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 Hearing Aids

JCIH 2007 www.jcih.org

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.

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!! 1 - 3 - 6 PA EHDI Program Goals which are consistent with national goals.

Why the rush??

Anu Sharma Ph.D. Cortical Auditory Potentials

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 , moeller@boystown.org

Anatomy and Physiology

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

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

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

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

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

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?

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

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”)

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

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

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

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

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

Deciphering the Audiogram Horizontal axis: Frequency information (pitch)

Deciphering the Audiogram Vertical axis: Sound energy (loudness)

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.

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

Audiogram of Familiar Sounds

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

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

Conductive Hearing Loss

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

Sensorineural Hearing Loss

Hearing loss and Prosthesis Simulator HeLPS 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 -

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

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

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

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

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

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

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

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

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

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

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

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

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)

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

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

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

Contact information: jzeigler@liu18.org Thank You!