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33 Managing Infants and Children with Auditory Neuropathy Spectrum Disorder

Madell et al., Pediatric Audiology: Diagnosis, Technology, and Management, 3rd Ed. (ISBN 978-1-62623-401-7), copyright © 2019 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.

as an alternate measurement of auditory function that, together with the PEACH questionnaire, can help guide the management of

infants with ANSD over the ﬁrst year after birth.

33.3.1 Objective and Functional Approach

CAEPs are a series of waves recorded on the scalp generated by the auditory cortex. In infants, the CAEP is dominated by a positive-polarity peak with a latency of around 200 ms after stimulus onset, and it can be recorded from infants within the

ﬁrst few months after birth.41 The peak amplitude of P1 is rela-

tively large in infants, and it lasts for hundreds of milliseconds, in contrast to the small peaks occurring every 1 to 2 ms for the ABR. This means that while small disruptions in neural synchrony can result in abnormalities of the ABR, they have less impact on the CAEP. Whereas ABR may be absent in infants with ANSD, CAEPs are often detectable. This makes these responses particularly relevant to use when assessing individuals with ANSD.

Hearing thresholds in babies have been challenging to estimate accurately using CAEPs, which is likely to be due to the high myogenic noise levels relative to the small-amplitude CAEP at

threshold. Nevertheless, testing at intensities of 55 to 75 dB sound pressure level (SPL) in the free ﬁeld can help to narrow down

the range of the degree of hearing loss, which can assist in the

decision of whether or not to ﬁt ampliﬁcation.

Research has shown a correlation between stimulus sensation level and CAEP response detection in infants.42 When unaided

CAEPs are present at 55 dB SPL in the free ﬁeld, it is likely that the

behavioral hearing thresholds lie within the normal to mild hearing loss range at the frequency band tested. Similarly, when CAEPs

are present at 65 dB SPL but absent at 55 dB SPL, present at 75 dB SPL but absent at 65 dB SPL, or absent at 75 dB SPL, the hearing

thresholds are likely to be in the mild to moderate, moderate to moderately severe, and severe to profound range, respectively. It

is important to keep in mind that up to 32% of infants show no

CAEP even when the sound is audible to them.43 For this reason an absent CAEP should be interpreted together with measures of functional auditory behavior and behavioral response audiometry (BOA) to establish whether there is consistency between behavioral and objective measures.

A number of studies have used the speech sounds /m/, /g/

and /t/, which have spectral emphasis at around 250, 1,500, and

3,000 Hz, respectively, as speech stimuli for CAEP testing. Using

a combination of these sounds presented at dierent intensities, it is possible to establish the general degree and conﬁguration of the hearing loss. If unaided CAEPs are absent at 55 dB HL, and

baseline PEACH scores are not age appropriate, aiding should be considered in consultation with the family and early intervention therapist working with the child.

Pearl

CAEPs and tests of functional auditory behavior can be used to

assist in the early tting of amplication before VRA results can

be obtained.

Tests of functional auditory behavior, such as the PEACH questionnaire, have a number of beneﬁts. First, there are validated normative data that can be used as a reference point for

unctional auditory development.

f repeated over time to ensure that the infant is progressing as expected or showing signs of plateauing or deterioration. Third, it engages parents in the observation and assessment process. A positive correlation has been shown between aided CAEP results and PEACH score in infants with SNHL and ANSD, whereby infants with CAEPs present to a wider range of stimuli, showing higher PEACH scores. morphology have been linked to lower IT-MAIS scores in infants and to lower auditory skills and speech discrimination scores in children. perception demonstrates the value of combining CAEP testing and formal measures of functional auditory behavior to complement the audiologic assessment battery in these cases.

46,47

In a similar vein, abnormal CAEP latencies and

48,49

The relationship between cortical detection and

33.3.2 Hearing Aids

Once a decision to aid is reached, the initial estimated audiogram based on CAEPs and/or functional auditory behavior can be used to derive prescriptive targets according to a prescriptive procedure for children, such as the National Acoustic Laboratories (NAL) or the Desired Sensation Level (DSL) procedure. Real-ear measurements are required to verify that the prescriptive targets

are met in hearing devices. After veriﬁcation, the eectiveness of the ampliﬁcation in providing audibility should be evaluated

using aided CAEP and PEACH assessments, both in infants with

43,50

SNHL monotonic pattern to speech stimuli, whereby an increase in the stimulus sensation level results in an increase in the detection rate of the CAEP. The same monotonic pattern has been seen in some,42 but not all,46 infants with ANSD.

Evaluation Procedure

CAEP testing for infants is typically carried out in an acoustically

treated room. Speech stimuli can be presented from a free-ﬁeld

loudspeaker at a calibrated level. An infant is positioned in a high chair or a parent’s lap at 0° azimuth, at a distance of about 1 meter from the loudspeaker. Three electrodes are placed on the scalp: a recording electrode on the vertex of the head, a reference electrode on the mastoid, and a ground electrode on the forehead. In aided testing, the infant wears his or her hearing aids,

which must have been veriﬁed to match prescriptive targets.

and in those with ANSD.42 CAEPs typically show a

44,45

Second, the PEACH can be

351

IV Educational and Clinical Management of Hearing Loss in Children

her newborn hearing screen and was diagnosed with ANSD. At 2 months corrected age, CAEPs were measured in the unaided condition to estimate hearing sensitivity and the need for ampli-

ﬁcation. Results revealed no detectable responses to speech sound stimuli /t/ /m/ /g/ at 75 dB SPL. Hearing aids were then ﬁtted to a severe degree of hearing loss in both ears. Aided CAEPs and PEACH were used to evaluate the ampliﬁcation, and the

hearing aids were then adjusted accordingly. Subsequent evaluation results showed limited progress in the child’s functional and language development. The family was advised to consider referral for CI candidacy evaluations.

with the development of speech perception, production, and language should be monitored regularly to determine whether the progress is adequate and age appropriate or whether an alternate

Fig. 33.4 Potential scenarios that can occur during CAEP testing. Scenario 1 shows absent unaided CAEPs at conversational levels, which

are present when aided, thus demonstrating aided benet. Scenario 2

shows absent unaided and aided responses, including the four possible explanations for this result.

Fig. 33.4 shows the possible scenarios that can occur. The ﬁrst

scenario shows absent unaided but present aided CAEP responses.

This demonstrates aided beneﬁt in the frequency band tested, and

the results can be used to assure the parents that their child is

“detecting” the sound at conversational level. Although this is not a test of thresholds or speech discrimination, parents can ﬁnd this

early management very reassuring. As the child grows, it will then

be possible to perform VRA (to assist in ﬁne tuning of the hearing

aid gain settings) and, eventually, formal measures of speech discrimination ability (to monitor development).

The second scenario shows that no response is seen at 65 dB

SPL in both the unaided and aided conditions. This can mean one

of four things. First, the infant is underﬁtted, so the clinician can

consider reestimating the hearing levels and adjusting the hearing aids accordingly before repeating the test. Second, the infant has a

severe to profound hearing loss, in which case ampliﬁcation is likely

to provide only limited access to the speech range in the same way as it would for a case with SNHL. Third, the infant may be one of those with an absent CAEP even when the sound is audible. Finally, based on previous studies on children with ANSD showing an association between absence of CAEPs and poor speech discrimination ability,49 it may be an indication that the infant has poor speech discrimination ability even though the sound is audible.

Pearl

Absent CAEPs do not necessarily mean the infant cannot hear the sound.

Case Study

Case AN1 presented in Chapter 28 shows how CAEPs and the PEACH diary were used to assist in the decision to refer for cochlear implant candidacy. The infant referred bilaterally on

mode of communication or alternate technology such as CI are

indicated. As many children with ANSD experience diculties

when listening in noisy situations, such as the classroom, clinicians should also consider frequency modulation (FM) or remote microphone (RM) systems. These can be used in isolation (e.g., in the case of a child with normal hearing sensitivity) or in addition to hearing aids or CIs.

33.3.3 Cochlear Implant

Studies investigating outcomes with cochlear implants in subjects with ANSD have, on the whole, been positive, although not all have had successful outcomes.53 The reasons

why outcomes have varied can be explained by dierences in the

underlying pathology. Individuals with peripheral sites of lesion (IHC, ribbon synapse, or terminal dendrites) are likely to have more successful outcomes than those with pathology involving spiral ganglion cells, axons, or hypoplasia of the cochlear nerve.

Implant Evoked Electrical ABR

Implant evoked electrical ABR (ImpEABR) testing has helped to provide objective evidence of pre- versus postsynaptic sites of lesion in children with ANSD and to predict children who are more likely to progress well with their CI. The testing technique is similar to standard ABR testing except that electrical pulses are delivered from the implant to elicit the response. Fig. 33.5

shows examples of ImpEABR results for three dierent children with ANSD. The ﬁrst set of results is from a child who suered signiﬁcant hypoxia at birth and shows present wave V responses

on all electrodes. The second set of traces on Fig. 33.5 is from

a child with late-onset ANSD due to riboavin transporter deﬁciency. The morphology and latency of the response are

inconsistent across the array and are in keeping with the neural pathology underlying this condition. The third set of results is from a child with hypoplastic cochlear nerves. In this case responses are seen only on two electrodes at the basal end of the

cochlea, suggesting that a small patch of cochlear nerve ﬁbers

are present and capable of responding to electrical stimuli. Recent research has shown that children who demonstrated clear ImpEABR waveforms demonstrated speech perception and receptive and expressive language scores similar to those of implanted SNHL children.

In addition to assessing speech detection, the child’s progress

51,52

352

33 Managing Infants and Children with Auditory Neuropathy Spectrum Disorder

Medical Imaging

Radiologic imaging of the temporal bones prior to cochlear implantation is important in determining whether there are any structural abnormalities that may impac t on the insertion of the CI array and to identify whether there is a reduction in the size of the cochlea r nerve that may prevent a complete sign al from the CI b eing transferred along the auditory brainstem pathways. Magnetic resonance imaging (MRI) using a cross-sectional/parasagittal view of t

he internal auditory meatus is recommended. This allows the radiologist to compare the diameter of the cochlear nerve relative to the facial nerve and the two branches of the vestibular nerve. Fig. 33.6 shows the parasagittal view on MRI for a child with a full complement of nerves in the bundle (left); a child with only two nerves (center); and a child with only one nerve (right). Birman

et al analyzed the results of 50 children with varying degrees of

abnormality of the cochlear nerve and reported higher scores on

tests of auditory perception for those with small but identiﬁable

nerves than for children where the nerve was either absent or fused with other nerves in the bundle.53 Other researchers have reported a similar pattern of results whereby the greater the degree of the abnormality, the more limited the outcomes with a CI.56 This information is useful when counseling families about realistic expectations following cochlear implantation and the likely need to integrate visual forms of communication such as sign language into the child’s early intervention program.

Fig. 33.5 Implant evoked electrical auditory brainstem responses (ImpEABRs) elicited from individual electrodes for a 22-channel Nucleus CI (Cochlear Ltd., Sydney, Australia). The responses for electrodes range from the apical end of the cochlea (e.22, top) to those at the basal end (e.1, bottom). The set of traces on the left are for a

child who suered signicant hypoxia who is likely to have auditory

neuropathy spectrum disorder (ANSD) due to damage of the inner hair cells (IHCs); in the center for a child with a neurologic condition

associated with riboavin transporter deciency; and on the right for

a child showing a hypoplastic cochlear nerve on magnetic resonance imaging (MRI).

Pearl

MRIs need to be performed with a parasagittal view to check for hypoplasia of the cochlear nerve in ANSD.

Mild-Moderate Hearing Loss

It has been reported that some children with ANSD who have a mild to moderate pure tone loss demonstrated speech recognition ability below that expected for their degree of hearing loss, even when wearing hearing aids that were optimally

ﬁtted.49 These children could potentially beneﬁt from cochlear

implantation. It is important for clinicians to include measures of speech discrimination or recognition ability when establishing an audiologic management plan and not exclude the option of cochlear implantation based on the pure tone thresholds alone if

discrimination/recognition is signiﬁcantly aected.

33.3.4 Family-Centered Multidisciplinary

Assessment and Rehabilitation

Family-centered early intervention (FCEI) enhances parental engagement in the management and monitoring process and can help to optimize outcomes for children with hearing loss. Clinicians are referred to the consensus statement describing the ten principles of FCEI published by Moeller et al,57 which describes the validated evidence base behind these principles and clinician behaviors that support them.

Fig. 33.6 Parasagittal magnetic resonance imaging (MRI) views of the nerve bundle within the internal auditory meatus. On the left, all four nerves (cochlear, facial, and two branches of the vestibular) are present in the bundle. In the center only two nerves are present, and on the right only one nerve.

As mentioned previously, the majority of infants diagnosed with ANSD have experienced a very stormy start to life. As a result, parents can feel overwhelmed with perinatal health issues even before the diagnosis of ANSD is made.58 Parents have iden-

tiﬁed additional stressors associated with the diagnosis of ANSD, including conicting information from the professionals working

with them and uncertainty around the prognosis for their child.59 Parents will inevitably search on the Internet for further information, which can further exacerbate the situation if they are not able to critically evaluate the information presented. It is particularly important for clinicians to spend time with the family to gain an understanding of their current level of knowledge and establish the parents’ goals and preferences for their child. In addition, families need to be provided with comprehensive information on all the options available, including any advantages, disadvantages, and uncertainties around each one.

Pearl

Clinicians need to provide parents with good-quality, evidence-based information to reduce the parental stress associ-

ated with the provision of conicting messages.

353

IV Educational and Clinical Management of Hearing Loss in Children

Ching et al8 reported that 30% of the ANSD population in their study were found to have at least one other disability in addition to hearing loss, including developmental delay, physical disabilities such as cerebral palsy, and autism spectrum disorder. For this reason it is not unusual for a child to need to access input from a number of other professionals including physiotherapy, occupational therapy, and ear, nose and throat specialists. Audiologists need to work closely with speech and language therapists, in particular, to obtain formal information about each child’s progress with speech and language development.

33.4 Conclusion

The prevalence of ANSD is estimated to be around 10% of all

children diagnosed with permanent hearing loss. Appropriate test procedures need to be used for the accurate diagnosis of ANSD using ABR, and repeat testing performed to check for improvement in ABR morphology over time. Clinicians should refer older children for ABR testing if they demonstrated poorer than expected speech discrimination/recognition ability or pre-

sented with OAEs despite signiﬁcant hearing loss, so that cases of late-onset ANSD can be identiﬁed.

The majority of children with ANSD have spent time in the NICU and have medically related risk factors; however, genetic

causes have been identiﬁed in approximately 40% of cases. The dierent underlying causes, and associated sites of lesion, are

likely to explain the variations seen in pure tone sensitivity, speech discrimination/recognition ability, and outcomes follow-

ing ampliﬁcation or cochlear implantation.

The use of CAEPs together with functional auditory assessments, such as the PEACH, enable us to reach decisions about the

need to provide ampliﬁcation and to proceed with the early ﬁtting

of hearing aids in infants with ANSD. In addition, these methods

enable us to evaluate the eectiveness of ampliﬁcation and identify infants who would beneﬁt from a signing mode of communi-

cation or referral for CI candidacy evaluations. Where available,

speech discrimination/recognition ability with ampliﬁcation

should also be considered. In the workup for cochlear implantation, an MRI scan using a parasagittal view should be arranged to check for hypoplasia of the cochlear nerve. By using a combination of objective testing and parent ratings of auditory behavior in real life, clinicians are now in a better position than before to provide early intervention to infants diagnosed with ANSD.

33.5 Acknowledgment

We thank Vicky Zhang for her assistance with preparation of the manuscript.

Discussion Questions

1. What are some of the medical risk factors associated with

ANSD?

2. What are some of the genetic mutations associated with late-onset ANSD?

3. What pattern of results are you likely to see on CAEP and PEACH assessments for an infant who has inadequate

amplication?

4. What are some of the reasons why some children with ANSD perform better with a CI than others?

5. Which multidisciplinary team members are likely to need to be involved for a child with ANSD and cerebral palsy?

6. Where do parents potentially source information on ANSD

that can provide conicting information and management

recommendations?

References

[1] Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI. Auditory neuropathy. Brain

1996;119(Pt 3):741–753

[2] Berlin CI, Hood L, Rose K. On renaming auditory neuropathy as auditory dys-syn-

chrony. Audiology Today. 2001;13(6):15–17

[3] Berlin CI, Hood LJ, Cecola RP, Jackson DF, Szabo P. Does type I aerent neuron

dysfunction reveal itself through lack of eerent suppression? Hear Res 1993;65(1-2):40–50

[4] Rapin I, Gravel J. “Auditory neuropathy”: physiologic and pathologic evi-

dence calls for more diagnostic speciﬁcity. Int J Pediatr Otorhinolaryngol 2003;67(7):707–728

[5] Amatuzzi M, Liberman MC, Northrop C. Selective inner hair cell loss in prematu-

rity: a temporal bone study of infants from a neonatal intensive care unit. J Assoc Res Otolaryngol 2011;12(5):595–604

[6] Hayes D, Sininger Y, Northern J. Guidelines for identiﬁcation and management of

infants and young children with auditory neuropathy spectrum disorder. Paper

presented at: Proceedings of the Guidelines Development Conference at NHS; June 19–21, 2008; Cernobbio, Italy

[7] Berlin CI, Hood LJ, Morlet T, et 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

[8] Ching TYC, Day J, Dillon H, et al. Impact of the presence of auditory neuropathy

spectrum disorder (ANSD) on outcomes of children at three years of age. Int J Audiol 2013;52(Suppl 2):S55–S64

[9] Xoinis K, Weirather Y, Mavoori H, Shaha SH, Iwamoto LM. Extremely low

birth weight infants are at high risk for auditory neuropathy. J Perinatol

2007;27(11):718–723

[10] Bielecki I, Horbulewicz A, Wolan T. Prevalence and risk factors for auditory neu-

ropathy spectrum disorder in a screened newborn population at risk for hearing loss. Int J Pediatr Otorhinolaryngol 2012;76(11):1668–1670

[11] Sininger YS. Identiﬁcation of auditory neuropathy in infants and children. Semin

Hear 2002;23(3):193–200

[12] Rodríguez-Ballesteros M, Reynoso R, Olarte M, et al. A multicenter study on the

prevalence and spectrum of mutations in the otoferlin gene (OTOF) in subjects with nonsyndromic hearing impairment and auditory neuropathy. Hum Mutat

2008;29(6):823–831

[13] Santarelli R, Cama E, Scimemi P, Dal Monte E, Genovese E, Arslan E. Audio-

logical and electrocochleography ﬁndings in hearing-impaired children with connexin 26 mutations and otoacoustic emissions. Eur Arch Otorhinolaryngol 2008;265(1):43–51

354

33 Managing Infants and Children with Auditory Neuropathy Spectrum Disorder

[14] Trevisi P, Ciorba A, Aimoni C, Bovo R, Martini A. Outcomes of long-term

audiological rehabilitation in CHARGE syndrome. Acta Otorhinolaryngol Ital 2016;36(3):206–214

[15] Jutras B, Russell LJ, Hurteau AM, Chapdelaine M. Auditory neuropathy in siblings

with Waardenburg’s syndrome. Int J Pediatr Otorhinolaryngol 2003;67(10):1133– 1142

[16] Rance G, Corben L, Delatycki M. Auditory processing deﬁcits in children with

Friedreich ataxia. J Child Neurol 2012;27(9):1197–1203

[17] Ceranić B, Luxon LM. Progressive auditory neuropathy in patients with Leber’s

hereditary optic neuropathy. J Neurol Neurosurg Psychiatry 2004;75(4):626–630

[18] Rance G, Ryan MM, Bayliss K, Gill K, O’Sullivan C, Whitechurch M. Auditory

function in children with Charcot-Marie-Tooth disease. Brain 2012;135(Pt

5):1412–1422

[19] Menezes MP, O’Brien K, Hill M, et al. Auditory neuropathy in Brown-Vialetto-Van

Laere syndrome due to riboavin transporter RFVT2 deﬁciency. Dev Med Child Neurol 2016;58(8):848–854

[20] Bahmad F Jr, Merchant SN, Nadol JB Jr, Tranebjaerg L. Otopathology in

Mohr-Tranebjaerg syndrome. Laryngoscope 2007;117(7):1202–1208

[21] Attias J, Raveh E, Aizer-Dannon A, Bloch-Mimouni A, Fattal-Valevski A. Auditory

system dysfunction due to infantile thiamine deﬁciency: long-term auditory

sequelae. Audiol Neurootol 2012;17(5):309–320

[22] Marlin S, Feldmann D, Nguyen Y, et al. Temperature-sensitive auditory neuropa-

thy associated with an otoferlin mutation: Deafening fever! Biochem Biophys Res Commun 2010;394(3):737–742

[23] Bidichandani SI, Delatycki MB. Friedreich ataxia. GeneReviews. https://www.

ncbi.nlm.nih.gov/books/NBK1281/. Updated June 2017. Accessed January 9, 2018

[24] Delettre-Cribaillet C, Hamel C, Lenaers G. Optic atrophy type 1. GeneReviews.

https://www.ncbi.nlm.nih.gov/books/NBK1248/. Updated November 12, 2015. Accessed January 9, 2018

[25] Colomban C, Micallef J, Lefebvre M-N, et al. Clinical spectrum and gender

dierences in a large cohort of Charcot-Marie-Tooth type 1A patients. J Neurol Sci 2014;336(1-2):155–160

[26] Sininger Y, Oba S. Patients with auditory neuropathy: who are they and what can

they hear? In: Sininger Y, Starr A, eds. Auditory neuropathy: A new perspective

on hearing disorders. San Diego, CA: Singular; 2001:15–35

[27] Rance G, Beer DE, Cone-Wesson B, et al. Clinical ﬁndings for a group of infants

and young children with auditory neuropathy. Ear Hear 1999;20(3):238–252

[28] Rance G. Auditory neuropathy/dys-synchrony and its perceptual consequences.

Trends Amplif 2005;9(1):1–43

[29] Zeng FG, Kong YY, Michalewski HJ, Starr A. Perceptual consequences of disrupted

auditory nerve activity. J Neurophysiol 2005;93(6):3050–3063

[30] Walker E, McCreery R, Spratford M, Roush P. Children with auditory neuropathy

spectrum disorder ﬁtted with hearing aids applying the American Academy of Audiology Pediatric Ampliﬁcation Guideline: Current practice and outcomes. J Am Acad Audiol 2016;27(3):204–218

[31] Roush P, Frymark T, Venediktov R, Wang B. Audiologic management of auditory

neuropathy spectrum disorder in children: a systematic review of the literature. Am J Audiol 2011;20(2):159–170

[32] 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 Otorhinolaringol 2015;81(1):85–96

[33] Humphriss R, Hall A, Maddocks J, Macleod J, Sawaya K, Midgley E. Does cochlear

implantation improve speech recognition in children with auditory neuropathy spectrum disorder? A systematic review. Int J Audiol 2013;52(7):442–454

[34] Soares I, Menezes P, Carnaúba AT, de Andrade KC, Lins OG. Study of cochle-

ar microphonic potentials in auditory neuropathy. Braz J Otorhinolaringol

2016;82(6):722–736

[35] Berlin CI, Hood LJ, Morlet T, et al. Absent or elevated middle ear muscle reexes

in the presence of normal otoacoustic emissions: a universal ﬁnding in 136 cases of auditory neuropathy/dys-synchrony. J Am Acad Audiol 2005;16(8):546–553

[36] Kei J. Acoustic stapedial reexes in healthy neonates: normative data and test-re-

test reliability. J Am Acad Audiol 2012;23(1):46–56

[37] Psarommatis I, Riga M, Douros K, et al. Transient infantile auditory neuropathy

and its clinical implications. Int J Pediatr Otorhinolaryngol 2006;70(9):1629– 1637

[38] Madden C, Rutter M, Hilbert L, Greinwald JH Jr, Choo DI. Clinical and audi-

ological features in auditory neuropathy. Arch Otolaryngol Head Neck Surg 2002;128(9):1026–1030

[39] Moore JMP, Thompson G, Folsom RCP. Auditory responsiveness of premature in-

fants utilizing visual reinforcement audiometry (VRA). Ear Hear 1992;13(3):187–

194

[40]

Punch S, Van Dun B, King A, Carter L, Pearce W. Clinical experience of using

cortical auditory evoked potentials in the treatment of infant hearing loss in Australia. Semin Hear 2016;37(1):36–52

[41] Kushnerenko E, Ceponiene R, Balan P, Fellman V, Huotilaine M, Näätäne R.

Maturation of the auditory event-related potentials during the ﬁrst year of life. Neuroreport 2002;13(1):47–51

[42] Gardner-Berry K, Chang H, Ching TYC, Hou S. Detection rates of cortical

auditory evoked potentials at dierent sensation levels in infants with sensory/

neural hearing loss and auditory neuropathy spectrum disorder. Semin Hear

2016;37(1):53–61

[43] Van Dun B, Carter L, Dillon H. Sensitivity of cortical auditory evoked potential

detection for hearing-impaired infants in response to short speech sounds.

Audiology Res 2012;2(1):65–76

[44] Ching TY, Hill M. The Parents’ Evaluation of Aural/oral Performance of Children

(PEACH) scale: Normative data. J Am Acad Audiol 2007;18(3):220–235

[45] Bagatto MP, Scollie SD. Validation of the Parents’ Evaluation of Aural/Oral Perfor-

mance of Children (PEACH) Rating Scale. J Am Acad Audiol 2013;24(2):121–125

Gardner-Berry K, Purdy SC, Ching TY, Dillon H. The audiological journey and

[46]

early outcomes of twelve infants with auditory neuropathy spectrum disorder from birth to two years of age. Int J Audiol 2015;54(8):524–535

[47] Golding M, Pearce W, Seymour J, Cooper A, Ching T, Dillon H. The relationship

between obligatory cortical auditory evoked potentials (CAEPs) and functional measures in young infants. J Am Acad Audiol 2007;18(2):117–125

[48] Sharma A, Cardon G, Henion K, Roland P. Cortical maturation and behavioral

outcomes in children with auditory neuropathy spectrum disorder. Int J Audiol 2011;50(2):98–106

[49] Rance G, Cone-Wesson B, Wunderlich J, Dowell R. Speech perception and

cortical event related potentials in children with auditory neuropathy. Ear Hear 2002;23(3):239–253

[50] Chang HW, Dillon H, Carter L, van Dun B, Young ST. The relationship between

cortical auditory evoked potential (CAEP) detection and estimated audibility in infants with sensorineural hearing loss. Int J Audiol 2012;51(9):663–670

[51] Leigh J, Rance G, Dettman S, Dowell R. Cochlear implant outcomes for children

with auditory neuropathy spectrum disorder. SIG 9 Perspect Hear Hear Disord Child 2009;19(2):75–84

[52] Teagle HFB, Roush PA, Woodard JS, et al. Cochlear implantation in children with

auditory neuropathy spectrum disorder. Ear Hear 2010;31(3):325–335

[53] Birman CS, Powell HR, Gibson WP, Elliott EJ. Cochlear implant outcomes in

cochlea nerve aplasia and hypoplasia. Otol Neurotol 2016;37(5):438–445

[54] Rance G, Starr A. Pathophysiological mechanisms and functional hearing conse-

quences of auditory neuropathy. Brain 2015;138(Pt 11):3141–3158

[55]

Rance G, Barker EJ. 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

[56] Jeong SW, Kim LS. Auditory neuropathy spectrum disorder: predictive value of

radiologic studies and electrophysiologic tests on cochlear implant outcomes and

its radiologic classiﬁcation. Acta Otolaryngol 2013;133(7):714–721

[57] Moeller MP, Carr G, Seaver L, Stredler-Brown A, Holzinger D. Best practices in

family-centered early intervention for children who are deaf or hard of hearing: an international consensus statement. J Deaf Stud Deaf Educ 2013;18(4):429–445

[58] 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):186–193

[59] Uus K, Young A, Day M. Parents’ perspectives on the dilemmas with intervention

for infants with auditory neuropathy spectrum disorder: a qualitative study. Int J Audiol 2015;54(8):552–558

355

34 Working with Multicultural Families of Young Children with Hearing Loss

34 Working with Multicultural Families of Young Children

with Hearing Loss

Ellen A. Rhoades

Summary

Immigration trends across the 21st century signiﬁcantly inuence services provided by audiologists and other auditory-based practitioners. Although cultural diversity is currently the Americ an norm, fundament al inequalities remain, par ticularly in the delivery of audiologic services to families and their children of color or of low-income status. Pediatric clinicians strive to overcome personal biases and learn about each family’s culture

in order to develop eective alliances with parents. Language,

one indicator of diversity, can bridge cultural divides; hence,

appropriate use of qualiﬁed interpreters is critical. Bilingualism, deﬁned as proﬁcient conversational uency in two languages, is

a reality for increasingly more American children with hearing

loss. Given parent commitment and consistent situational boundaries, many children with hearing loss eectively learn

to speak the family’s home/minority language as well as spoken

English. Bimodal bilingualism, involving the use of two dierent

sensory-dependent communication systems, can be more challenging for children with hearing loss, particularly if the parent’s

goal is to have their child eectively learn to understand and use

a spoken language with ease.

Keywords

cultural diversity, biases, expectations, multicultural competence, family system, introspection, mimicry, interpreters, bilingualism

Key Points

Implicit biases negatively aect the developmental outcomes

•

of minority children. Complex families, including children of color or of low-income

•

status, are underserved and underrepresented. Audiologists, providing culturally responsive services, learn

•

about each child’s family culture.

Audiologists strive to develop an eective alliance with all

•

parents. Each child is actively encouraged to learn the family’s heri-

•

tage language. With parent commitment and consistent situational boundar-

•

ies, many children with hearing loss can speak two languages.

34.1 Culture and Bias in America

Immigration trends across the 21st century signiﬁcantly inuence services provided by audiologists and other audi-

tory-based practitioners. Although diversity is the American

norm, fundamental inequalities remain. Language is only one

indicator of diversity; understanding cultural dierences as

well as communication modes is also important. Bilingualism,

deﬁned as proﬁcient conversational uency in two languages, is

a reality for increasingly more American children. This chapter

briey reviews how multiculturalism challenges audiologists and how multiculturalism is manifested in language-speci

evelopmental patterns of children with hearing loss.

Culture, typically not in our consciousness, is uid in that it is

always subject to change. Cultural characteristics include country of origin, race, religion, socioeconomic status, language, sexual orientation, and value system as well as family structure and relationships. As reviewed elsewhere, culture also includes one’s problem-solving capacities and sense of wholeness (e.g., existing disabilities). because it embraces behaviors that result in power/privilege

dierentiations, resistances, and innovations. While culture enables people to have a sense of belonging (in-group aliations/

favoritisms), culture may also facilitate negative attitudes toward out-group members.

Since the founding of the United States, waves of immi-

gration have been reshaping America. Immigrants and their

present-day U.S. population. Marked family-related changes have occurred with respect to American women in the labor force, living arrangements, social expectations, and cultural values. Consequently, there is no dominant form against which families can now be measured or judged; family diversity is the 21st-century norm.

Stereotypes drive biases that cause unjustiﬁed discrimination

and harmful behavior. Even when implicit, individual and societal prejudices deleteriously aect self-perceptions as well as educational and developmental outcomes of children, resulting in a lower quality of life for their families.

limited knowledge of cultural dierences; hence, many people

feel threatened by change or are fearful or disrespectful of cultural

dierences and minority groups.

For example, Hispanic Americans, comprising approximately

one-ﬁfth of America’s population,4 may share a single language,

but their dialects, lifestyles, family structures, religions, and skin colors vary. Thus, it is important not to view Hispanic Americans

as a monolithic, uniﬁed group.5 It is essential to oer options in

the delivery of audiologic services that accommodate and honor the cultural dierences of Hispanic Americans and also to personalize their learning.

A similar example often occurs when typically hearing parents

ﬁrst hear the diagnostic label of deafness assigned to their child.

Perceptions of that label, often based on erroneous stereotypes and resultant stigma with lowered expectations, may cause some parents to react in despair, and other parents to rely on varied

coping strategies. Diverse parental perceptions have signiﬁcant

Culture, then, is multifaceted and conict-laden

1,2,3

Many Americans have

ﬁc

1,6

357

IV Educational and Clinical Management of Hearing Loss in Children

implications for audiologists who rst make concerted eorts to educate themselves about cultural dierences and then

educate parents about hearing loss, hearing devices, and their

ramiﬁcations.

Cultural characteristics of each family have direct relevance to

their understanding of speciﬁc domains of child development. Such characteristics inuence adult–child interactions, the way

children are raised and educated, and parents’ long-term goals for their children. Moreover, the ways that children play, socialize, learn, solve problems, communicate, and perceive the world are culturally grounded.1 When audiologists better understand cultural dierences, miscommunications and under-/overrepresentation of minority students receiving audiologic services are likely to decrease.

1,7,8

34.2 Underserved and Underrepresented Families and Their Children

There are profound social inequalities in America. Racism and

poverty prevail, particularly aecting single-parent households that include children under 6 years of age.1 Approximately one-third of America’s children identiﬁed with hearing loss are Hispanic, and approximately two-ﬁfths of America’s children

with hearing loss are from low-income families.9 Some parents are unemployed or in poor health; some children are abused or

have many special needs. Poverty is selective in how it aects

the delivery of services, child learning, and developmental outcomes as well as parental attitudes toward inclusion and special education.

A review of the literature1 shows that children of color or from low socioeconomic status families are (1) at greater risk of having hearing loss or delays in communication and other behaviors;

(2) less likely to be early identiﬁed and less likely to receive

audiologic/early intervention services; (3) more likely to have the diagnosis of hearing loss confer stress on all family members;

(4) less likely to be eectively ﬁtted with hearing aids or to make optimum use of either hearing aids or cochlear implants; (5) more

likely to have higher rates of postoperative complications, worse follow-up compliance, and lower rates of bilateral implantation;

and (6) more likely to report poorer health status, use Medicaid,

live in single-parent households, and reside below the poverty level. Restated, the following characteristics are associated with fewer or less eective audiologic or other early intervention services: (1) the color of the child’s skin, (2) the low socioeconomic status of the child’s family, and (3) the child’s home language not being English. treatment inequities.

Despite the trend toward multicultural families in American schools, audiologists and other practitioners serving families and their children with hearing loss remain overwhelmingly white English speakers from traditional Anglo-Western culture.

Although some eorts have been made to culturally sensitize

clinicians since the last decade of the 20th century, the reality is that diversity continues to present considerable challenges for 21st-century audiologists.

10,11,12,13

7,14

Audiologists must make assertive eorts to avoid

1,15

34.3 Meeting the Challenges of Multicultural Audiology

Becoming open to diversity is a ﬁrst imperative step for audiologists. In the quest to understand the meaning of unearned white privilege and subtle racism,16 Caucasian audiologists are encouraged to develop a willingness and interest in exploring other cultures and interacting with people of color. Because interracial

contact is eective in reducing inherent biases and facilitating

White Empathy,16 it is important to befriend ﬁnancially impoverished persons and to understand their daily household routines as well as their need and desire for such resources as books, toys, and digital materials.

The child cannot be appropriately served when the child’s culture (i.e., social context) is poorly understood. As reviewed elsewhere by the present author,17 understanding the family system enables audiologists to view the strengths of each culture as

cultural capital; a deﬁcit perspective is untenable. The following

sections discuss three steps that audiologists can take to develop a minimal level of competency in serving diverse populations: engage in introspection; adopt a family-systems approach; and make an active commitment to diversity.

34.3.1 Step 1: Engage in Introspection

Exploring one’s own beliefs and biases is an imperative.16 The

initial step toward developing a minimal level of competency in

diversity necessitates that audiologists ﬁrst look within to better

understand themselves and to strive toward becoming nonjudgmental with all individuals from minority groups. Overcoming implicit racial biases and cultural attitudes requires that we

ﬁrst understand personal attitudes for what they are. Diversity-

sensitive counseling challenges one to confront personal fears, myths, stereotypes, faulty assumptions, and negative attitudes

and to redeﬁne whiteness and the Eurocentric perspective.

Awareness of one’s own ignorance and subconscious beliefs can, in turn, cause vigilance and conscious refusal to act on them.1 An ongoing concerted eort, facilitated by knowledge and understanding, develops tolerance.

Beyond self-analysis, interracial contact can be highly eective

for reducing inherent biases. Audiologists are encouraged to actively seek minority individuals to inquire about issues that directly bear on inherent biases. For example, to understand

others’ experiences better, ask people of dierent races and

cultures what racism means to them. Shadow a minority child

for at least a school day. Attend ﬁlms and read books authored

by minority persons about their cultures. Attend local cultural festivals and other celebratory events as well as those places of worship frequented by minority families.1 Become an advocate for social justice.

Pitfall

An audiologist’s inherent biases and cultural misperceptions can

negatively aect the delivery of services.

358

34 Working with Multicultural Families of Young Children with Hearing Loss

34.3.2 Step 2: Adopt a Family-Systems Approach

The second step toward attaining a minimal level of competency in understanding diversity is for audiologists to develop a family-systems perspective. A review of the literature17 shows family-systems theory has to do with the complex interrelationships and negotiations of each family member; an invisible web of complementary demands and expectations regulates family and individual behavior. The family provides the primary social context in which to view the young child.

Core terminology pertaining to the dynamics of family relationships needs to be understood. When audiologists implement simple family-supportive strategies, optimizing the child’s poten-

tial and minimizing the secondary negative eects of hearing loss

are more likely to occur.17 Alternately stated, when audiologists demonstrate counseling competence during their interactions with caregivers, positive change can be facilitated for the child.

An eective counseling strategy that audiologists can incorporate into communicating with a child’s caregivers is that of mimicry. Mimicry occurs when an audiologist, while addressing a family, selectively and appropriately adopts that family’s language level, mannerisms, communicative style, speech patterns, and familial colloquialisms; the audiologist conforms to the family’s

aective range. Also known as mimesis, mimicry tends to facilitate mutual rapport. Developing trustworthy audiologist–parent alliances beneﬁts children with hearing loss by identifying par-

ents as experts on their children.

Financially impoverished families are likely to be driven by survival above and beyond any special needs; they typically need a stronger and wider supportive network than do other families.1 Given that multicultural families are part of this mix, a huge variety of childrearing practices presents to audiologists, who must then seek an even wider base of practitioner and community

collaboration. When ﬁnancially feasible, audiologic services for

at-risk families must be made more frequent, more intensive, and

more convenient—without sacriﬁcing the quality of audiologic

services being delivered. It is important that service providers

reect greater diversity of disciplines and cultural backgrounds. More attention is given to facilitating parental self-ecacy; this

includes expanding the parental knowledge base, providing parents with a culturally similar peer support network, and improv-

ing parent–child communications. The many long-term negative eects of ﬁnancial impoverishment on children can be mitigated

when the support of extended family and peers is enlisted.

34.3.3 Step 3: Make a Commitment to

Diversity

Beyond developing an awareness of and sensitivity to cultural

dierences, eective audiologists demonstrate cultural responsiveness. Due to intercultural dierences in learning assump-

tions and principles, teaching strategies that promote learning in children from the Anglo-Western culture do not necessarily result in greater gains among children from minority cultures. Viewpoints as to whether audiologists are guardians of knowledge or facilitators of learning are culturally based.

As soon as all minority groups and languages represented in

a caseload are identiﬁed, consultants and trained peer advocates

from minority groups are secured. Audiologists strive to be nonjudgmental and show empathy when listening to parents by trying t

o see the world as they see it. Audiologists also strive to develop awareness of communication obstacles that are often culturally controlled. For example, before informing parents of a child’s hear-

ing loss, culturally responsive audiologists ﬁrst make small talk with caregivers to develop rapport and impart conﬁdence and then

inquire about each family’s culture and belief system regarding disabilities.

aect the choice of words used in speech perception assessments (e.g., some immigrant children might not associate “football” with

an image of American football or associate an image of the game

they have always known as football with the word “soccer”).

Toward that end, parents can be asked a series of questions that provide audiologists with a framework of information relevant to audiologic diagnosis and treatment. Such questions involve ascertaining the family’s cultural and linguistic status as well as identifying the communicative style within the family system. The audiologist who does not speak the child’s home language can

observe the family’s communicative style in the oce or waiting room, noting uency and variety of communication skills used by

child and caregivers. During this questioning process, regardless of whether or not the services of an interpreter or translator are available, the audiologist demonstrates warmth while initially avoiding prolonged eye or physical contact. In a trustworthy, compassionate, and personable manner, both closed-ended and

open-ended questions are asked of caregivers, using sucient

pauses to allow time to think and respond.

Audiologists providing culturally relevant services facilitate multicultural knowledge among all families, including those who are Anglo-Western. In addition to promoting interracial contact, multiculturally competent audiologists include the conscious use of marketing/educational collateral and picture books that positively feature children of the same ancestry as the family. Families and their children are more likely to thrive when audiologists demonstrate cross-cultural competence in thinking, feeling, and behaving. Ultimately, audiologists understand that the goal of becoming sensitive and knowledgeable across many cultures is a lifelong quest.

1,18

Learning about each child’s family background can

34.4 Interpreters for Caregiver-

Audiologist Communications

Some Anglo practitioners do not feel competent when working with linguistic minorities.1 Although language interpreters are used more frequently than in past decades, they are used with

less conﬁdence. If children with hearing loss are to be optimally served, eective, ongoing communication between their parents

and audiologists is critical.

The linguistic diversity caused by the demographic shift of the past two decades results in daunting challenges.18 Although family members are often used as interpreters when professional interpreters are unavailable, relying on children in this capacity is not a good idea; family biases and relationships can negatively

aect the audiologic session.

When enlisting the assistance of a professional interpreter, audiologists try to use the same one for the same family so that an

359

IV Educational and Clinical Management of Hearing Loss in Children

ongoing relationship can be developed. Interpreters are selected for a variety of attributes including honesty, reliability, neutrality, conﬁdentiality, cultural proﬁciency, and consistency in availability. These attributes are in addition to the highly critical attribute

of bilingual proﬁciency. Ideally, interpreters are of the same

ethnicity as that of the family and are familiar with the family’s heritage culture; the goal is cultural and language concordance.

Before using interpretive services, the audiologist meets with

the interpreter to reiterate the need for conﬁdentiality, to develop

rapport, and to set some ground rules such as the provision of exact translations for parents. Interpreters are advised to avoid gestures and other cues that could unfairly assist the child during administration of audiologic tests. Audiologists spend time orienting interpreters to the subject matter, procedures, and goals of audiologic services and to review all technical terms. These consultations, completed prior to meeting with the family, also involve discussions on the relevance of materials and language expressions to be used.

Interpreters are asked to speak in the ﬁrst person and to carry

a notepad so that they can take notes as needed. Interpreters are encouraged to ask questions when unsure of a term or phrase that was used. Interpreters are also encouraged to project clearly and to mirror the audiologist’s overall tone and vocal stresses. Finally, interpreters are advised to refrain from engaging in tangent dialogues with families and from attempting to serve as advocates or

mediators in the audiologist–parent dialogue.

During each actual interpreted session, the audiologist faces parents while talking to them; the interpreter is asked to sit next to and slightly behind the audiologist, so that parents are more likely to make eye contact with the audiologist. Audiologists make

concerted eorts to avoid using slang, idioms, and metaphors

while casually monitoring the process of interpreting, particularly

watching that interpreters do not appear to be oering their own

thoughts to parents.

Subsequent to each meeting with child or family, the audiol-

ogist has a debrieﬁng session with the interpreter. The primary

purpose of each postmeeting session is to ensure that the interpreter’s perceptions are shared with the audiologist. The audiologist reviews notes and progress, discussing any interpreting issues

with the interpreter. In short, the time and eort expended by audiologists in learning how to use interpreters eectively can reap great beneﬁts for families and their children.

and social interaction skills. In contrast to the acquisition of

signed language, spoken language is based on, ﬁrst, hearing the

auditory information. Beyond acoustic accessibility, the child must develop listening skills that, in turn, enable comprehension of spoken words. Listening necessitates intentionality that is manifested by the child; this is displayed as eortful, purposeful listening and auditory attentional focus. spoken is then typically dependent on the quality and quantity of sound/auditory information received and perceived by the child. In other words, competent audiologists set the stage for the acquisition of spoken language by making auditory information

available to the child’s brain through the use of ampliﬁcation

technology.

Speciﬁcally pertaining to children with signiﬁcant hearing loss,

scoping and systematic reviews yield inconclusive ﬁndings as to

whether the child’s home language should be spoken or signed. Regardless, it is neurobiologically uncontestable that the brains of

children who rely on a signed language dier signiﬁcantly from

those of children relying on a spoken language. ing are other data-driven insights:

1. Early auditory exposure to the mother tongue is critical for the development of speech perception.

2. Some children with signiﬁcant hearing loss, as a result of inten- sive auditory-based early intervention, develop auditory domi- nance (i.e., for them, auditory input has a privileged processing status).

3. Psychological research demonstrates that high expectations of child performance are often realized as truths.

Moreover, studies show that children with hearing loss who

were more eective in understanding and using spoken language

demonstrated improved developmental outcomes relative to those children with hearing loss whose knowledge and use of spoken language was poorer.

The choice of signed or spoken language for children with hearing loss remains a controversial issue among audiologists and other practitioners. the choice is not theirs to make. Parents’ preference for their child’s home language is to be respected and honored by all clinicians.

The parental decision-making process involves dierent stages

supporting audiologists’ data-driven input. In short, the child’s primary caregivers select the language to be used at home, and this becomes the child’s heritage language regardless of whether it is spoken or signed, English or another spoken language.

27,28

29,30,31,32

Nevertheless, as reviewed by Porter,33

How language is

23,24,25,26

The follow-

21,22

Pearl

34.6 Learning a Second Spoken

Language is a cultural characteristic; diversity characterizes 21st-century America.

On a global level, speaking two languages (bilingualism) is the rule rather than the exception. All languages are equal; that is, no one spoken language is harder for children to learn than

34.5 Language of Choice: Signed or Spoken

Language, a culturally based activity, serves as an anchor for family cohesion while facilitating the child’s literacy, learning,

another.35 A review of studies19 involving bilingualism shows bilingualism to be a treasured asset for the child and society by conferring advantages involving improved learning capacities.

language. When parents attempt to communicate with their

360

Language

The home or heritage language should be the child’s native

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