Prevalence of auditory neuropathy (AN) among infants and young children with severe to profound hearing loss

Talaat, Hossam Sanyelbhaa; Kabel, Abdelmagied Hasn; Samy, Hesham; Elbadry, Mohamed

doi:10.1016/j.ijporl.2009.03.009

Cited by 56 publications

(38 citation statements)

References 10 publications

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“…The summated amplitude of these responses is the product of the total of the individual hair cells [6] . However, cochlear microphonics are used in the diagnosis of hearing impairment, deafness or auditory neuropathy [13,14] and they are often used as a diagnostic instrument also at high sound pressure levels [9,10,[14][15][16][17][18] .…”

Section: Discussionmentioning

confidence: 99%

“…Both in deaf patients whose cochlea has normal fluid content, and in patients with postmeningitic deafness who have an ossified or obliterated cochlea, it proved possible to record cochlear microphonics down to 80 dB [8] . As cochlear microphonics are often used in the diagnosis of hearing impairment or deafness [9,10] , the aim of the present study was to shed light on the question of whether these potentials should be viewed as artifacts or as indicating residual cochlear function.…”

Section: Introductionmentioning

confidence: 99%

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Validity of Cochlear Microphonics at High Sound Pressure Levels as an Important Clinical Aspect

Teschner

Lenarz²,

Battmer³

2012

ORL

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Introduction: Cochlear microphonics are electrical stimulus responses of the inner ear, generated by mechanical displacement of the hair cells caused by acoustic stimulation. As cochlear microphonics are often used in the diagnosis of hearing impairment and deafness, in preliminary investigations it was seen that obliteration or ossification have no effect on the extent to which cochlear microphonics can be recorded at high sound pressure levels. As artifacts at high sound pressure levels suggested, measurements were subsequently conducted using temporal bone specimens. Methods: In a test setup equivalent to that for electrocochleography, a needle electrode was placed on the cochlear promontory and used to record potentials following application of an acoustic stimulus. Results: Curves comparable to cochlear microphonics were registrable down to a threshold of 80 dB HL. Additional measurements conducted on damp cloths yielded comparable findings. Conclusions: Registration of cochlear microphonics at high sound pressure levels does not serve as an indicator of hair cell function, but should instead be regarded as artifacts. The possible sources are discussed.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Validity of Cochlear Microphonics at High Sound Pressure Levels as an Important Clinical Aspect

Teschner

Lenarz²,

Battmer³

2012

ORL

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“…7 A prevalência descrita da NA em doentes pediátricos com hipoacúsia severa ou profunda é de cerca de 13,4%. 8 De entre os fatores genéticos, muito estudados, estão estabelecidas algumas mutações em genes classicamente descritos e relacionados com a NA, como o OTOF e o PJVAK. 4 Acredita-se que tais genes possam ser responsáveis por disfunções relacionadas com as CCI.…”

Section: Introductionunclassified

“…Tal situação pode ocorrer em até 30% dos doentes com NA, pelo que a ausência de OEA não é um fator de exclusão para o diagnóstico de NA. 8,24 O MC é outra forma de verificar o funcionamento e integridade cóclea. Trata-se de um potencial de corrente alternada que aparece durante a estimulação sonora e reflete o movimento da membrana basilar.…”

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“…Como já foi referido, a pesquisa do MC é fundamental na avaliação desses doentes, principalmente na ausência de OEA. 8,24 Em alguns programas de triagem auditiva neonatal, como o do Newborn Hearing Screening Programme, a presença do MC num exame de PEATE incita a investigação ativa de patologias cocleares, como a NA. 24 Sabe-se que as células ciliadas externas são amplificadoras primárias da cóclea, e que podem auxiliar na modulação da sensibilidade das células ciliadas internas.…”

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Neuropatia Auditiva: Avaliação Clínica e Abordagem Diagnóstica

Carvalho¹,

Leão²,

Ramos³

et al. 2016

Acta Med Port

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RESUMOIntrodução: A neuropatia auditiva é uma condição na qual há alteração na condução neuronal do estímulo sonoro. Este trabalho pretende descrever e caracterizar a casuística de doentes com neuropatia auditiva. Material e Métodos:Realizámos um estudo transversal, retrospetivo, com descrição de uma série de casos consecutivos. O diagnóstico da neuropatia auditiva foi definido nas seguintes situações: Presença de otoemissões acústicas com potenciais auditivos de tronco encefálico ausente ou anormal e presença do microfonismo coclear independentemente da presença de otoemissões acústicas. Resultados:Foram avaliados 34 doentes com perda auditiva bilateral, 67% deles do sexo masculino. O aparecimento dos sintomas foi congênito em 80% dos casos. Na pesquisa das otoemissões acústicas, a resposta foi ausente em 67% dos doentes. O microfonismo coclear foi detetado em 79% dos doentes. Antecedentes gestacionais, perinatais ou ambientais relevantes estiveram presentes em 35,3% dos casos. Discussão:A literatura médica ainda apresenta grande variabilidade nos achados relacionados com a neuropatia auditiva, tanto na sua etiologia quanto nos dados epidemiológicos. Conclusão:A neuropatia auditiva apresenta um amplo espectro de alterações que podem resultar em disfunções leves a severas no funcionamento da via auditiva. Na nossa amostra, observámos que 80% das neuropatias auditivas terão tido origem congênita e/ou apresenta microfonismo coclear, 91% dos doentes apresenta défice auditivo significativo e 53% sofrem de surdez severa ou profunda. Palavras-chave: Células Ciliadas Auditivas; Células Ciliadas Auditivas Externas; Células Ciliadas Auditivas Internas; Potenciais Evocados Auditivos do Tronco Encefálico; Neuropatia Auditiva. ABSTRACT Introduction:Auditory neuropathy is a condition in which there is a change in the neuronal transmission of the auditory stimuli. Our objective was to describe the patients' series within the clinical spectrum of auditory neuropathy. Material and Methods:We designed a transversal, retrospective study, with a description of a consecutive case series. Auditory neuropathy was defined by the presence of acoustic otoemissions plus absent/abnormal auditory brainstem responses with cochlear microphonism.Results: 34 patients with bilateral hearing loss, 23 males and 11 females, were included in the study. Eighty percent of the cases had congenital onset of hearing loss. Acoustic otoemissions were absent in 67% of them. Cochlear microfonism was present in 79% of all cases. Prenatal, perinatal or ambiental factors were present in 35.2% of the cases.Discussion: Medical literature shows great variability in findings related to auditory neuropathy, both in its etiology and epidemiological data. Conclusion:Auditory neuropathy presents a broad spectrum of changes that may result from mild to severe changes in the functioning of the auditory pathway, and in our sample we observed that 80% of Auditory neuropathy have congenital onset of hearing loss and/ or with cochlear microphonism identified. 91% of patient...

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Children with Hearing Loss

Prelock

Hutchins

2018

Best Practices in Child and Adolescent Behavioral Health Care

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Prevalence of auditory neuropathy (AN) among infants and young children with severe to profound hearing loss

Cited by 56 publications

References 10 publications

Validity of Cochlear Microphonics at High Sound Pressure Levels as an Important Clinical Aspect

Validity of Cochlear Microphonics at High Sound Pressure Levels as an Important Clinical Aspect

Neuropatia Auditiva: Avaliação Clínica e Abordagem Diagnóstica

Children with Hearing Loss

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