Prevalence of auditory neuropathy spectrum disorder in an auditory health care service

Penido, Rosimar Costa; Isaac, Myriam Lima

doi:10.5935/1808-8694.20130077

Cited by 33 publications

(28 citation statements)

References 27 publications

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“…Estimates of how frequently hearing impairment is caused by auditory neuropathy vary substantially, from ≤1% 7,8 to almost 10% 9 of individuals with hearing impairment. Some of this variance is attributed to the inclusion in these studies of populations with different types of hearing impairment.…”

Section: Prevalencementioning

confidence: 99%

Auditory neuropathy — neural and synaptic mechanisms

2016

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Section: Prevalencementioning

confidence: 99%

Auditory neuropathy — neural and synaptic mechanisms

2016

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“…Em cinco casos (14%) foram detetadas alterações genéticas em genes potencialmente envolvidos na génese da NA, porém, em nenhum dos casos do nosso estudo houve evidência clínica ou laboratorial de uma relação de causa e efeito. 19,21 Estudos tanto clássicos como recentes defendem que a etiologia da NA está relacionada, em 42% das vezes com patologias neurológicas hereditárias, em 10% dos casos com situações diversas (tóxicos, metabólicas, infeciosas, …) e que, nos restantes 48% dos casos, a NA é idiopáti-ca. 19,[21][22][23] Na nossa amostra, quase 40% dos doentes tinha histó-ria gestacional/perinatal ou antecedentes ambientais relevantes, tendo sido os mais prevalentes a prematuridade e a história de internamento UCI neonatal.…”

Section: Discussionunclassified

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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“…Generally, patients still have difficulty in speech discrimination, inconsistent with the pure tone audiogram findings [1,2] .…”

Section: Introductionmentioning

confidence: 99%

“…Auditory neuropathy (AN) is a condition caused by a deficiency of synchronous neural activity of the cochlear nerve and is related to injuries that can affect the inner hair cell synapse, spiral ganglion, axon, the myelin sheath, and nerve dendrite [1][2][3] .…”

Section: Introductionmentioning

confidence: 99%

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Performance of Cochlear Implants in Pediatric Patients with Auditory Neuropathy Spectrum Disorder

Carvalho¹,

Ramos²,

Arthur³

et al. 2016

Int Adv Otol

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OBJECTIVE:To describe the performance and results of CIs (cochlear implant) in patients with AN (auditory neuropathy) and to present a medical literature review. MATERIALS and METHODS:Retrospective chart review of patients with AN who were treated with CI. The mesh terms used for the review in the Pubmed and Scopus databases were as follows: "hearing loss, cochlear implants, rehabilitation of persons with hearing impairment, auditory neuropathy". Statistical Analyses: The Mann-Whitney test was performed. RESULTS:The sample consisted of 10 patients. The mean age at surgery was 4.3 years, range 2-16 years. The average length of CI use was 5.2 years. The comparison of hearing levels before and after CI use showed a significant improvement in all patients, with p<0.05. All of them also reported an increase in overall satisfaction 1 year after the procedure. A CI is the standard treatment for the hearing rehabilitation of patients with severe profound hearing loss who do not benefit from conventional hearing aids. There are diseases such as AN that also invoke a discussion in the literature regarding CI benefits. CONCLUSION:Individuals with an demonstrated a significant gain in hearing levels and language use with CI. KEYWORDS:Auditory neuropathy spectrum disorder, evoked auditory brainstem response, otoacoustic emissions, GJB2, hearing loss, cochlear implants Guilherme Machado de Carvalho, Priscila Ramos, Castilho Arthur, Alexandre Guimarães, Edi SartoratoDepartment of Ear Nose Throat Head and Neck Surgery, UNICAMP (Campinas University, São Paulo, Brazil), Campinas, Brazil (GMDC, CA, AG) Centro de Biologia Molecular e Engenharia Genética, UNICAMP (Campinas University, São Paulo, Brazil), Campinas, Brazil (PR, ES) 8 Cochlear implants (CI) currently has ample evidence for its efficacy in the treatment of various forms of deafness, from unilateral to bilateral cases with severe to profound hearing loss. It also has been shown as an alternative for hearing rehabilitation in many conditions, such as in deafness with residual hearing at low frequencies and AN. In AN cases, CI is an option when the patient has not had a good response to conventional hearing aids and an adequate development of speech [1,3,5,10,11] .Although it has been demonstrated that the earlier use of CI in children with AN with severe or profound hearing loss leads to an important benefit in most cases, the situation is still challenging in cases when the hearing loss is moderate [1,3] . Thus, CI has shown conflicting results worldwide, probably because AN must be heterogeneous and have different etiologies [5] .A recent systematic review evaluated the improvement of speech development in children with AN treated with CIs and showed favorable results after CI regarding hearing and speech parameters but concluded that the evidence for CI is still weak and further studies are needed to clarify where and when CI could be applied as a good option for pediatric patients with AN [12] .Regarding the etiologic heterogeneity of AN, it is not surprising that ...

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Prevalence of auditory neuropathy spectrum disorder in an auditory health care service

Abstract: ANSD was seen in 1.2% of the individuals with SNHL included in this study.

Cited by 33 publications

References 27 publications

Auditory neuropathy — neural and synaptic mechanisms

Auditory neuropathy — neural and synaptic mechanisms

Neuropatia Auditiva: Avaliação Clínica e Abordagem Diagnóstica

Performance of Cochlear Implants in Pediatric Patients with Auditory Neuropathy Spectrum Disorder

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