress related to alterations in parental role (p = 0.003) and NICU sights and sounds (p = 0.01), respectively. Higher stress levels were associated with higher depressive scores (p = 0.001). Maternal mental health factors, demographic factors, pregnancy factors and infant characteristics were not associated with increased stress. CONCLUSION: Elevated stress levels and depressive symptoms are already present in mothers of preterm infants upon NICU admission. Being married or living long distance from the hospital is associated with higher stress. Future work is needed to develop effective interventions for alleviating stress in NICU mothers and preventing its potential development into postnatal depression.

Impact of the NICU environment on language deprivation in preterm infants.

UNLABELLED: It is unclear whether the atypical language development commonly seen in preterm infants is a consequence of language deficiency experienced during their prolonged NICU stay. This review provides a novel viewpoint, which highlights the potential impact of the NICU design on the developmental origin of language disabilities in preterm infants. CONCLUSION: Further research is needed to identify evidence-based design solutions for providing preterm infants with a healthier linguistic hospital environment that aids growth and development.

Linking prenatal experience to the emerging musical mind.

The musical brain is built over time through experience with a multitude of sounds in the auditory environment. However, learning the melodies, timbres, and rhythms unique to the music and language of one's culture begins already within the mother's womb during the third trimester of human development. We review evidence that the intrauterine auditory environment plays a key role in shaping later auditory development and musical preferences. We describe evidence that externally and internally generated sounds influence the developing fetus, and argue that such prenatal auditory experience may set the trajectory for the development of the musical mind.

Weight gain velocity in very low-birth-weight infants: effects of exposure to biological maternal sounds.

OBJECTIVE: To examine the effects of biological maternal sounds (BMS) on weight gain velocity in very low-birth-weight (VLBW) infants (≤ 1,500 g). STUDY DESIGN: An exploratory study with a matched-control design. A prospective cohort of VLBW infants exposed to attenuated recordings of BMS during their neonatal intensive care unit hospitalization were compared with retrospective controls matched 1:1 for sex, birth weight, gestational age, scores for neonatal acute physiology and perinatal extension (SNAPPE - II) scores (n = 32). RESULTS: A linear mixed model controlling for gestational age, chronic lung disease, and days to regain birth weight revealed that infants receiving BMS significantly improved their weight gain velocity compared matched controls (p < 0.001) during the neonatal period. No differences were found on days spent nothing by mouth (p = 0.18), days until full enteral feeds (p = 0.51), total fluid intake (p = 0.93), or caloric intake (p = 0.73). CONCLUSION: Exposure to BMS may improve weight gain velocity in VLBW infants. Further research is needed to evaluate the effectiveness of this noninvasive intervention during the neonatal period.

Improved motor sequence retention by motionless listening.

This study examined the effect of listening to a newly learned musical piece on subsequent motor retention of the piece. Thirty-six non-musicians were trained to play an unfamiliar melody on a piano keyboard. Next, they were randomly assigned to participate in three follow-up listening sessions over 1 week. Subjects who, during their listening sessions, listened to the same initial piece showed significant improvements in motor memory and retention of the piece despite the absence of physical practice. These improvements included increased pitch accuracy, time accuracy, and dynamic intensity of key pressing. Similar improvements, though to a lesser degree, were observed in subjects who, during their listening sessions, were distracted by another task. Control subjects, who after learning the piece had listened to nonmusical sounds, showed impaired motoric retention of the piece at 1 week from the initial acquisition day. These results imply that motor sequences can be established in motor memory without direct access to motor-related information. In addition, the study revealed that the listening-induced improvements did not generalize to the learning of a new musical piece composed of the same notes as the initial piece learned, limiting the effects to musical motor sequences that are already part of the individual's motor repertoire.

Auditory brain development in premature infants: the importance of early experience.

Preterm infants in the neonatal intensive care unit (NICU) often close their eyes in response to bright lights, but they cannot close their ears in response to loud sounds. The sudden transition from the womb to the overly noisy world of the NICU increases the vulnerability of these high-risk newborns. There is a growing concern that the excess noise typically experienced by NICU infants disrupts their growth and development, putting them at risk for hearing, language, and cognitive disabilities. Preterm neonates are especially sensitive to noise because their auditory system is at a critical period of neurodevelopment, and they are no longer shielded by maternal tissue. This paper discusses the developmental milestones of the auditory system and suggests ways to enhance the quality control and type of sounds delivered to NICU infants. We argue that positive auditory experience is essential for early brain maturation and may be a contributing factor for healthy neurodevelopment. Further research is needed to optimize the hospital environment for preterm newborns and to increase their potential to develop into healthy children.

The multisensory brain and its ability to learn music.

Playing a musical instrument requires a complex skill set that depends on the brain's ability to quickly integrate information from multiple senses. It has been well documented that intensive musical training alters brain structure and function within and across multisensory brain regions, supporting the experience-dependent plasticity model. Here, we argue that this experience-dependent plasticity occurs because of the multisensory nature of the brain and may be an important contributing factor to musical learning. This review highlights key multisensory regions within the brain and discusses their role in the context of music learning and rehabilitation.

Exposure to biological maternal sounds improves cardiorespiratory regulation in extremely preterm infants.

OBJECTIVE: Preterm infants experience frequent cardiorespiratory events (CREs) including multiple episodes of apnea and bradycardia per day. This physiological instability is due to their immature autonomic nervous system and limited capacity for self-regulation. This study examined whether systematic exposure to maternal sounds can reduce the frequency of CREs in NICU infants. METHODS: Fourteen preterm infants (26-32 weeks gestation) served as their own controls as we measured the frequency of adverse CREs during exposure to either Maternal Sound Stimulation (MSS) or Routine Hospital Sounds (RHS). MSS consisted of maternal voice and heartbeat sounds recorded individually for each infant. MSS was provided four times per 24-h period via a micro audio system installed in the infant's bed. Frequency of adverse CREs was determined based on monitor data and bedside documentation. RESULTS: There was an overall decreasing trend in CREs with age. Lower frequency of CREs was observed during exposure to MSS versus RHS. This effect was significantly evident in infants ≥ 33 weeks gestation (p=0.03), suggesting an effective therapeutic window for MSS when the infant's auditory brain development is most intact. CONCLUSION: This study provides preliminary evidence for short-term improvements in the physiological stability of NICU infants using MSS. Future studies are needed to investigate the potential of this non-pharmacological approach and its clinical relevance to the treatment of apnea of prematurity.

The effects of noise on preterm infants in the NICU.

Preterm infants in the neonatal intensive care unit (NICU) are constantly exposed to ambient noise that often exceeds recommended levels. There is a growing concern that such noise puts preterm infants at high risk for adverse health effects. This review looks at the effects of NICU noise on the cardiovascular, respiratory, auditory and nervous systems. Loud transient noise has negative short-term effects on the cardiovascular and respiratory systems of preterm infants, although direct evidence linking noise to neonatal pathology is still unclear. Further controlled trials with larger sample sizes are needed to determine the effects of more extensive exposure to NICU noise on early brain maturation and long-term developmental outcomes.

Simulation of prenatal maternal sounds in NICU incubators: a pilot safety and feasibility study.

OBJECTIVE: This pilot study evaluated the safety and feasibility of an innovative audio system for transmitting maternal sounds to NICU incubators. METHODS: A sample of biological sounds, consisting of voice and heartbeat, were recorded from a mother of a premature infant admitted to our unit. The maternal sounds were then played back inside an unoccupied incubator via a specialized audio system originated and compiled in our lab. We performed a series of evaluations to determine the safety and feasibility of using this system in NICU incubators. RESULTS: The proposed audio system was found to be safe and feasible, meeting criteria for humidity and temperature resistance, as well as for safe noise levels. Simulation of maternal sounds using this system seems achievable and applicable and received local support from medical staff. CONCLUSION: Further research and technology developments are needed to optimize the design of the NICU incubators to preserve the acoustic environment of the womb.