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Hearing and Children with PKS

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

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Hearing and Children with PKS

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  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 www.jcih.org

  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.

  5. EHDI Program Goals • 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!! 1 - 3 - 6

  6. Why the rush??

  7. Anu Sharma Ph.D. Cortical Auditory Potentials

  8. 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 3 LanguageAge (yrs) 2 1 0 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 , moeller@boystown.org

  9. Anatomy and Physiology

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

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

  12. 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

  13. 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

  14. 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

  15. 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?

  16. 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

  17. 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”)

  18. 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

  19. 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

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

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

  22. 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

  23. Deciphering the AudiogramHorizontal axis: Frequency information (pitch)

  24. Deciphering the AudiogramVertical axis: Sound energy (loudness)

  25. Frequency Low Pitch to High Pitch Loudness Soft to Loud

  26. Normal Hearing Mild Loss Moderate Loss Moderate Severe Loss Severe Loss Profound Loss

  27. Audiogram of Familiar Sounds

  28. Plotting Results on an Audiogram White area is inaudible Tan area is audible From: Glen R. Meier, M.S., CCC-A, FAAA http://www.audiologyawareness.com/hearinfo_audiogramread.asp

  29. 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

  30. Conductive Hearing Loss

  31. 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

  32. Sensorineural Hearing Loss

  33. Hearing loss and Prosthesis SimulatorHeLPS Demo #1 Severity of sensorineural hearing loss http://www.sens.com/helps/helps_d01.htm Demo # 3 Mild Conductive and Mild Sensorineural hearing loss comparison http://www.sens.com/helps/helps_d03.htm -

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

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

  36. 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

  37. 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

  38. 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

  39. 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

  40. 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

  41. 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

  42. 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

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

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

  45. 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

  46. 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)

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

  48. 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

  49. Resources You tube video on hearing: Brandon Pletsch http://www.youtube.com/watch?v=PeTriGTENoc&NR=1 Hearing screening information for parents: www.babyhearing.org American Speech,Language and Hearing Association www.asha.org American Academy of Audiology www.audiology.org Pennsylvania Early Hearing Detection and Intervention www.paearlyhearing.org

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