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Speech perception and spoken language of children with auditory neuropathy spectrum disorder: a systematic literature review

ABSTRACT

Purpose:

to analyze the test results of speech perception and spoken language in children with hearing loss and auditory neuropathy spectrum disorder, users of hearing aids or cochlear implants.

Methods:

a systematic review of the literature based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Consultation was performed in databases, considering studies from 1996 to 2021, selecting the studies that presented the results of speech perception or spoken language in children with bilateral auditory neuropathy spectrum disorder, with no structural alterations of the ear and/or vestibulocochlear nerve, or other associated impairments. Descriptive analysis was performed.

Literature Review:

among 1,422 studies found, 15 were included. Variability in the sample size, types of studies, evaluation procedures and methodological questions were observed. The cochlear implants and hearing aids contributed to the development of speech perception and spoken language in children with auditory neuropathy spectrum disorder, since skills such as auditory comprehension and speech intelligibility, were achieved.

Conclusion:

15 studies suggest that cochlear implants and hearing aids may be effective for speech perception and spoken language development in children with auditory neuropathy spectrum disorder, with no other associated impairments. The need for further research with a high methodological rigor is highlighted.

Keywords:
Cochlear Implants; Hearing Aids; Hearing Loss; Speech Perception; Child Language

RESUMO

Objetivo:

analisar os resultados de testes percepção da fala e linguagem falada de crianças com espectro da neuropatia auditiva, usuárias de aparelho de amplificação sonora individual ou implante coclear.

Métodos:

revisão sistemática da literatura, baseada no guideline Preferred Reporting Items for Systematic Reviews and Meta-Analyses. Levantaram-se, em bases de dados, estudos entre 1996 e 2021, que apresentavam os resultados de percepção da fala ou linguagem falada de crianças com espectro da neuropatia auditiva bilateral, sem alterações estruturais da orelha/nervo vestíbulo-coclear, ou comprometimentos associados. Os dados foram analisados descritivamente.

Revisão da Literatura:

dentre 1422 estudos encontrados, 15 foram incluídos. Observou-se variabilidade no tamanho das amostras, tipos de estudos, procedimentos de avaliação e questões metodológicas. O implante coclear e o aparelho de amplificação sonora individual contribuíram para o desenvolvimento da percepção da fala e linguagem falada de crianças com espectro da neuropatia auditiva, pois foram alcançadas habilidades, como a compreensão auditiva e inteligibilidade da fala.

Conclusão:

15 estudos sugeriram que o implante coclear e o aparelho de amplificação sonora individual podem ser efetivos para o desenvolvimento da percepção da fala e linguagem falada de crianças com espectro da neuropatia auditiva sem outros comprometimentos associados. Ressalta-se a necessidade de pesquisas com alto rigor metodológico.

Descritores:
Implantes Cocleares; Auxiliares de Audição; Perda Auditiva; Percepção da Fala; Linguagem Infantil

INTRODUCTION

The term “Auditory Neuropathy” was first published by Starr et al. (1996)11. Star A, Picton TW, Sininger Y, Hood LJ, Berlin CI. Auditory neuropathy. Brain. 1996;119(Pt 3):741-53. https://doi.org/10.1093/brain/119.3.741. PMID: 8673487.
https://doi.org/10.1093/brain/119.3.741...
to describe a possible impairment of the inner hair cells, the auditory nerve and/or the failure between the synapses of the inner hair cells and the auditory nerve11. Star A, Picton TW, Sininger Y, Hood LJ, Berlin CI. Auditory neuropathy. Brain. 1996;119(Pt 3):741-53. https://doi.org/10.1093/brain/119.3.741. PMID: 8673487.
https://doi.org/10.1093/brain/119.3.741...
,22. Doyle KJ, Sininger Y, Star A. Auditory neuropathy in childhood. Laryngoscope. 1998;108(9):1374-7. https://onlinelibrary.wiley.com/doi/abs/10.1097/00005537-199809000-00022. PMID: 9738760.
https://onlinelibrary.wiley.com/doi/abs/...
. Over the years, studies in the area have used different nomenclatures, such as “Auditory Neuropathy”, “Auditory Dyssynchrony” and “Auditory Neuropathy Spectrum Disorder” - ANSD11. Star A, Picton TW, Sininger Y, Hood LJ, Berlin CI. Auditory neuropathy. Brain. 1996;119(Pt 3):741-53. https://doi.org/10.1093/brain/119.3.741. PMID: 8673487.
https://doi.org/10.1093/brain/119.3.741...
,33. Berlin CI, Hood L, Rose K. On renaming auditory neuropathy as auditory dys-synchrony. Audiology Today. 2001;13(6):15-7.,44. Berlin CI, Hood LJ, Morlet T, Wilensky D, Li L, Mattingly KR et al. Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony. Int J Audiol. 2010;49(1):30-43. https://doi.org/10.3109/14992020903160892. PMID: 20053155.
https://doi.org/10.3109/1499202090316089...
.

The diagnosis of auditory neuropathy is verified by the presence of Evoked Otoacoustic Emission, register of abnormal or absent Brainstem Auditory Evoked Potentials (BAEPs) in the neural response and/or the presence of Cochlear Microphonism, observed in the BAEPs or in the Electrocochleography55. Santarelli R, Arslan E. Electrocochleography in auditory neuropathy. Hear Res. 2002;170(1-2):32-47. https://doi.org/10.1016/S0378-5955(02)00450-1. PMID: 12208539.
https://doi.org/10.1016/S0378-5955(02)00...
,66. Raveh E, Buller N, Badrana O, Attias J. Auditory neuropathy: clinical characteristics and therapeutic approach. Am J Otolaryngol. 2007;28(5):302-8. https://doi.org/10.1016/j.amjoto.2006.09.006. PMID: 17826530.
https://doi.org/10.1016/j.amjoto.2006.09...
. In addition, when there is a hearing loss, the degree can vary from mild to profound and the difficulty in understanding speech in silence and, especially, in noise, is disproportionate to the hearing loss presented44. Berlin CI, Hood LJ, Morlet T, Wilensky D, Li L, Mattingly KR et al. Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony. Int J Audiol. 2010;49(1):30-43. https://doi.org/10.3109/14992020903160892. PMID: 20053155.
https://doi.org/10.3109/1499202090316089...
,77. Zeng F, Oba S, Garde S, Sininger Y, Starr A. Temporal and speech processing deficits in auditory neuropathy. Neuroreport. 1999;10(16):3429-35. https://doi.org/10.1097/00001756-199911080-00031. PMID: 10599857.
https://doi.org/10.1097/00001756-1999110...
.

The variability found in the Auditory Neuropathy Spectrum (ANS) diagnostic tests demonstrates the heterogeneity of its pathophysiology88. Pham NS. The management of pediatric hearing loss caused by auditory neuropathy spectrum disorder. Curr Opin Otolaryngol Head Neck Surg. 2017;25(5):396-9. https://doi.org/10.1097/moo.0000000000000390. PMID: 2870863.
https://doi.org/10.1097/moo.000000000000...
,99. Yawn RJ, Nassiri AM, Rivas A. Auditory neuropathy: bridging the gap between hearing aids and cochlear implants. Otolaryngol Clin North Am. 2019;52(2):349-55. https://doi.org/10.1016/j.otc.2018.11.016. PMID: 30765091.
https://doi.org/10.1016/j.otc.2018.11.01...
. Individuals with ANS may present alterations in the perception of sound stimuli and in the ability of temporal auditory processing, due to the failure in neural synchrony77. Zeng F, Oba S, Garde S, Sininger Y, Starr A. Temporal and speech processing deficits in auditory neuropathy. Neuroreport. 1999;10(16):3429-35. https://doi.org/10.1097/00001756-199911080-00031. PMID: 10599857.
https://doi.org/10.1097/00001756-1999110...
,1010. Starr A, McPherson D, Patterson J, Don M, Luxford W, Shannon R et al. Absence of both auditory evoked potentials and auditory percepts dependent on timing cues. Brain. 1991;114(Pt 3):1157-80. https://doi.org/10.1093/brain/114.3.1157. PMID: 2065245.
https://doi.org/10.1093/brain/114.3.1157...
.

The use of electronic devices to access speech sounds, such as the Hearing Aid (HA) and the Cochlear Implant (CI), brings the possibility of minimizing the impact of auditory sensory deprivation and enabling the development of spoken language1111. Moret ALM, Bevilacqua MC, Costa OA. Cochlear implant: hearing and language in pre-lingual deaf children. Pró-Fono R. Atual. Cient. 2007;19(3):295-304. https://doi.org/10.1590/S0104-56872007000300008. PMID: 17934605.
https://doi.org/10.1590/S0104-5687200700...
,1212. Fernandes NF, Yamaguti EH, Morettin M, Costa OA. Speech perception in users of hearing aid with auditory neuropathy spectrum disorder. CoDAS. 2016;28(01):22-6. https://doi.org/10.1590/2317-1782/20162014157. PMID: 27074185.
https://doi.org/10.1590/2317-1782/201620...
.

When the results provided by the HA regarding auditory speech perception are limited, the CI may be indicated, since this device partially replaces the function of the cochlea hair cells, compensating for the alteration of neural synchrony1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
,1414. Rajput K, Saeed M, Ahmed J, Chung M, Munro C, Patel S et al. Findings from aetiological investigation of Auditory Neuropathy Spectrum Disorder in children referred to cochlear implant programs. Int J Pediatr Otorhinolaryngol. 2019;116:79-83. https://doi.org/10.1016/j.ijporl.2018.10.010. PMID: 30554714.
https://doi.org/10.1016/j.ijporl.2018.10...
. However, the literature reinforces the importance of considering the individuality of each case, and caution is necessary in relation to the conduct of indicating the cochlear implant66. Raveh E, Buller N, Badrana O, Attias J. Auditory neuropathy: clinical characteristics and therapeutic approach. Am J Otolaryngol. 2007;28(5):302-8. https://doi.org/10.1016/j.amjoto.2006.09.006. PMID: 17826530.
https://doi.org/10.1016/j.amjoto.2006.09...
,99. Yawn RJ, Nassiri AM, Rivas A. Auditory neuropathy: bridging the gap between hearing aids and cochlear implants. Otolaryngol Clin North Am. 2019;52(2):349-55. https://doi.org/10.1016/j.otc.2018.11.016. PMID: 30765091.
https://doi.org/10.1016/j.otc.2018.11.01...
,1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
.

A previous study systematically reviewed the existing evidence regarding the results of the auditory abilities of children using CI with auditory neuropathy spectrum disorder, verifying advances in the detection of speech, in the discrimination and recognition of words and sentences after surgery, and no differences were observed in these abilities when compared to children with sensorineural hearing loss who used cochlear implants1515. Fernandes NF, Morettin M, Yamaguti EH, Costa OA, Bevilacqua MC. Performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implant: a systematic review. Braz J Otorhinolaryngol. 2015;81(1):85-96. https://doi.org/10.1016/j.bjorl.2014.10.003. PMID: 25458263.
https://doi.org/10.1016/j.bjorl.2014.10....
.

However, further research is still needed in order to understand in more detail the effectiveness of the intervention with HA and/or CI in the performance of speech perception and spoken language of children with ANSD.

Studies that systematize the evidence produced over the years in this area may support the establishment of guidelines and scientific parameters that assist in the conduct of the auditory rehabilitation process, mainly related to the technological device to access to speech.

In view of the above, this study aimed to verify the results of speech perception and spoken language of children with hearing loss and auditory neuropathy spectrum disorder, users of hearing aids or cochlear implant.

METHODS

Research Strategy

Systematic review of the literature registered in the International Prospective Register of Systematic Reviews (PROSPERO) system, under number CRD420212735621616. International Prospective Register of Systematic Reviews - PROSPERO. Centre for Reviews and Dissemination. Available at: https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42021273562.
https://www.crd.york.ac.uk/prospero/disp...
, following the recommendations of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines1717. Page MJ, McKenzie JE, Bossuyt PM, Boutron I, Hoffmann TC, Mulrow CD et al. The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. BMJ. 2021;372(1):71. https://doi.org/10.1136/bmj.n71.
https://doi.org/10.1136/bmj.n71...
.

The review was conducted based on the guiding question: Were the cochlear implant and the hearing aid effective for the development of the perception of speech and spoken language in children with auditory neuropathy spectrum disorder?

Initially, a search was made in the Cochrane Library, Regional Portal of the Virtual Health Library (VHL) and PROSPERO databases, without verifying review studies with the proposed theme.

Thus, a bibliographic search was carried out in national and international journals indexed in the electronic databases: PubMed/MEDLINE, Web of Science, SCOPUS, Embase, Scientific Electronic Library Online (SciELO), VHL and Brazilian Digital Library of Theses and Dissertations (BDTD).

Health Sciences Descriptors (DeCS) in Portuguese and Spanish and their English counterparts in the Medical Subject Headings (MeSH) were used, as well as keywords related to the theme of the study. The descriptors and words were combined using the Boolean operators "OR" and "AND", as shown in Chart 1.

Chart 1
Search strategy

Study Selection Criteria

For the selection of primary studies, the following criteria were considered:

  • Types of studies: those in Portuguese, English or Spanish, published in the period from 1996 to 2021. The delimitation of studies starting from 1996 is justified by the fact that it was in this year the term "auditory neuropathy" and its description was first published. When publication was available, dissertations and theses were replaced by published work.

  • Population: children with bilateral auditory neuropathy spectrum disorder, inserted in the context of spoken language, with no structural alterations of the ear or vestibulocochlear nerve (CN VIII) or other associated impairments, such as syndromes.

  • Intervention: use of the bilateral hearing aid or unilateral or bilateral cochlear implant or use of the unilateral cochlear implant with contralateral (bimodal) hearing aid.

  • Clinical outcomes: results expressed as a percentage of correct answers or classifications of scales that demonstrate the performance in the perception of speech and/or spoken language.

Data Analysis

The studies were extracted from the databases and organized using the EndNote software (online version), and repeated work was manually excluded.

The evaluation took place in two stages, by the Rayyan selection platform.

In the first stage, a blind evaluation by two reviewers selected the studies with potential relationship with the theme addressed by reading the titles and abstracts. In the second stage, two reviewers through blind evaluation analyzed the full text of the studies in which inclusion or exclusion was not possible only by reading the title and abstract, so as to define which studies fulfilled the selection criteria.

When there were disagreements among the judges regarding the inclusion or exclusion of the studies in the previous stages, another judge was called as a tie-breaking criterion. The third judge was able to analyze the title, abstract and full text (when it was not possible to include or exclude the study only by reading the title and abstract).

A protocol was used to analyze the research resulting from the previous stages, containing the following information: authors, year of publication, objectives, population, procedures for assessing speech perception and/or language, main results, level of evidence of the studies and risk of bias. One author organized these data, which were analyzed by another author and, if there were disagreements, a discussion was held to reach consensus.

To determine the level of evidence, the classification adapted by Cox (2004) was used1818. Cox RM. Waiting for evidence-based practice for your hearing aid fittings? It's here! Hear. J. 2004;57(8):10-7. http://dx.doi.org/10.1097/01.HJ.0000292854.24590.8d.
http://dx.doi.org/10.1097/01.HJ.00002928...
.

To assess the risk of bias, the critical appraisal tools appropriate for this type of study from the Joanna Briggs Institute were used1919. Critical appraisal tools for use in JBI systematic reviews. Joanna Briggs Institute. Disponível em: https://jbi.global/critical-appraisal-tools. Accessed 2021 dec.
https://jbi.global/critical-appraisal-to...
. The risk of bias was characterized according to the frequency of "yes" answers, which meant adequacy of the study. The risk of "high" bias corresponded to the frequency of "yes" responses less than or equal to 49%, "moderate" between 50% and 69% and "low" greater than 70%. These tools were applied by the same reviewers who evaluated the articles in the previous stages, through consensus and, in case of disagreements, a discussion was held to reach consensus.

It is worth noting that the literature review has been updated in order to contemplate all possible studies that were published by the end of 2021.

LITERATURE REVIEW

Considering the importance of monitoring the results of the intervention, both with the hearing aid and with the CI, this study aimed to analyze the results of tests of speech perception and spoken language of children with auditory neuropathy spectrum disorder. This objective aims to answer the guiding question of the study, which analyzes the effectiveness of the devices in this population.

Figure 1 shows the diagram of the results of this search.

Figure 1
Diagram of systematic review results

The research identified 1,422 studies in the databases used. After the duplicates were removed, 845 papers were screened. Of these, four dissertations/theses had already been published, being disregarded from the analysis and 765 studies did not contemplate the selection criteria.

After reading the title and abstract, 76 studies showed a relationship with the proposed theme and in only one the full text was not recovered, totaling 75 studies included for the second stage. After full analysis, 60 studies were excluded for different reasons, such as language, population and clinical outcomes, and 15 were included in this study. Taking into account the period elapsed between the first description of auditory neuropathy and the current advances in audiological diagnosis and intervention, it can be inferred that the number of studies found is relatively low.

Chart 2 shows the synthesis of the characteristics of the included studies.

Chart 2
Summary of included studies

Regarding the population of the included studies, the chronological age ranged from two years and two months to 12 years and two months, with a sample size between one and 136 participants. Regarding the degree of hearing loss, two studies included participants with mild hearing loss, two studies addressed the moderate degree, seven studies evaluated children with severe hearing loss, 12 encompassed the profound degree, one article mentioned the nomenclature "degree of severe hearing loss" and one study did not mention the degree of hearing loss.

When analyzing the criteria related to the population included in this study, we selected the studies with children with no structural alterations of the ear or vestibulocochlear nerve (cranial nerve VIII) and with no other associated impairments, such as syndromes.

It is known that the diagnosis of the Auditory Neuropathy Spectrum Disorder may be accompanied by other comorbidities, due to risk factors, genetic factors and peri- or postnatal complications, which makes the population extremely heterogeneous88. Pham NS. The management of pediatric hearing loss caused by auditory neuropathy spectrum disorder. Curr Opin Otolaryngol Head Neck Surg. 2017;25(5):396-9. https://doi.org/10.1097/moo.0000000000000390. PMID: 2870863.
https://doi.org/10.1097/moo.000000000000...
,99. Yawn RJ, Nassiri AM, Rivas A. Auditory neuropathy: bridging the gap between hearing aids and cochlear implants. Otolaryngol Clin North Am. 2019;52(2):349-55. https://doi.org/10.1016/j.otc.2018.11.016. PMID: 30765091.
https://doi.org/10.1016/j.otc.2018.11.01...
,2020. Uus K, Young A, Day M. Auditory neuropathy spectrum disorder in the wider health context: experiences of parents whose infants have been identified through newborn hearing screening programme. Int J Audiol. 2012;51(3):186-93. https://doi.org/10.1590/S1516-80342008000100009. PMID: 22107446.
https://doi.org/10.1590/S1516-8034200800...
.

In addition, it is worth noting that the term "spectrum" brought an expansion to the concept of the lesion site, which contemplates other sites of alteration, in addition to the auditory nerve2121. Hayes D, Sininger YS. Guidelines for identification and management of infants and young children with Auditory Neuropathy Spectrum Disorder. Newborn Hearing Systems (NHS) Conference. 2008. Available at: https://www.childrenscolorado.org/globalassets/departments/ear-nose-throat/ansd-monograph.pdf. Accessed oct 2021.
https://www.childrenscolorado.org/global...
. Although the pathophysiological mechanism of auditory neuropathy is not yet fully understood, the literature reports that the lack of synchronization of neural discharges along the auditory pathway causes changes in the accuracy of the coding or transmission of auditory information, which may explain this condition77. Zeng F, Oba S, Garde S, Sininger Y, Starr A. Temporal and speech processing deficits in auditory neuropathy. Neuroreport. 1999;10(16):3429-35. https://doi.org/10.1097/00001756-199911080-00031. PMID: 10599857.
https://doi.org/10.1097/00001756-1999110...
,99. Yawn RJ, Nassiri AM, Rivas A. Auditory neuropathy: bridging the gap between hearing aids and cochlear implants. Otolaryngol Clin North Am. 2019;52(2):349-55. https://doi.org/10.1016/j.otc.2018.11.016. PMID: 30765091.
https://doi.org/10.1016/j.otc.2018.11.01...
,1010. Starr A, McPherson D, Patterson J, Don M, Luxford W, Shannon R et al. Absence of both auditory evoked potentials and auditory percepts dependent on timing cues. Brain. 1991;114(Pt 3):1157-80. https://doi.org/10.1093/brain/114.3.1157. PMID: 2065245.
https://doi.org/10.1093/brain/114.3.1157...
.

Thus, in view of the different manifestations that individuals with ANSD may present, the referred criteria for the population were established aiming at greater homogeneity of the participants of the included studies, which may be related to the number of studies found.

Among the included studies, the number of the sample varied widely, observing case report studies1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
,2222. Fei J, Chen A, Hong M, Shi W, Li J, Yang SJ. Cochlear implantation in a child with auditory neuropathy spectrum disorder. J. Otol. 2011;6(2):29-37. https://doi.org/10.1016/S1672-2930(11)50019-4.
https://doi.org/10.1016/S1672-2930(11)50...

23. Lin C, Chen Y, Wu J. Cochlear implantation in a Mandarin Chinese-speaking child with auditory neuropathy. Eur Arch Otorhinolaryngol. 2005;262(2):139-41. https://doi.org/10.1007/s00405-004-0757-5. PMID: 14999509.
https://doi.org/10.1007/s00405-004-0757-...
-2424. Kim L, Jung S, Park Y, Heo M. Cochlear implantation in pediatric auditory neuropathy. Cochlear Implants Int. 2013;5(1):224-5. http://dx.doi.org/10.1179/cim.2004.5.Supplement-1.224.
http://dx.doi.org/10.1179/cim.2004.5.Sup...
that evaluated the speech perception and language of children who received unilateral cochlear implant and a multicenter study2525. Daneshi A, Mirsalehi M, Hashemi SB, Ajalloueyan M, Rajati M, Ghasemi MM et al. Cochlear implantation in children with auditory neuropathy spectrum disorder: a multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2018;108:12-6. https://doi.org/10.1016/j.ijporl.2018.02.004. PMID: 29605339.
https://doi.org/10.1016/j.ijporl.2018.02...
, which proposed to evaluate the auditory performance and speech production of 136 children who received the cochlear implant between 2003 and 2015, performing a follow-up in the period of two years post-implantation. Due to the longitudinal character, the authors obtained the complete evaluations of 79 participants.

Regarding the degree of hearing loss, which ranged from mild to profound, only two studies involved participants with ANSD and mild hearing loss1212. Fernandes NF, Yamaguti EH, Morettin M, Costa OA. Speech perception in users of hearing aid with auditory neuropathy spectrum disorder. CoDAS. 2016;28(01):22-6. https://doi.org/10.1590/2317-1782/20162014157. PMID: 27074185.
https://doi.org/10.1590/2317-1782/201620...
,2626. Rance G, Barker EJ. Speech perception in children with auditory neuropathy/dyssynchrony managed with either hearing aids or cochlear implants. Otol Neurotol. 2008;29(2):179-82. https://doi.org/10.1097/mao.0b013e31815e92fd. PMID: 18165792.
https://doi.org/10.1097/mao.0b013e31815e...
and two studied children with ANSD and moderate hearing loss2626. Rance G, Barker EJ. Speech perception in children with auditory neuropathy/dyssynchrony managed with either hearing aids or cochlear implants. Otol Neurotol. 2008;29(2):179-82. https://doi.org/10.1097/mao.0b013e31815e92fd. PMID: 18165792.
https://doi.org/10.1097/mao.0b013e31815e...
,2727. Praveena J, Prakash H, Rukmangathan TM. Language outcomes using hearing aids in children with auditory dyssynchrony. Audiol Res. 2014;4(1)36-39. https://doi.org/10.4081/audiores.2014.80. PMID: 26557348.
https://doi.org/10.4081/audiores.2014.80...
. This result emphasizes the importance of neonatal hearing screening programs, which involve both evoked otoacoustic emissions and brainstem auditory evoked potentials, because the combination of both tests will avoid errors, allowing to verify babies with possible findings compatible with auditory neuropathy spectrum disorder44. Berlin CI, Hood LJ, Morlet T, Wilensky D, Li L, Mattingly KR et al. Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony. Int J Audiol. 2010;49(1):30-43. https://doi.org/10.3109/14992020903160892. PMID: 20053155.
https://doi.org/10.3109/1499202090316089...
,2828. Assessment andmManagement of Auditory Neuropathy Spectrum Disorder (ANSD) in young infants. British Society of Audiology (BSA). 2019. Available at: https://www.thebsa.org.uk/resources/assessment-and-management-of-auditory-neuropathy-spectrum-disorder-ansd-in-young-infants/. Accessed 2021 oct.
https://www.thebsa.org.uk/resources/asse...
,2929. Joint Committee on Infant Hearing (JCIH). Year 2019 position statement: principles and guidelines for early hearing detection and intervention programs. J. Early Hear. Detect. Interv. 2019;4(2):1-44. https://doi.org/10.15142/fptk-b748.
https://doi.org/10.15142/fptk-b748...
.

Most of the children evaluated had severe or profound hearing loss and used cochlear implants1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
,2222. Fei J, Chen A, Hong M, Shi W, Li J, Yang SJ. Cochlear implantation in a child with auditory neuropathy spectrum disorder. J. Otol. 2011;6(2):29-37. https://doi.org/10.1016/S1672-2930(11)50019-4.
https://doi.org/10.1016/S1672-2930(11)50...

23. Lin C, Chen Y, Wu J. Cochlear implantation in a Mandarin Chinese-speaking child with auditory neuropathy. Eur Arch Otorhinolaryngol. 2005;262(2):139-41. https://doi.org/10.1007/s00405-004-0757-5. PMID: 14999509.
https://doi.org/10.1007/s00405-004-0757-...

24. Kim L, Jung S, Park Y, Heo M. Cochlear implantation in pediatric auditory neuropathy. Cochlear Implants Int. 2013;5(1):224-5. http://dx.doi.org/10.1179/cim.2004.5.Supplement-1.224.
http://dx.doi.org/10.1179/cim.2004.5.Sup...

25. Daneshi A, Mirsalehi M, Hashemi SB, Ajalloueyan M, Rajati M, Ghasemi MM et al. Cochlear implantation in children with auditory neuropathy spectrum disorder: a multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2018;108:12-6. https://doi.org/10.1016/j.ijporl.2018.02.004. PMID: 29605339.
https://doi.org/10.1016/j.ijporl.2018.02...
-2626. Rance G, Barker EJ. Speech perception in children with auditory neuropathy/dyssynchrony managed with either hearing aids or cochlear implants. Otol Neurotol. 2008;29(2):179-82. https://doi.org/10.1097/mao.0b013e31815e92fd. PMID: 18165792.
https://doi.org/10.1097/mao.0b013e31815e...
,3030. Melo TM, Moret ALM, Bevilacqua MC. Speech production outcomes in children with multichannel cochlear implants. Rev Soc Bras Fonoaudiol. 2008;13(1):45-51. https://doi.org/10.1590/S1516-80342008000100009.
https://doi.org/10.1590/S1516-8034200800...

31. Carvalho ACM, Bevilacqua MC, Sameshima K, Costa Filho OA. Auditory Neuropathy / Auditory Dyssynchrony in children with cochlear implants. Braz J Otorhinolaryngol. 2011;77(4):481-7. https://doi.org/10.1590/S1808-86942011000400012. PMID: 21860975.
https://doi.org/10.1590/S1808-8694201100...

32. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...

33. Attias J, Greenstein T, Peled M, Ulanovski D, Wohlgelernter J, Raveh E. Auditory performance and electrical stimulation measures in cochlear implant recipients with auditory neuropathy compared with severe to profound sensorineural hearing loss. Ear Hear. 2017;38(2):184-93. https://doi.org/10.1097/aud.0000000000000384. PMID: 28225734.
https://doi.org/10.1097/aud.000000000000...

34. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...

35. Fukushima K, Kataoka Y, Maeda Y, Kariya S, Tominaga S, Nagayasu R et al. Cochlear implantation for children with auditory neuropathy among Japanese language users. In: Kaga k, Starr A. Neuropathies of the Auditory and Vestibular Eighth Cranial Nerves. Springer, Tokyo, 2009. p. 71-5. https://doi.org/10.1007/978-4-431-09433-3_8.
https://doi.org/10.1007/978-4-431-09433-...
-3636. Yamaguti EH. Avaliação da percepção da fala com ruído competitivo em crianças portadoras de deficiência auditiva neurossensorial com espectro da neuropatia auditiva usuárias de implante coclear [dissertation]. Bauru (SP): Universidade de São Paulo; 2013.. Studies show that the cochlear implant can bring greater neural synchrony1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
,2424. Kim L, Jung S, Park Y, Heo M. Cochlear implantation in pediatric auditory neuropathy. Cochlear Implants Int. 2013;5(1):224-5. http://dx.doi.org/10.1179/cim.2004.5.Supplement-1.224.
http://dx.doi.org/10.1179/cim.2004.5.Sup...
,3131. Carvalho ACM, Bevilacqua MC, Sameshima K, Costa Filho OA. Auditory Neuropathy / Auditory Dyssynchrony in children with cochlear implants. Braz J Otorhinolaryngol. 2011;77(4):481-7. https://doi.org/10.1590/S1808-86942011000400012. PMID: 21860975.
https://doi.org/10.1590/S1808-8694201100...
, becoming an alternative treatment when the hearing aid is not effective for the development of auditory skills and language2323. Lin C, Chen Y, Wu J. Cochlear implantation in a Mandarin Chinese-speaking child with auditory neuropathy. Eur Arch Otorhinolaryngol. 2005;262(2):139-41. https://doi.org/10.1007/s00405-004-0757-5. PMID: 14999509.
https://doi.org/10.1007/s00405-004-0757-...
.

However, the CI indication process should be thorough, considering that a common clinical conduct is not possible for this group of children, due to the fact that the ANSD presents different degrees of impairment along the auditory pathway, with fluctuation in auditory performance, making the decision-making process about the best rehabilitation option for this population challenging2626. Rance G, Barker EJ. Speech perception in children with auditory neuropathy/dyssynchrony managed with either hearing aids or cochlear implants. Otol Neurotol. 2008;29(2):179-82. https://doi.org/10.1097/mao.0b013e31815e92fd. PMID: 18165792.
https://doi.org/10.1097/mao.0b013e31815e...
,3131. Carvalho ACM, Bevilacqua MC, Sameshima K, Costa Filho OA. Auditory Neuropathy / Auditory Dyssynchrony in children with cochlear implants. Braz J Otorhinolaryngol. 2011;77(4):481-7. https://doi.org/10.1590/S1808-86942011000400012. PMID: 21860975.
https://doi.org/10.1590/S1808-8694201100...
,3333. Attias J, Greenstein T, Peled M, Ulanovski D, Wohlgelernter J, Raveh E. Auditory performance and electrical stimulation measures in cochlear implant recipients with auditory neuropathy compared with severe to profound sensorineural hearing loss. Ear Hear. 2017;38(2):184-93. https://doi.org/10.1097/aud.0000000000000384. PMID: 28225734.
https://doi.org/10.1097/aud.000000000000...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
.

Two studies showed that children who use hearing aids may present benefits in speech perception skills, with regard to auditory recognition and comprehension, when evaluating children with degrees of hearing loss that ranged from mild to severe1212. Fernandes NF, Yamaguti EH, Morettin M, Costa OA. Speech perception in users of hearing aid with auditory neuropathy spectrum disorder. CoDAS. 2016;28(01):22-6. https://doi.org/10.1590/2317-1782/20162014157. PMID: 27074185.
https://doi.org/10.1590/2317-1782/201620...
,2626. Rance G, Barker EJ. Speech perception in children with auditory neuropathy/dyssynchrony managed with either hearing aids or cochlear implants. Otol Neurotol. 2008;29(2):179-82. https://doi.org/10.1097/mao.0b013e31815e92fd. PMID: 18165792.
https://doi.org/10.1097/mao.0b013e31815e...
. However, another study, which evaluated the language development of children using hearing aids with moderate to severe hearing loss, found superior performance for participants with lower degree of hearing loss, stating that amplification can help in hearing, but is not enough to modify the neural coding pattern that is altered in the different degrees of hearing loss, especially in the severe degree2727. Praveena J, Prakash H, Rukmangathan TM. Language outcomes using hearing aids in children with auditory dyssynchrony. Audiol Res. 2014;4(1)36-39. https://doi.org/10.4081/audiores.2014.80. PMID: 26557348.
https://doi.org/10.4081/audiores.2014.80...
.

To this extent, it is emphasized that a careful preoperative evaluation is necessary, which takes into account different aspects, such as the individual benefits of hearing aids, the parents' perception of the child's development, the parents' expectations regarding the results of the CI and the active participation of the family in the rehabilitation process1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
,2222. Fei J, Chen A, Hong M, Shi W, Li J, Yang SJ. Cochlear implantation in a child with auditory neuropathy spectrum disorder. J. Otol. 2011;6(2):29-37. https://doi.org/10.1016/S1672-2930(11)50019-4.
https://doi.org/10.1016/S1672-2930(11)50...
,3232. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...
.

It is worth mentioning that, after the conduct is established, the success of the devices (HA and CI) for the development of auditory and language skills involves factors such as the motivation to use the device, family support, speech therapy, among others3131. Carvalho ACM, Bevilacqua MC, Sameshima K, Costa Filho OA. Auditory Neuropathy / Auditory Dyssynchrony in children with cochlear implants. Braz J Otorhinolaryngol. 2011;77(4):481-7. https://doi.org/10.1590/S1808-86942011000400012. PMID: 21860975.
https://doi.org/10.1590/S1808-8694201100...
,3737. Moog JS, Geers AE. Speech and language acquisition in young children after cochlear implantation. Otolaryngol Clin North Am. 1999;32(6):1127-41. https://doi.org/10.1016/s0030-6665(05)70199-7. PMID: 10523457.
https://doi.org/10.1016/s0030-6665(05)70...
.

Regarding the clinical outcomes of speech perception, it was possible to observe that results were obtained through procedures that involved inventories answered by the guardians, scales scored by the evaluator, tests with the child him/herself or the procedures agreed upon.

Among the inventories answered by the parents/guardians, the Meaningful Auditory Integration Scale (MAIS) was applied in two studies2222. Fei J, Chen A, Hong M, Shi W, Li J, Yang SJ. Cochlear implantation in a child with auditory neuropathy spectrum disorder. J. Otol. 2011;6(2):29-37. https://doi.org/10.1016/S1672-2930(11)50019-4.
https://doi.org/10.1016/S1672-2930(11)50...
,3232. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...
and the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) in three studies1212. Fernandes NF, Yamaguti EH, Morettin M, Costa OA. Speech perception in users of hearing aid with auditory neuropathy spectrum disorder. CoDAS. 2016;28(01):22-6. https://doi.org/10.1590/2317-1782/20162014157. PMID: 27074185.
https://doi.org/10.1590/2317-1782/201620...
,3232. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...
,3333. Attias J, Greenstein T, Peled M, Ulanovski D, Wohlgelernter J, Raveh E. Auditory performance and electrical stimulation measures in cochlear implant recipients with auditory neuropathy compared with severe to profound sensorineural hearing loss. Ear Hear. 2017;38(2):184-93. https://doi.org/10.1097/aud.0000000000000384. PMID: 28225734.
https://doi.org/10.1097/aud.000000000000...
. The use of these procedures is relevant, considering that the report of the parents/guardians is an important tool to evaluate the effectiveness of the devices in significant contexts of daily life, especially for young children2222. Fei J, Chen A, Hong M, Shi W, Li J, Yang SJ. Cochlear implantation in a child with auditory neuropathy spectrum disorder. J. Otol. 2011;6(2):29-37. https://doi.org/10.1016/S1672-2930(11)50019-4.
https://doi.org/10.1016/S1672-2930(11)50...
.

The scores on these scales ranged from zero to 44% before CI surgery 2222. Fei J, Chen A, Hong M, Shi W, Li J, Yang SJ. Cochlear implantation in a child with auditory neuropathy spectrum disorder. J. Otol. 2011;6(2):29-37. https://doi.org/10.1016/S1672-2930(11)50019-4.
https://doi.org/10.1016/S1672-2930(11)50...
,3333. Attias J, Greenstein T, Peled M, Ulanovski D, Wohlgelernter J, Raveh E. Auditory performance and electrical stimulation measures in cochlear implant recipients with auditory neuropathy compared with severe to profound sensorineural hearing loss. Ear Hear. 2017;38(2):184-93. https://doi.org/10.1097/aud.0000000000000384. PMID: 28225734.
https://doi.org/10.1097/aud.000000000000...
, while after surgery scores of up to 100% were achieved1212. Fernandes NF, Yamaguti EH, Morettin M, Costa OA. Speech perception in users of hearing aid with auditory neuropathy spectrum disorder. CoDAS. 2016;28(01):22-6. https://doi.org/10.1590/2317-1782/20162014157. PMID: 27074185.
https://doi.org/10.1590/2317-1782/201620...
,2222. Fei J, Chen A, Hong M, Shi W, Li J, Yang SJ. Cochlear implantation in a child with auditory neuropathy spectrum disorder. J. Otol. 2011;6(2):29-37. https://doi.org/10.1016/S1672-2930(11)50019-4.
https://doi.org/10.1016/S1672-2930(11)50...
,3232. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...
,3333. Attias J, Greenstein T, Peled M, Ulanovski D, Wohlgelernter J, Raveh E. Auditory performance and electrical stimulation measures in cochlear implant recipients with auditory neuropathy compared with severe to profound sensorineural hearing loss. Ear Hear. 2017;38(2):184-93. https://doi.org/10.1097/aud.0000000000000384. PMID: 28225734.
https://doi.org/10.1097/aud.000000000000...
. In one study2222. Fei J, Chen A, Hong M, Shi W, Li J, Yang SJ. Cochlear implantation in a child with auditory neuropathy spectrum disorder. J. Otol. 2011;6(2):29-37. https://doi.org/10.1016/S1672-2930(11)50019-4.
https://doi.org/10.1016/S1672-2930(11)50...
, the results were obtained after three and seven months of use of the implant, achieving development of hearing skills, with a maximum score of 81% at the end of this period. Another study found that children implanted before 24 months of life had higher scores for the MAIS and IT-MAIS scales than children implanted after this period3232. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...
.

The early age for cochlear implant surgery is reported in the literature as a factor capable of bringing benefits to the speech perception of children with ANSD 2525. Daneshi A, Mirsalehi M, Hashemi SB, Ajalloueyan M, Rajati M, Ghasemi MM et al. Cochlear implantation in children with auditory neuropathy spectrum disorder: a multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2018;108:12-6. https://doi.org/10.1016/j.ijporl.2018.02.004. PMID: 29605339.
https://doi.org/10.1016/j.ijporl.2018.02...
,3232. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
. Other authors, who evaluated the auditory skills of this group, through the Categories of Auditory Performance (CAP) scale, found superior results for participants implanted early2525. Daneshi A, Mirsalehi M, Hashemi SB, Ajalloueyan M, Rajati M, Ghasemi MM et al. Cochlear implantation in children with auditory neuropathy spectrum disorder: a multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2018;108:12-6. https://doi.org/10.1016/j.ijporl.2018.02.004. PMID: 29605339.
https://doi.org/10.1016/j.ijporl.2018.02...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
.

Regarding the tests applied with the child him/herself, the results showed that different skills were evaluated through a variety of evaluation procedures, as shown in Chart 2.

One of the main aspects considered was to understand whether there were differences in performance in speech perception between children with ANSD who used hearing aids or CI and children with sensorineural hearing loss1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
,2626. Rance G, Barker EJ. Speech perception in children with auditory neuropathy/dyssynchrony managed with either hearing aids or cochlear implants. Otol Neurotol. 2008;29(2):179-82. https://doi.org/10.1097/mao.0b013e31815e92fd. PMID: 18165792.
https://doi.org/10.1097/mao.0b013e31815e...
,3333. Attias J, Greenstein T, Peled M, Ulanovski D, Wohlgelernter J, Raveh E. Auditory performance and electrical stimulation measures in cochlear implant recipients with auditory neuropathy compared with severe to profound sensorineural hearing loss. Ear Hear. 2017;38(2):184-93. https://doi.org/10.1097/aud.0000000000000384. PMID: 28225734.
https://doi.org/10.1097/aud.000000000000...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
,3636. Yamaguti EH. Avaliação da percepção da fala com ruído competitivo em crianças portadoras de deficiência auditiva neurossensorial com espectro da neuropatia auditiva usuárias de implante coclear [dissertation]. Bauru (SP): Universidade de São Paulo; 2013.. It was noted that four studies found similar results between the groups with regard to the abilities of detection of speech sounds1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
, attention to environmental and speech sounds of everyday life3333. Attias J, Greenstein T, Peled M, Ulanovski D, Wohlgelernter J, Raveh E. Auditory performance and electrical stimulation measures in cochlear implant recipients with auditory neuropathy compared with severe to profound sensorineural hearing loss. Ear Hear. 2017;38(2):184-93. https://doi.org/10.1097/aud.0000000000000384. PMID: 28225734.
https://doi.org/10.1097/aud.000000000000...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
, auditory discrimination1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
,3333. Attias J, Greenstein T, Peled M, Ulanovski D, Wohlgelernter J, Raveh E. Auditory performance and electrical stimulation measures in cochlear implant recipients with auditory neuropathy compared with severe to profound sensorineural hearing loss. Ear Hear. 2017;38(2):184-93. https://doi.org/10.1097/aud.0000000000000384. PMID: 28225734.
https://doi.org/10.1097/aud.000000000000...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
, recognition in closed and open set1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
,3333. Attias J, Greenstein T, Peled M, Ulanovski D, Wohlgelernter J, Raveh E. Auditory performance and electrical stimulation measures in cochlear implant recipients with auditory neuropathy compared with severe to profound sensorineural hearing loss. Ear Hear. 2017;38(2):184-93. https://doi.org/10.1097/aud.0000000000000384. PMID: 28225734.
https://doi.org/10.1097/aud.000000000000...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
,3636. Yamaguti EH. Avaliação da percepção da fala com ruído competitivo em crianças portadoras de deficiência auditiva neurossensorial com espectro da neuropatia auditiva usuárias de implante coclear [dissertation]. Bauru (SP): Universidade de São Paulo; 2013., sequential memory1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
, background figure1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
and auditory comprehension1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
.

However, one study found different results, in which children with ANSD who used CI or HA had lower performance in the ability to recognize phonemes, when compared to children with sensorineural hearing loss2626. Rance G, Barker EJ. Speech perception in children with auditory neuropathy/dyssynchrony managed with either hearing aids or cochlear implants. Otol Neurotol. 2008;29(2):179-82. https://doi.org/10.1097/mao.0b013e31815e92fd. PMID: 18165792.
https://doi.org/10.1097/mao.0b013e31815e...
.

These different findings in the literature show that knowing the site of the alteration along the auditory pathway is fundamental in assisting in the choice of the technological resource for auditory rehabilitation and in understanding the prognosis of the individual with ANSD. When there is a sensory impairment, a favorable prognosis is expected with the use of hearing aids, but if there is a dysfunction that also involves the neural component, the auditory responses may be unpredictable, regardless of the device used (HA or CI) and, specifically with the CI, the results may depend on the ability of the auditory nervous system to deal with electrical stimulation1212. Fernandes NF, Yamaguti EH, Morettin M, Costa OA. Speech perception in users of hearing aid with auditory neuropathy spectrum disorder. CoDAS. 2016;28(01):22-6. https://doi.org/10.1590/2317-1782/20162014157. PMID: 27074185.
https://doi.org/10.1590/2317-1782/201620...
.

It is worth noting that among the tests applied with the child, only two studies evaluated the perception of speech in the presence of competitive noise. In both studies, children with CI and ANSD were compared with children with CI and sensorineural hearing loss, in relation to the recognition of sentences in noise3333. Attias J, Greenstein T, Peled M, Ulanovski D, Wohlgelernter J, Raveh E. Auditory performance and electrical stimulation measures in cochlear implant recipients with auditory neuropathy compared with severe to profound sensorineural hearing loss. Ear Hear. 2017;38(2):184-93. https://doi.org/10.1097/aud.0000000000000384. PMID: 28225734.
https://doi.org/10.1097/aud.000000000000...
,3636. Yamaguti EH. Avaliação da percepção da fala com ruído competitivo em crianças portadoras de deficiência auditiva neurossensorial com espectro da neuropatia auditiva usuárias de implante coclear [dissertation]. Bauru (SP): Universidade de São Paulo; 2013. and spondaic words3333. Attias J, Greenstein T, Peled M, Ulanovski D, Wohlgelernter J, Raveh E. Auditory performance and electrical stimulation measures in cochlear implant recipients with auditory neuropathy compared with severe to profound sensorineural hearing loss. Ear Hear. 2017;38(2):184-93. https://doi.org/10.1097/aud.0000000000000384. PMID: 28225734.
https://doi.org/10.1097/aud.000000000000...
, in which the performance of these groups was similar.

Considering that the monitoring of the development of the auditory skills of children who use devices is an important factor to verify the effectiveness of the intervention with the HA and the CI and that this population can reach the plateau in the tests applied in silence, the evaluation with competitive noise becomes relevant, because it inserts a listening situation closer to everyday life1212. Fernandes NF, Yamaguti EH, Morettin M, Costa OA. Speech perception in users of hearing aid with auditory neuropathy spectrum disorder. CoDAS. 2016;28(01):22-6. https://doi.org/10.1590/2317-1782/20162014157. PMID: 27074185.
https://doi.org/10.1590/2317-1782/201620...
,2525. Daneshi A, Mirsalehi M, Hashemi SB, Ajalloueyan M, Rajati M, Ghasemi MM et al. Cochlear implantation in children with auditory neuropathy spectrum disorder: a multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2018;108:12-6. https://doi.org/10.1016/j.ijporl.2018.02.004. PMID: 29605339.
https://doi.org/10.1016/j.ijporl.2018.02...
,3838. Melo RC, Menezes DC, Pacífico FA, Advíncula KP, Griz SMS. Brazilian Portuguese Hearing in Noise Test (HINT): different interpretation criteria for individuals' responses. CoDAS. 2017;29(1):e20160082. https://doi.org/10.1590/2317-1782/20172016082. PMID: 28300960.
https://doi.org/10.1590/2317-1782/201720...
.

Regarding the clinical outcomes of spoken language, the receptive and expressive aspects were evaluated, using different procedures, as shown in Chart 2.

In relation to the receptive aspect, only one study investigated this ability, through the Language Development Assessment (ADL) procedure. Three children using bilateral hearing aids, with moderate to severe hearing loss, who underwent speech therapy with emphasis on hearing and spoken language, were evaluated, verifying differences in receptive age between the pre- and post-therapy moments, which ranged from six months to three years2727. Praveena J, Prakash H, Rukmangathan TM. Language outcomes using hearing aids in children with auditory dyssynchrony. Audiol Res. 2014;4(1)36-39. https://doi.org/10.4081/audiores.2014.80. PMID: 26557348.
https://doi.org/10.4081/audiores.2014.80...
. Regarding the expressive aspect, the same study obtained results by ADL before and after the intervention, with differences in expressive age ranging from one year to one year and seven months2727. Praveena J, Prakash H, Rukmangathan TM. Language outcomes using hearing aids in children with auditory dyssynchrony. Audiol Res. 2014;4(1)36-39. https://doi.org/10.4081/audiores.2014.80. PMID: 26557348.
https://doi.org/10.4081/audiores.2014.80...
.

Although the number of participants was low, this study demonstrates the importance of speech therapy, with emphasis on the development of hearing and spoken language, as children with hearing loss, using devices, need an intervention that helps in the use of hearing information obtained by hearing aids or CI for the establishment of effective oral communication2424. Kim L, Jung S, Park Y, Heo M. Cochlear implantation in pediatric auditory neuropathy. Cochlear Implants Int. 2013;5(1):224-5. http://dx.doi.org/10.1179/cim.2004.5.Supplement-1.224.
http://dx.doi.org/10.1179/cim.2004.5.Sup...
,2727. Praveena J, Prakash H, Rukmangathan TM. Language outcomes using hearing aids in children with auditory dyssynchrony. Audiol Res. 2014;4(1)36-39. https://doi.org/10.4081/audiores.2014.80. PMID: 26557348.
https://doi.org/10.4081/audiores.2014.80...
.

It is worth mentioning that the main expressive aspect evaluated in the studies was speech intelligibility, in which four studies2222. Fei J, Chen A, Hong M, Shi W, Li J, Yang SJ. Cochlear implantation in a child with auditory neuropathy spectrum disorder. J. Otol. 2011;6(2):29-37. https://doi.org/10.1016/S1672-2930(11)50019-4.
https://doi.org/10.1016/S1672-2930(11)50...
,2525. Daneshi A, Mirsalehi M, Hashemi SB, Ajalloueyan M, Rajati M, Ghasemi MM et al. Cochlear implantation in children with auditory neuropathy spectrum disorder: a multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2018;108:12-6. https://doi.org/10.1016/j.ijporl.2018.02.004. PMID: 29605339.
https://doi.org/10.1016/j.ijporl.2018.02...
,3232. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
used the Speech Intelligibility Rating (SIR) scale. One study3535. Fukushima K, Kataoka Y, Maeda Y, Kariya S, Tominaga S, Nagayasu R et al. Cochlear implantation for children with auditory neuropathy among Japanese language users. In: Kaga k, Starr A. Neuropathies of the Auditory and Vestibular Eighth Cranial Nerves. Springer, Tokyo, 2009. p. 71-5. https://doi.org/10.1007/978-4-431-09433-3_8.
https://doi.org/10.1007/978-4-431-09433-...
applied a speech intelligibility test based on percentage and one study3030. Melo TM, Moret ALM, Bevilacqua MC. Speech production outcomes in children with multichannel cochlear implants. Rev Soc Bras Fonoaudiol. 2008;13(1):45-51. https://doi.org/10.1590/S1516-80342008000100009.
https://doi.org/10.1590/S1516-8034200800...
used the ABFW phonology test.

The literature reports that intelligibility is an aspect that can bring representative results about the child's competence for spoken language, quickly, reflecting their performance in a more natural communication situation3939. Chin BS, Finnegan KR, Chung BA. Relationships among types of speech intelligibility in pediatric users of cochlear implants. J Commun Disord. 2001;34(3):187-205. https://doi.org/10.1016/S0021-9924(00)00048-4. PMID: 11409603.
https://doi.org/10.1016/S0021-9924(00)00...
,4040. Chin BS, Tsai PP, Gao S. Connected speech intelligibility of children with cochlear implants and children with normal hearing. Am J Speech Lang Pathol. 2003;12(4):440-51. https://doi.org/10.1044/1058-0360(2003/090. PMID: 14658996.
https://doi.org/10.1044/1058-0360(2003/0...
.

All studies that evaluated speech intelligibility were performed with implanted children and verified that the intervention with CI led to an increase in the scores obtained with the scales used2222. Fei J, Chen A, Hong M, Shi W, Li J, Yang SJ. Cochlear implantation in a child with auditory neuropathy spectrum disorder. J. Otol. 2011;6(2):29-37. https://doi.org/10.1016/S1672-2930(11)50019-4.
https://doi.org/10.1016/S1672-2930(11)50...
,2525. Daneshi A, Mirsalehi M, Hashemi SB, Ajalloueyan M, Rajati M, Ghasemi MM et al. Cochlear implantation in children with auditory neuropathy spectrum disorder: a multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2018;108:12-6. https://doi.org/10.1016/j.ijporl.2018.02.004. PMID: 29605339.
https://doi.org/10.1016/j.ijporl.2018.02...
,3030. Melo TM, Moret ALM, Bevilacqua MC. Speech production outcomes in children with multichannel cochlear implants. Rev Soc Bras Fonoaudiol. 2008;13(1):45-51. https://doi.org/10.1590/S1516-80342008000100009.
https://doi.org/10.1590/S1516-8034200800...
,3232. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
,3535. Fukushima K, Kataoka Y, Maeda Y, Kariya S, Tominaga S, Nagayasu R et al. Cochlear implantation for children with auditory neuropathy among Japanese language users. In: Kaga k, Starr A. Neuropathies of the Auditory and Vestibular Eighth Cranial Nerves. Springer, Tokyo, 2009. p. 71-5. https://doi.org/10.1007/978-4-431-09433-3_8.
https://doi.org/10.1007/978-4-431-09433-...
. Considering that the development of spoken language in children who use hearing aids may also be influenced by the quality of the acoustic signal provided by the device, impacting on the expressive aspect, the evaluation of speech intelligibility in this group is also relevant2727. Praveena J, Prakash H, Rukmangathan TM. Language outcomes using hearing aids in children with auditory dyssynchrony. Audiol Res. 2014;4(1)36-39. https://doi.org/10.4081/audiores.2014.80. PMID: 26557348.
https://doi.org/10.4081/audiores.2014.80...
.

Two studies compared speech intelligibility between children with ANSD who used CI and implanted children with sensorineural hearing loss, finding no differences in performance between the groups3030. Melo TM, Moret ALM, Bevilacqua MC. Speech production outcomes in children with multichannel cochlear implants. Rev Soc Bras Fonoaudiol. 2008;13(1):45-51. https://doi.org/10.1590/S1516-80342008000100009.
https://doi.org/10.1590/S1516-8034200800...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
. These findings demonstrate that the access to speech sounds provided by the adequate adaptation of the device can bring benefits to the spoken language of children with hearing loss.

It was also pointed out in the studies that children with higher scores for speech intelligibility had shorter sensory deprivation time and had undergone CI surgery at an early age2525. Daneshi A, Mirsalehi M, Hashemi SB, Ajalloueyan M, Rajati M, Ghasemi MM et al. Cochlear implantation in children with auditory neuropathy spectrum disorder: a multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2018;108:12-6. https://doi.org/10.1016/j.ijporl.2018.02.004. PMID: 29605339.
https://doi.org/10.1016/j.ijporl.2018.02...
,3232. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
. In addition, there was an evolution in the performance of speech intelligibility, as a function of the duration of the post-adaptation follow-up period with the CI2525. Daneshi A, Mirsalehi M, Hashemi SB, Ajalloueyan M, Rajati M, Ghasemi MM et al. Cochlear implantation in children with auditory neuropathy spectrum disorder: a multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2018;108:12-6. https://doi.org/10.1016/j.ijporl.2018.02.004. PMID: 29605339.
https://doi.org/10.1016/j.ijporl.2018.02...
. These results show the importance of early intervention and systematic monitoring of the development of this group.

Regarding the level of evidence, eight studies were classified in "level 4", which consists of descriptive studies, five case-control studies2626. Rance G, Barker EJ. Speech perception in children with auditory neuropathy/dyssynchrony managed with either hearing aids or cochlear implants. Otol Neurotol. 2008;29(2):179-82. https://doi.org/10.1097/mao.0b013e31815e92fd. PMID: 18165792.
https://doi.org/10.1097/mao.0b013e31815e...
,3030. Melo TM, Moret ALM, Bevilacqua MC. Speech production outcomes in children with multichannel cochlear implants. Rev Soc Bras Fonoaudiol. 2008;13(1):45-51. https://doi.org/10.1590/S1516-80342008000100009.
https://doi.org/10.1590/S1516-8034200800...
,3333. Attias J, Greenstein T, Peled M, Ulanovski D, Wohlgelernter J, Raveh E. Auditory performance and electrical stimulation measures in cochlear implant recipients with auditory neuropathy compared with severe to profound sensorineural hearing loss. Ear Hear. 2017;38(2):184-93. https://doi.org/10.1097/aud.0000000000000384. PMID: 28225734.
https://doi.org/10.1097/aud.000000000000...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
,3636. Yamaguti EH. Avaliação da percepção da fala com ruído competitivo em crianças portadoras de deficiência auditiva neurossensorial com espectro da neuropatia auditiva usuárias de implante coclear [dissertation]. Bauru (SP): Universidade de São Paulo; 2013., two cohort studies2525. Daneshi A, Mirsalehi M, Hashemi SB, Ajalloueyan M, Rajati M, Ghasemi MM et al. Cochlear implantation in children with auditory neuropathy spectrum disorder: a multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2018;108:12-6. https://doi.org/10.1016/j.ijporl.2018.02.004. PMID: 29605339.
https://doi.org/10.1016/j.ijporl.2018.02...
,3232. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...
and one cross-sectional study3131. Carvalho ACM, Bevilacqua MC, Sameshima K, Costa Filho OA. Auditory Neuropathy / Auditory Dyssynchrony in children with cochlear implants. Braz J Otorhinolaryngol. 2011;77(4):481-7. https://doi.org/10.1590/S1808-86942011000400012. PMID: 21860975.
https://doi.org/10.1590/S1808-8694201100...
, while seven studies were classified in "level 5", which represents the case studies1212. Fernandes NF, Yamaguti EH, Morettin M, Costa OA. Speech perception in users of hearing aid with auditory neuropathy spectrum disorder. CoDAS. 2016;28(01):22-6. https://doi.org/10.1590/2317-1782/20162014157. PMID: 27074185.
https://doi.org/10.1590/2317-1782/201620...
,1313. Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol. 2000;11(6):309-15. https://doi.org/10.1055/s-0042-1748059. PMID: 10858002.
https://doi.org/10.1055/s-0042-1748059...
,2222. Fei J, Chen A, Hong M, Shi W, Li J, Yang SJ. Cochlear implantation in a child with auditory neuropathy spectrum disorder. J. Otol. 2011;6(2):29-37. https://doi.org/10.1016/S1672-2930(11)50019-4.
https://doi.org/10.1016/S1672-2930(11)50...

23. Lin C, Chen Y, Wu J. Cochlear implantation in a Mandarin Chinese-speaking child with auditory neuropathy. Eur Arch Otorhinolaryngol. 2005;262(2):139-41. https://doi.org/10.1007/s00405-004-0757-5. PMID: 14999509.
https://doi.org/10.1007/s00405-004-0757-...
-2424. Kim L, Jung S, Park Y, Heo M. Cochlear implantation in pediatric auditory neuropathy. Cochlear Implants Int. 2013;5(1):224-5. http://dx.doi.org/10.1179/cim.2004.5.Supplement-1.224.
http://dx.doi.org/10.1179/cim.2004.5.Sup...
,2727. Praveena J, Prakash H, Rukmangathan TM. Language outcomes using hearing aids in children with auditory dyssynchrony. Audiol Res. 2014;4(1)36-39. https://doi.org/10.4081/audiores.2014.80. PMID: 26557348.
https://doi.org/10.4081/audiores.2014.80...
,3535. Fukushima K, Kataoka Y, Maeda Y, Kariya S, Tominaga S, Nagayasu R et al. Cochlear implantation for children with auditory neuropathy among Japanese language users. In: Kaga k, Starr A. Neuropathies of the Auditory and Vestibular Eighth Cranial Nerves. Springer, Tokyo, 2009. p. 71-5. https://doi.org/10.1007/978-4-431-09433-3_8.
https://doi.org/10.1007/978-4-431-09433-...
. It is possible to observe that the 15 included studies are between levels 4 and 5 of the classification proposed by Cox (2004) 1818. Cox RM. Waiting for evidence-based practice for your hearing aid fittings? It's here! Hear. J. 2004;57(8):10-7. http://dx.doi.org/10.1097/01.HJ.0000292854.24590.8d.
http://dx.doi.org/10.1097/01.HJ.00002928...
, which consist of lower levels related to the quality of the study.

Chart 3 shows the risk of bias of the studies, analyzed by the critical appraisal tools of the Joanna Briggs Institute1919. Critical appraisal tools for use in JBI systematic reviews. Joanna Briggs Institute. Disponível em: https://jbi.global/critical-appraisal-tools. Accessed 2021 dec.
https://jbi.global/critical-appraisal-to...
.

Chart 3
Risk of bias of included studies

The risk of bias in three studies was classified as moderate, with blanks in the description of the patients' history2424. Kim L, Jung S, Park Y, Heo M. Cochlear implantation in pediatric auditory neuropathy. Cochlear Implants Int. 2013;5(1):224-5. http://dx.doi.org/10.1179/cim.2004.5.Supplement-1.224.
http://dx.doi.org/10.1179/cim.2004.5.Sup...
, pairings of cases and controls3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
, description of the method used to evaluate clinical outcomes2424. Kim L, Jung S, Park Y, Heo M. Cochlear implantation in pediatric auditory neuropathy. Cochlear Implants Int. 2013;5(1):224-5. http://dx.doi.org/10.1179/cim.2004.5.Supplement-1.224.
http://dx.doi.org/10.1179/cim.2004.5.Sup...
, description of intervention procedures2424. Kim L, Jung S, Park Y, Heo M. Cochlear implantation in pediatric auditory neuropathy. Cochlear Implants Int. 2013;5(1):224-5. http://dx.doi.org/10.1179/cim.2004.5.Supplement-1.224.
http://dx.doi.org/10.1179/cim.2004.5.Sup...
and lack of identification of confounding factors, as well as strategies for resolving these factors3030. Melo TM, Moret ALM, Bevilacqua MC. Speech production outcomes in children with multichannel cochlear implants. Rev Soc Bras Fonoaudiol. 2008;13(1):45-51. https://doi.org/10.1590/S1516-80342008000100009.
https://doi.org/10.1590/S1516-8034200800...
,3434. Alzhrani F, Yousef M, Almuhawas F, Almutawa H. Auditory and speech performance in cochlear implanted ANSD children. Acta Otolaryngol. 2019;139(3):279-83. https://doi.org/10.1080/00016489.2019.1571283. PMID: 30947614.
https://doi.org/10.1080/00016489.2019.15...
.

There were also 12 studies identified with low risk of bias, although five studies presented methodological aspects that were not contemplated, such as: whether there was the inclusion of all participants of interest from the place where the research was developed1212. Fernandes NF, Yamaguti EH, Morettin M, Costa OA. Speech perception in users of hearing aid with auditory neuropathy spectrum disorder. CoDAS. 2016;28(01):22-6. https://doi.org/10.1590/2317-1782/20162014157. PMID: 27074185.
https://doi.org/10.1590/2317-1782/201620...
,3535. Fukushima K, Kataoka Y, Maeda Y, Kariya S, Tominaga S, Nagayasu R et al. Cochlear implantation for children with auditory neuropathy among Japanese language users. In: Kaga k, Starr A. Neuropathies of the Auditory and Vestibular Eighth Cranial Nerves. Springer, Tokyo, 2009. p. 71-5. https://doi.org/10.1007/978-4-431-09433-3_8.
https://doi.org/10.1007/978-4-431-09433-...
, clear description of the demographic characteristics of the participants2727. Praveena J, Prakash H, Rukmangathan TM. Language outcomes using hearing aids in children with auditory dyssynchrony. Audiol Res. 2014;4(1)36-39. https://doi.org/10.4081/audiores.2014.80. PMID: 26557348.
https://doi.org/10.4081/audiores.2014.80...
, results of speech and/or language perception before the intervention with the devices2525. Daneshi A, Mirsalehi M, Hashemi SB, Ajalloueyan M, Rajati M, Ghasemi MM et al. Cochlear implantation in children with auditory neuropathy spectrum disorder: a multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2018;108:12-6. https://doi.org/10.1016/j.ijporl.2018.02.004. PMID: 29605339.
https://doi.org/10.1016/j.ijporl.2018.02...
,3232. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...
, clearly reported clinical outcomes3535. Fukushima K, Kataoka Y, Maeda Y, Kariya S, Tominaga S, Nagayasu R et al. Cochlear implantation for children with auditory neuropathy among Japanese language users. In: Kaga k, Starr A. Neuropathies of the Auditory and Vestibular Eighth Cranial Nerves. Springer, Tokyo, 2009. p. 71-5. https://doi.org/10.1007/978-4-431-09433-3_8.
https://doi.org/10.1007/978-4-431-09433-...
, description of the reasons for the loss of participants throughout the period in which the research was developed, as well as strategies for adjusting the analysis of the outcomes, when patients did not participate in the entire study period2525. Daneshi A, Mirsalehi M, Hashemi SB, Ajalloueyan M, Rajati M, Ghasemi MM et al. Cochlear implantation in children with auditory neuropathy spectrum disorder: a multicenter study on auditory performance and speech production outcomes. Int J Pediatr Otorhinolaryngol. 2018;108:12-6. https://doi.org/10.1016/j.ijporl.2018.02.004. PMID: 29605339.
https://doi.org/10.1016/j.ijporl.2018.02...
,3232. Liu Y, Dong R, Li Y, Xu T, Li Y, Chen X et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014;41(6):502-6. https://doi.org/10.1016/j.anl.2014.06.001. PMID: 25194855.
https://doi.org/10.1016/j.anl.2014.06.00...
.

Although variability was observed in relation to sample size, types of studies, evaluation procedures used and methodological aspects, the results of this literature review demonstrated that CI and HA can be effective for the development of speech perception and spoken language in children with auditory neuropathy spectrum disorder, once complex levels of these skills were achieved, such as listening comprehension and speech intelligibility. Due to the heterogeneity of the studies, it was not possible to perform a meta-analysis.

Considering the specificities of ANSD, the continuity of research that seeks to evaluate the speech perception and spoken language of this population, with standardized methods and longitudinal character, may add information about the effectiveness of hearing aids and CI and contribute to the intervention in this pathology.

CONCLUSION

Considering the heterogeneity of the auditory neuropathy spectrum, over 25 years of research, 15 studies were verified suggesting that CI and HA may be effective for the development of speech perception and spoken language of children with auditory neuropathy spectrum disorder with no other associated impairments. The need for studies with high methodological rigor that demonstrate the effectiveness of the intervention with the devices (HA and/or CI) in the performance of speech perception and language of children with ANSD, is emphasized.

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  • Study conducted at Universidade de São Paulo, Hospital de Reabilitação de Anomalias Craniofaciais - HRAC/USP, Bauru, São Paulo, Brazil.
  • Financial support: Nothing to declare.

Publication Dates

  • Publication in this collection
    02 June 2023
  • Date of issue
    2023

History

  • Received
    10 Oct 2022
  • Accepted
    30 Mar 2023
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