Specialization of the brain for language is early emerging and essential for language learning in young children. Fragile X Syndrome (FXS) is a neurogenetic disorder marked by high rates of delays in both expressive and receptive language, but neural activation patterns during speech and language processing are unknown. We report results of a functional Near Infrared Spectroscopy (fNIRS) study of responses to speech and nonspeech sounds in the auditory cortex in a sample of 2- to 10-year-old...

We conducted a six-center, prospective, randomized, open-label trial to assess whether an early standardized educational protocol provided from 42 to 48 months of age improved the progression of oral language and phonological development in children born preterm. A total of 552 children with phonological fragility were included in this study. The children were randomized to receive the educational protocol (guided arm, n = 87) or not (non-guided arm, n = 78). In the guided arm, the oral language...

Noise-induced cochlear synaptopathy has been studied for over 25 years with no known diagnosis for this disorder in humans. This type of "hidden hearing loss" induces a loss of synapses in the inner ear but no change in audiometric thresholds. Recent studies have shown that by two months post synaptopathy-inducing noise exposure, synapses in some animal species can regenerate. Animal studies to date have focused primarily on peripheral hearing measures to diagnose ribbon synapse loss, while...

Background: Chiari malformation is a condition involving caudal descent of the hindbrain which herniates the cerebellar tonsils through the foramen magnum. The purpose of this study was to quantify auditory deficits in an affected individual and to explore the hypothesis that cerebellar malformation specifically disrupts binaural processing. Methods: We present audiometric, electrophysiologic, imaging and auditory perceptual findings for a 17-year-old female with Chiari 1 malformation and for a...