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OBJECTIVE To describe a group of children exhibiting electrophysiologic responses characteristic of auditory neuropathy (AN) who were subsequently identified as having absent or small cochlear nerves (i.e., cochlear nerve deficiency). DESIGN A retrospective review of the clinical records, audiological testing results, and magnetic resonance imaging (MRI)(More)
OBJECTIVE To describe the internal auditory canal (IAC) and inner ear morphologic characteristics of children with cochlear nerve (CN) deficiency. STUDY DESIGN Retrospective case series. SETTING Tertiary referral center. PATIENTS Fourteen children with small or absent (deficient) CNs have been identified by means of high-resolution magnetic resonance(More)
Nitric oxide (NO) mediates the effects of the excitatory amino acids in the central nervous system. Excitatory amino acids, in particular L-glutamate, are thought to be the neurotransmitter(s) present at the cochlear hair cell-afferent nerve synapse. To our knowledge, no studies to date have documented the presence of NO in the cochlea nor attempted to(More)
Nitric oxide is known to function as a neurotransmitter in the central nervous system. It is also known to be involved in the central nervous system excitatory amino acid neurotransmission cascade. Activation of excitatory amino acid receptors causes an influx of calcium, which activates nitric oxide synthase. The resulting increase in intracellular nitric(More)
Atlas-building from population data is widely used in medical imaging. However, the emphasis of atlas-building approaches is typically to estimate a spatial alignment to compute a mean/median shape or image based on population data. In this work, we focus on the statistical characterization of the population data, once spatial alignment has been achieved.(More)
OBJECTIVE To explore interrater and intrarater reliability (R (inter) and R (intra), respectively) of a standardized scale applied to nasoendoscopic assessment of velopharyngeal (VP) function, across multiple centers. DESIGN Multicenter blinded R (inter) and R (intra) study. SETTING Eight academic tertiary care centers. PARTICIPANTS Sixteen(More)
Young children with upper airway problems are at risk for hypoxia, respiratory insufficiency and long term morbidity. Computational models and quantitative analysis would reveal airway growth patterns and benefit clinical care. To capture expected growth patterns we propose a method to build a pediatric airway atlas as a function of age. The atlas is based(More)
Minimally invasive imaging of upper airway obstructions in children and adults is needed to improve clinical decision-making. Toward this goal, we demonstrate an anatomical optical coherence tomography (aOCT) system delivered via a small-bore, flexible endoscope to quantify the upper airway lumen geometry. Helical scans were obtained from a(More)
This article is the second in a 2-part series reviewing neuroimaging in childhood SNHL. Previously, we discussed the clinical work-up of children with hearing impairment, the classification of inner ear malformations, and congenital nonsyndromic causes of hearing loss. Here, we review and illustrate the most common syndromic hereditary and acquired causes(More)
OBJECTIVES To assess the effect of an intraoperative perilymph fluid gusher during cochlear implantation on speech perception abilities in pediatric patients with labyrinthine anomalies. SETTING Tertiary care academic referral center. METHODS Seventy subjects with labyrinthine malformations who received a cochlear implant were identified in our(More)