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 and toddler 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.
Diagnoses of Fragile X Syndrome, Typical Development, or History of Premature Birth
able to sit independently
English is spoken at home
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
Single group
Speech discrimination
Other interventionTwo different speech sounds are played at the same sound intensity.
Change in Mullen Scales of Early Learning
change in Mullen Scales scores (age-corrected) from baseline on all subscales, including Expressive Language, Receptive Language, FIne Motor, Gross Motor, and Visual Reception. T-Scores from the Mullen Scales of Early Learning (MSEL) have a mean of 50 and a standard deviation of 10. Range=20-80. Higher scores indicate more advanced developmental skills.
Changes in oxygenated and deoxygenated hemoglobin concentration in response to sounds in language regions of the brain
Relative increase in oxygenated versus deoxygenated hemoglobin for no sounds, low intensity sounds, and medium intensity sounds. Measured via functional Near Infrared Spectroscopy with optodes placed over frontal, temporal, and parietal language regions.
Changes in amplitude of mismatch negativity response during sound discrimination
Electroencephalography is measured over the scalp while participant listens to speech sounds with infrequent "oddball" stimuli. Amplitude of the P100 for all stimuli as well as amplitude of the mismatch negativity response (frequent minus infrequent response) as well as change in these metrics over development are tracked in all groups.
Changes in hearing thresholds
Hearing thresholds in dB as assessed using Conditioned Play Audiometry (CPA) or Visual Reinforced Audiometry (VRA) dependent on child age and developmental ability.
Sensory profile 2 Auditory Processing subtest
parent-report measure of child's behavioral responses to sounds in their environment
Sensory Profile 2 Attentional subtest
parent-report measure of child's awareness of and responses to sensory cues in their environment.
Click on the lead sponsor to view all of their trials.
