Auditory neuropathy/dyssynchrony: its diagnosis and management

CI Berlin, T Morlet, LJ Hood - Pediatric Clinics, 2003 - pediatric.theclinics.com
Otoacoustic emissions, middle ear muscle reflexes, and auditory brain stem response (ABR)
often are presented as ''objective and infallible hearing screening tools.''They are not …

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 …

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 …

Clinical uses of electrically evoked auditory nerve and brainstem responses

CJ Brown - Current opinion in otolaryngology & head and neck …, 2003 - journals.lww.com
The introduction of cochlear implants with the capability of measuring the response of the
auditory nerve to electrical stimulation from an intracochlear electrode has tremendous …

Cochlear implantation in patients with auditory neuropathy of varied etiologies

JC Mason, A De Michele, C Stevens… - The …, 2003 - Wiley Online Library
Abstract Objectives/Hypothesis Auditory neuropathy is a relatively recently described pattern
of hearing loss characterized by preservation of outer hair cell function despite absent …

Evidence for surviving outer hair cell function in congenitally deaf ears

PA Rea, WPR Gibson - The Laryngoscope, 2003 - Wiley Online Library
Abstract Objective/Hypothesis: The hypotheses of the study were that congenital hearing
impairment in infants can result from the isolated loss of inner hair cells of the cochlea and …

Bilateral cochlear implantation in children

K Vermeire, JPL Brokx, PH Van de Heyning… - International Journal of …, 2003 - Elsevier
Aims: to determine the benefit of bilateral cochlear implantation in a child on speech and
language development. Method: This child got her first implant, a Nucleus 24-system, on the …

Cochlear implants in young children

JK Niparko, R Blankenhorn - Mental retardation and …, 2003 - Wiley Online Library
The cochlear implant is best characterized as a device that provides access to the sound
environment. The device enables the hearing pathway to respond to environmental and …

Primary auditory neuropathy–an enigma

N Shivashankar, P Satishchandra… - Acta neurologica …, 2003 - Wiley Online Library
Objectives–To report audiological and neuroradiological findings in patients with primary
auditory neuropathy (PAN) from a tertiary institution in south India. Material and methods …