1. Diagnostic Pediatric Audiology from Birth to Intervention
Karen M. Ditty, M.S.
NCHAM
Antonia Brancia Maxon, Ph.D.
NECHEAR

2. Timely and Appropriate Diagnosis of Hearing Loss
Newborns screened by 1 month
Infants with hearing loss diagnosed by 3 months
Amplification use begins within 1 month of diagnosis
Benchmarks (JCIH, 2000)

3. Timely and Appropriate Diagnosis of Hearing Loss
Infants enrolled in family-centered early intervention by 6 months
Ongoing audiological management - not to exceed 3 month intervals
Professionals working with these infants are knowledgeable about all aspects
Benchmarks (JCIH, 2000)

4. Newborns screened by 1 month
Approximately 90% of all newborns in the United States have their hearing screened at birth
The number of infants referred for diagnostic audiological evaluations has dramatically increased .

5. Infants with hearing loss diagnosed by 3 months
Progress has been made however it is affected by
Testing site may influence age of diagnosis
Goal is often met in hospital clinics
Less likely in non-hospital centers
Geographic access to services may influence age of diagnosis
Rural communities are less likely to meet the goal
Although this goal is often easily met in hospital based audiological clinics, Audiologists in non hospital based centers or rural sites are having trouble achieving this goal.

6. Impediments to Lowering Diagnostic Age
Audiologists lack experience with very young infants
uncomfortable making the final diagnosis.
Defer to and refer for second opinion
Facilities do not have the equipment needed to assess very young infants.
Frequency specific ABR
AC and BC ABR
High frequency tympanometry
Many audiologists are uncomfortable with the interpretation of their test findings and will put off a diagnosis or refer to a hospital based center for a 2nd opinion.
There are currently not enough audiologists in the United States that have the training or expertise to work with the “newborn”.
May mention that there is now a push for Pediatric Specialty Certification in Audiology

7. Impediments to Lowering Diagnostic Age
Audiologists are not familiar with clinical protocols necessary for making accurate diagnosis with very young infants.
Do not have “norms”
Cannot “read” ABR for this population
Inadequate number of audiologists with pediatric expertise
No academic training to work with very young infants
No clinical training to work with very young infants
Many audiologists are uncomfortable with the interpretation of their test findings and will put off a diagnosis or refer to a hospital based center for a 2nd opinion.
There are currently not enough audiologists in the United States that have the training or expertise to work with the “newborn”.
May mention that there is now a push for Pediatric Specialty Certification in Audiology

8. Aids to Lowering the Age of Diagnosis
Although there are no national protocols or standards many states have guidelines for their audiologists.
These guidelines can be obtained via the following link on the NCHAM website
For many audiologists, ABR test protocols for the adult population are well known and accepted. However, when addressing infants, protocols and practices need to be revised. Eventually, standardized infant protocols will be developed and will become widely accepted. Let’s look at this particular benchmark that JCIH set up and discuss what is the appropriate infant diagnostic test battery

9. Aids to Lowering the Age of Diagnosis
Audiologists can get training through continuing education provided by national associations
NCHAM audiology training
Pediatric Diagnostics
Covers the initial diagnostic procedure
Pediatric Amplification Fitting
Covers behavioral assessment, hearing aid selection, fitting and validation and cochlear implants
For many audiologists, ABR test protocols for the adult population are well known and accepted. However, when addressing infants, protocols and practices need to be revised. Eventually, standardized infant protocols will be developed and will become widely accepted. Let’s look at this particular benchmark that JCIH set up and discuss what is the appropriate infant diagnostic test battery

10. Pediatric Audiologist
Have the appropriate audiological equipment and protocols for testing newborns and young infants.
Can evaluate a child’s hearing within a short period of time after being contacted for an appointment.
Specializes in working with infants and young children.
Wants to work with infants and young children.
Has worked with Part C program in their state
How can you determine if you can meet the time-consuming and unique needs of this population, including their families?
There can be many answers, but we have put together a few criteria we think make up a Diagnostic Pediatric Audiologist.

11. Pediatric Audiologist
Is familiar with the procedures of the Part C system, including IFSP development and procedures for acquiring hearing aids or assistive technology.
If dispenses hearing aids:
can make earmolds,
has loaner hearing aids available
provides hearing aids on a trial basis
has resources to repair hearing aids quickly

12. Pediatric Audiologist
Is willing to review the test results of the audiological evaluation face to face with the family, respecting the Cultural Differences of family units.
Is willing to provide a comprehensive written report with a copy of the test findings in a timely manner.
Is willing to continue to explain results at follow-up evaluations

13. Pediatric Diagnostic Test Battery
Comprehensive Case History
Frequency-Specific Auditory Brainstem Response
High Frequency Probe Tone Tympanometry
Transient and/or Distortion Product Otoacoustic Emissions
Hearing aid Fitting with Real Ear Measurements
Behavioral Audiometry
Referrals
So you fit the criteria and know you could be that Pediatric Audiologist we need What about equipment?

14. Comprehensive Case History

15. Frequency Specific Auditory Brainstem Response
Air Conduction Clicks
Abrupt or rapid onset of a broad frequency bandwidth .
Greatest agreement in the Hz frequency range.
Not enough information across the frequency range
Low frequencies absent

16. Frequency Specific Auditory Brainstem Response
Tonebursts
Provides information for narrower frequency regions
Better relates to pure tone audiogram
Bone-Conducted Clicks
Should get when either the click or 500-Hz tonebursts responses are not present at expected normal levels.

17. Frequency-Specific ABR
Accuracy of pure tone threshold estimates with tone burst ABR
High correlation (>.94) for infants and older children (Stapells, et al, 1995)
90% of ABR thresholds within 20 dB of PT thresholds with most within 10 dB
audiometric configuration does not affect accuracy of match (Oates and Stapells, 1998)
Click and/or tonal stimuli can be used to determine hearing status and loss configuration.
Tone pips for frequency specific information
Clicks are Hz region
DON’T use only click - will misdiagnose

18. Frequency Specific Auditory Brainstem Response
Auditory Steady State Response (ASSR)
An electrophysiologic response, similar to ABR
Generated by rapid modulation of “carrier” pure tone amplitude or frequency.
Signal intensity can be as high as 120 dB

19. Frequency Specific Auditory Brainstem Response
Auditory Steady State Response (ASSR)
Done in conjunction with ABR Clicks, or on a separate occasions
Major advantage is it estimates severe-to-profound HL
Best used in conjunction with ABR and tone burst testing.

20. ABR (Click and Tone Burst) versus ASSR: Clinical Application
Advantages
Estimates normal hearing
thresholds
Ear-specific BC findings
Diagnosis of AN
Estimates severe to
profound HL
Disadvantages
Can’t estimate profound HL
Skilled analysis required
Limited BC intensity levels
No ear-specific BC findings
Requires sleep or sedation
ABR
ASSR
R. Ruth, 2003

21. Pediatric Sedation for ABR
Who and When
4 months to 5 years
Options
conscious sedative
mild general anesthesia
Monitoring
administered and managed by nurse
monitor O2, HR and BP
crash cart and suction available
(J. Hall, 2001)

22. Pediatric Sedation for ABR
Negative outcomes associated with
overdoses, drug interactions
non-trained personnel
injuries on the way to facility (administered at home)
drugs with long half-lives (chloral hydrate, pentobarbital)
(J. Hall, 2001)

23. Pediatric ABR summary
Air conduction measures should be done with insert earphones
Headphones can affect latency of waveform
Bone conduction measures are needed to rule out conductive loss or find conductive component.
Use B-70 bone vibrator
Use mastoid placement
Key Points to remember with Pediatric ABR
B-70 vibrator
Mastoid placement
Earlobe inverting electrodes
Alternating tone burst to minimize artifact
Slower rate (e.g., 11.1/sec) enhances Wave I
Begin near maximum intensity (50 dB nHL)
Identify Wave I in ipsi ear to verify test ear
Plot I-L function of Wave V vs AC

24. Pediatric ABR summary Use earlobe inverting electrodes
Use alternating tone burst to minimize artifact
A slower rate (e.g., 11.1/sec) enhances Wave I
Begin testing near maximum intensity (50 dB nHL)
Allows good waveform to be seen
Identify Wave I in ipsilateral ear to verify test ear
Plot I-L function of Wave V
Key Points to remember with Pediatric ABR
B-70 vibrator
Mastoid placement
Earlobe inverting electrodes
Alternating tone burst to minimize artifact
Slower rate (e.g., 11.1/sec) enhances Wave I
Begin near maximum intensity (50 dB nHL)
Identify Wave I in ipsi ear to verify test ear
Plot I-L function of Wave V vs AC

25. Pediatric ABR summary
Air conduction measures should include frequency specific tone bursts and/or ASSR as part of a battery of electrophysiological tests.
Of the audiological test battery, only an ABR can help determine an auditory neuropathy case; therefore, ASSR should not be performed alone, but as part of a battery of electrophysiological tests.
Key Points to remember with Pediatric ABR
B-70 vibrator
Mastoid placement
Earlobe inverting electrodes
Alternating tone burst to minimize artifact
Slower rate (e.g., 11.1/sec) enhances Wave I
Begin near maximum intensity (50 dB nHL)
Identify Wave I in ipsi ear to verify test ear
Plot I-L function of Wave V vs AC

26. High Frequency Probe Tone Tympanometry
Tympanometry provides information about middle ear status
add information to BC results
May be affected by conditions in very young infant’s ears
Ear canal and eardrum are very compliant
Use of high frequency probe tone (800 Hz or greater) increases reliability and accuracy in young infants.

27. Transient & Distortion Product Otoacoustic Emissions
Infants and young children with normal hearing have robust
transient evoked otoacoustic emissions (TEOAE)
distortion product otoacoustic emissions (DPOAE)
TEOAEs and DPOAEs are easily measured in infants and children.
Reflect Outer Hair Cell integrity, whereas the audiogram measure hearing. Is pre-neural, whereas the audiogram is dependent on retrocochlear pathways.
Essential component of Pediatric Diagnostic test battery

28. Middle Ear Effects on OAEs
Middle ear effusion may
obliterate emission
eliminate low frequency component
Negative middle ear pressure may
reduce amplitude, particularly in high frequencies
Complications can arise with OAEs, and the importance of the battery approach is critical when working with newborns and young infants

29. OAE Summary OAEs are objective evidence of healthy cochlear function
The vast majority of hearing impairment in the low-risk
population is a result of malfunction of the outer hair
cells
the most sensitive and vulnerable part of the
hearing mechanism tested by OAEs.
OAEs provide meaningful information when
retrocochlear lesions and/or auditory neuropathy
are a concern.

30. Amplification Assessment and Fitting
Initiate amplification process immediately after diagnosis.
Includes medical clearance
Federal regulation - ENT
Includes earmolds
overnight mailing to get within 1 week
continue to remake to avoid fitting problems

31. Pediatric amplification fitting
Does not require exhaustive audiological data
Target audiogram
Individual ear information
Ability to conduct real-ear measures
Scheduling flexibility and immediacy
Experience with functional measures of benefit

32. Real Ear to Coupler Difference Procedure (RECD)
The infant ear is smaller than an adult ear
More SPL for same input compared to adult
Differences can be as large as dB
Many hearing-aid fitting algorithms do not take these differences into account.
RECD affects estimates of
Threshold
Real-ear gain and output

33. Real ear measurement The insert phone is coupled to the earmold
The probe microphone is placed into the ear canal
The earmold is inserted into the ear
Test stimulus is presented
Total test time 5-10 minutes per ear

34. RECD
After the RECD is obtained, all hearing aid testing can be done in the test box
RECD values are entered into the hearing aid fitting program to provide a more accurate estimate of real-ear aided gain and output
The RECD will change as the child grows. A good rule of thumb is to obtain a new RECD when a new earmold is needed

35. Basic Audiological Information Used to Fit Amplification
Hearing Sensitivity
ABR frequency specific information - low, mid and high frequency
Individual ear measures: insert phones
Middle Ear Status
Tympanometry - high frequency
BC to rule out conductive loss

36. Basic Audiological Information Used to Fit Amplification
Cochlear status
ABR intensity-latency function
OAEs
Behavioral Responses
target audiogram
speech awareness

37. Behavioral Response Audiometry
Provides information about how an infant or young child uses hearing
Behavioral observation techniques can be used to give functional information
Sometimes only suprathreshold information is obtained
will get better responses to speech than tones
Can look at amplification benefit

38. Behavioral Response Audiometry
Look at amplification benefit
Need to provide speech at greater than detection level
Cannot learn language with threshold-only information
All of normal conversational level speech needs to reach child through amplification

39. Speech Sounds Range from softest to loudest speech sound = 30 dB
“th” – “ah”
Low frequencies carry suprasegmental, vowel, and voicing information.
High frequencies carry consonant, perceptual, and syntactic cues.

40. Referral to and Enrollment in Early Intervention
Know established Part C guidelines in state
Know child eligibility criteria
automatic enrollment - diagnosed condition
significant developmental delay
Know state guidelines for selecting a program

41. Enrollment in Early Intervention
Develop Individualized Family Service Plan (IFSP)
All services
speech and language development
auditory development
assistive technology
Goals and objectives
Timelines

42. Components of IFSP for I/T with Hearing Loss
Amplification provision
Parent education
Audiological monitoring
Development of auditory skills
Communication development
listening skills - speech perception
speech production
language development
Monitoring middle ear status

43. Status of EHDI Programs Early Intervention
Many of the programs in the current system designed to serve infants with bilateral severe-profound losses
BUT, majority of those identified have mild, moderate, and unilateral losses
Programs and professionals not appropriate for children and families
Therefore, Part C of IDEA is severely under utilized

44. Status of EHDI Programs Early Intervention
State Coordinators estimate
Only 53% of infants with hearing loss are enrolled in EI programs before 6 months of age
Only 31% of states have adequate range of choices for EI programs

45. Barriers to Early Intervention
30-40% of children with hearing loss demonstrate additional disabilities that may affect communication and related development.
Families who live in under-served areas may have less accessibility, fewer professional resources, deaf or hard of hearing role models, or sign language interpreters available to assist them.
A growing number of children with hearing loss in the United States are from families that are non-native English Speaking.
JCIH, 2000

46. Pediatric Audiology
Pediatric Audiology with newborns and young infants can be challenging!

47. Pediatric Audiology
But also rewarding!

48. Some babies are born listeners..
If we
use the elements of an effective EHDI program
use the JCIH 2000 Benchmarks
use appropriate diagnostic protocols and procedures
refer to early intervention
are active participants in early intervention