Pediatrics: What's Old, What's New?

1 Pediatrics: Whats Old, Whats New? Alison M. Grimes, AuD Director, Audiology and Newborn Hearing UCLA Health 3 Whats Old (Foundational Principles that Will Never Change) Early diagnosis matters And

protocols matter Counseling matters Amplification that provides maximal audibility matters And there is only ONE way to ensure maximal audibility Early intervention matters Parents matter And are the most critical key in Early Intervention success

Whats New? JCIH (almost) EHDI, 2016Most Recent Data 90% of infants are screened 1.7% not-pass outcome 9.7% ultimately diagnosed with hearing loss 76% of babies with hearing loss diagnosed before 3 months of age 25% Lost-to-Follow-Up/Lost-to-Documentation

What Else is New? Bilingual children with bilateral hearing loss are at increased risk for poor expressive and expressive language development A particularly UCSF vulnerable population 2018 Parents refusing many/all services in the Nursery, including NBHS

8 California Geography is a Problem Geographic distribution of pediatric diagnostic audiologists in California is a real problem CCS audiologists serving hearing impaired infants using best practices are concentrated in a few key areas But there are too many CCS providers? Tele-practice needed but significant obstacles exist Pediatric Audiology and Amplification

One area that is ours alone Right? Or is it? Will third party plans make the distinction? What does it mean to be a contracted provider? 10 Hearing Aid Breakthrough in WSJ 8/26/17 Over-the-Counter hearing aids Will be great for consumers Including children?

What Happens if Children Get OTC Hearing Aids? Can they? (their parents can) Will they? If funding is a concern What will the outcome be? Over- or under-amplification?

Delayed referral for EI? Speech therapy? Delayed referral for CI? The law does not prohibit their use by individuals under the age of 18 OTC devices are intended for adults Nothing prevents parents from purchasing Parents Must Obtain Hearing Aids for their Child Wealthy? No problem Low-income? No problem (thank you CCS)

Middle income? No coverage! Mandate for insurance coverage for children has failed in CA government 3 times Another effort underway now 14 How will Parents Select/Fit an OTC Device for their Child?

Audiogram? What about RECD? How does that sound? What about Auditory Neuropathy? How to accommodate conductive component? How will parents even determine that childs hearing fluctuates with MEE? 15

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a restricted stimulus bandwidth can negatively affect the perception of /s/ and /z/ spoken by female talkers. Given the importance of these phonemes in the English language and the tendency of early caregivers to be female, an inability to perceive these sounds correctly may have a negative impact on both phonological and morphological development. 23 Audiology Practices Changing Corporate-owned practices Physician-owned practices

Out-sourcing cochlear implant services to CI companies Reimbursement is so poor for audiology services post-implant surgery CI-company run center within Audiology Clinic Only surgery is profitable, not audiology services Is pediatric audiology and treatment immune to these trends?

25 Hearing Aid Third-Party Administrators How does this work for kids? More plans use 3PA for hearing aid benefits Including pediatric hearing aids Whats our responsibility to work with or not work with these hearing aids? Legally? Ethically?

26 What Can We Do to Continue to Own Pediatric Audiology? (ASHA says hearing testing is in the SOP of SLP) Practice according to best-practices and standards of care Support and work in NBHS, EHDI, public school programs Be more than the diagnosing, hearing aid-dispensing audiologist

Collaborate with parents, EHDI professionals, teachers Research, advocacy and education 27 But No One Else Really Wants Pediatric Audiology Time-consuming Charting and paperwork

Frequent re-evaluations that may not be covered by insurance Expensive diagnostic equipment Higher rate of cancellations/no-shows? Need for test-assistant Emotionally-challenging Poor reimbursement Increased possibility of malpractice lawsuit?

28 Hearing Loss or Hearing Level? One of many current controversies 29 Whats a Hearing Loss in an Infant? Everything Mild Minimal Unilateral Transient/Fluctuating

Middle Ear Effusion for any period of time Auditory Neuropathy Progressive Central/cortical 30 Is Auditory Deprivation is a Hearing Loss? The infant who isnt spoken to The infant who is raised in a chronically noisy environment The infant who is raised by a TV

31 Infants WANT to Hear and Develop Speech and Language From birth, infants prefer their mothers voices over others voices Reciprocal communication encourages more reciprocal communication Infants prefer listening to infant-directed speech (IDS) (motherese) There is a positive association between IDS and language development including

hearing-impaired infants wearing hearing aids 32 Management of Pediatric Hearing EHDIEarly Hearing Detection and Intervention In California this is Early Start The entire process Birth Screen Diagnosis (audiological, medical, genetic) Treatment Referral

genetics (audiological/hearing devices), medical if indicated to other specialties including developmental pediatrics, 33 Management Intervention Provided in LEA, Regional Center, dually-served Includes Parent support

Ongoing Audiology to monitor Progressive/fluctuating Management Cochlear Pediatric hearing loss of hearing aids implant candidacy evaluation otology

Many resources exist 34 35 EHDI State-Federal partnership Wealth of information and resources for parents and professionals on EHDI website Considerable research that supports the importance of early detection, early intervention Program has grown

Starting Now in just a few statesRhode Island, Colorado all states and territories Landscape may change EHDI Programs Managed by NCHAM (Contractor) National Center for Hearing Assessment and Management SLC,

UT Karl White, Director Parent Resources Audiologist Resources Technical Resources for state EHDI programs State statistics available 37 38 Prevalence of Infant Hearing Impairment

Generally 2-3/1,000 Lower in WBN Higher in NICU Most common birth condition Screening meets the criteria for an efficient and effective screening program: Condition is prevalent Condition is easily screened, and results are objective/valid Robust evidence that early diagnosis and early intervention

leads to improved outcomes From the CDC 40 EHDI--History Centers for Disease Control established in 1946 EHDI initiated in 1997 Clearninghouse for statistics, program development and support. Major supporter of JCIH 41

EARLY Matters Conclusively shows that the earlier the better Diagnosis Amplification Intervention: by parents, teachers, SLPs, LISLs, Teachers of the Deaf/ASL teachers, other EHDI professionals 1,2,3 in place of 1,3,6?

Typical/Normal hearing child Hears from ~20th week gestation Surrounded by language at birth Recognizes mothers voice Prefers native language 42 EHDI--Age at Intervention Matters NAL study (National Acoustics LaboratoryAustralia) Prospective study of 350 children Children amplified at 3 months had better outcomes at

age 5 than those amplified at 24 months Difference larger for children with 70 dB as compared with children having 50 dB hearing losses Children who were implanted at 24 months had poorer language than those implanted at 6 months Follow-up Matters (orthe purpose of screening is not just to screen) Average age of intervention has declined ~6-8

months with wide variation Early identification vs early intervention Identification doesnt ensure intervention Factors leading to delayed diagnosis/treatment Parent denial/parent not understanding urgency Limited access to pediatric audiologists Appointment wait-times Presence of medical co-morbidities

Presence of mild or unilateral HL Pediatricians and PCPs can help or hinder 44 More Challenges/Barriers #1 problem: multiple re-screenings and diagnostic evaluations by physicians, audiologists Necessitates changing from one center to another, often necessitates sedation/anesthesia Standardized protocols for re-screens, diagnostic

evaluations not always followed Physician and barriers: wait and see, avoidance/denial Hearing loss is not visible! Lack of family-support systems Low SES barriers (transportation, employment, financial) 45 And a NEW Challenge/Barrier Parental Refusal for Treatments, Procedures, and Vaccines in the Newborn Nursery

Burton, et al. 2018 Advances in Pediatrics Parent refusal to have baby screened invasive un-natural I know he can hear Refusal of standardized care occurring more frequently 46 Additional Causes of Delay

Audiologists tasked with infant/pediatric responsibilities without adequate knowledge, skills, equipment, time Poor communication among providers Lack of 3d party payments For multiple appointments For pediatric hearing aids Deductibles and co-pays Being uninsured

OME Parents report baby responds to sound 47 Solutions? (In My Perfect World) All parents would receive education about NBHS prior to babys birth California protocol No discharge until re-screen appointment is scheduled Re-screens (not just diagnostics) would be covered by insurance or CCS Parent-education for all not-pass screen outcomes would be provided by pediatric audiologists in the nursery

Pediatricians would support Audiologists Re-screens would occur in accessible locations Scheduled in such a way that diagnostic could immediately follow 48 My Perfect World, Continued Parents of D/HH kids would immediately be paired with parents of newly-diagnosed children ASL families LISL families

Spanish- and other-language speaking families paired up Pediatrician/PCP and otologic physicians would support audiologists No child would have OME to compound diagnostic challenges Audiologists who are contracted providers for healthplans but not pediatric-savvy would not be required to evaluate infants 49 Diagnostic Tools Would Continue to Be Refined Greater use of automated algorithms for ABR

Wideband reflectance would supplant tympanometry CAEP would come into wider use 50 CAEP Recording Holds Promise for Earlier Detection/Intervention (Mehta, et al., 2017) Compared diagnosis of hearing loss via CAEP vs ABR Use of CAEP reduced median age of hearing aid fitting for all hearing levels from 9.2 months to 3.9 months Children with profound hearing losses were referred earlier for cochlear implantation 51

Whats a Pediatric Audiologist? ABA PASC Certification California: CCS Designation Private health plans: no distinction among audiologists Access to, and use of, all equipment necessary for evaluation, treatment Function as part of the Team approach Knowledge of and use of best clinical practices, algorithms, Referral pathways Parent- and patient-centered care 52

Pediatric Outcomes: 3 Major Studies US (Boystown)OCHL (Outcomes of Children with Hearing Loss) Mary Pat Moeller, Ryan McCreery AustraliaLOCHI (Longitudinal Outcomes of Children with Hearing Impairment) Terese Ching Geers, et al., 2017. - Prospective, multi-site, longitudinal

study Little evidence-based research with ASL 53 OCHL: Outcomes of Children with Hearing Loss NIH-funded 5-year, multi-center (3) Designed to address/answer 3 questions 1. Impact of specific factors on timely diagnosis and follow-up SES

Hearing loss severity 2. Consistency of appropriate care re 1-3-6 3. Reasons for delays between various steps in the EHDI process 54 OCHL: Open-Access Ear and Hearing 2015

35% of children had aided SII significantly below that recommended/possible Either severe-profound HL and targets not attainable OR Deviations from prescribed targets Importance of either Referring

to CI center when audibility with well-fitted hearing aids is insufficient And/or ensuring that independent measures show maximum audibility given degree/configuration of hearing loss 55 LOCHI: Longitudinal Outcomes of Children with Hearing Impairment PI, Terese Ching at NAL, Australia >450 children enrolled Longitudinalstudy initiated in 2005 Most

recent data just published 5-2018 Regular monitoring of the speech, language, hearing and psycho-social development of children who wear hearing aids and/or cochlear implants. 56 LOCHI 5-Year Data (2018) The key message emanating from the outcomes presented in these papers is clear early fitting of hearing aids or cochlear implants is critical to achieving the best potential outcomes. Parental involvement

Early device fitting Adherence to achieving amplification targets 57 LOCHI The findings lend support to the American Academy of Audiology guidelines for best practice in paediatric amplification (American Academy of Audiology Task Force on Pediatric Amplification 2013 American Academy of Audiology Task Force on Pediatric Amplification. 2013. American Academy of Audiology Clinical Practice Guidelines: Pediatric Amplification. Reston, VA: American Academy of Audiology.

[Google Scholar]). 58 Lessons from LOCHI (Smith, et al., 2016) Provide hearing aid amplification, and implant early when indicated Aid children with ANSD early/earlier Use behavioral thresholds Parent-report (e.g., PEACH) Use CAEP (not in widespread use in US but growing) to permit earlier

HA amplification Use spoken-language only Work with an inter-disciplinary team particularly when child has multiple impairments/disabilities Have a high-educational level mother Maximize audibility in hearing aid fitting Australia does it well Publicly-funded diagnostic, hearing aid and cochlear implant services. Birth to 7 months: estimated hearing levels by ABR, ASSR, ECochG One

low frequency (500 or 1000) and one high frequency (2000 or 4000 Hz) Or click-evoked to estimate hearing in the 2000-4000 range BOA to teach parents how to observe auditory behavior/responsiveness at home VRA asap 60 Australia

Hearing aids are no-cost. Hearing aid management and CI evaluation proceed simultaneously If unilateral cochlear implantation, hearing aid use continues in non-implanted ear Implantation occurs early (6 months+/-) Consistent use of best-practice protocols for diagnosis and hearing aid/cochlear implant management 61

PEACH (Ching and Hill, 2007) A NAL/LOCHI product Parent Evaluation of Aural/Oral Performance in Children Designed to evaluate effectiveness of amplification 13 questions for parents to answer Device use/Loudness Discomfort Listening in Different Situations Responses to Environmental Sounds 62 Australian Hearing Protocols for

Management of Infants with AN Approximately 50% of children with AN will benefit from hearing aids Must have behavioral (not electrophysiologic) tests demonstrating elevated thresholds to fit hearing aids Recommendation of Australian as well as AAA Protocols Fitting low gain-low output hearing aids (in the absence of behavioral thresholds) NOT indicated Hearing fluctuation can occur (e.g., with changes in temperature)

The 3d Study was a Bombshell 64 Early Sign Language Exposure and Cochlear Implantation Benefits--Geers, et al., 2017 Prospective, Longitudinal, Multi-Site study CDaCI Investigative Team (Childhood Development after Cochlear Implantation) 3 groups of implanted children who differed in duration of early sign language exposure compared longitudinally Outcomes

Children without early sign exposure achieved Better speech recognition skills Significant advantage in spoken language and reading Children whose families signed for the first 3 years after implantation averaged considerably less intelligible speech Children without sign language scored significantly better on literacy skills 66

Children exposed to sign language Performed more poorly on auditory-only speech recognition Speech intelligibility Spoken language Reading Is exposure to sign language a Cause (i.e., children were not progressing with auditory skills so Sign was initiated or continued)? Effect (i.e., sign reliance resulted in less impetus to develop

spoken language skills) Caveats Parents were normal-hearing and not fluent signers All sign was recognized (ASL, Total, pidgin, Baby Sign, SEE, etc.) There may have been differences among groups that werent identified More than half of the mothers in this study were college graduates, and results may not be applicable to other SES groups.

This study was viewed with alarm by some EHDI personnel University of Western Ontario Child Amplification Lab Susan Scollie, Director (formerly Richard Seewald) Its product DSLDesired Sensation Level Research has shown relatively equal outcomes in pediatric speech-language development DSL

NAL (Canadian kids do better with DSL, Australian kids with NAL ) The best single figure for audiologists fitting pediatric hearing aids 69 University of Western Ontario DSLIO Aided SII by Hearing Loss (mean,+/- 1 sd) McCreery, Bentler, Roush 2013: MultiSite Study

195 Children: 3 sites (academic medical centers) Assessed proximity of fitting re DSL targets, 500-4000 Hz Approximately 26% of the children had aided audibility less than 0.65 on the Speech Intelligibility Index (SII). More than half of children had at least one ear that deviated by >5dB from targets 71 Characteristics of Hearing Aid Fittings in Infants and Young Children. McCreery, Ryan; Bentler, Ruth; Roush, Patricia Ear & Hearing. 34(6):701-710, November/December 2013. DOI: 10.1097/AUD.0b013e31828f1033

Fig. 4 . SII values for soft speech input levels (50 dB SPL; top panels) and average speech input levels (65 dB SPL; bottom panels) as a function of left ear (left panels) and right ear (right panels) PTA. The closed circles are fittings with 5 dB RMS error. For the average speech input levels, the normative SII range from the study by Bagatto et al. (2011) is plotted as solid (mean) and dashed (confidence intervals) lines. PTA indicates pure-tone average; RMS, root-mean-square; SII, Speech Intelligibility Index. 2013 by Lippincott Williams & Wilkins. Published by Lippincott Williams & Wilkins, Inc. 2 Audibility Matters! Speech-language development

Speech perception Decisions about implantation need to be made based on evidence of BEST POSSIBLE audibility Audibility must be optimized, using evidence-based protocols. NOT First Fit Hearing aid manufacturer proprietary software algorithm And it must be measured in the ear, or else

73 When Does Diagnosis and Hearing Aid Fitting Need to Happen? NO AGE is too young JCIH is proposing *1-3-6 is the standard *for those centers who have achieved 1-3-6, strive for 1-2-3! 75

1,3,6 Its hard to achieve screening completed by 1 month of age when The middle ear is still full of amniotic fluid The baby is in the NICU The baby was severe preemie and too young for automated testing (<34 weeks GA) Or when Parents, pediatricians are not supportive

76 Hard to Achieve Hearing Aid Amplification by 3 months when Chronic MEE AN with no waveform to base HA fit on Baby cannot or will not sleep in clinic for ABR Insurance wont issue authorization Parents dont keep appointments Physicians dont reinforce the message to parents about the importance of the diagnosis 77

INFANT EAR WITH MEE226 vs 1000 Hz PROBE TONE No Wave V Threshold Because Theres No Wave V! Hard to Achieve 6 months when Parent or physician denial Parents resist referrals for hearing aids, early intervention Physicians wont give medical clearance But

do we need it? Insurance doesnt cover hearing aids Its summer and school isnt in session 80 UCLA Protocols to Address Delays Evaluations in NICU Any child who is 3 months of age Pre-set appointments for hospitals making referrals

Infants discharged on weekends Set re-screens in a diagnostic appointment Yes it can result less-than-optimal time management for clinic Fit loaner hearing aids without waiting for medical clearance Coordinated Audiology, SLP, HNS, Social Work 81

Infant Diagnostics Reliable and valid when established protocols followed Thresholds (behavioral or tone-burst ABR) 500 Hz 2000 Hz Air- and bone conduction Each ear separately With this information, hearing aids can be fitted.

Dont wait for an 8-frequency audiogram! When there is a NR ABRlook for AN 82 ABR Toneburst Interpretation McCreery, et al., 2015 Argues against using a single correction factor Agreement between ABR and behavioral threshold varied as a function of degree of hearing loss Use of a correction factor taking into account the degree of hearing loss on the difference between ABR and behavioral threshold Make

hearing better for low-intensity (<50 dB) stimuli Make hearing worse for high-intensity (>70 dB) stimuli ABR underestimates thresholds for moderate-profound loss, overestimates thresholds for normal-mild. AN Protocols Australian (2005) and AAA (2012) are highly similar ANY

no-response ABR Or any atypical-morphology ABR High-intensity click ABR with condensation and rarefaction polarities separated to reveal cochlear microphonic CM will be 180 degrees OOP

OAE assessment (will be present) Acoustic reflex assessment (will be present) Caveats: OME will obscure OAE and AR Not all AN looks the same 84 Classic ANABR

Alternating polarity stimulus hides cochlear microphonic Single-polarity reveals CM response 2 phases 85 AN-Single Polarities AN-Alternating AN Has Multiple Etiologies Hyperbilirubinemia

High ICP/Hydrocephalus (can be reversible) Twin-to-twin Transfusion Syndrome Temperature-Dependent Familial/Genetic Cochlear Nerve deficiency RDS/Anoxia Hence sometimes referred to as spectrum 88 ABRProtocols, and Noise, Matter Starting intensity can be a hearing test! Noise is the enemy Access to drugs helps

Bad information is worse than no information If its a real response, it will repeat. 89 Auditory Neuropathy 10-15% of infants with hearing loss have AN Outcomes with cochlear implants the same whether child has AN or cochlear hearing loss (Ching, et al, 2017) Assuming delay

no associated medical conditions or developmental 93 AN and Hearing Aids Defer hearing aid fitting (if any) until such time as child responds behaviorally to frequency-specific signals Hearing aids cannot be fitted unless theres a W5 Degree of hearing loss may vary from none/normal to profound Loss may fluctuate

Unless you can establish threshold responses via CAEP Fitting a low gain hearing aid may be worse than no hearing aid at all 94 Amplification for Children with AN When there are behavioral responses/thresholds to estimate gain and output ABR cannot be used Walker, et al., 2016 recommend

HA usage in AN when Thresholds can be measured Thresholds are elevated sufficiently to impede access to speech at conversational levels. Functional

outcomes are similar to peers with SNHL CAEP will speed amplification fitting 95 AN Challenges Delayed diagnosis Failure to use appropriate ABR protocols When and how to fit hearing aid amplification

Behavioral responses to sound needed Whether to evaluate for CI Concomitant morbidities can confound/delay audiologic management 96 Purpose of Early Diagnosis and Intervention is Language Development Speech/spoken language What about ASL?

What is audiologists role in the language discussion? Provide information Language, whether signed, spoken or both, is the goal All children must have competent expressive and receptive language Should audiologists engage in the methods conversation with parents? 97

Audism Free America Audism Free America (AFA) is a grassroots Deaf activist organization in the US, which advocates for Deaf American rights, cultural resurgence, and seeks primarily to challenge the ideological foundations of audism in America. Audism is attitudes and practices based on the assumption that behaving in the ways of those who speak and hear is desired and best. It produces a system of privilege, thus resulting in stigma, bias, discrimination, and prejudicein overt or covert waysagainst Deaf culture, American Sign Language, and Deaf people of all walks of life. AFA is committed to: 1. human and linguistic rights of Deaf people 2. unmasking audism and media misrepresentation 3. advocating for future generations

98 99 Use of Best Practices Imperative in Determining Pediatric Practices Literature and clinical practice guidelines exist From our professional organization AAA Pediatric Diagnosis AAA Pediatric Amplification AAA CI document coming close to completion

From PubMed From multi-disciplinary organizations JCIHJoint Committee on Infant Hearing 100 Joint Committee on Infant Hearing ( (often confused with Joint Commissionoften referred to by its former name JCAHO Established by Marion Downs 40+ years ago Multi-organizational professional group AAA, ASHA, AAO, AAP, AG

(CDC-EHDI, HRSA) Bell, CED, Dips, Federal Entities Issues periodic best-practices statements Fields questions from hospitals, clinics 101 Joint Committee on Infant Hearing (JCIH) Last document 2013 (Early Intervention) New full document in final stages of editing

No major changes Updated references Publication in Pediatrics in 2019 102 JCIH Preview Risk Factors: largely unchanged Use a by 9 months or 7-9 months of age for re-evaluation of conditions with possible progression, delayed onset Deleted isolated ear tags/pits as a risk factor

Added Zika Emphasized cCMV Screening protocol in the WBN OK to rescreen by OAE when first fail was by ABR ! Why? Push for 1,2,3 when 1-3-6 is met But 1,3,6 is still often not met What ELSE is New in JCIH 2018?

Surveillance of babies with risk factors for late-onset or progressive hearing loss By 9 months of age Exceptions: ECMO grads no later than 3 months after occurrence cCMV no later than 3 months after infection

Meningitis, encephalitis no later than 3 months after infection Head trauma or Chemotherapy no later than 3 months after occurrence 104 What Isnt New but is Still Important? Diagnosis the earlier the better Risk Factors are important but only tell the story about half the time Theres no substitute for high-quality ABR for diagnosis of hearing loss Use of best-practice protocols in pediatric amplification Maximizes

Speeds audibility for speech/language learning decision-making regarding implantation 105 And the #1 Risk Factor for Infant Hearing Loss Caregiver concern regarding hearing, speech, language, developmental delay and/or developmental regression

106 Risk Factor 1 A history of family members being deaf or hard of hearing with onset in childhood Causes of Deafness 30% Syndromic 50% Genetic

70% Non-Syndromic >400: Examples include Alport Syndrome Usher Syndrome Pendred Syndrome Waardenburg Syn. Branchio-Oto-Renal Syndrome 75-85% Autosomal Recessive

~50% have 2 GJB2 deafness variants Recurrence: 25% 15-24% Autosomal Dominant Recurrence: 50% Deafness/hoh 1-2%

X-Linked Recurrence: variable <1% 25% Recurrence: variable Environmental Recurrence:0.1-0.3%

25% Mitochondrial Idiopathic Recurrence: 10-18% Infections (CMV) Risk Factor 2 NICU admission > 5 days 10-15% of all births have NICU admission

Risk Factor 3 Hyperbilirubinemia requiring exchange transfusion Risk Factor 4 Aminoglycoside administration of >5 days Administration of aminoglycoside antibiotics for less than 5 days is considered a risk factor only if toxic blood levels are identified or if there is a family history of a mitochondrial genetic mutation associated with aminoglycoside sensitivity for sensorineural hearing loss Risk Factor 5 Perinatal asphyxia

Risk Factor 6 Extracorporeal membrane oxygenation (ECMO) Risk Factor 7 In-utero infections (i.e., herpes, rubella, syphilis and toxoplasmosis). CMV infection is a leading cause of congenital infection, and is a leading cause of non-genetic unilateral or bilateral early, progressive and delayed onset sensorineural hearing loss. cCMV Recent systematic review (Bartlett, et al., Sept, 2017)

90% of cCMV infections are not clinically apparent at birth At risk for developing abnormalities (SNHL and/or neurodevelopmental impairment) throughout childhood. Onset, course and severity of HL is variableno marker to determine which infants will develop sequalae Cumulative incidence of SNHL 7-11% Surveillance of all children with cCMV regardless of presentation is necessary Cochlear implant performance in children deafened by congenital cytomegalovirus a systematic review 2018 The available evidence reveals that cCMV children

often have worse cochlear implant performance compared to noncCMV children, which can be attributed to cCMV related comorbidities. We urge physicians to take into account the cCMV related comorbidities in the counselling of pediatric CI users deafened by cCMV. Zika virus has been added to the list of in-utero infections associated with hearing loss. Risk Factor 8 All craniofacial conditions and physical conditions associated with hearing loss

Risk Factor 9 More than 400 syndromes and/or genetic disorders associated with hearing loss 119 Risk Factor 10 Family/caregiver concern regarding hearing, speech, language, or development requires attention. One of the most important risk factors Risk Factor 11 Perinatal or post-natal bacterial and/or viral meningitis or encephalitis

121 Risk Factor 12 Post-natal trauma Especially head trauma involving mastoid 122 What Should the Audiologists Role Be in Risk-Factor Surveillance? Parent-education

Physician-education Written recommendations for surveillance per JCIH recommendations included in reports Review risk factors at all pediatric appointments (Some are late-onset) Audiologists Roles in EHDI--Screening Developing and managing hospital-based UNHS programs CA requires

Monitoring an audiologist-consultant for birthing hospitals statistics (too many fails, not enough fails) Training screeners, assessing competencies Providing pediatrician/PCP education Developing protocols and policies Types

of screening devices (well-baby vs NICU) Training and assuring competence of screeners Resources How for re-screens and diagnostic evaluations to manage long-term NICU/PICU residents?

124 Audiologists Roles in EHDI Rescreening and Diagnosis OP Re-screening (Protocols? OAE vs ABR?) Diagnosis Meeting the 3 month timeline for diagnosis Meeting the 4 month timeline for amplification Using best practices in equipment, protocols, parent-counseling Providing surveillance of hearing levels and auditory skills

125 Audiologists Roles in EI/Surveillance Referrals to EI Audiologist REQUIRED to make referral to EI within 7 days (parents are not obligated to accept services) Long-term Intervention Direct provision of services and/or

Collaboration with Intervention Team, School-based or RCbased EI personnel Managing equipment Surveillance Hearing aids? Or time for CI? Development of auditory skills 126

How Are We Doing? Better Vast improvement in past decades Not good enough Children are still late diagnosed and treated (Speech and) Language Development still lags 127

Early Hearing Detection and Vocabulary of Children with Hearing Loss Yoshinaga, et al., 2017 Examination of Vocabulary Learning in children meeting 1-3-6 guidelines 6 Main Findings: Children with Higher Vocabulary Actually MET the1-3-6 guidelines Younger at age of diagnosis and hearing aid treatment No additional disabilities Mild-moderate hearing loss Had parents who were deaf or hard of hearing

Mothers with higher levels of education 129 1-3-6 vs 1-2-3 Guidelines In Yoshinaga study, only 1/2 to 2/3 of children met 1-3-6 Independent variables only explained 41% of the variance Other factors that need to be considered and examined: Family involvement Parent-child interaction Compliance

with amplification use Intensity of intervention services Characteristics of early intervention providers and programs 130 Opportunities for Improvement Family-mentors Transportation (at least in CA) Centers of excellence to manage all diagnoses and hearing aid fittings Robust, accessible parent-to-parent support Both

for LISL and ASL Coordinated centers Physicians, audiologists, educators, parent-support, social services, transportation 131 From Diagnosis to Hearing Aid Fitting Coordinated, not separate EMI at ABR appointment

Loaner hearing aids medical clearance? Partnering with EI audiologist or D/HH Encouraging parents Hands and Voices AG Bell/NAD

Local parent-support 132 Infant Hearing AidsWhere to Start? Must have valid thresholds ABR or Behavioral Must have (at least an estimate of) RECD Use average RECD

Use 1 ear measured RECD for the other ear Unless anatomy is clearly unusual/different Gain within 5 dB of target Target matching most important for 50 dB SPL inputs OSPL no more than 3 dB above recommended 133 Roadblocks to Early Hearing Aid Fitting $$$

Insurance roadblocks Physician reluctance/lack of knowledge Parent reluctance or denial Mixed messages from audiologists Required features vs high-level/premium Audiologists who are not comfortable with infants Audiologists

who dont have infant-specific protocols 134 Infants in Families with Hearing Aid Benefit from a Third-Party Plan 135 Objective, Evidence-Based InfantPediatric Hearing Aid Protocols NAL, UWO Similarities greater than differences Common threads:

Use of evidence-based prescriptive targets (not manufacturerderived/specific) Australia: NAL-NL2 (National Acoustic Laboratory-Non-Linear v2) University of Western OntarioDSL v5.0 (Desired Sensation Level) Some evidence that DSL prescribes higher target gain and output with better audibility for soft speech

136 AAA Pediatric Amplification Guideline (2013) (currently being revised) Evidence-based Best practices The specific goal is to provide a set of statements, recommendations, and strategies for best practices specific to the application of amplification as part of a comprehensive treatment plan for the audiologic management of children with hearing loss. Covers all aspects of amplification Candidacy, selection, verification, validation Air- and bone conduction devices, FM/remote microphone,

cochlear implant candidacy 137 What About Manufacturers Default or Proprietary Fitting Formulae? Why? Manufacturers Proprietary Formulae Not evidence-based May employ features not routinely required Frequency

shifting Auto-directionality How can fitting be independently verified? Every fitting requires verification and modification based on an evidence-based protocol DSL, NAL 138

Each Seeks to Maximize Insofar audibility across the frequency spectrum as childs hearing loss AND hearing aid technology permits For soft, average and loud speech inputs Reduce noise Minimize distortion

Supports the use of pediatric-friendly features Visual battery indicator Small size, earhook FM-capable 139 What are the Differences Between the US and Canada/Australia? Canada and Australia have single-payer healthcare In California Single-payer

for low-income only Limited control over quality of HA selection/fitting HA fitting and CI Candidacy evaluation occur simultaneously in Australia Not in the US, necessarily 140 Australian Protocol for Mild, Unilateral HL

BAH not used for UHL Only bilateral atresia FM may be used in lieu of hearing aid if appropriate Directly aiding impaired ear always best option 141 Considerations for Type-Degree of Hearing Loss UHL Conductive

vs Sensory vs Mixed vs Neural vs absence of VIII vs unaidable/profound Unilateral microtia/atresia Bilateral microtia/atresia AN Bilateral SNHL: Degree Minimal/Mild-severe Profoundshould child/family quickly move to CI?

ALL losses result in speech-language-psychosocial issues 142 UHL asymmetric hearing loss Lack of stimulation may doom poor/deaf ear Evidence that brain re-organizes Auditory pathway only recognizes hearing ear Stimulation of deaf ear may enable function May restore binaural/spatial hearing Is there a critical period?

UHL is often late-identified Importance minimized Parents may not feel urgency Approaches to UHL (SSD) Ignore FM CROS Hearing Aid BAH Cochlear Implant

Children With Single-Sided Deafness Use Their Cochlear Implant. (Polenko, et al., Ear and Hearing, 2017) 144 UHL Known to cause speech and language delays in many but not all cases As many as 13/1,000 (higher prevalence than bilateral HL) Brookhauser,

1996 2004 Meta-analysis (Lieu), covering 40 years of studies 22% 12% to 35% rate of repeating at least one grade to 41% receiving additional educational assistance Speech and language delays Surveillance of Hearing Levels * Delayed-onset or progressive HL is common Reflects

characteristics of screening tools? 2/1000 babies has PCHL at time of NBHS/early diagnosis 5/1000 children have PCHL at Kindergarten-entry Fitzpatrick, 2017 37% of UHL showed progression of loss in the unilateral ear and/or opposite ear Audiologists responsibility to provide surveillance of hearing levels

146 Recent Meta-Analysis (Anne, et al., 2017) A total of 429 studies of children with UHL 13 met inclusion criteria and were reviewed. Overall, 7 studies showed poorer scores on various speech and language tests effects more pronounced for children with severe to profound hearing loss Four studies did not demonstrate any difference in testing results between patients with unilateral hearing loss and those

with normal hearing. Two studies that evaluated effects on speech and language longitudinally showed initial speech problems, with improvement in scores over time. Goal is BINAURAL Hearing (or at least bilateral) Only HA or CI can potentially achieve this Significantly asymmetrical hearing losses preclude binaural hearing Consider CI in poorer ear Candidacy criteria should embrace this application for

infants/young children Only opportunity to develop binaural skills BAH or CROS does not yield binaural hearing Both run the risk of bringing unwanted noise into the normal ear (masking) Binaural Hearing Allows Localization Binaural Summation

Elimination of Head Shadow Effect Binaural Squelch All necessary in a classroom All necessary for psycho-social functioning All necessary for safety Unilateral Hearing Loss Often late-diagnosed Significance often down-played By parents, physicians, school fail first then do something about it is a common approach

Use of hearing aid or other equipment often less-thanoptimal Causes of UHL Cochlear nerve deficiency Termed by far the most common cause of UHL in children after studying 480 children with UHL (Laury, et al., 2010) Cytometalovirus GJB2 abnormality Bacterial meningitis Congenital aural atresia with cochlear abnormality Head trauma

151 Cochlear Nerve Deficiency (CND) Demonstrated by both CT and MRI MRI more sensitive CT can be normal in presence of CND Current recommendation is that children receive BOTH CT and MRI prior to CI CN Deficiency? How

small is too small? How does size relate to function? Answer is not apparent from the literature 152 UHLOutcomes with Various Approaches to Amplification (AAO-HNS) Systematic review (Appachi, et al., 2017)249 studies currently there is no consensus Looked at

Hearing aid FM CROS Bone-conduction Best outcomes for speech-in-noise with FM Flaw.. All UHL are not equal! Degree of hearing loss Whether normal cochlea (conductive) or not Mixed Purely S/N

Dead ear Absent VIII Appachi, 2017 failed to distinguish among unilateral conductive, mixed or SSD when advocating for BC devices for moderate to profound UHL. 154 UHL-Etiology? Isolatedidiopathic

UHL CT/MRI in 102 children with USNHL abnormalities were identified in 49% of cases most frequently affected site was the labyrinth (29%) cochlear nerve (9%) brain (7%) No significant difference in the number or type of abnormalities was found for the degree of hearing loss or age categories Van Beek Calkoen, et al., 2017

155 CI for UHL? In CHILDREN? (its not even approved for adults!) No other way to stimulate and keep stimulated a congenitally-impaired (deaf or unaidable) ear. The only opportunity to create binaural hearing In adults, CI for UHL Relieves Improves

tinnitus sound localization Speech understanding in quiet and noise (Tokita, et al. 2014) Microtia-Atresia Dont screen! If normal cochlea BA device asap If unilateral profound deafness

BAH or CROS NOT indicated Potential for introduction of unwanted noise into normal ear (AAA Pediatric Amplification Guideline) Beware the surgeon who wants to fix it

157 Bilateral Severe-Profound Deafness Not as common as decades past No more rubella Relative to mild-severe hearing loss, less common Earlier diagnosed Lack of behavioral response at home more apparent

Can be considered developmental emergency 158 Multiple Etiologies Connexin 26 Pre-natal viral infection Idiopathic 159 Rehabilitation, Education, Communication What is diagnosing-audiologists role?

Children must have language Whether signed, spoken, both LEAD-K initiative (now law in California) We want all Deaf kids to be kindergarten-ready! 160 LEAD-K in Californiafirst state to pass SB 210 (passed in 2015) Language developmental milestones from existing standardized norms will be shared in a resource for use by parents to monitor

their D/HH childs language acquisition and developmental stages toward English An assessment tool will be used to assess the language and literacy development of D/HH children If a child does not demonstrate progress in expressive and receptive language skills, the childs IFSP or IEP team will explain the reasons why, and will recommend specific strategies, services, and programs to assist the childs success toward English literacy. 161 Mellon et al., Should All Deaf Children Learn Sign Language? (2015) Multi-author paper combining literature review and expert

opinion The benefits of learning sign language clearly outweigh the risks Children need to learn language. They must learn it from parents, teachers, and their community... Deaf children of hearingparents are unique among all children in the world in that they cannot easily or naturally learn the language that their parents speak. Hearing parents of deaf children are unique in that they are asked to learn, at least in a rudimentary way, a new and foreign language to communicate with their children. On the Other Hand Early Sign Language Exposure and Cochlear Implantation

Benefits Multi-site longitudinal study (Geers, et al., 2017) Published in Pediatrics Children without early sign language exposure achieved better speech recognition skills over the first 3 years postimplant and exhibited a statistically significant advantage in spoken language and reading near the end of elementary grades over children exposed to sign language there was no advantage to parents' use of sign language either before or after CI. 163

LANGUAGE is a Requirement How to get there is parent choice ASL Spoken Language Combination All children need an excellent language model(s) to develop communication A great majority (>90-95%) of Deaf children have normal/typical hearing and use spoken language Not providing language is not an option 164 JCIH Statement on Early Intervention

(2013) Goal 3: All Children who are D/HH from birth to 3 Years of age and their families have EI providers who have the professional qualifications and core knowledge and skills to optimize the childs development and child/family wellbeing Goal 3a: Intervention services to teach ASL will be provided by professionals who have native or fluent skills and are trained to teach parents/families and young children Goal 3b: intervention services to develop Listening and Spoken language will be provided by professionals who have specialized skills and knowledge Considerations in Children with Specific

Conditions/Diagnoses Cleft Palate CDC EHDI just completed pediatrician-education information sheet for pediatricians Theoretically medically treatable But often not, or not consistently Down Syndrome Autism Spectrum Disorder

Significant Developmental Delay Chemotherapy-induced HL 166 Autism Spectrum Disorder (ASD) AAP called for referral to an audiologist for ALL children suspected of being on the autism spectrum !! If not behaviorally testable to R/O hearing loss OAE ABR under anesthesia/sedation

167 Ototoxicity Monitoring In pediatric oncology (Cisplatin) Commonly-utilized Highly in pediatric cancers ototoxic In NICU (aminoglycoside antibiotics) Genetic

variant makes some infants highly susceptible to ototoxic effects even with standard dosages New paper (Garinis, 2017) recommends Use higher frequency (>4K Hz) to monitor ototoxicity Use a protocol that builds on findings to determine next test sequence 168

Newly-Published Monitoring Protocol Brooks and Knight, 2017: Ototoxicity monitoring in children treated with platinum chemotherapy Most commonly-treated cancers Medulloblastoma Osteosarcoma Hepatoblastoma Neuroblastoma Germ cell tumors Ototoxicity is the primary toxicity of cisplatin 60-70%

of children affected If co-treated with carboplatin: 80-90% ototoxicity 169 Baseline Monitoring evaluation after every cycle End-of-treatment evaluation (hearing changes may occur after cessation of drug) Monitoring serves 2 purposes Inform treatment management

Inform family so that intervention can be initiated promptly has parent materials 170 Ototoxic Monitor Protocol Initial evaluation Establish 8000 Hz)

all frequencies possible (i.e., octave frequencies 500- For monitoring after initial evaluation Start at 4000 Hz If WNL Go to 8000 Hz If elevated Go to 2000 Hz If elevated, go to 1000 Hz

171 Protocol for Pure Tone Monitoring (Behavioral or TB ABR) 172 Pediatric Hearing Aid Amplification General goals Audibility Low Distortion

Immediacy Consistent Use 173 AAA Pediatric Amplification Guideline Rev 2013 Depends on evidence-base for recommendations and conclusions Many aspects do not have clear and rigorous evidence 174

Criteria for Amplification for Children Unilateral or bilateral If unilateral, preference for hearing aid ON poorer ear CROS not supported by research Possibility of putting unwanted noise in the normal-hearing ear

Degree of lossall Type of lossall (unless CND or absence) Fluctuating conductive If child/parents can discern when its necessary Permanent conductive (e.g., ear canal stenosis, congenital stapes fixation, etc) 175 AAA Pediatric Amplification Protocol

The primary goal of amplification is to provide audibility across the long-term average speech spectrum (LTASS), without delivering any signal that is of an intensity that would be either uncomfortable or unsafe. Only ONE way to determine this 176 Real-Ear Probe Microphone Measures using Child-Specific RECD Optimized to a validated, pediatric-specific target DSL-IO NAL-NL2

Using childs own earmolds 177 First Fit No RECD 39% AII Optimized with Individualized RECD: AII=76% Do Children WEAR Their Hearing Aids? Dependent on many factors Parent

level of education Family factors (denial) Degree of childs hearing loss Presence of other handicapping conditions

Depression influences hours of daily use In Hispanic Community (Munoz, et al., 2017) Need for interpreter Need referrals to connect with Spanish-speaking parents Fear

of losing hearing aids Difficulty in seeing/determining whether HA benefitting their child Datalogginga tool to improve, not a tool to punish 180 Surveillance is Necessary Throughout the Childs Lifetime Regular, reliable and valid measures of a childs

progress in meeting early intervention goals Without regular assessment of (unaided) hearing opportunities for modification to best meet the childs needs may be missed. Challenges when child is managed by audiologist who doesnt Refer for CI Know the referral criteria Know but doesnt follow referral criteria

181 How? 1. Regular audiologic monitoring, particularly in first months-years after diagnosis May require ABR in general anesthesia/sedation 2. Regular hearing aid checks Real-ear changes

measures and re-programming with all earmold 3. Regular audiological evaluation of unaided hearing 4. Communication among diagnostic-hearing aidcochlear implant audiologist, early-intervention or school-based personnel, parents/caregivers 182 CI for Children with AN? Outcomes are similar for children with sensorineural hearing loss and with AN (Ching et al.) Depends in part on the etiology of the AN

183 Cochlear Implantation: How Young? The younger the better Our FDA criteria are not keeping up with current research 184 Age at Intervention (from Ching) Prospective study of 350 children Multiple regression analyses

Children amplified at 3 months had better outcomes at age 5 than those amplified at 24 months Difference larger for children with 70 dB as compared with children having 50 dB hearing losses Children who were implanted at 24 months had poorer language than those implanted at 6 months Age at intervention and 5-year language Ching et al, (2017) Pediatrics 140(3) e20164274

-0.7 SD -0.4 SD -0.3 SD THIS is only possible when NBHS is accurate Diagnostic follow-up occurs immediately following failed screening UCLA combines re-screen and dx appointment Hearing aid fitting is not delayed

Loaner vs purchased Medical clearance?? Parents are on board 187 Who ISNT a CI Candidate? Children without a cochlear nerve

Even deficient nerves may permit success with CI 97% of children with a No Response ABR ultimately underwent cochlear implantation (Hang, et al., 2015) Remainder were not recommended for CI due to co-morbidities No child demonstrated sufficient progress in speech and language development with hearing aids to contraindicate

implantation. HA trials do not significantly change clinical outcomes after implantation but can substantially delay fitting of CI. 188 Older Children-Teens Hearing loss due to Chronic MEE, PET, chronically infected ears

EVA Progressive Noise HL exposure 6-19% of teens have signs of NIHL Chemotherapy

Idiopathic (Functional) 189 Noise Exposure: Hidden Hearing Loss in Teens? HHLcochlear synaptopathy demonstrated in teenagers with noise exposure College-age listeners with normal hearing thresholds History of noise exposure Significant correlation between performance on a speech-innoise test and an electrophysiological measure of the auditory

nerve (SP/AP ratio) Also poorer speech recognition In noise In time-compression In

reverberation Hypersensitivity--hyperacusis 190 For ALL Children Language stimulation Starts in utero Opportunity for parent-education BEFORE baby is born

Why should I talk to him when he cant talk? Part of the pre-birth education of parents should include information on the importance of auditory and speech/language stimulation Impact on future literacy and school-success No child is too young to be read to! Or

sung to! 191 And if Children are Read to They develop a love of books! Infant Auditory Development: What Do We Know? Starts in utero Babies prefer mothers voice to others voices at or shortly after birth Babies prefer mothers language to foreign languages Infants with normal hearing progress predictably

through stages of auditory development Startle Localization Increasing auditory discrimination over time Hearing loss = atypical auditory development Joint Committee on Infant Hearing Supplement to the JCIH 2007 position statement: principles and guidelines for early intervention after confirmation that a child is deaf or hard of hearing. Pediatrics, 2013

Goal 1 All children who are D/HH and their families have access to timely and coordinated entry into EI programs supported by a data management system capable of tracking families and children from confirmation of hearing loss to enrollment into EI services Goal 2 All children who are D/HH and their families experience timely access to service coordinators who have specialized knowledge and skills related to working with individuals who are D/HH

Goal 3 All children who are D/HH from birth to 3 years of age and their families will have EI providers who have the professional qualifications and core knowledge and skills to optimize the childs development and child/family well-being Goal 3a Intervention Services to teach ASL will be provided by professionals who have native or fluent skills and are trained to teach parents/families and young children Goal 3b Intervention Services to develop listening and spoken

language will be provided by professionals who have specialized skills and knowledge Goal 4 All children who are D/HH with additional disabilities have access to specialists who have the professional qualifications and specialized knowledge and skills to support and promote optimal developmental outcomes Goal 5 All children who are D/HH and their families from culturally diverse backgrounds and/or from non-English speaking homes have access to culturally competent services with provision of the same quality and quantity

of information given to families from the majority culture Goal 6 All children who are D/HH should have their progress monitored every 6 months from birth to 36 months, through a protocol that includes the use of standardized, norm-referenced developmental evaluations, for language (spoken and/or signed), the modality of communication (auditory, visual and/or augmentative), social-emotional, cognitive, and fine and gross motor skills Goal 7 All children who are identified with hearing loss of any

degree, including those with unilateral or slight hearing loss, those with AN, and those with progressive or fluctuating hearing loss, receive appropriate monitoring and immediate follow-up intervention services where appropriate Goal 8 Families will be active participants in the development and implementation of EHDI systems at the state/territory and local levels Goal 9 All families will have access to other families who have children who are D/HH and who are appropriately

trained to provide culturally and linguistically sensitive support, mentorship, and guidance. Goal 10 Individuals who are D/HH will be active participants in the development and implementation of EHDI systems at the national, state/territory and local levels; their participation will be an expected and integral component of the EHDI systems Goal 11 All children who are D/HH and their families have access to support, mentorship and guidance from individuals who are D/HH

Goal 12 As Best Practices are increasingly identified and implemented, all children who are D/HH and their families will be ensured of fidelity in the implementation of the intervention they receive How to Determine Quality of Amplification? Aided audiogram? No, for so many reasons!

Aided speech perception? Yes, when child has sufficient speech-language Objective measures Best Estimate of audibility relative to hearing aid gain/output SII is visible in Verifit software Frequency lowering? If

targets can be met, why lower frequencies? This IS a form of distortion 211 Top 10 Technical Tune-Ups for Pediatric and Child Amplification (Scollie, 2017) Routine use of real-ear verification Use SII values using the normed scoresheet Use earmolds for VRA Use RECD corrections in interpreting VRA thresholds Loaner hearing aids available Use pediatric outcome measures

Verify frequency-lowering in the ear Later-Onset HL: Common! Either late-onset or progressive Surveillance of auditory development by parents/PCP important Chronic MEE Ototoxicity Noise-Exposure Genetic Idiopathic

213 Later-Onset HL: EVA Enlarged Vestibular Aqueduct (EVA) is one of the most common congenital malformations associated with sensorineural or mixed hearing loss. Association between hearing loss and EVA is syndromic or non-syndromic disorders Mutations of the SLC26A4 gene. The audiological phenotype of the EVA syndrome is heterogeneous, the type and entity of hearing loss may vary and vertigo episodes might also be present. (Aimoni, et al., 2017).

214 Deaf Community Considerations Importance of respecting Deaf Community This is a culture, not just a language Strong belief that children/families, regardless of degree of hearing loss (Its not a loss if you never had it, hearing level) Should have access to ASL

Should have access to the Deaf Community Is CI=Genocide? 215 Whats in the Future? My Wishlist Every baby gets screened And we understand the real intensity level that we are screening Even within a single manufacturer, it has been reported that

calibration intensities and spectra differed between two devices designed to screen infant hearing, with a significant difference in referral rate that was attributed to these dissimilarities (Hofmann, Luts, Poelmans, & Wouters, 778 2012). More rapid progression from NBHS to diagnosis, from diagnosis to treatment with hearing aids, cochlear implants, other devices With easy access to sedation/anesthesia for diagnostic procedures Parents will become familiar with typical auditory and

speech-language development 216 Wishlist Continues CI for UHL in children Hybrid CI for children Centers of Audiology Excellence will provide the infantpediatric services using EB Protocols. Including Social Work services, insurance facilitators, family counseling, peer-to-peer support Pediatricians, NPs, Family Practice physicians will be familiar with signs of atypical auditory development

Every parent is able to meet other parents early in the process Babies wont get screens Parents will talk and read to their children 217 Audiologists Roles Take ownership of NBHSP Ensure that 1,3,6 or better is met Educate physicians, nurses, families Make prompt referral to pediatric audiology center if unable to determine hearing status promptly When necessary, ABR with anesthesia/sedation Bad

information is worse than no information Implement a seamless clinical pathway Screening-diagnosis-intervention (hearing aids, cochlear implants, language intervention) Roles Refer to CI center for all children with bilateral moderate-profound SNHL Advocate for local, state and federal funding of programs EHDI Medicaid

Educate and advocate with private insurance for services and devices Write op-ed pieces 219 220 Importance of Licensure (Certification) Licensure is required to practice Attend the meetings!

Certification is icing on the cake Not required (including TJC) Specialty certification Be a careful consumer of certification Trends in Licensure Move toward public (non-professional) majority on boards North Carolina teeth whitening case Licensure

is designed to protect public Not to protect/limit practice Public member majority Suggestion that licensure is restraint of trade Importance of Multi-Disciplinary, InterDisciplinary Care Hearing loss/deafness doesnt occur in isolation Etiologies of HL are also etiologies of Intellectual

disability, autism Physical disability Sensory impairment (e.g., blindness/vision loss) Can impact intervention Each child has a family, community Roadblocks Poor support or antagonism from PCP Rural areaslack of choice in education systems Transportation Cultural/linguistic barriers including illiteracy

Economic barriers, life-stressors Poor understanding of implications of hearing loss and delayed language development on development, education Insurance Confusion about EHDI process Disparities in Care: Non-English Speaking and/or With Public Insurance Su, et al., 2017 Parents less likely to know type or degree of childs hearing loss Children were older on presentation to clinic Children ages 2-7 less likely to perform at expected

levels in auditory/oral function Less likely to receive CI Increased difficulty obtaining hearing aids, educational services 225 In Closing Pediatric audiologywe own it and need to advocate for research, development and maintenance of excellence in programs EHDI Early Intervention Licensure

Practice Use evidence-based literature to guide decision-making (not marketing) Work in a trans-disciplinary fashion Be active in state licensure, professional organizations Be active in systems that impact Services we provide Patients/families that benefit from our services Be active in state and national professional organization(s) Be politically aware and active Enjoy your kids!

227 Wish-List Insurance will cover hearing aids for all children Every family has the opportunity to meet another family that (1 of each) Has a HOH child Has a deaf child Has a Deaf child Family is Deaf

Has a child with a CI Has a child who successfully wears hearing aids in school, uses FM, has friends and is socially and educationally successful 228 Wish-List part 2 Every family can observe A residential Deaf School A successful auditory-oral (LISL) program Audiologists will refer infants and children to pediatric

centers when they dont have equipment and protocols And insurance would pay reasonable reimbursement for evaluations And repeat evaluations And insurance would NOT provide infant/pediatric hearing aids from on-line or OTC sites 229 What I Fear Pediatricians will cancel NBHS in the hospital and do

them in their offices Families without insurance coverage will seek out and use OTC devices Insurance coverage for hearing aids for children will be provided by on-line sites NBHS programs will increasingly be run by equipment manufacturing companies without independent oversight As state/fed budgets shrink, will EHDI survive? 230 What Can You Do? Be aware of challenges in your community, state

Be confident in reporting clear malpractice to state licensing board Ethics: Practice according to ethics principles Participate in systems-changes Be a change-agent rather than accepting the status quo Professional activities Attend

your state licensing board meetings Volunteerstate and national organizations Run for state office/national office 231 Practice to the TOP of Your Profession Pediatric hearing aidslifelong impacts Include parent-counseling at every visit Connect with school audiologist and teacher Counsel parents about language-stimulation Narrating activities

Reading Re-phrasing and reinforcing all attempts at speech Reduce screen time (ALL screens) 232 SLPAHADB, ABA, PASC Very important to stay tuned to what the board is doing Governs HOW and IF you can practice

Governs what you can and cannot do ABA is an important credential Do you have the CCC? Why? ABA is equivalent And is undergoing reconstruction to strengthen its value

PASC Specialty certification Examination-based Identifies audiologists who specialize in Pediatric Audiology 233 AND Your State Organization California Academy of Audiology AAA does a lot Only the state organization can be responsive to local needs

Ask not what your state organization can do for you ask what YOU can do for your state organization! 234 Parent EducationIn a Perfect World Forget the childbirth classes Prepare parents for newborn hearing screening Audiologist to counsel all parents after failed screen re importance of follow-up Work with parents to learn, know, practice Language stimulation

Reading to Baby Singing to Baby 235 Parent Resources Hands and Voices A Resource Guide for Parents of Infants and Toddlers Who Are Deaf or Hard of Hearing California Department of Education

2013 Audiologists Best Path Audiologists own NBHS, EHDI Audiologists who practice according to standards of care Audiologists who seek and earn PASC Best practices research (independent) High-quality hearing aids available to ALL children. Parents have easy access to both Deaf families and families with a child who is fully-mainstreamed in order

to be fully-informed about what their future looks like 237 Conclusions Early diagnosis and early excellent treatment leads to best outcomes Provision of audiology services by an experienced pediaudiologist Audiologists role is important, but not as important as the parent-caregivers roles Evidence (independent, published in peer-reviewed

literature) must undergird our clinical practices If we dont verify, and validate, our pediatric amplification choices, are we better than OTC?

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