Presentation on theme: "Hearing and Children with PKS"— Presentation transcript:
1 Hearing and Children with PKS James Zeigler Au.D.
2 Thank YouProtocol for screening and evaluating hearing loss in infants and young childrenConductive and sensorineural hearing lossesAmount of hearing lossAudiogram overviewHearing Tests - abr and sound booth testsHearing Aids
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 treatEHDI is an integrated system that brings multiple disciplines together for the single purpose of detecting and treating hearing loss as early in life as possibleThere 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 conditionEarly diagnosis and treatment improves outcomesMultiple disciplines and agencies are involved in making EHDI a successHearing 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 ageDiagnose hearing loss by 3 months of ageLink the child to intervention by 6 months of age to maximize developmental, educational and communication outcomesProvide support and education for families about the importance of detection and treatment of newborn hearing lossRemember!!PA EHDI Program Goals which are consistent with national goals.
9 Boys Town National Research Hospital Study of Earlier vs. Later 129 deaf and hard-of-hearing children assessed 2x each year6Identified <6 mos (n = 25)5Identified >6 mos (n = 104)4Language Age (yrs)3184.108.40.206.220.127.116.11.24.8Age (yrs)Moeller, M.P. (1997).Personal communication,
11 Anatomy and Physiology of Hearing Divided into 4 partsOuter EarMiddle EarInner EarCentral Auditory Nervous System
12 Structures of the Outer Ear PinnaGathers sound wavesAids in localization
13 Structures of the Outer Ear External Auditory Canal or Ear CanalApprox. 1 inch long“S” shapedAmplifies sound by 3 to 6 dBIsolates the eardrum from physical damageCerumen glands moisten/soften skinPresence of some cerumen is normal
14 Structures of the Outer Ear Tympanic Membrane or Ear DrumThin membraneForms boundary between outer and middle earVibrates in response to sound wavesChanges acoustical energy into mechanical energy
15 Structures of the Middle Ear OssiclesOssicular chain = malleus, incus & stapesFocus/amplify vibration of TM to smaller areaEnables vibration of cochlear fluidsMalleus = HammerAttaches to TMIncus = AnvilConnectorStapes = StirrupSmallest bone in the bodyFootplate inserts in oval window of the cochlea
16 Structures of the Middle Ear Eustachian TubeConnects middle ear cavity to nasopharynx“Equalizes” air pressure in middle earNormally closed, opens under certain conditionsMay allow a pathway for infectionChildren “grow out of” most middle ear problems as this tube lengthens and becomes more verticalPKS and high arch palate?
17 Structures of the Inner Ear CochleaSnail shaped cavity within mastoid bone2 ½ turns3 fluid-filled chambersContains Organ of CortiConverts mechanical energy to neuro-electrical energy
18 Structures of the Inner Ear Organ Of CortiEnd organ of hearing3 rows of Outer Hair Cells1 row of Inner Hair CellsTectorial and Basilar MembranesCochlear fluids(From Augustana College, “Virtual Tour of the Ear”)
19 Structures of the Inner Ear Hair CellsFrequency specificLow pitches = apex of cochleaHigh pitches = base of cochleaFluid movement causes deflection of nerve endingsNerve impulses (electrical energy) are generated and sent to the brain
20 Structures of the Central Auditory System VIIIth Cranial Nerve or Auditory NerveBundle of nerve fibers (25-30K)Travels from cochlea through internal auditory meatus to skull cavity and brain stemCarry signals from cochlea to primary auditory cortex, with continuous processing along the way
21 Structures of the Central Auditory System Auditory CortexWernicke’s Area within Temporal Lobe of the brainSounds interpreted based on experience/association
22 Anatomy of Hearing Loss Site ofConductiveLossSite of Sensori-neural Loss
23 Types of Hearing Loss Conductive = Outer and/or Middle Ear Sensorineural = Inner EarMixed = Outer and/or Middle Ear and Inner EarAuditory Neuropathy Spectrum Disorder (AKA Auditory Neuropathy / Dys-synchrony) = Central Auditory SystemUnilateralBilateral
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 LoudnessSoft to LoudSPEAKER’S NOTESThis 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 GraphicThe 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 GraphicA 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
32 Sensorineural Hearing Loss (SNHL) Caused by damage, disease, or malformation of the inner earSensory loss associated with inner ear damageNeural loss if hearing nerve cannot send the impulse to the brainSound perceived as distorted AND weak
34 Hearing loss and Prosthesis Simulator HeLPS Demo #1 Severity of sensorineural hearing lossDemo # 3 Mild Conductive and Mild Sensorineural hearing loss comparison-
35 Hearing Test Categories Objective tests or physiologic testsDo not require child's participationSubjective testsRequire the child to participate in evaluation
36 Types of Subjective Auditory Tests Sound Booth Tests Visual Reinforcement Audiometry (VRA)Play AudiometryConventional AudiometryHearing for SpeechAll subjective tests evaluate HEARING: requires consistent responses from the child
37 Types of Objective Auditory Tests Tympanometry - middle earOtoacoustic Emissions (OAEs) - inner earAuditory Brainstem Response (ABR)-auditory pathways in brainstemDO NOT evaluate Hearing
38 TympanometrySpecial earplug placed in the ear canal produces a puff of airThe technique measures the response of the eardrum to the change in air pressureTympanometry evaluates only the middle ear and does not provide information about hearingUsed 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 canalCochlea responds by producing a soundProbe detects this cochlear emission, which is recorded on computerOAEs detect presence or absence of hearing loss, but cannot assess degree of hearing impairmentMethod for newborn hearing screening
40 Auditory Brainstem Response (ABR) Special earphones deliver sound to earElectrodes on the scalp measure brain activity in response to soundResulting waves provide information on the integrity of the neural pathwayABRs can estimate degree of hearing loss at different frequenciesUsed for infants and children who cannot consistently respond to subjective testing
41 JCIH - Changes in 2007Audiologic EvaluationShould be performed by audiologists experienced in pediatric hearing assessmentInitial audiologic test battery to confirm hearing loss must include:physiologic measures – objective testswhen developmentally appropriate, behavioral methodsCompleted in both ears regardless of the results of screening tests
42 JCIH 2007Audiologic Evaluation – Birth to 6 months (Developmental Age)Child and family historyFrequency-specific AC (air conduction) ABR;Bone conduction, frequency specific ABR, when indicatedClick-evoked ABRif infant has risk indicators for neural HL,any infant demonstrating no response on FS-ABR requires click-evoked ABROAE (DPOAE or TEOAE)Tympanometry using 1000-Hz probe toneObservation of auditory behaviorAs cross-check; not for assessment or amplification fitting
43 JCIH 2007 Audiologic Evaluation – 6 months to 36 months Child and family historyParent report of auditory and visual behaviors and communication milestonesBehavioral audiometry (VRA, CPA), including:Pure-tone audiometry across the frequency range for each earSpeech detection and speech recognition measuresOAE testingTympanometry & Acoustic Reflex ThresholdsABR testing if responses to behavioral audiometry are not reliable, OR if ABR testing has not been performed previously
44 JCIH 2007AmplificationInfants 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 developmentCommunication modalitiesEmotionalSocialCognitiveWhoEarly interventionistsSpeech-language therapists – specialized in hearing impairmentEducators for the hearing impaired
47 Monitoring and managing hearing loss Hearing can change – can get worsePlan for future needs - amplification flexibilityMonitor hearing aid/cochlear implant function – trouble shootProvide educational input andconsultation (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 MessageInfants can and should be assessed as soon as possible to maximize development of maturing auditory skills; sets the stage for language developmentFamily 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 AssociationAmerican Academy of AudiologyPennsylvania Early Hearing Detection and Intervention