Definition of the clinical spectrum of kernicterus and bilirubin-induced neurologic dysfunction (BIND)

SM Shapiro - Journal of perinatology, 2005 - nature.com
Kernicterus, currently used to describe both the neuropathology of bilirubin-induced brain
injury and its associated clinical findings, is a complex syndrome. The neurobiology of …

Auditory neuropathy/dys-synchrony and its perceptual consequences

G Rance - Trends in amplification, 2005 - journals.sagepub.com
Auditory neuropathy/dys-synchrony is a form of hearing impairment in which cochlear outer
hair cell function is spared but neural transmission in the auditory pathway is disordered …

Multi-site diagnosis and management of 260 patients with Auditory Neuropathy/Dys-synchrony (Auditory Neuropathy Spectrum Disorder*)

CI Berlin, LJ Hood, T Morlet, D Wilensky… - … journal of audiology, 2010 - Taylor & Francis
Test results and management data are summarized for 260 patients with diagnoses of
Auditory Neuropathy Spectrum Disorder (ANSD). Hearing aids were tried in 85 of these …

Perceptual consequences of disrupted auditory nerve activity

FG Zeng, YY Kong… - Journal of …, 2005 - journals.physiology.org
Perceptual consequences of disrupted auditory nerve activity were systematically studied in
21 subjects who had been clinically diagnosed with auditory neuropathy (AN), a recently …

Speech perception and cortical event related potentials in children with auditory neuropathy

G Rance, B Cone-Wesson, J Wunderlich… - Ear and …, 2002 - journals.lww.com
Objectives 1) To investigate the unaided and aided speech perception abilities of children
with auditory neuropathy (AN) and to compare their performance to children with …

Cochlear implantation in children with auditory neuropathy spectrum disorder

HFB Teagle, PA Roush, JS Woodard, DR Hatch… - Ear and …, 2010 - journals.lww.com
Objective: To report the patient's characteristics, preoperative audiological profiles, surgical
outcomes, and postoperative performance for children with auditory neuropathy spectrum …

Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene

R Varga, PM Kelley, BJ Keats, A Starr… - Journal of medical …, 2003 - jmg.bmj.com
It is estimated that about 1 in 500 children are born with a significant hearing loss. 1 Non-
syndromic recessive hearing loss (NSRHL) represents a major aetiologic factor in childhood …

Chronic bilirubin encephalopathy: diagnosis and outcome

SM Shapiro - Seminars in Fetal and Neonatal Medicine, 2010 - Elsevier
Chronic bilirubin encephalopathy (kernicterus) can be diagnosed using semi-objective
criteria based on history, physical and neurological examination and laboratory findings …

Auditory neuropathy in patients carrying mutations in the otoferlin gene (OTOF)

M Rodríguez‐Ballesteros, FJ del Castillo… - Human …, 2003 - Wiley Online Library
Inherited hearing impairment affects one in 2,000 newborns. Nonsyndromic prelingual forms
are inherited mainly as autosomal recessive traits, for which 16 genes are currently known …

Pathology and physiology of auditory neuropathy with a novel mutation in the MPZ gene (Tyr145→Ser)

A Starr, HJ Michalewski, FG Zeng, S Fujikawa‐Brooks… - Brain, 2003 - academic.oup.com
We studied a family with hereditary sensory motor neuropathy and deafness accompanying
a missense mutation in the MPZ gene. Pathological examination of the cochlea in one of the …