Presentation on theme: "Early Hearing Detection and Intervention – The Role of the Primary Care Physician AAP CME Teleconference, Part I October 15, 2003."— Presentation transcript:
1 Early Hearing Detection and Intervention – The Role of the Primary Care Physician AAP CME Teleconference, Part I October 15, 2003
2 Types of Congenital/Early Onset Hearing Loss and Why It Is Important to Know the Difference Gravel
3 Hearing Loss Characterized broadly by degree, configuration and type, Degree: amount of hearing loss in relationship to normal auditory functionConfiguration: overall ‘shape’ or pattern of the hearing loss as displayed on the conventional audiogram in dB HL as a function of frequencyType: site (location) of the auditory disorder
4 Degree: Categories of HL NormalBorderline (Minimal)MildModerateModerate-severeSevereProfound-10 to 15 dB HL16 to 25 dB HL26 to 40 dB HL41 to 55 dB HL56 to 70 dB HL71 to 90 dB HL> 90 dB HLNew York State Department of Health
5 Hearing Loss Characterized broadly by degree, configuration and type, Degree: amount of hearing loss in relationship to normal auditory functionConfiguration: overall ‘shape’ or pattern of the hearing loss as displayed on the conventional audiogram in dB HL as a function of frequencyType: site (location) of the auditory disorder
6 Hearing Level (HL) in dB Configuration: Sloping High frequency thresholds >20 dB poorer than low frequencyFrequency in HzHearing Level (HL) in dBStach, 1998
7 Hearing Loss Characterized broadly by degree, configuration and type, Degree: amount of hearing loss in relationship to normal auditory functionConfiguration: overall ‘shape’ or pattern of the hearing loss as displayed on the conventional audiogram in dB HL as a function of frequencyType: site (location) of the auditory disorder
8 Types of Hearing Loss: Conductive Reduction of air-conductive sound delivered to the normal cochlea during transmission through a disordered outer ear and/or middle earSound reaching cochlea attenuated to some degree (in OME or debris ~25 dB HL; in complete atresia, maximally ~60 dB HL)Excellent speech perception when incoming acoustic signal is sufficiently intense
9 Type of Hearing Loss: Sensory Damage to outer or outer and inner hair cells of the cochleaDiffering impact on speech perception depending on degree and configuration of hearing lossMultiple audiometric configurationsAny degree of hearing loss
10 Types of Hearing Loss: Mixed Both sensory component and overlying conductive componentExample: child with sensory loss who experiences OME
11 Types of Auditory Disorders: NeuralOuter ear, middle ear and cochlea (OHCs) intactDeficit in neural transmission (auditory neuropathy)CentralConductive, sensory & neural pathway intactProcessing deficit at higher levels of the central nervous system
12 Hearing Loss: Characterized by Ear(s) Affected BilateralUnilateral (in one ear only)
13 What are the major genetic and environmental causes of congenital hearing loss ? Vohr
14 Characteristics of Children with Hearing Loss Site RateWell Baby Nursery 1 per 1000NICU 10 per 1000Total population 2-3 per 1000# infants ident annually US 8,000-16,000Average career pediatrician patients
15 JCIH Risk Factors for Infants birth to 28 days Any illness requiring admission to the NICU for > 48 hours.Stigmata associated with a syndrome known to include SNHL or conductive HLCraniofacial anomalies including the pinna and ear canalIn utero infections including CMV, herpes, toxoplasmosis and rubellaFamily history of permanent HL
16 Most Common Specific Environmental Risk Causes CMV > 1 risk factorMeningitis Perinatal AsphyxiaPrematurity < 1500 g Ototoxic medications
17 Some Infants pass their hearing screen and have late onset hearing loss Some of these infants have a risk factor and some have no risk factor known to the family or physician.
18 Risk indicators from 29 d to 2 years of age Stigmata or Syndrome associated with HLIn-utero infectionsPostnatal infectionsNeonatal indicators such as ECMONeurodegenerative disordersHead traumaRecurrent or persistent OM
19 Risk Indicators obtained from the family Parent or caregiver concern regarding hearing, speech, language, or developmental delay (parent concern has been shown to be a good predictor).Family history of permanent HL in first or second degree relatives with onset by 30y or age.
20 Causes of Permanent Hearing Loss in 100 Infants 50% Environmental 5050% Genetic30% syndromes (>300) 3020% >75 genes ident 20½ are GJB2 – Connexin 26
21 Genetic Causes Single gene Connexin 26 Gene + environment Mitochondrial + ototoxicGene + gene Gene + other gene
22 The Connexin 26 GeneIt is estimated that % of all autosomal recessive congenital deafness may to due to mutations in th3e Cx-26 gene on chromosome 13q11-q12The Cx gene produces a gap junction protein expressed between the outer hair cells and supporting cells and is involved with auditory transduction.The estimated carrier frequency in the general population is 1 in (Estivill et al, 1998)
23 Genetic TestingObtaining an adequate sample for DNA testing is now quite easyBilateral buccal smears with a Q tip provide adequate genetic material for testing.The follow-up genetic counseling is key to the success of genetic testing.
24 New technologies used in hearing screening Gravel
25 Otoacoustic Emissions (OAE) By-product of the active processing of healthy OHCRecording of an OAEIndicates healthy OHCs (cochlea)Presence highly correlated with normal hearing sensitivity or no greater than a mild hearing lossSensorineural hearing losses of greater than about 30 dB HL generally result in absent OAE.Since recording OAE requires normal forward and backward transmission of energy to and from the cochlea, conductive hearing loss associated with middle or outer ear abnormality can result in absent OAE
26 Spontaneous OAEs Evoked OAEs Types of OAE Transient OAE (TEOAE, TOAE, or click-OAE: COAE)Distortion Product (DPOAE)
27 TEOAE Elicited by transients or brief stimuli Clicks: rapid onset, broadband stimulus containing energy from low through high frequencies (i.e., across speech frequency range)Tone bursts: more frequency-specific
28 Distortion-Product OAE (DPOAE) Occur as a result of nonlinear processes of the cochleaWhen 2 tones are presented to the cochlea, “distortion” occurs in the form of other tones (harmonics) that are not present in the 2 eliciting tones
29 Screening Technologies - Neonates Evoked Otoacoustic Emissions EOAE Advantages:QuickInexpensiveFrequency-specificIdentifies cochlear and conductive losses: mild-mod?Pass-refer screening devices availableEOAE Disadvantages:Sensitive to ear canal and middle ear conditionsSensitive to noise (internal & external)Cannot identify neural disorders including auditory neuropathyHigh fail rates in some programs.
30 Device Options for OAE Screening TypesHandheldPortable screening devicesPC-based hybridsPC-based clinical systems
31 Auditory Brainstem Response (ABR) Recording (through surface electrodes) of the micro-volt electrical activity generated by the cochlea and transmitted by the auditory nerve and brainstem pathways in response to brief clicks.Clicks produce a synchronized response from neural fibers; a tracing of the response is a series of waves
32 Screening Technologies – Neonates Screening (Automated) Auditory Brainstem Response (SABR or AABR) ABR Advantages:Identifies cochlear, conductive and neural losses: ? mild-mod.Pass-refer screening devices. Some test both ears simultaneously.Relatively insensitive to transient ear canal, middle ear & external noise.? Lower fail rate than OAEABR Disadvantages:Test timeDisposable costsInfant state/myogenic artifactRequires electrode prep, placement & removalClick can miss unusual configurations of HL
34 In-Hospital Screening: Two Technology Protocol OAE + ABR Advantages:Low fail rateDepending on test order, identifies cochlear, neural and conductive lossesReduced effects of noisePass-refer screening devices available for both technologiesOAE + ABR Disadvantages:TimeEquipment and disposable costs
35 Why is diagnostic confirmation by an audiologist skilled in evaluating infants and young children important? Vohr
36 A failed hearing screen may be a false positive or an actual fail A failed hearing screen may be a false positive or an actual fail. These 2 findings need totally different management.Therefore, an accurate diagnosis of normal hearing, SNHL, auditory neuropathy or conductive loss is important as soon as possible to minimize parental stress and to decrease the time interval between screen fail and starting treatment.Parents of late identified children have feelings of guilt and frustration.
37 Early Identification of Hearing Loss is Important because Delayed identification, even of mild HL results inlanguage delaysdevelopmental skill delays, andbehavior problems.Subsequent delays in literacy, and academic performance
38 Reading Comprehension Scores of Hearing and Deaf Students Grade EquivalentsAge in YearsDeaf Children in America 1986
39 Unilateral or Mild Loss 50% of children either repeat a grade or need resource support in schoolIncreased behavioral and linguistic problems compared to hearing controls.Bess F, Pediatrics 1984
40 Early “Early Intervention” for Hearing Loss is Important because There are dramatic benefits associated with early identification and intervention for hearing loss before 6 months of age.Children identified and receiving services < 6 m have larger vocabularies, better comprehension and better expressive language than children identified > 6 m.
41 Are Interventions Available to Improve Outcome? White - language scores of sev to profound (14 vs 26 m)Apizzo - better language scores at 4 if ident <2 mMoeller D/HH children with early ident - better outcomesYoshinago-Itano - better scores at 36 m if ident <6 mEarly “Early Intervention” is better !!!!
42 AAP & JCIH Recommendations Components of EHDI Programs in the US Universal Newborn Hearing Screening< 1 mEffective Tracking and Follow-up as a part of the Public Health SystemAppropriate and Timely Diagnosis of the HL < 3mPrompt Enrollment in Appropriate EI < 6mAll infants will have a medical home
43 Importance of Diagnostic Audiologic Confirmation of Hearing Loss Gravel
45 “Facilities that lack the expertise or “Audiologists should have experience with the assessment of infants & children with HL and the knowledge and equipment necessary for use with current pediatric assessment methods”.“Facilities that lack the expertise orequipment for assessing infants & childrenshould establish consortial arrangementswith those that do”.Pediatric Working Group, 1996
46 What are the components of the medical home work-up for children with congenital hearing loss? Vohr
47 EHDI and the Medical Home Hospital ScreeningAudiologyParent Groups Mental HealthPrimary Provider Child/FamilyENT3rd Party PayorsDeaf CommunityInterpretersEI TherapistsGeneticsDeaf Services
48 The Medical WorkupComplete prenatal & perinatal hxFamily Hx of onset of HL < age 30Physical for stigmata, ear tabs, cleft palate, cardiac, skeletal, microcephalyRefer to ENT / CT of temporal bonesRefer to Genetics and OpthalmologyOther: CMV, EKG
49 What are some of the questions to ask ? Antenatal history- maternal illness during the pregnancy or deliveryNeonatal complications, prematurity, jaundice, asphyxia, assisted ventilation, ECMO
50 Examination for Causes Evaluate for dysmorphic features, minor and major stigmata and syndromesOther anomalies – visual, facial, endocrine, cardiac, kidney, hair, and skinParticular attention to the head and neck. HL may be associated with abn. pinna, atresia or stenosis of the ear canal, ear tags, and bony growths in the ear canal.Cleft lip and palate may have middle ear fluid
51 What to ask about family history ? Is there a family hx of onset of permanent HL < 30 years of age ( over 3 generations)Are there other family members with syndromes or anomalies ?
52 Which families may benefit from a Genetic Referral ? All families with a child with congenital or late onset hearing lossFamilies of a child with stigmata or a syndrome will benefit from the information.Families with a child with non-syndromic HL want to know the cause ?Some parents who are culturally deaf wish information on the risk or cause of HL.
53 When to refer to Ophthalmology First- Follow periodicity schedule for all patientsSome syndromes with permanent HL have specific eye findings such as heterochromia in Wardenburgs.In Ushers the child is at risk of late onset vision loss secondary to retinitis pigmentosa. (If sign is the primary communication mode they will obviously have a problem.
54 When to get an EKG ?This is ordered to rule out long QT ( Jervell and Lange-Nielsen )syndrome.This syndrome may manifest itself with apnea, passing out episodes, or a history of sudden death in a relative.
55 Should there be additional workup ? This needs to be individualized.For example, A NICU infant with IUGR should have an MRI and TORCH titers to rule out CMV, toxoplasmosis etc.
56 Children with Cochlear Implants and Meningitis The incidence of meningitis is higher among children with cochlear implants.The incidence of Streptococcus pneumoniae meningitis was 30 times the incidence in the general population. NEJM, July 2003
57 Recommendations for children with Cochlear Implants Children < age 2 years should get pneumococcal conjugate vaccine (Prevnar) according to the routine schedulesThere are guidelines for older children (CDC)Children with cochlear transplants should be monitored and treated promptly for any bacterial infections.
58 Amplification Choices for Families including Hearing Aids, FM Systems and Cochlear Implantation Gravel
59 When are Hearing Aids Fit? What is the Goal ofHearing Aid FittingEnsure children receive full-time use and consistent audibility of the speech signal at safe and comfortable listening levels as soon as hearing loss is confirmed.PWG, 1996When are Hearing Aids Fit?For newborns identified by UNHS,fit hearing aids within one month ofconfirmation of hearing loss, preferably before4 months and no later than 6 months of age.JCIH, 2000
60 Which infants are candidates for amplification? Significant, permanent bilateral peripheral hearing loss.Mild hearing loss in some casesSome children with unilateral hearing loss, and minimal HLNeed based on audiogram plus additional information:Family choiceother disabilities/on-going medical issuesperformancePWG, 1996
61 Pre-Selection - Children’s Hearing Aids Should Include: Binaural fitting unless clear contraindicationBehind-the-ear style of choicePWG, 1996
62 Pre-Selection - Children’s Hearing Aids Should Include: Flexible response characteristicsCompression to limit overall output sound pressure level of the hearing aid (safety and comfort)PWG, 1996
63 Directional Microphone Technology Improves directional hearing abilitiesHear parent speaking from front; noise from shopping mall at back of child reduced.
64 Multiple Memory Hearing Instruments Allows storage of more than one electroacoustic response settingAllows switching between memories for various listening situations
65 Digital Signal Processing (DSP) Technology Newer hearing aids that use digital processing of incomingNo studies, to date, demonstrate better performance of digital instruments over conventional hearing aids in either adults or childrenConsiderably more expensive
66 What Other Features Should Children’s Hearing Aids Include? Safety-related features: tamper resistantbattery compartmentvolume controlPhysical fit (size) and colorEarmolds made of soft materialPWG, 1996
67 How Do We Fit Hearing Aids to Infants & Young Children? Use computerized prescriptive fitting procedureRequires only minimal threshold data to begin, so fitting can begin early.From: Seewald, 2003
68 Prescriptive Fitting Procedure Incorporate simple probe microphone measurementsAllow audiologist to:Pre-select the response characteristics of the hearing aidsRefine or ‘individualize’ the hearing aid for the unique acoustic characteristics of each infant’s earVerify that the prescriptive frequency-gain and output targets have been achievedFrom:
69 Goals of FM FittingChild hears primary talker at level that is consistently audible above the background noiseChild able to monitor his/her own voiceChild hears voices of others who are not wearing the FM microphoneLewis et al., 1998 (Phonak AG)
70 Infants and young children with congenital/early onset hearing loss Why Does the Acoustic Climate (of the Home, Daycare Setting, etc.) Need to be Considered?Infants and young children with congenital/early onset hearing lossAre learning language for the first timeHave greater difficulty understanding speech in background noise than adultsRequire a more audible (intense) signal than adults to understand speech
71 Fail Neonatal Screening Fail Outpatient screeningFS-ABR,EOAE AC & BC, tympsRepeat FS-ABR, EOAE,RECD withinsert, for HA selection, tympsBehavioral & tymps(with mold to insert coupling)1m2m3m4m5m6m7m8m9m10m11m12mCounselingCounseling; medical/ENT referralBegin processes for HA procurementMold impressions, EI ProgramObserve auditory behaviors & tympsHA FittingHA Check & (molds)Review habilitation, language milestonesRECD,HA modification, (molds)Review habilitation, language milestonesBehavioral & tymps(with mold-to-insert coupling)Behavioral & tymps(with mold-to-insert coupling)RECD,HA modification, (molds)Review habilitation, language milestonesBehavioral & tymps(with mold-to-insert coupling)RECD, HA Check, (molds)Validation measures, language milestonesSet habilitation goals for year 2Gravel, 2000
72 Qualifications of Audiologists and Facilities No facility should fit hearing aids to children if it lacks the equipment for behavioral, electrophysiologic, electroacoustic, and probe-microphone/real-ear evaluation.Facilities that lack the expertise or equipment should establish consortialarrangements with centers that do.PWG, 1996
73 Considerations in Determining the Appropriateness of Cochlear Implantation including Risks, Benefits & TimingGravel
74 What are the Candidacy Criteria for a Cochlear Implant? From:Limited benefit from conventional amplification following a minimum of 3-6 months useMay be sooner following deafness from meningitisProfound hearing loss:12-18 monthsSevere-to-Profound hearing loss>18 monthsMotivated, involved family with child enrolled in an intervention program emphasizing spoken (oral-aural) language development
75 Cochlear ImplantsAcoustic signal picked up by microphone located in headset worn at ear levelCord carries sound from microphone to a speech processorSpeech processor digitizes sound into coded signalsCoded signals sent up to the transmitting coilCoil sends coded signals as FM radio signals to CI under the skinCI delivers electrical energy to the electrode array inserted into the cochleaElectrodes along the array stimulate remaining nerve fibers in cochleaCochlear Corp. 2002
76 Selecting a Cochlear Implant Center Experienced cochlear implant teamAudiologist, speech-language pathologist, surgeonOthers: educator of the deaf, psychologist, social workerComprehensive program covering eligibility, surgery, device activation, and long-term habilitation.Multi-disciplinary, family centered approach.Knowledgeable regarding deafness, child development, and speech, language and auditory developmentOffers intervention program and continuedFollow-up for changes in cochlear implant mapping
77 BenefitsSimilar to early amplification provision, children implanted at early ages with more experience tend to do better than older children who receive implants after greater period of deafness
78 Listening to Parental Concern About Delayed Language Development Gravel & Vohr
79 Listening to parent concern about language development Parent concerns about hearing, speech, language, or developmental delays are strong predictors of an actual problem.Providers must avoid statements like “Babies develop at different rates. Lets take another look in about 6 months”
80 Clinical signs of Hearing Loss Delayed early language milestonesUnintelligible speechUncharacteristic voice patternsChild turns TV volume very loudChild only responds to loud sounds/words or in a very quiet environment
81 Myths about hearing and early speech language delay We don’t have to worry because:His older brothers and sisters talk for himBoys develop speech much later than girlsTwins always have language delaysGrandma says that her Dad did not speak until he was 3 years old.She has great motor milestonesI know he hears because he gets upset every time I turn on the vacuum.
82 Assessment of language delay Administer a speech language screenCheck middle ear status for MEE: RxMEE for > 3 m refer to otolaryngologyIf receptive /expressive delay refer back to audiology for repeat diagnosticRefer to early intervention for speech language therapyAssess for other possible dx: PDD, autismContinue to follow-up on speech/language
83 It is important to respond to concerns about language immediately ! Most children with delays of speech and language respond to appropriate medical, audiologic, and educational interventions.A successful early screening, identification, and intervention program will ultimately permit every child with HL to develop to his/her potential.
84 All in-hospital screening failures should receive follow-up Failure rate at discharge, once high has decreased to 2% or less.Probability 1 in 5 that infant who fails NHS has hearing lossRegardless of screening outcome, if parent is concerned regarding hearing or speech-language development, child should be referred for audiologic evaluation
86 Early Hearing Detection and Intervention CME Teleconference Series, Part II November 12, :00 – 1:00 pm, Central Standard TimeTopics:Implementing newly developed AAP guidelines; important referrals; the role of early intervention; primary care’s role in coordination of services; parenting issues and reactions; knowledge of and support for communication choices; cost and reimbursement issues; and national resources.Faculty: Al Mehl and Mary Pat Moeller