Tracking Early Emergence of Sound Perception Impairments in FXS With Multimodal fNIRS/EEG-Preschool Age
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An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- The protocol lists: Speech discrimination.
- Who it may be relevant to
- Registry conditions: Fragile X Syndrome. Basic parameters: 24 months — 4 years · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- United States
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
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Official title
Tracking Early Emergence of Sound Perception Impairments in FXS With Multimodal fNIRS/EEG- Preschool Age
Overview
Individuals with Fragile X Syndrome show differences in how they understand and learn language from infancy. They frequently have lifelong delays in speech and language as well. In addition, they experience other auditory symptoms, including being very sensitive to certain sounds as well as being more sensitive than others to loud sounds. The underlying brain activity for sound perception and speech learning in Fragile X is not well understood, especially in the infant, toddler, and preschool years. This study uses behavioral assessment of speech and language abilities, neuroimaging, and hearing tests to understand how speech and hearing are different in children with Fragile X Syndrome.
Detailed description
Fragile X Syndrome (FXS) is the leading monogenic cause of intellectual disability and autism and is associated with extremely high risk for early delays in speech and language. While early childhood is essential for speech and language development, neural mechanisms for language impairments have been studied entirely in older children and adults with FXS. Therefore, markers for speech and language impairments are unavailable in young children with FXS to predict severity, test potential mechanisms, and track response to intervention. The investigators have identified a hallmark brain-based phenotype of hyperresponsiveness to sounds in adolescents and adults with FXS. This fundamental alteration in cortical responses to sound could influence early language delays, but this phenotype has not been explored in infants or toddlers with FXS.
Specifically, in this study the investigators will use simultaneous EEG/fNIRS during presentation of simple speech, stories, and nonspeech sounds to quantify and localize auditory hypersensitivity and neural differentiation in 30 preschoolers with FXS. The investigators will assess specificity through comparison with 30 typically developing controls and 30 mental-age matched children with a history of premature birth and language delays.
Interventions
- Other Speech discrimination
Two different speech sounds are played at the same sound intensity.
Primary outcome measures
- Change in Mullen Scales of Early Learning [Time frame: at single study visit between ages of 2-4 years inclusive]
- Changes in oxygenated and deoxygenated hemoglobin concentration in response to sounds in language regions of the brain [Time frame: at single study visit between ages of 2-4 years inclusive]
- Changes in amplitude of mismatch negativity response during sound discrimination [Time frame: at single study visit between ages of 2-4 years inclusive]
- Changes in hearing thresholds [Time frame: at single study visit between ages of 2-4 years inclusive]
- Otoacoustic Emissions (OAEs) [Time frame: at single study visit between ages of 2-4 years inclusive]
- Changes in tympanometric pressure profile in the inner ear [Time frame: at single study visit between ages of 2-4 years inclusive]
Secondary outcome measures (2)
- Sensory profile 2 Auditory Processing subtest [Time frame: at single study visit between ages of 2-4 years inclusive]
- Sensory Profile 2 Attentional subtest [Time frame: at single study visit between ages of 2-4 years inclusive]
Eligibility criteria
Inclusion criteria
- Diagnoses of Fragile X Syndrome, Typical Development, or History of Premature Birth
- able to sit independently
- English is spoken at home
Exclusion criteria
- For all participants: no seizures in the past 6 months
- For typical development group and Fragile X group: not born prior to 32 weeks gestation
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: Yes
Study design
- Allocation
- N/A
- Model
- Single group
- Masking
- Open label
- Primary purpose
- Basic science
Study locations
United States · 1 center
- Cincinnati Children'S Hospital — Cincinnati
Identifiers
NCT: NCT05957549 · K23HD109375 Aim 1 · K23HD109375