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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)
BACKGROUND Children with complex airway problems see multiple specialists. To improve outcomes and coordinate care, we developed a multidisciplinary Children's Airway Center. For children with tracheostomies, aspects of care targeted for improvement included optimizing initial hospital discharge, promoting effective communication between providers and(More)
OBJECTIVES/HYPOTHESIS Mutations in GJB2 are a common cause of congenital sensorineural hearing loss. Many children with these mutations receive cochlear implants for auditory habilitation. The purpose of the study was to compare the speech perception performance of cochlear implant patients with GJB2-related deafness to patients without GJB2-related(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)
BACKGROUND The greater palatine canal (GPC) local injection is used to limit posterior bleeding during sinus surgery in adults. Given the potential for causing iatrogenic damage to the intraorbital contents, this procedure is not commonly used in the pediatric population. No studies have described the anatomic development of the GPC during facial growth. By(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)
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)
OBJECTIVE To report the patient's characteristics, preoperative audiological profiles, surgical outcomes, and postoperative performance for children with auditory neuropathy spectrum disorder (ANSD) who ultimately received cochlear implants (CIs). DESIGN Prospective, longitudinal study of children with ANSD who received CIs after a stepwise management(More)
Sleep disordered breathing syndromes in pediatric patients can lead to adverse effects in the cardiovascular system, neurocognitive function, growth, and behavior. These syndromes occur more frequently in patients with craniofacial disorders. A high index of suspicion as well as early recognition, detection, and treatment of these syndromes are considered(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)