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European Annals of Otorhinolaryngology, Head and Neck Diseases
Volume 135, n° 1S
pages 41-48 (février 2018)
Doi : 10.1016/j.anorl.2017.12.008
International Consensus (ICON) - IFOS Paris 2017 - ENT World Congress

International consensus (ICON) on audiological assessment of hearing loss in children
 

A. Farinetti a, , A. Raji b, H. Wu c, B. Wanna d, C. Vincent e
a Department of Pediatric Otolaryngology, Hôpital La Timone Enfants, AP–HM, 264, avenue Saint-Pierre, 13005 Marseille, France 
b Department of Otolaryngology, Mohammed VI Hospital, avenue Ibn Sina Amerchich, BP2360 Marrakech-principal, Morocco 
c Department of Otolaryngology Head and Neck Surgery, Shanghai Ninth People's Hospital & Shanghai Jiaotong University School of Medicine, 639, Zhizaoju Road, 200011 Shanghai, China 
d Department of Otolaryngology Head and Neck, Middle East Institute of Health–University Hospital, Bsalim main road, Mezher street, 60387 Bsalim-Metn, Lebanon 
e Department of Otolaryngology, Hôpital Roger-Salengro, Centre Hospitalier Régional de Lille, rue du Professeur-Emile-Laine, 59000 Lille, France 

Corresponding author.
Abstract

The prevalence of hearing loss in newborns and infants is estimated between 1 to 3.47 cases per 1000 live births. Early diagnosis and rehabilitation of congenital hearing loss are mandatory in order to achieve a satisfactory linguistic and cognitive development. Without appropriate opportunities to learn language, these children will fall behind their normal hearing peers in communication, cognition, reading and socio-emotional development. After promising results, neonatal screening for hearing loss and audiological evaluation are becoming more extensively carried out. In planning universal neonatal hearing screening programs, transient evoked otoacoustic emissions and auditory brainstem responses are the gold standard for the screening and diagnosis program. However, there is no consensus regarding the use of audiometry and other electrophysiological tests (such as auditory steady-state responses) in current practices. Several screening and audiological assessment procedures have been described and advocated all around the world. But, a systematic scheme of performing diagnosis in the pediatric audiology population is lacking. A consensus conference was held at the International Federation of Oto-rhino-laryngological Societies Congress, in June 2017, to discuss the different current practices and to identify the best neonatal hearing screening and audiological assessment management. This article is intended to provide professionals with recommendations about the “best practice” based on consensus opinion of the session's speakers, and a review of the literature on the efficacy of various assessment options for children with hearing loss.

The full text of this article is available in PDF format.

Keywords : Hearing loss, Children, Hearing screening, Audiometry, Otoacoustic emissions, Auditory brainstem responses


Introduction

The prevalence of hearing loss (HL) in newborns and infants is estimated between 1 to 3.47 cases per 1000 live births. The goal of early HL detection and intervention is to maximize linguistic competence and literacy development for children with hearing impairment [1]. Without appropriate opportunities to learn language, these children will fall behind their normal hearing peers in communication, cognition, reading and socio-emotional development.

The identification of HL through the neonatal hearing screening (NHS) is essential for early intervention. If HL is diagnosed before three months of age, and intervention is initiated before the age of six months, significant changes in cognitive and linguistic development of deaf individuals can be obtained [1]. Several screening and audiological assessment procedures have been described and advocated all around the world. But, a systematic scheme of performing diagnosis in the pediatric audiology population is lacking. The use of automated auditory brainstem response (aABR) and otoacoustic emissions (OAE) is implemented in many countries to allow early identification and timely intervention of babies with HL. In European countries, HL screening consists of either a two-stage OAE testing, or the use OAE as a first step, followed by aABR. There is still no consensus on recommended screening techniques [2, 3, 4]. Even in France, the national recommendation of 2014 did not arbitrate and allow either OAEs or aABRs except in NICU [5].

In case of abnormal hearing screening, an audiological evaluation is required to confirm and characterize the HL, based on several tests. This evaluation includes in particular objective electrophysiological measures (auditory brainstem response [ABR], auditory steady-state response [ASSR]) and/or behavioral methods to estimate hearing thresholds. Recommendations are still differ between countries, and early behavioral audiometry in children younger than 6 months is still controversial [6, 7].

Setting of the consensus conference

An International Consensus Conference (ICON) was held at the International Federation of Oto-rhino-laryngological Societies Congress, in June 2017, in Paris, France.

The members of the panel were Pr Abdelaziz Raji (Marocco), Pr Hao Wu (China), and the Dr Bernard Wanna (Lebanon). The discussion was led by the moderators, Pr Christophe Vincent and Dr Anne Farinetti (France). Based on a review of the literature, a questionnaire about “audiological assessment of hearing loss in children” was sent to the panelists and their answers were presented and discussed at the conference (Appendix 1).

The objective was to provide professionals with recommendations about the best practice, based on experts’ opinion and scientific evidence regarding the efficacy of various assessment options for young children with HL.

The review of the literature used an evidence-based approach to provide balanced and objective classification for making informed decisions about assessment options.

Each article has been assessed using the GRADE scoring system. This rating indicates the amount, general quality and clinical applicability of scientific evidence used for each recommendation, ranged from A to D.

Neonatal Hearing Screening

The identification of children with HL through the NHS is essential for early intervention (GRADE D) [1]. To maximize hearing outcomes, it is recommended (1) To screen more than 95% of all newborns by 1 month of age, (2) To perform a comprehensive audiological and medical evaluation at no later than 3 months in case of failed-screening, (3) And to receive appropriate intervention at no later than 6 months of age from health care and educational professionals with expertise in HL and deafness management in infants and young children, in case of confirmed HL (GRADE D) [1].

Currently, two physiological procedures are recommended as NHS for early detection of HL: evoked OAE and aABR.

What procedure should we use?
OAEs

Evoked OAE are low-level sounds primarily generated by the outer hair cells in the cochlea and are recorded in the external auditory canal after stimulation. This procedure is fast and inexpensive, has a high rate of false-positive results (middle ear effusion) and false-negative results (auditory neural spectrum disorders).

These emissions are usually classified according to the generating stimulus:

transient-evoked (TEOAEs) are evoked using a click (broad frequency range) (CEOAEs) or tone burst (brief duration pure tone) (TBOAEs) stimulus at one level of 80dB SPL;
stimulus frequency OAEs (SFOAEs) are measured during the application of a pure tone stimulus, and detected by the vectorial difference between the stimulus waveform and the recorded waveform;
distortion product OAEs (DPOAEs) are evoked using a pair of primary tones ƒ1 and ƒ2 with particular intensity (usually one level of L1 and L2 65/55dB SPL at least at four frequencies).

TEOAEs are present in preterm and full-term infants (range from 82 to 100%), and are thus theorically feasible from 30th week after conception (GRADE B) [8, 9], (GRADE C) [10]. However, it is recommended to use the aABR to not miss ANSD.

The most useful clinically OEA are the TEOAEs and the DPOAEs (GRADE D) [1]. There are numerous differences between both methods, which are important to help us decide to perform either TEOAE or DPOAE.

Hearing level thresholds detection (GRADE B) [11]

When TEOAEs are present, hearing thresholds equal to or better than 20dB HL would be predicted in case of hearing loss related to endocochlear dysfunction with outer hair cells dysfunction. TEOAEs are inevitably absent in cases with sensorineural hearing loss exceeding 40dB HL or in cases of middle ear pathology. A mild hearing loss with thresholds ranging from 25 to 35dB HL is considered in the zone of uncertainty, where the interpretation of TEOAEs is not clear.

Zone of uncertainty (GRADE C) [11]

The zone of uncertainty is wider in DPOAE recordings than in TEOAEs, ranging from 25 to 50–60dB HL. This zone of uncertainty could explain the risk of higher rate of false-negative in case of use of DPOAEs instead of TEOAEs.

Frequency range

TEOAEs are most effective in sampling cochlear function in the mid-frequency region (1000 to 2000Hz), and CEOAEs are almost as frequency-specific as TBOAEs (GRADEC) [12, 13]. On the other hand, DPOAEs can be measured over a broad range of frequencies, they perform better than TEOAEs at 4000Hz or more, but are not accurate predictors of hearing status at lower frequencies. DPOAEs are superior to TEOAEs at frequencies above 2kHz (GRADE A) [14, 15, 16]. In conclusion, neither OAEs nor DPOAEs can show clear superiority.

Influence of SNR

CEOAEs (Chirp) has a relatively high false-positive rate, often due to infant physiological and background noise adversely affecting the emission recording, leading to a “refer” screening result, especially for the low frequencies (below 1000Hz). In attempt to reduce these false-positive screening, TBOAEs (Tone Burst) may elicit a greater signal to noise ratio than CEOAEs.

In this way, the introduction of combined CEOAE and TBOAE protocols may assist in the reduction of “refer” results, and hence the false-positive rates of UNHS programs.

In conclusion, there is no recommendations showing the superiority of TEOAEs over DPOAEs in hearing screening protocols (GRADE B) [8], (GRADE C) [10].

Automated ABRs

It is important to use different screening protocols according to the term of the child in order not to miss neural disorders.

OAEs reflect the status of peripheral auditory system extending to the cochlear outer hair cells (OHC). In contrast, automated ABRs (aABRs) reflect the status of the peripheral auditory system, the eighth nerve, and the brainstem auditory pathway. According to the JCIH recommendations in 2007, both techniques can be used to detect cochlear hearing loss (GRADE D) [1]. On the other hand, OAEs cannot be used to detect neural dysfunction that requires aABRs (GRADE C) [17], (GRADE D) [1].

However, both may be affected by outer or middle ear dysfunction. Consequently, TEOAEs may result in a failed-screening test result in the presence of normal cochlear and/or neural function. Because OAEs are low-amplitude acoustic signals, even small middle ear status changes can be sufficient to reduce OAE amplitude or prevent the OAE from being recorded (GRADE C) [3].

What is the most appropriate screening protocol?

In 2016, Vos et al. reported a high level of diversity in newborn hearing screening programmes in the European Union. The differences concerned levels of policy decision-making and designs of the programmes. More than 59% of the European participating countries chose a different protocol for weel-infant nurseries and for NICU, while other countries (33%) chose only one test for all their newborns [18].

When performing a two-stage screening protocol, different schemes are reported in the literature: either using OEA for the test and retest, or using OEA as a first step followed by aABR. The authors are not unanimous on this subject.

It is recommended to perform a neonatal hearing screening in all newborns in order to identify infants born with hearing loss soon after birth, but this screening is not yet available to all countries (GRADE D) [1].
It is recommended to use aABRs in NICU, in order to not miss auditory neural disorders (GRADE D) [1].

Newborns without risks

For newborns without risks, the international pediatric otolaryngology group (IPOG) in 2016 recommends to perform a two-stage testing with OAEs alone as the initial screening in nurseries. However, some institutions no longer stratify based on risk and instead screen all infants with aABR in order to detect all cases of ANSD (GRADE D) [19].

There are international recommendations on the two-step screening procedure, but controversies persist over the type of screening material to be used (OAEs or aABRs), and on the indication of a systematic control at 1 month of life (OAEs or aABRs), before performing ABR as a diagnostic confirmation test [1, 19].

Newborns at risks

For infants at risks, the Consensus statement of JCIH 2007 recommended the use of a combination of OAEs and aABRs for all infants, while recommending aABRs alone for infants in NICU, in order not to miss neural hearing loss [1]. Moreover, when a child, with conditions associated with potential HL (hyperbilirubinemia that requires exchange transfusion or culture-positive sepsis), is readmitted to a healthcare facility in the first month of life, a repeated hearing screening is recommended before discharge (GRADE D) [1].

On the other hand, the IPOG recommend screening high risk neonates with both OAEs and aABRs, and not only with aABRs, in order to suspect ANSD [19]. The panelists all agreed with the recommendations of the JCIH, in cases where nurseries have aABRs.

Screening protocols (GRADE D) [19]

The first screening can be performed with either OAEs or aABRs according to nurseries’ protocols.

For infants who do not pass either OAEs or aABRs, the French authorities recommend to perform a control in both cases, in the first month of life [5].

Outpatient screening should also be available to infants who were discharged before receiving the birth admission screening or who were born outside a healthcare center. At this stage, testing both ears is recommended, even if only one ear failed the initial screening.

All panelists agreed that a two-stage procedure is mandatory for hearing screening, either with TEOAEs alone, or TEOAEs as a first step and aABR for rescreening in the neonate period. In case of abnormal screening, an evaluation should be performed in an audiological referral center at 1 month of life.

Audiological assessment
Audiologic Test Battery

After hearing screening, and when hearing loss is suspected, a systematic clinical examination must be done with recording of child and family history and the evaluation of developmental milestones, then followed by an otoscopy.

The order and selection of tests to be included in the audiological test battery vary based on the age of the child: OAEs, acoustic immitance, behavioral audiometry, ABR and ASSR.

Otoscopy

Otoscopy is used to ensure that there are no contra-indications to placing an earphone or probe in the external auditory canal (EAC):

verify that the external auditory canal is free of obstruction and that there is no drainage from the middle ear;
examining the tympanic membrane characteristics regarding color, position, and anatomical abnormalities.

Visual inspection should look for structural abnormalities: ear pits, ear tags, atresia, low-set ears of the pinna and/or EAC.

OAEs

TEOAEs then should be followed by tympanometry. If the tympanogram is abnormal, a conductive hearing loss is likely.

OAE tests are useful for assessing cochlear amplification (GRADE C) [20].

TEOAEs more qualitatively assess cochlear function and are therefore more suited for topographic diagnostics. DPOAEs provide more quantitative information about the hearing loss. DPOAE audiograms are able to assess cochlear hearing loss more precisely than behavioral tests in infants where the conditioned free-field audiogram does not reflect the real threshold.

DPOAEs are not present at frequencies where the hearing loss is higher than 50dB. In cases where DPOAEs are not measurable – that concern only a few cases – ASSRs have to be measured in order to get frequency-specific information on the hearing loss in the entire range of hearing [20].

Acoustic immitance

Tympanometry and acoustic reflex testing are used in conjunction to assess middle ear effusion.

Tympanometry

A higher probe-tone frequency (1000Hz) appears to provide a more valid test to diagnose middle ear effusion (MEE) in children under 6 months of corrected age. With children between 5 and 7 months of corrected age, false-negative tympanograms in ears with MEE are possible (GRADE D) [1], (GRADE B) [21, 22]. A 226Hz probe-tone is more appropriate for most children over 6 months of age.

This test should be a part of UNHS test battery, because high-frequency tympanometry shows a significant relation between otologic evaluation and TEOAE test results (GRADE C) [23, 24]. All panelists advocated that otoscopy and tympanometry are both essential before rescreening in order to eliminate MEE.

Acoustic (Stapedial) Reflex Testing (ART)

Ipsi- and contra-lateral ART should be done at 500, 1000 and 2000Hz and are recommended for screening of children over 6 months of age (GRADE D) [1]. The reflex is absent or not measurable in the presence of middle ear effusion, nonintact membrane, cerumen impaction, cochlear HL grater than 80dB or some hearing losses of retrocochlear origin. Its presence confirms both the intact nature of the neural reflex arc and the absence of middle ear effusion, and allows to eliminate in the majority of cases a severe to profound HL. However, no recommendation exists on its systematic use in audiological evaluation in children.

Wideband Acoustic Reflectance

Wideband acoustic reflectance is interesting as a clinical tool to assess MEE in young infants, but further investigation is still needed (GRADE D) [1].

Behavioral audiometry

Behavioral techniques

Different age-appropriate behavioral techniques exist. Visual reinforcement audiometry (VRA) is typically used in patients younger than 24 months old, while conditioned play audiometry (CPA) is used for children between 2 and 5 years old.

Conventional audiometry is used for all patients above 5 years of age and these techniques are the gold standard to quantify and describe hearing loss in children (Table 1) (GRADE D) [1, 25].

Behavioral testing under the age of 6 months

Under six months of age, measurements are based on the observation of the reflex reactions triggered by the perception of an acoustic stimulus.

Numerous protocols have been established in different countries.

In France, it is recommended that the assessment of newborns (under the age of 5 months) should not been restricted to objective tests but should also include bone- and air-conduction behavioral tests adjusted to developmental stage and performed in the presence of the parents (GRADE C) [6, 26].

This audiometric assessment procedure is known as “the Delaroche protocol”. In addition to quantitative information, the method of Delaroche highlights information on the development of the child, his or her relational and communication competences, all of which are determining factors for the organization and prognosis of intervention. In 2008, Madell developed the “Sucking Test Protocol”, to provide direct measures of hearing in children younger than 6 months (GRADE D) [7].

All panelists agreed that audiometry in children younger than 6 months is not available in all referral centers due to lack of time or lack of experienced audiophonologists. In these cases, they prefer to use ABRs as a complementary test to diagnose hearing loss in neonates.

Objective electrophysiological tests

It is likely that today ABR could easily be replaced by the ASSR testing. ASSR testing has emerged as an alternative electrophysiological technique that can, as ABR testing, be used to confirm behavioral audiometry thresholds, particularly for children who are too young for reliable behavioral testing.

ABR

The ABRs allow a specific stimulation of the auditory system by an optimal nerve fiber synchronization over the 2–4kHz frequency range.

The click stimulus provides estimation in a broad frequency range between 1000 to 4000Hz. Although, we need a complete evoked potential evaluation which contains reliable ear specific and frequency-specific information with the tone burst stimuli [27, 28].

The tone burst (TB) is a brief tone stimulation more frequency-specific. Like click produced ABR, TB ABR is an excellent predictor of hearing in neonates and infants. The differences are (1) That accelerates the acquisition of ABR responses, and (2) That click permits to assess high-frequency hearing, while low-frequency (500Hz) TB assesses low-frequency hearing [29].

With click stimulus, the difficulty is due to the cochlear travel wave delay whereby frequency areas are stimulated one after the other. The CE-Chirp is a family of stimuli designed to compensate the cochlear travel wave delay and provide enhanced neural synchronicity [30, 31].

The TB ABR is the current gold standard test recommended by the JCIH 2007 for estimation the audiogram for infants between 1 and 3 months (Fig. 1). On the other hand, the NHS recommends tone-pip clicks stimulation and now accepts Chirp stimuli to evaluate hearing thresholds [32].

It is recommended to evaluate hearing thresholds with ABR for estimation the audiogram for infants between 1 and 3 months.
There is no universal recommendations about the type of stimulus which is different according to the national Guidelines (GRADE D) [1, 32].



Fig. 1


Fig. 1. 

Screening and rescreening protocols (GRADE D) [1, 19]. AABR: automated auditory brainstem response; OEA: otoacoustic emission; ABR: auditory brainstem response; ANSD: auditory neuropathy spectrum disorder; HL: hearing loss.

Zoom

The JCIH and the NHS suggest that the clinicians use air conduction TB stimuli to record ABR and, when thresholds are elevated, bone-conduction TB stimuli in order to differentiate among sensory, conductive and mixed HL and to determine the configuration of HL in each ear (GRADE D) [1, 32].

Gorga in 2006 recommended the use of the combination of click-ABR and low-frequency TB-evoked ABR threshold measurements to quickly provide clinical information for both ends of the audiogram (250–500Hz for TB, 2 to 4kHz for click) (GRADE B) [28].

ASSR

ASSR testing has many advantages over ABR.

This test permits estimation of hearing thresholds in the low to upper frequencies (e.g. 500 to 4000Hz) (GRADE B) [32], and so, is a valuable tool to detect residual hearing. François et al., in 2016, recommended to perform ASSR before ABR when hearing threshold rather than latency is to be determined (GRADE C) [33].

This test is an objective response detection method using an automatic detection algorithm, and strong correlations have been demonstrated between air conduction ASSR, ABR and Pure Tone Audiometry (PTA) thresholds (with a difference from 5 to 15dB). ASSR testing has the advantage of being able to determine responses to both ears at multiple frequencies simultaneously (dichotic multiple-stimulus technique).

Unfortunately, this test is more time consuming than the ABR, there are fewer correlations between ASSR and PTA thresholds on the 500Hz and the 4000Hz frequencies, and fewer correlations in children with ANSD [34, 35, 36, 37, 38, 39, 40]. Furthermore, the maximum presentation level usually does not exceed 90 to 105dB HL to avoid saturation. The possibility of artefactual responses in intensities above 110dB HL can be minimized by the use of insert earphones. Absent ABR is consistent with significant hearing impairment but cannot distinguish between severe to profound HL.

ASSR should complement while not replacing the OEAs and ABRs in preterm infants or infants with increasing risk of ANSD (GRADE B) [41, 42].
It is not recommended including high intensities (>120dB HL) in routine evaluation of pediatric cochlear implant (CI) candidates because these very high intensities may damage the cochlea and the clinician may be unaware of behavioral test results, as the electrophysiological tests usually precede the behavioral tests. (GRADE B) [43].
It is recommended to perform an electrocochleography (ECochG) if ABR responses are unreliable or in cases of suspicion of ANSD (GRADE D) [25]

Auditory neuropathy spectrum disorder (ANSD)

ANSD occurs in about 10% of individuals who have a dys-synchronous ABR, or an ABR consistent with an estimate of severe or profound HL [37, 44, 45].

Both TEOAEs and abnormal positive ABR may occur in more than 40% of hearing-impaired children in NICU setting [46].

ANSD is a hearing disorder resulting from lesions involving auditory nerve fibers themselves (postsynaptic ANSD), the inner hair cells (IHCs) or their synapses with auditory nerve terminals (presynaptic ANSD) [47].

Risk indicators

The risk factors for the development of an auditory neuropathy are only speculative.

The exact mechanisms which underlie auditory neuropathy development in case of environmental risk factors are not completely elucidate. Factors associated with ANSD in newborns include hyperbilirubinemia, prematurity, ototoxic drug therapy, and positive familial history. Genetic causes of ANSD are heterogenous, and can be syndromic, non syndromic or mitochondrial related (such as otoferline, pejvakine…) (GRADE C) [47, 48].

In infants and children, risk indicators of ANSD are the difficulty of understanding speech, particularly in the presence of noise, a fluctuating hearing ability, or delays in speech and language development (GRADE A) [49, 50, 51, 52, 53, 54].

Diagnosis [55]

Hearing loss in cases of ANSD is usually bilateral and of any severity degree, with poor speech perception.

The audiological battery test has to test the preservation of cochlear OHC receptors activity (presence of OAEs and/or Cochlear Microphonic potentials CMs), and the disruption of auditory nerve function (abnormal ABR) (GRADE C) [47]. Acoustic Reflex Testing reports absent responses to the ipsi- and contra-lateral tones at 110dB HL [54, 56].

Some infants who show ANSD characteristics at birth could develop ABRs over the first year of life, because The ABRs continue to mature through the first 18 months of age [34, 57].

When the diagnosis of ANSD is made, these children should have regular hearing assessments within the first two years of life (with ABR, OAEs, and behavioral audiometry) (GRADE C) [47].

Madden reported children diagnosed as having ANSD in the neonate period whose thresholds resolved to normal levels within the first year of life (7% for Berlin) [47, 56].

The audiological test results in ANSD children are summarized in the Table 2.

Auditory amplification

Providing a clearer signal to an auditory system that cannot cope with interference from noise, as occurs in ANSD, can be particularly helpful in those patients with some residual word recognition in quiet environments.

Hearing aids

Benefits with hearings aids (HAs) are variable from 39 to 75% (in terms of speech comprehension and language acquisition) [52, 58, 59, 60].

The use of OAEs during the follow-up is recommended to detect damage to the OHCs while the children are using HAs (GRADE C) [61, 62].

Frequency modulation systems

The use of a FM system is helpful in noisy settings [49].

Cochlear implantation

CI allows significant improvement of speech perception in quiet and in noise but expectations must be lower than those for children with cochlear loss (GRADE C) [47, 58, 63].

Cochlear implant is accepted as the standard treatment option for ANSD in cases where there is no spontaneous improvement and where limited benefit is obtained with a HA (GRADE C) [64, 65, 66, 67]:
Benefits: speech and language development, communication ability;
Period of 3 months of hearing aid rehabilitation is recommended;

In these cases, side-specific ECochG should be used to ensure the diagnosis of ANSD and verify the indication for a CI (GRADE B) [68, 69].

In cases of severe to profound HL and poor response to amplification with HAs, side-specific ECochG is used to ensure the diagnosis of ANSD and verify the indication for a CI. Specific waveform ECochG patterns would be helpful to outline ANSD to a delay of maturation of the auditory brainstem (post synaptic lesion) or to OHC damage (presynaptic lesion), as this may have implications for the outcome of CI.

Conclusion

The development of universal newborn screening for HL allows for earlier diagnosis of HL and thus earlier auditory rehabilitation using HAs or CIs. While this intervention is extremely important at early stages of development, it is also important to accurately determine hearing thresholds, the degree and type of HL. Overall, the panelists expressed minor differences in daily practices and agreed on audiological assessment protocols, except for electrophysiological tests, which are not feasible in every country. In clinical practice, pediatric audiologists use different age-appropriate behavioral techniques to estimate thresholds at different frequencies. The neonatal hearing screening program using automated ABR and OAE are implemented in most countries to allow early identification of babies with HL. After failure of rescreening, newborns are referred for comprehensive audiological evaluation. In children younger than 6 months, ABR is still the gold standard in most countries. After 6 months of corrected age, ABR and behavioral audiometry are used together to assess hearing thresholds. The ASSRs are not yet used in the current practice for all the panelists, because this test is expensive and time consuming.

Disclosure of interest

The authors declare that they have no competing interest.


Appendix 1. Panelits's questionnaire about “Audiological Assessment of Hearing Loss in Children”

Neonatal Hearing Screening

1.
What is the most appropriate neonatal hearing screening protocol and which test do you use: TEOAEs, DPOAEs, automated ABR or ABR?
2.
Is it relevant to use different screening protocol for neonates with risk factors, and why?
3.
In case of suspicion of hearing impairment on neonatal screening, what is your appropriate management to assess it?
-
Do you refer failed-screening to a referral center or an ENT private practice?
-
After a failed-screening, do you recommend testing with automated ABR or do you perform initially diagnostic tests such as audiometry/ABR and ASSR?

Hearing Evaluation

4.
Is behavioral audiometry reliable in infants younger than 6 months or does it have to be replaced by objective measures?

Regarding audiological tests:

5.
According to you, what is the value of Auditory Steady-State Response (ASSR) in the diagnosis of hearing loss? And, do you think that ASSR could be a good test to replace ABR?
6.
In which circumstances do you use speech perception tests in noise or spatial localization tests in the management of hearing-impaired children?

Children with Auditory Neuropathy Spectrum Disorder

7.
When do you suspect an auditory neuropathy in children?
8.
What do you use to diagnose auditory neuropathy in children: behavioral tests and/or electrophysiological tests, and if so, which one?
9.
Regarding rehabilitation: what results can be expected with hearing aids and with cochlear implantation?

References

Joint Committee on Infant Hearing Year 2007 position statement: principles and guidelines for early hearing detection and intervention programs Pediatrics 2007 ;  120 : 898-921
Da Silva D.P., Suman Lopez P., Espíndola Ribeiro G., and al. The importance of retesting the hearing screening as an indicator of the real early hearing disorder Braz J Otorhinolaryngol 2015 ;  81 (4) : 363-367 [cross-ref]
El-Refaie A., Parker D., Bamford J. Otoacoustic emission versus ABR screening: the effect of external and middle ear abnormalities in a group of SCBU neonates Br J Audiol 1996 ;  30 (1) : 3-8 [cross-ref]
Smyth V., Mcpherson B., Kei J., and al. Otoacoustic emission criteria for neonatal hearing screening Int J Pediatr Otorhinolaryngol 1999 ;  48 : 9-15 [cross-ref]
Arrêté du 3 novembre 2014 relatif au cahier des charges national du programme de dépistage de la surdité permanente néonatale  :  (2014). 1-6[Available from: www.legifrance.gouv.fr/].
Delaroche M., Gavilan-Cellié I., Maurice-Tison S., and al. Is behavioral audiometry achievable in infants younger than 6 months of age? Int J Pediatr Otorhinolaryngol 2011 ;  75 : 1502-1509 [cross-ref]
Madell J.R. Using behavioral observation audiometry to evaluate hearing in infants from birth to 6 months  diagnosing hearing disorders in infants and children :  (1974). 54-64
Cavalcante J.M., De Lima Isaas M. Analysis of otoacoustic emissions in neonates at term and preterm Braz J Otorhinolaryngol 2013 ;  79 (5) : 582-588 [cross-ref]
Norton S., Gorga M., Widen J., and al. Identification of neonatal hearing impairment: evaluation of transient evoked otoacoustic emission, distorsion product otoacoustic emission, and auditory brainstem response test performance Ear Hear 2000 ;  21 (5) : 508-528 [cross-ref]
Brienesse P., Maertzdorf W., Anteunis L., and al. Click-evoked oto-acoustic emission measurement in preterm infants Eur J Pediatr 1998 ;  157 (12) : 999-1003 [cross-ref]
Balatsouras D.G., Kaberos A., Kloutsos G., and al. Correlation of transiently evoked to distortion-product otoacoustic emission measures in healthy children Int J Pediatr Otorhinolaryngol 2006 ;  70 : 89-93 [cross-ref]
Bonfils P., Dumont A., Marie P., and al. Evoked otoacoustic emissions in newborn hearing screening Laryngoscope 1990 ;  100 (2) : 186-189 [cross-ref]
Kemp D., Ryan S., Bray P. A guide to the effective use of otoacoustic emissions Ear Hear 1990 ;  11 (2) : 93-105 [cross-ref]
Prieve B.A., Gorga M.P., Schmidt A., and al. Analysis of transient-evoked otoacoustic emissions in normal-hearing and hearing impaired ears J Acoust Soc Am 1993 ;  93 (6) : 3308-3319 [cross-ref]
Gorga M., Neely S., Bergman B., and al. A comparison of transient-evoked and distortion product otoacoustic emissions in normal-hearing and hearing-impaired subjects J Acoust Soc Am 1993 ;  94 (5) : 2639-2648 [cross-ref]
Santarelli R. Information from cochlear potentials and genetic mutations helps localize the lesion site in auditory neuropathy Genome Med 2010 ;  2 (91) : 1-10
Rhee C., Park H., Jang Y. Audiological evaluation of neonates with severe hyperbilirubinemia using transiently evoked otoacoustic emissions and auditory brainstem responses Laryngoscope 1999 ;  109 : 2005-2008 [cross-ref]
Vos B., Senterre C., Lagasse R., and al. Organisation of newborn hearing screening programmes in the European Union: widely implemented, differently performed Eur J Public Health 2016 ;  26 (3) : 505-510 [cross-ref]
Liming B.J., Carter J., Cheng A., and al. International pediatric otolaryngology group (IPOG) consensus recommendations: hearing loss in the pediatric patient Int J Pediatr Otorhinolaryngol 2016 ;  90 : 251-258 [cross-ref]
Janssen T. A review of the effectiveness of otoacoustic emissions for evaluating hearing status after newborn screening Otol Neurotol 2013 ;  34 : 1058-1063 [cross-ref]
Margolis R., Bass-Ringdahl S., Hanks W., and al. Tympanometry in newborn infants – 1kHz norms J Am Acad Audiol 2017 ;  14 (7) : 383-392
Kei J. Acoustic stapedial reflexes in healthy neonates: normative data and test-retest reliability J Am Acad Audiol 2012 ;  23 (1) : 46-56
Kilic A., Baysal E., Karatas E., and al. The role of high frequency tympanometry Eur Rev Med Pharmacol Sci 2012 ;  16 : 220-223
Swanepoel D., Erasmus H. Auditory steady-state responses for estimating moderate hearing loss Eur Arch Otorhinolaryngol 2007 ;  264 : 755-759 [cross-ref]
Sterkers-Artières F., Vincent C. Consensus formalisé d’experts concernant l’audiométrie de l’adulte et de l’enfant  Rapport de la SFORL :  (2014). 1-21
Delaroche M., Thiebaut R., Dauman R. Behavioral audiometry: validity of audiometric measurements obtained using the “Delaroche protocol” in babies aged 4–18 months suffering from bilateral sensorineural hearing loss Int J Pediatr Otorhinolaryngol 2006 ;  70 : 993-1002 [cross-ref]
Baldwin M., Watkin P. Predicting the degree of hearing loss using click auditory brainstem response in babies referred from newborn hearing screening Ear Hear 2013 ;  34 : 361-369 [cross-ref]
Gorga M.P., Johnson T.A., Kaminski J.K., and al. Using a combination of click- and toneburst-evoked auditory brainstem response measurements to estimate pure-tone thresholds Ear Hear 2006 ;  27 (1) : 60-74 [cross-ref]
McCreery R., Kaminski J., Beauchaine K.L., and al. The impact of degree of hearing loss on auditory brainstem response predictions of behavioral thresholds Ear Hear 2015 ;  36 (3) : 309-319 [cross-ref]
Elberling C., Callø J., Don M. Evaluating auditory brainstem responses to different chirp stimuli at three levels of stimulation J Acoust Soc Am 2010 ;  128 (1) : 215-223 [cross-ref]
Elberling C., Don M. Auditory brainstem responses to a chirp stimulus designed from derived-band latencies in normal-hearing subjects J Acoust Soc Am 2008 ;  124 (5) : 3022-3037 [cross-ref]
Stevens J., Sutton G., Wood S., and al. Guidelines for the early audiological assessment and management of babies referred from the newborn hearing screening programme NHSP Early Assess Guidel 2013 ;  31 : 1-44
Francois M., Dehan E., Carlevan M., and al. Use of auditory steady-state responses in children and comparison with other electrophysiological and behavioral tests Eur Ann Otorhinolaryngol Head Neck Dis 2016 ;  133 : 331-335 [cross-ref]
Dimitrijevic A., John M., Van Roon P., and al. Estimating the audiogram using multiple auditory steady-state responses J Am Acad Audiol 2002 ;  13 (4) : 205-224
Swanepoel D., Schmulian D., Hugo R. Establishing normal hearing with the dichotic multiple-frequency auditory steady-state response compared to an auditory brainstem response protocol Acta Otolaryngol 2004 ;  124 : 62-68 [cross-ref]
Picton T., John M. Avoiding electromagnetic artifacts when recording auditory steady-state responses J Am Acad Audiol 2004 ;  15 (8) : 541-554 [cross-ref]
Rance G., Beer D., Cone-Wesson B., and al. Clinical findings for a group of infants and young children with auditory neuropathy Ear Hear 1999 ;  20 (3) : 238-252 [cross-ref]
Shinn J., Musiek F. The auditory steady state response in individuals with neurological insult of the central auditory nervous system J Am Acad Audiol 2007 ;  18 (10) : 826-845 [cross-ref]
Gorga M.P., Neely S.T., Hoover B.M., and al. Determining the upper limits of stimulation for auditory steady-state response measurements Ear Hear 2004 ;  25 (3) : 302-307 [cross-ref]
Small S., Stapells D. Artifactual responses when recording auditory steady-state Ear Hear 2004 ;  25 (6) : 611-623 [cross-ref]
Silva D.P.C., Lopez P.S., Montovani J.C. Auditory steady state response in hearing assessment in infants with cytomegalovirus Rev Paul Pediatr 2013 ;  31 (4) : 550-553 [cross-ref]
Luts H., Desloovere C., Wouters J. Clinical application of dichotic multiple-stimulus auditory steady-state responses in high-risk newborns and young children Audiol Neurotol 2006 ;  11 (1) : 24-37 [cross-ref]
Grasel S.S., de Almeida E.R., Beck R., and al. Are auditory steady-state responses useful to evaluate severe-to-profound hearing loss in children? Biomed Res Int 2015 ; 1-7 [cross-ref]
Berlin C., Hood L., Morlet T., and al. Auditory neuropathy/dys-synchrony: diagnosis and management Ment Retard Dev Disabil Res Rev 2003 ;  9 (4) : 225-231 [cross-ref]
Lee J.S.M., Mcpherson B., Yuen K.C.P., and al. Screening for auditory neuropathy in a school for hearing impaired children Int J Pediatr Otorhinolaryngol 2001 ;  61 : 39-46 [cross-ref]
Rea P., Gibson W. Evidence for surviving outer hair cell function in congenitally deaf ears Laryngoscope 2003 ;  113 (11) : 2030-2034
Madden C., Rutter M., Hilbert L., and al. Clinical and audiological features in auditory neuropathy Arch Otolaryngol Head Neck Surg 2002 ;  128 : 1026-1030 [cross-ref]
Manchaiah V.K.C., Zhao F., Danesh A.A., and al. The genetic basis of auditory neuropathy spectrum disorder (ANSD) Int J Pediatr Otorhinolaryngol 2011 ;  75 (2) : 151-158 [cross-ref]
Hood L.J. Auditory neuropathy/dys-synchrony disorder: diagnosis and management Otolaryngol Clin N Am 2015 ;  48 (6) : 1027-1040 [inter-ref]
Starr A., Picton T., Sininger Y., and al. Auditory neuropathy Brain 1996 ;  119 : 741-753 [cross-ref]
Starr A., Sininger Y., Pratt H. The varieties of auditory neuropathy J Basic Clin Physiol Pharmacol 2000 ;  11 (3) : 215-230 [cross-ref]
Berlin C., Hood L., Morlet T., and al. Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony (auditory neuropathy spectrum disorder) Int J Audiol 2010 ;  49 (1) : 30-43 [cross-ref]
Zeng F., Oba S., Garde S., and al. Temporal and speech processing deficits in auditory neuropathy Neuroreport 1999 ;  10 (16) : 3429-3435 [cross-ref]
Sanyelbhaa H., Hamed L., Khafagy A.H., and al. Persistence of otoacoustic emissions in children with auditory neuropathy spectrum disorders Int J Pediatr Otorhinolaryngol 2013 ;  77 (5) : 703-706
Kaga K. Auditory nerve disease and auditory neuropathy spectrum disorders Auris Nasus Larynx 2016 ;  43 (1) : 10-20 [cross-ref]
Berlin C., Bordelon J., St John P., and al. Reversing click polarity may uncover auditory neuropathy in infants Ear Hear 1998 ;  19 (1) : 37-47 [cross-ref]
Dowley A., Whitehouse W., Mason S., and al. Auditory neuropathy: unexpectedly common in a screened newborn population Dev Med Child Neurol 2009 ;  51 (8) : 642-646 [cross-ref]
Raveh E., Buller N., Badrana O., and al. Auditory neuropathy: clinical characteristics and therapeutic approach Am J Otolaryngol 2007 ;  28 : 302-308 [cross-ref]
Rance G., Barker E. Speech and language outcomes in children with auditory neuropathy/dys-synchrony managed with either cochlear implants or hearing aids Int J Audiol 2009 ;  48 (6) : 313-320 [cross-ref]
Ching T., Day J., Dillon H., and al. Impact of the presence of auditory neuropathy spectrum disorder (ANSD) on outcomes of children at three years of age Teresa Int J Audiol 2013 ;  52 (2) : 1-22
Deltenre P., Mansbach A., Bozet C., and al. Auditory neuropathy with preserved cochlear microphonics and secondary loss of otoacoustic emissions Audiology 1999 ;  38 (4) : 187-195 [cross-ref]
Ngo R.Y.S., Tan H.K.K., Balakrishnan A., and al. Auditory neuropathy/auditory dys-synchrony detected by universal newborn hearing screening Int J Pediatr Otorhinolaryngol 2006 ;  70 : 1299-1306 [cross-ref]
Rance G., Barker E. Speech perception in children with auditory neuropathy/dyssynchrony managed with either hearing AIDS or cochlear implants Otol Neurotol 2008 ;  29 (2) : 179-182 [cross-ref]
Jeong S., Kim L. Auditory neuropathy spectrum disorder: predictive value of radiologic studies and electrophysiologic tests on cochlear implant outcomes and its radiologic classification Acta Otolaryngol 2013 ;  133 : 714-721 [cross-ref]
Trautwein P.G., Sininger Y.S., Nelson R., and al. Cochlear implantation of auditory neuropathy J Am Acad Audiol 2000 ;  11 (6) : 309-315
Shallop J.K., Peterson A., Facer G.W., and al. Cochlear implants in five cases of auditory neuropathy: postoperative findings and progress Laryngoscope 2001 ;  111 (4 Pt 1) : 555-562 [cross-ref]
Breneman A., Gifford R., Dejong M. Cochlear implantation in children with auditory neuropathy spectrum disorder: long term outcomes J Am Acad Audiol 2012 ;  23 (1) : 5-17
Stuermer K., Beutner D., Foerst A., and al. Electrocochleography in children with auditory synaptopathy/neuropathy: diagnostic findings and characteristic parameters Int J Pediatr Otorhinolaryngol 2015 ;  79 (2) : 139-145 [cross-ref]
McMahon C., Patuzzi R., Gibson W., and al. Frequency-specific electrocochleography indicates that presynaptic and postsynaptic mechanisms of auditory neuropathy exist Ear Hear 2008 ;  29 (3) : 314-325 [cross-ref]



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