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Early Hearing Detection and Intervention – The Role of the Primary Care Physician AAP CME Teleconference, Part I October 15, 2003.

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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

3 Hearing Loss Characterized broadly by degree, configuration and type,
Degree: amount of hearing loss in relationship to normal auditory function Configuration: overall ‘shape’ or pattern of the hearing loss as displayed on the conventional audiogram in dB HL as a function of frequency Type: site (location) of the auditory disorder

4 Degree: Categories of HL
Normal Borderline (Minimal) Mild Moderate Moderate-severe Severe Profound -10 to 15 dB HL 16 to 25 dB HL 26 to 40 dB HL 41 to 55 dB HL 56 to 70 dB HL 71 to 90 dB HL > 90 dB HL New 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 function Configuration: overall ‘shape’ or pattern of the hearing loss as displayed on the conventional audiogram in dB HL as a function of frequency Type: site (location) of the auditory disorder

6 Hearing Level (HL) in dB
Configuration: Sloping High frequency thresholds >20 dB poorer than low frequency Frequency in Hz Hearing Level (HL) in dB Stach, 1998

7 Hearing Loss Characterized broadly by degree, configuration and type,
Degree: amount of hearing loss in relationship to normal auditory function Configuration: overall ‘shape’ or pattern of the hearing loss as displayed on the conventional audiogram in dB HL as a function of frequency Type: 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 ear Sound 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 cochlea Differing impact on speech perception depending on degree and configuration of hearing loss Multiple audiometric configurations Any degree of hearing loss

10 Types of Hearing Loss: Mixed
Both sensory component and overlying conductive component Example: child with sensory loss who experiences OME

11 Types of Auditory Disorders:
Neural Outer ear, middle ear and cochlea (OHCs) intact Deficit in neural transmission (auditory neuropathy) Central Conductive, sensory & neural pathway intact Processing deficit at higher levels of the central nervous system

12 Hearing Loss: Characterized by Ear(s) Affected
Bilateral Unilateral (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 Rate Well Baby Nursery 1 per 1000 NICU 10 per 1000 Total population 2-3 per 1000 # infants ident annually US 8,000-16,000 Average 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 HL Craniofacial anomalies including the pinna and ear canal In utero infections including CMV, herpes, toxoplasmosis and rubella Family history of permanent HL

16 Most Common Specific Environmental Risk Causes
CMV > 1 risk factor Meningitis Perinatal Asphyxia Prematurity < 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 HL In-utero infections Postnatal infections Neonatal indicators such as ECMO Neurodegenerative disorders Head trauma Recurrent 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 50 50% Genetic 30% syndromes (>300) 30 20% >75 genes ident 20 ½ are GJB2 – Connexin 26

21 Genetic Causes Single gene Connexin 26
Gene + environment Mitochondrial + ototoxic Gene + gene Gene + other gene

22 The Connexin 26 Gene It is estimated that % of all autosomal recessive congenital deafness may to due to mutations in th3e Cx-26 gene on chromosome 13q11-q12 The 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 Testing Obtaining an adequate sample for DNA testing is now quite easy Bilateral 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

25 Otoacoustic Emissions (OAE)
By-product of the active processing of healthy OHC Recording of an OAE Indicates healthy OHCs (cochlea) Presence highly correlated with normal hearing sensitivity or no greater than a mild hearing loss Sensorineural 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 cochlea When 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: Quick Inexpensive Frequency-specific Identifies cochlear and conductive losses: mild-mod? Pass-refer screening devices available EOAE Disadvantages: Sensitive to ear canal and middle ear conditions Sensitive to noise (internal & external) Cannot identify neural disorders including auditory neuropathy High fail rates in some programs.

30 Device Options for OAE Screening
Types Handheld Portable screening devices PC-based hybrids PC-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 OAE ABR Disadvantages: Test time Disposable costs Infant state/myogenic artifact Requires electrode prep, placement & removal Click can miss unusual configurations of HL

33 Examples of screening ABR technology

34 In-Hospital Screening: Two Technology Protocol
OAE + ABR Advantages: Low fail rate Depending on test order, identifies cochlear, neural and conductive losses Reduced effects of noise Pass-refer screening devices available for both technologies OAE + ABR Disadvantages: Time Equipment 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 in language delays developmental skill delays, and behavior problems. Subsequent delays in literacy, and academic performance

38 Reading Comprehension Scores of Hearing and Deaf Students
Grade Equivalents Age in Years Deaf Children in America 1986

39 Unilateral or Mild Loss
50% of children either repeat a grade or need resource support in school Increased 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 m Moeller D/HH children with early ident - better outcomes Yoshinago-Itano - better scores at 36 m if ident <6 m Early “Early Intervention” is better !!!!

42 AAP & JCIH Recommendations Components of EHDI Programs in the US
Universal Newborn Hearing Screening< 1 m Effective Tracking and Follow-up as a part of the Public Health System Appropriate and Timely Diagnosis of the HL < 3m Prompt Enrollment in Appropriate EI < 6m All infants will have a medical home

43 Importance of Diagnostic Audiologic Confirmation of Hearing Loss

44 Gravel, 2000; Gravel & Hood, 1998

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 or equipment for assessing infants & children should establish consortial arrangements with 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 Screening Audiology Parent Groups Mental Health Primary Provider Child/Family ENT 3rd Party Payors Deaf Community Interpreters EI Therapists Genetics Deaf Services

48 The Medical Workup Complete prenatal & perinatal hx Family Hx of onset of HL < age 30 Physical for stigmata, ear tabs, cleft palate, cardiac, skeletal, microcephaly Refer to ENT / CT of temporal bones Refer to Genetics and Opthalmology Other: CMV, EKG

49 What are some of the questions to ask ?
Antenatal history- maternal illness during the pregnancy or delivery Neonatal complications, prematurity, jaundice, asphyxia, assisted ventilation, ECMO

50 Examination for Causes
Evaluate for dysmorphic features, minor and major stigmata and syndromes Other anomalies – visual, facial, endocrine, cardiac, kidney, hair, and skin Particular 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 loss Families 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 patients Some 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 schedules There 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

59 When are Hearing Aids Fit?
What is the Goal of Hearing Aid Fitting Ensure 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, 1996 When are Hearing Aids Fit? For newborns identified by UNHS, fit hearing aids within one month of confirmation of hearing loss, preferably before 4 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 cases Some children with unilateral hearing loss, and minimal HL Need based on audiogram plus additional information: Family choice other disabilities/on-going medical issues performance PWG, 1996

61 Pre-Selection - Children’s Hearing Aids Should Include:
Binaural fitting unless clear contraindication Behind-the-ear style of choice PWG, 1996

62 Pre-Selection - Children’s Hearing Aids Should Include:
Flexible response characteristics Compression to limit overall output sound pressure level of the hearing aid (safety and comfort) PWG, 1996

63 Directional Microphone Technology
Improves directional hearing abilities Hear 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 setting Allows switching between memories for various listening situations

65 Digital Signal Processing (DSP) Technology
Newer hearing aids that use digital processing of incoming No studies, to date, demonstrate better performance of digital instruments over conventional hearing aids in either adults or children Considerably more expensive

66 What Other Features Should Children’s Hearing Aids Include?
Safety-related features: tamper resistant battery compartment volume control Physical fit (size) and color Earmolds made of soft material PWG, 1996

67 How Do We Fit Hearing Aids to Infants & Young Children?
Use computerized prescriptive fitting procedure Requires only minimal threshold data to begin, so fitting can begin early. From: Seewald, 2003

68 Prescriptive Fitting Procedure
Incorporate simple probe microphone measurements Allow audiologist to: Pre-select the response characteristics of the hearing aids Refine or ‘individualize’ the hearing aid for the unique acoustic characteristics of each infant’s ear Verify that the prescriptive frequency-gain and output targets have been achieved From:

69 Goals of FM Fitting Child hears primary talker at level that is consistently audible above the background noise Child able to monitor his/her own voice Child hears voices of others who are not wearing the FM microphone Lewis 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 loss Are learning language for the first time Have greater difficulty understanding speech in background noise than adults Require a more audible (intense) signal than adults to understand speech

71 Fail Neonatal Screening
Fail Outpatient screening FS-ABR,EOAE AC & BC, tymps Repeat FS-ABR, EOAE,RECD with insert, for HA selection, tymps Behavioral & tymps (with mold to insert coupling) 1m 2m 3m 4m 5m 6m 7m 8m 9m 10m 11m 12m Counseling Counseling; medical/ENT referral Begin processes for HA procurement Mold impressions, EI Program Observe auditory behaviors & tymps HA Fitting HA Check & (molds) Review habilitation, language milestones RECD, HA modification, (molds) Review habilitation, language milestones Behavioral & tymps (with mold-to-insert coupling) Behavioral & tymps (with mold-to-insert coupling) RECD, HA modification, (molds) Review habilitation, language milestones Behavioral & tymps (with mold-to-insert coupling) RECD, HA Check, (molds) Validation measures, language milestones Set habilitation goals for year 2 Gravel, 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 consortial arrangements with centers that do. PWG, 1996

73 Considerations in Determining the Appropriateness of Cochlear Implantation including Risks, Benefits & Timing Gravel

74 What are the Candidacy Criteria for a Cochlear Implant?
From: Limited benefit from conventional amplification following a minimum of 3-6 months use May be sooner following deafness from meningitis Profound hearing loss: 12-18 months Severe-to-Profound hearing loss >18 months Motivated, involved family with child enrolled in an intervention program emphasizing spoken (oral-aural) language development

75 Cochlear Implants Acoustic signal picked up by microphone located in headset worn at ear level Cord carries sound from microphone to a speech processor Speech processor digitizes sound into coded signals Coded signals sent up to the transmitting coil Coil sends coded signals as FM radio signals to CI under the skin CI delivers electrical energy to the electrode array inserted into the cochlea Electrodes along the array stimulate remaining nerve fibers in cochlea Cochlear Corp. 2002

76 Selecting a Cochlear Implant Center
Experienced cochlear implant team Audiologist, speech-language pathologist, surgeon Others: educator of the deaf, psychologist, social worker Comprehensive 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 development Offers intervention program and continued Follow-up for changes in cochlear implant mapping

77 Benefits Similar 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 milestones Unintelligible speech Uncharacteristic voice patterns Child turns TV volume very loud Child 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 him Boys develop speech much later than girls Twins always have language delays Grandma says that her Dad did not speak until he was 3 years old. She has great motor milestones I know he hears because he gets upset every time I turn on the vacuum.

82 Assessment of language delay
Administer a speech language screen Check middle ear status for MEE: Rx MEE for > 3 m refer to otolaryngology If receptive /expressive delay refer back to audiology for repeat diagnostic Refer to early intervention for speech language therapy Assess for other possible dx: PDD, autism Continue 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 loss Regardless of screening outcome, if parent is concerned regarding hearing or speech-language development, child should be referred for audiologic evaluation

85 Question-and-Answer

86 Early Hearing Detection and Intervention CME Teleconference Series, Part II November 12, :00 – 1:00 pm, Central Standard Time Topics: 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

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