1. Hearing and Children with PKS
James Zeigler Au.D.

2. Thank You
Protocol for screening and evaluating hearing loss in infants and young children
Conductive and sensorineural hearing losses
Amount of hearing loss
Audiogram overview
Hearing Tests - abr and sound booth tests
Hearing Aids

3. JCIH 2007

4. Early Hearing Detection and Intervention (EHDI): What is it?
Hearing loss is the most common disorder screened in the newborn period and one of the most cost effective to treat
EHDI is an integrated system that brings multiple disciplines together for the single purpose of detecting and treating hearing loss as early in life as possible
There must be a seamless integration of universal newborn screening, prompt diagnosis, effective intervention and tracking.
Through the EHDI process, we are trying to bring together everyone involved in helping with hearing loss. A coordinated EHDI program is necessary because:
Hearing loss is a frequent condition
Early diagnosis and treatment improves outcomes
Multiple disciplines and agencies are involved in making EHDI a success
Hearing loss is an important public health concern. Each year in the United States, more than 12,000 babies are born with a hearing loss, making it the most frequently occurring condition identified through newborn screening. Left undetected, hearing loss in infants can negatively impact speech and language acquisition, academic achievement and social and emotional development. Even mild hearing loss can significantly interfere with the reception of spoken language and education performance. If hearing loss is detected early, however, these negative impacts can be diminished and even eliminated through early intervention.

5. EHDI Program Goals Remember!! 1 - 3 - 6
Screen all newborns by 1 month of age
Diagnose hearing loss by 3 months of age
Link the child to intervention by 6 months of age to maximize developmental, educational and communication outcomes
Provide support and education for families about the importance of detection and treatment of newborn hearing loss
Remember!!
PA EHDI Program Goals which are consistent with national goals.

6. Why the rush??

7. Anu Sharma Ph.D. Cortical Auditory Potentials

9. Boys Town National Research Hospital Study of Earlier vs. Later
129 deaf and hard-of-hearing children assessed 2x each year 6
Identified <6 mos (n = 25) 5
Identified >6 mos (n = 104) 4
Language Age (yrs) 3 2 1
0.8
1.2
1.8
2.2
2.8
3.2
3.8
4.2
4.8
Age (yrs)
Moeller, M.P. (1997).
Personal communication ,

10. Anatomy and Physiology

11. Anatomy and Physiology of Hearing
Divided into 4 parts
Outer Ear
Middle Ear
Inner Ear
Central Auditory Nervous System

12. Structures of the Outer Ear
Pinna
Gathers sound waves
Aids in localization

13. Structures of the Outer Ear
External Auditory Canal or Ear Canal
Approx. 1 inch long
“S” shaped
Amplifies sound by 3 to 6 dB
Isolates the eardrum from physical damage
Cerumen glands moisten/soften skin
Presence of some cerumen is normal

14. Structures of the Outer Ear
Tympanic Membrane or Ear Drum
Thin membrane
Forms boundary between outer and middle ear
Vibrates in response to sound waves
Changes acoustical energy into mechanical energy

15. Structures of the Middle Ear
Ossicles
Ossicular chain = malleus, incus & stapes
Focus/amplify vibration of TM to smaller area
Enables vibration of cochlear fluids
Malleus = Hammer
Attaches to TM
Incus = Anvil
Connector
Stapes = Stirrup
Smallest bone in the body
Footplate inserts in oval window of the cochlea

16. Structures of the Middle Ear
Eustachian Tube
Connects middle ear cavity to nasopharynx
“Equalizes” air pressure in middle ear
Normally closed, opens under certain conditions
May allow a pathway for infection
Children “grow out of” most middle ear problems as this tube lengthens and becomes more vertical
PKS and high arch palate?

17. Structures of the Inner Ear
Cochlea
Snail shaped cavity within mastoid bone
2 ½ turns
3 fluid-filled chambers
Contains Organ of Corti
Converts mechanical energy to neuro-electrical energy

18. Structures of the Inner Ear
Organ Of Corti
End organ of hearing
3 rows of Outer Hair Cells
1 row of Inner Hair Cells
Tectorial and Basilar Membranes
Cochlear fluids
(From Augustana College, “Virtual Tour of the Ear”)

19. Structures of the Inner Ear
Hair Cells
Frequency specific
Low pitches = apex of cochlea
High pitches = base of cochlea
Fluid movement causes deflection of nerve endings
Nerve impulses (electrical energy) are generated and sent to the brain

20. Structures of the Central Auditory System
VIIIth Cranial Nerve or Auditory Nerve
Bundle of nerve fibers (25-30K)
Travels from cochlea through internal auditory meatus to skull cavity and brain stem
Carry signals from cochlea to primary auditory cortex, with continuous processing along the way

21. Structures of the Central Auditory System
Auditory Cortex
Wernicke’s Area within Temporal Lobe of the brain
Sounds interpreted based on experience/association

22. Anatomy of Hearing Loss
Site of
Conductive
Loss
Site of Sensori-neural Loss

23. Types of Hearing Loss Conductive = Outer and/or Middle Ear
Sensorineural = Inner Ear
Mixed = Outer and/or Middle Ear and Inner Ear
Auditory Neuropathy Spectrum Disorder (AKA Auditory Neuropathy / Dys-synchrony) = Central Auditory System
Unilateral
Bilateral

24. Deciphering the Audiogram Horizontal axis: Frequency information (pitch)

25. Deciphering the Audiogram Vertical axis: Sound energy (loudness)

26. Frequency Low Pitch to High Pitch
Loudness
Soft to Loud
SPEAKER’S NOTES
This is an audiogram. Frequency or pitch is shown across the top of the audiogram.
CLICK NOW FOR GRAPHIC.
The frequencies range from 125 Hertz (Hz) to 8000Hz. You can think of the frequency portion of the audiogram like a piano keyboard. Sounds on the left portion of the audiogram are low pitched, bass quality sounds; with the lowest pitch on the audiogram being 125Hz. As you move to the right, toward 8000Hz, sounds get higher in pitch and have a tenor quality.
Intensity or loudness is shown along left side of the audiogram.
The further you move down the audiogram, the louder sound becomes. For example, a 10dB sound is as soft as a whisper, while a 110dB sound is as loud as an airplane.
Familiar sounds are plotted on this audiogram, revealing at what frequency and loudness level these sounds occur. For example, a lawn mower is a very loud, low pitched sound, while the “s” sound is a very soft, high pitched sound.
The shaded area represents where the sounds of speech take place. If you look closely at this area, you will notice that the sounds of speech occur between approximately 15-50dB from approximately 250 to 8000Hz. Vowels tend to be lower pitched and louder than consonants.

27. Normal Hearing Mild Loss Moderate Loss Moderate Severe Loss
SPEAKER’S NOTES. The audiogram is broken down into different parts that reflect the degree of hearing loss a person has.
Click Now for Graphic
The normal range of hearing is 0-20dB.
A person has a mild loss if their thresholds are from 20-40dB.
A person has a moderate loss if their thresholds are from 40-55dB.
A person has a Moderate-Severe loss if their thresholds are from 55-70dB.
A person has a Severe loss if their thresholds are from 70-90dB.
Click Now for Graphic
A person has a profound loss if their thresholds are 90dB and greater.
As you can see, a person with a mild loss will be unable to hear sounds in the normal range and person with a moderate loss will be unable to hear sounds in the normal and mild range. This pattern will continue for each successive degree of hearing loss. A person with a profound hearing loss will be unable to hear any of the sounds of speech and most environmental sounds.
Profound Loss

28. Audiogram of Familiar Sounds

29. Plotting Results on an Audiogram
White area is inaudible
Tan area is audible
From: Glen R. Meier, M.S., CCC-A, FAAA

30. Conductive Hearing Loss (CHL)
Caused by damage, disease, or malformation of the outer or middle ear
Sound is prevented from reaching the inner ear
Sound perceived as muffled or weak

31. Conductive Hearing Loss

32. Sensorineural Hearing Loss (SNHL)
Caused by damage, disease, or malformation of the inner ear
Sensory loss associated with inner ear damage
Neural loss if hearing nerve cannot send the impulse to the brain
Sound perceived as distorted AND weak

33. Sensorineural Hearing Loss

34. Hearing loss and Prosthesis Simulator HeLPS
Demo #1 Severity of sensorineural hearing loss
Demo # 3 Mild Conductive and Mild Sensorineural hearing loss comparison -

35. Hearing Test Categories
Objective tests or physiologic tests
Do not require child's participation
Subjective tests
Require the child to participate in evaluation

36. Types of Subjective Auditory Tests Sound Booth Tests
Visual Reinforcement Audiometry (VRA)
Play Audiometry
Conventional Audiometry
Hearing for Speech
All subjective tests evaluate HEARING: requires consistent responses from the child

37. Types of Objective Auditory Tests
Tympanometry - middle ear
Otoacoustic Emissions (OAEs) - inner ear
Auditory Brainstem Response (ABR)
-auditory pathways in brainstem
DO NOT evaluate Hearing

38. Tympanometry
Special earplug placed in the ear canal produces a puff of air
The technique measures the response of the eardrum to the change in air pressure
Tympanometry evaluates only the middle ear and does not provide information about hearing
Used to determine if (temporary) middle ear abnormality contributes to hearing loss

39. Otoacoustic Emissions (OAEs)
Special earphones placed into the ear that deliver clicking sound within the ear canal
Cochlea responds by producing a sound
Probe detects this cochlear emission, which is recorded on computer
OAEs detect presence or absence of hearing loss, but cannot assess degree of hearing impairment
Method for newborn hearing screening

40. Auditory Brainstem Response (ABR)
Special earphones deliver sound to ear
Electrodes on the scalp measure brain activity in response to sound
Resulting waves provide information on the integrity of the neural pathway
ABRs can estimate degree of hearing loss at different frequencies
Used for infants and children who cannot consistently respond to subjective testing

41. JCIH - Changes in 2007
Audiologic Evaluation
Should be performed by audiologists experienced in pediatric hearing assessment
Initial audiologic test battery to confirm hearing loss must include:
physiologic measures – objective tests
when developmentally appropriate, behavioral methods
Completed in both ears regardless of the results of screening tests

42. JCIH 2007
Audiologic Evaluation – Birth to 6 months (Developmental Age)
Child and family history
Frequency-specific AC (air conduction) ABR;
Bone conduction, frequency specific ABR, when indicated
Click-evoked ABR
if infant has risk indicators for neural HL,
any infant demonstrating no response on FS-ABR requires click-evoked ABR
OAE (DPOAE or TEOAE)
Tympanometry using 1000-Hz probe tone
Observation of auditory behavior
As cross-check; not for assessment or amplification fitting

43. JCIH 2007 Audiologic Evaluation – 6 months to 36 months
Child and family history
Parent report of auditory and visual behaviors and communication milestones
Behavioral audiometry (VRA, CPA), including:
Pure-tone audiometry across the frequency range for each ear
Speech detection and speech recognition measures
OAE testing
Tympanometry & Acoustic Reflex Thresholds
ABR testing if responses to behavioral audiometry are not reliable, OR if ABR testing has not been performed previously

44. JCIH 2007
Amplification
Infants diagnosed with permanent hearing loss should be fit with amplification within one month of confirmation of hearing loss

45. Treatment and intervention for hearing loss
Medical intervention surgical treatment treatment for chronic middle ear disorder Hearing aids Cochlear implants FM systems

46. Treatment and intervention for hearing loss
Early intervention for overall development
Communication modalities
Emotional
Social
Cognitive
Who
Early interventionists
Speech-language therapists – specialized in hearing impairment
Educators for the hearing impaired

47. Monitoring and managing hearing loss
Hearing can change – can get worse
Plan for future needs - amplification flexibility
Monitor hearing aid/cochlear implant function – trouble shoot
Provide educational input and
consultation (classroom modifications,
FMs, educational strategies)

48. How to work collaboratively with audiologists
Out reach efforts can be individual or group Mutual information sharing Keep asking questions

49. Take Home Message
Infants can and should be assessed as soon as possible to maximize development of maturing auditory skills; sets the stage for language development
Family choices for intervention often includes hearing aids/cochlear implants AND early intervention (communication strategies)
Questions about hearing? Ask your Audiologist

50. Resources You tube video on hearing: Brandon Pletsch
Hearing screening information for parents:
American Speech,Language and Hearing Association
American Academy of Audiology
Pennsylvania Early Hearing Detection and Intervention

51. Contact information:
Thank You!