Prevalence and Audiological Characteristics of Auditory Neuropathy Spectrum Disorder in Pediatric Population: A Retrospective Study

Vignesh, S. S.; Jaya, V.; Muraleedharan, A.

doi:10.1007/s12070-014-0759-6

Cited by 25 publications

(11 citation statements)

References 16 publications

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“…8 Other reported data indicate the prevalence of ANSD to be 1.2%, 5.1%, or 8.4% depending on the population. 27–29 Forty percent (40%) of ANSD is estimated to have a genetic basis, with the remainder due to acquired causes like hypoxia, prematurity, and jaundice, underscoring the increased rate of ANSD in neonatal intensive care units. The list of causative genes for ANSD includes DIAPH3 , OTOF , PJVK , and mitochondrial DNA (mtDNA) variants (m.1095T>C) for nonsyndromic ANSD, and AIFM1 , DDDP , MPZ , OPA1, PMP22 , and TMEM126A for syndromic ANSD, although based on prevalence data there are likely other genes involved.…”

Section: The Current Nbhs: Strengths and Weaknessesmentioning

confidence: 99%

A proposal for comprehensive newborn hearing screening to improve identification of deaf and hard-of-hearing children

Shearer

Shen

Amr

et al. 2019

Genetics in Medicine

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Early intervention for newborns who are deaf or hard-of-hearing leads to improved language, communication, and social–emotional outcomes. Universal physiologic newborn hearing screening has been widely implemented across the United States with the goal of identifying newborns who are deaf or hard-of-hearing, thereby reducing time to diagnosis and intervention. The current physiologic newborn hearing screen is generally successful in accomplishing its goals but improvements could be made. In the past ten years, genetic testing has emerged as the most important etiological diagnostic test for evaluation of children with deafness and congenital cytomegalovirus has been recognized as a major cause of childhood deafness that may be treatable. A comprehensive newborn hearing screen that includes physiologic, genetic, and cytomegalovirus testing would have multiple benefits, including (1) identifying newborns with deafness missed by the current physiologic screen, (2) providing etiologic information, and (3) possibly decreasing the number of children lost to follow up. We present a framework for integrating limited genetic testing and cytomegalovirus screening into the current physiologic newborn hearing screening. We identify needed areas of research and include an overview of genome sequencing, which we believe will become available over the next decade as a complement to universal physiologic newborn hearing screening.

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Section: The Current Nbhs: Strengths and Weaknessesmentioning

confidence: 99%

A proposal for comprehensive newborn hearing screening to improve identification of deaf and hard-of-hearing children

Shearer

Shen

Amr

et al. 2019

Genetics in Medicine

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“…The site of lesion for this type of disorder is probably the inner hair cells of cochlea, spiral ganglia and the auditory nerve. Hyperbilirubinemia, anoxia/hypoxia, prenatal/neonatal infections, immune disorders are possible risk factors for this disorder [10]. So a detailed evaluation of antenatal, natal and postnatal history is important to identify this spectrum of disorders.…”

Section: Discussionmentioning

confidence: 99%

Assessment of Hearing Status by Pure Tone Audiogram—An Institutional Study

Balasubramanian¹,

Thirunavukkarasu²,

Kalyanasundaram³

et al. 2015

IJOHNS

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Aim: To assess the hearing status of the study subjects in terms of degree and type of hearing loss, and establish the burden of this disability in the society. Materials and methods: This is a prospective study conducted in patients who attend our OPD. After an otorhinolaryngeal examination, all the patients were subjected to pure tone audiometry using MAICA-MA52 audiometer. Results: Our study comprises 1012 males (64%) and 563 females (36%). Out of this, about 15% have conductive deafness and 42% have sensorineural hearing loss. About 29% suffer from mild hearing loss, 26% moderate and 11% severe hearing loss. The alarming information is that about 5% have total hearing loss of Sudden Sensorineural type (SSNHL). Conclusion: Pure tone audiometry is cost effective and easy to perform. Early diagnosis and timely intervention will reduce the morbidity of deafness in our country. Hence it is necessary to identify and treat sudden sensorineural hearing loss and noise induced hearing loss at an early stage.

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“…The prevalence of ANSD remains uncertain, and studies report prevalences ranging from less than 1% of hearing impaired patients up to 10% [1,12,14,[21][22][23]. This variability reflects the wide heterogeneity of clinical profiles of ANSD patients across studies [12].…”

Section: Prevalence Of Ansdmentioning

confidence: 99%

Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports

Siati

Rosenzweig

Gersdorff

et al. 2020

JCM

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Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by deteriorated speech perception, despite relatively preserved pure-tone detection thresholds. Affected individuals usually present with abnormal auditory brainstem responses (ABRs), but normal otoacoustic emissions (OAEs). These electrophysiological characteristics have led to the hypothesis that ANSD may be caused by various dysfunctions at the cochlear inner hair cell (IHC) and spiral ganglion neuron (SGN) levels, while the activity of outer hair cells (OHCs) is preserved, resulting in discrepancies between pure-tone and speech comprehension thresholds. The exact prevalence of ANSD remains unknown; clinical findings show a large variability among subjects with hearing impairment ranging from mild to profound hearing loss. A wide range of prenatal and postnatal etiologies have been proposed. The study of genetics and of the implicated sites of lesion correlated with clinical findings have also led to a better understanding of the molecular mechanisms underlying the various forms of ANSD, and may guide clinicians in better screening, assessment and treatment of ANSD patients. Besides OAEs and ABRs, audiological assessment includes stapedial reflex measurements, supraliminal psychoacoustic tests, electrocochleography (ECochG), auditory steady-state responses (ASSRs) and cortical auditory evoked potentials (CAEPs). Hearing aids are indicated in the treatment of ANSD with mild to moderate hearing loss, whereas cochlear implantation is the first choice of treatment in case of profound hearing loss, especially in case of IHC presynaptic disorders, or in case of poor auditory outcomes with conventional hearing aids.

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Prevalence and Audiological Characteristics of Auditory Neuropathy Spectrum Disorder in Pediatric Population: A Retrospective Study

Cited by 25 publications

References 16 publications

A proposal for comprehensive newborn hearing screening to improve identification of deaf and hard-of-hearing children

A proposal for comprehensive newborn hearing screening to improve identification of deaf and hard-of-hearing children

Assessment of Hearing Status by Pure Tone Audiogram—An Institutional Study

Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports

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