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 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
Gravel

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
Gravel

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
Gravel

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