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121 Cards in this Set
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- Back
indicators of aud neuropathy
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pts have nml OAEs and cochlear microphonic with absent/severly abnormal ABR
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tone thresholds of aud neuropathy
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nml or near nml sensitivity to severe impairment
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aud neuro must have all these characteristics
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1. evidence of poor auditory function
2. evidence of poor neural function (reflexes and/or ABR) 3. evidence of normal outer hair cell function (OAEs or CM) |
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noisy enviornments for aud neuropathy pts
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impaired
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factors predisposing children to aud neuropathy
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1. can occur in absence of an apparent medical system
2. other symptoms/conditions: infectious processes (mumps), transient causes: fever, immune disorders, various genetic & syndromal conditions (Charcot-Marie-Tooth syndrome), other: anoxia, hyperbilirubinemia, acidosis |
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possible physiological causes
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1. neural synchrony disorder
2. neural synchrony could also be affected if IHC’s, synapses betw IHC’s & auditory nerve dendrites, or both were affected 3. AN=cochlear afferent disorder vs. more common cochlear efferent disorder seen with OHC damage |
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Treatment considerations (list possibilities)
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1. HAs
2. CI 3. directed perceptual training 4. formal training with visual cues |
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Treatment of AN: HAs + considerations
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-Losing OAEs following HA use
-Prevailing treatment =conservative trials w/amplification w/diligent monitoring of amp output levels & OAEs -HA’s benefit only 50% of children w/AN (benefit determined by anecdotal reports & speech perception tests) - Using algorithmic approach to HA fitting & real-ear measurements to verify parameters of HA, permanent threshold shift can be avoided - Aim of HA fitting should be to make speech audible |
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Treatment of AN: CIs + considerations
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-Electrical stim can be effective in providing useful aud info for speech perception
- 5/6 children w/implants due to AN = successful (significant improvement in speech perception abilities); these children all had severe to profound HL |
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Treatment of AN: Directed perceptual training
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- speech-sound training can be used to modify perception.
e.g., people can be trained to hear sounds that do not occur in their native language |
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Treatment of AN: formal training with visual cues
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- could possibly use intact visual modality to enhance auditory perception (to hear w/one’s eyes)
e.g., Cued Speech, Total Communication (signed English systems) |
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impact of neuro on speech perception
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-Significantly affects speech perception, even w/near-nml thresholds
- Range = functionally deaf – relatively intact speech perception in quiet w/severely impaired perception in noise - Poor temporal processing, gap detection, binaural sig processing diff |
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mean onset age of AN
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9 years
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range of onset of AN
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birth-at least 60 years (largest group onset before 2)
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gender distribution of AN
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equal
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medical history of AN
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27% have no associated medical conditions or family hx
80% of 25 patients w/onset prior to age 2, had either family or neonatal risk factors |
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audiograms of AN
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all degrees, all configurations, may change over time
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speech discrimination of AN
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-Speech perception abilities are out of proportion w/pure tone loss
-Speech discrim scores for AN patients fall significantly below level expected by severity of loss -Results for AN are similar to those w/acoustic neuroma but patients w/AN have primarily a bilateral involvement & are younger |
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acoustic reflexes of AN
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Absent in 93.5% of cases; 6.5% present or elevated
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OAEs of AN: presence
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80% of AN patients have clear OAE; 9% never had OAE during evaluations; 11% OAE disappeared over time
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ABR results of AN
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70% of patients have complete absence of ABR
19% show wave V only (peak is poorly defined, latency is abn, amp is small) 6% ABR is abn but includes at least 2 of traditional peaks (usually waves III & V, waveform morphology is abn, peak latency & amp are abn) |
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AN relationship between degree of HL and severity
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-Patients w/ absent ABR show poorest pure tone avg thresholds
-Those w/several peaks in ABR, have best thresholds -Cannot use ABR to estimate hearing thresholds in patient w/AN |
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possible causes of abnormal ABR results
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1. demyeliniation of nerve fibers
2. slowing and/or loss of individual nerve fibers |
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Peripheral neuropathy & AN
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Peripheral neuropathy = neuropathic disorder of nerves going to the periphery; caused by many diff etiologies. 80% of patients over 15 yrs old, exhibited peripheral neuropathy as well
-sometimes peripheral neuropathy preceded auditory and vice versa |
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How do you diagnose peripheral neuropathy
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Diagnose peripheral neuropathy by one or more of the following: absent ankle jerks or loss or abnormal reduction of vibration sense in the feet, abnormal nerve conduction studies, abnormal sural nerve biopsy
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Psychoacousics of speech perception in AN its
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-Severe temporal processing impairment vs. relatively nml temporal processing seen w/cochlear HL
-Temporal processing deficit can account for abn speech recognition observed in patients w/AN -Poor freq discrimination at low & moderate freqs while having relatively nml intensity processing & nml freq discrim at high freqs |
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What do patients with AN need in their HAs?
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Need HAs that not only amplify sound to overcome audiometric HL but also need a HA that will accentuate temporal envelope fluctuations to compensate for impaired temporal processing at suprathreshold levels
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What is the key to treatment and management of HL?
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Early detection.
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Screening
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Preliminary acquisition of info for early detection of a condition
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total prevalence of HL
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5.7/1000
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Screening techniques
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most infant programs use either both ABR or OAEs
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Sensitivy and specificity of ABR as a screening tool
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>96%
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What must happen once an infant has been identified in screening process
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must have referral options for audiolgoic, medical, surgical, ed, psych, and other health/social services
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Cost of running universal newborn hearing screening program?
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$26.05/infant screened
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What should happen if no UNS (universal newborn screening) program in place?
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Newborns (birth – 28 days) should be screened if any condition on high risk register exists
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Infrastructure supporting infant hearing programs (9 components-list)
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1. screening/surveillance
2. audio logic follow-up/assessment 3. other assessments (genetic counseling, etc) 4. disclosure (share w/ parents and family in sensitive timely manner) 5.home-based support 6. hearing aids 7. early support (benefial <6 mo) 8. training (for audiologists and staff members) 9. quality assurance (monitor for quality) |
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Most common screening procedure
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ABR b/c relatively unaffected by sleep/sedation
-concentrate on wave V -can find threshold -can use tone bursts/bone conducted but less clear -also have automated |
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SSEP
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Steady State Evoked Potential (aka ASSR)
-modulation of a pure tone carrier vs. transient stim -response waveform locked to a whole # of period modulation=wave fq=mod fq -can determine up to 4 thresholds at a time |
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Biggest difference between ABR and SSEP
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ABR determined by visual inspection vs SSEP is determined bys at indicator
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OAES for screening
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1. TEOAEs
2. DPOAES (sweep across several diff fq regions) 3. OAEs will not be present w/ HL >30 dB |
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Most common technique for newborn screening
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Automated ABR and TEOAE
-sensitivy/specificity acceptable when trying to get losses |
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Screening preschoolers
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- Kids not always in class
-Looking for HL that can be remediated with med attention (ex. otitis media) -Pure tone play -Immitance screening |
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School screening ASHA recommendations
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Screening at 20 dB HL at 500, 1000, 2000, & 4000 Hz in each ear using behav test methods
500 Hz is optional if using immittance screenings as well |
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rational for identifying neonatal HL (6 reasons-list)
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1. Normal hearing is a prereq for development of optimum oral/aural comm
2. children with HL experience: speech-language deficits, lower academic achievement,poorer social-emotional development than peers w/nml hrg 3. evidence support imporantce of aud input early in infancy 4. average age of detection is bad w/o screening 5. prevalence and cost-effective considerations 6.principles of screening |
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Evidence that support importance of aud input early in infancy
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Those who experience early exposure to language (nml hrg) & then lose their hrg appear to acquire oral/aural communication more rapidly than those who did not have early aud experiences
Lots of literature on early cochlear implantation & the improved benefits: Levitt et al. (1987) fd that children who received special ed services before 3 yrs = better expressive communication Yoshinaga-Itano & Apuzzo (1995) found that infants w/HL who were identified & provided w/HAs or intervention before 6 mos were at age level on language tasks measured @ 40 mos compared to those that were identified & received remediation after 12 mos |
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Average age of detection w/o screening
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Traditionally = ~30 mos – related to degree of HL, lesser degrees going undetected the longest vs. sev to prof HL id betw ~11 & 17 mos
Bigger issue was once HL suspected there was lengthy delays between suspicion of the loss, confirmation of loss, & initiation of habilitation |
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Prevalaence and cost effective considerations
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Prevalence (# of cases of HL w/in population during specific time period)
Estimates vary widely; as few as 1:1000, as high as 6:1000 Cost-effectiveness = determined by computing cost per HL identified; greater cost per HL = less cost effective screening program |
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WHO principles of screening
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1 disease/condition must be significant – must result in sig consequences for individ & society at large;
2. clear and measurable definition of condition 3. easy to admin 4. comfortale for pt 5. short duration 6. inexpensive 7. high sens/spec (low refer rates) 8. appropriate to detect condition 9. treatment available 10. sufficient resources to implement and comply with testing and follow up |
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1960's and 1970's
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BOA, local use of high risk registers (HRR), studies of cardiovascular response
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JCIH 2000
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Endorses early hrg detection and intervention (EHDI) of infants w/HL through integrated, interdisciplinary state & national systems of UNHS, evaluation, & family centered intervention – 8 principles provide foundation for EHDI
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EHDI programs provide data to
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Monitor quality, demonstrate compliance w/legislation & regulations, determine fiscal accountability & cost effectiveness, support reimbursement for services, & mobilize & maintain community support
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Conferences/consensus statements (list)
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Nova Scotia Conference 1974
Saskatton Conference 1978 Healthy People 2000 NIH Consensus Conference 1993 |
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1970-1980
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HRR, arousal test, electric response audiometry (initial work w/ ABR), acoustic reflex, automated tests
(Crib-o-gram = automated method for behavioral hearing screening, recorded movement responses to high-level stim) |
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1980-1990
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ABR, AABR, TEOAE
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healthy people 2000
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1990 U.S. Dept. of Health & Human Services issued this report: Purpose = set goals directed at improving health of citizens of this country
Recommended age of id of children w/HL to 12 mos or less |
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1990+
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AABR, screening ABR systems, DPOAE & TEOAE screening protocols
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Healthy people 2010
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100% of newborns served by state-sponsored EHDI programs
100% of newborns access to screening Provide f/u audiologic/medical evals before 3mos Provide access to intervention before 6 mos for infants who are hard of hearing & deaf |
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JCIH 1994 position statement
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Endorsed goal of universal detection of infants w/HL as early as possible – ID by 3 mos, intervention by 6 mos
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status of UNHS to date
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3 states & District of Columbia have enacted legislation, which provides universal hearing screening to newborns
8 states do not have provisions requiring universal screening 6 states already screen the hearing of more than 95% of newborns on a voluntary basis (Arizona, Delaware, Idaho, Washington, North Dakota, Vermont) 1 state is mandated by regulation = Alabama thus, 1 has no provisions = South Dakota |
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current challenges for UNHS
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1. only 21 states do at home births
2. not always receive follow up 3. not always collect diag info 4. physicians think because must be 4+mo to be fit 5. states don't always evaluate programs |
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JCIH recommendations
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physiological measures
ABR or EOAE |
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JCIH ABR
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1. compares online responses from infant w/nml template response pattern – if test infant’s responses falls w/in normative values, automated instrument renders a “pass” decision; if response pattern falls outside acceptable response template, “refer” decision rendered
2. determine presence of response by ascertaining if measured variance ratio (an ABR SNR – magnitude of ABR response divided by magnitude of noise – often referred to as Fsp b/c calculated from F statistic at a single point – currently calculated from several points but still referred to as Fsp) exceeds criterion |
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JCIH EOAES
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1. Only practical & objective methods currently
2. neither is a test of behave hrg, but each is good predictor of aud function & is able to id HL 3. both tests require: nml periph aud function 4. ABR requires nml aud neurological function |
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factors to consider for hospital
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1. screening tools
2. program philosophy 3. severity and config of HL 4. prior experience 5. personnel 6. typical length of stay for mothers and infants 7. space (idea=own space near nursery that is quiet) 8 referral rates 9. funding |
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what do referral rates depend on
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1.expertise of screeners
2.environment 3.criteria chosen for “passing” test |
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Duties as an educational audiologist
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intake aud: get failed kids and audios
-decide/help with IEP -make ear molds -fit FM systems -CI's (not mapping!) -don't have to be ASHA certified |
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Qualities needed to work in schools
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-work well on team
-variety of kids -work with good and difficult parents well -need to interact with kids well -prepared (gotta lug your equipment everywhere) -like kids -be patient |
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OAEs of AN -amplitude
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Amplitude of OAEs = strong (not too surprising considering most patients in this group are children)
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Loss of OAEs in AN
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Reason for loss of emissions over time is unclear
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When does the cochlea have normal adult function?
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20 weeks gestational age
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Babies begin to hear at what gestational age?
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5th month of gestation
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It is easiest to test for startle and APR responses when a child is in what state?
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Light sleep or awake and quiet
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The moro reflex is a startle response where the knees and arms are drawn into the body
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True
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The auro-palpebral reflex is whole body movement in response to loud sound
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False: Auro-palpebral reflex is an eye blink response to loud sound
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Name three things that should be included in a case history
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Identifying information, Hearing/chief complaint, Speech or language concerns, Family history, Maternal health during pregnancy, Birth history, Medical history Developmental history, Psycho-social history, Educational history, Any special precautions
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What is the auditory behavior in infants aged 0 to 6 weeks
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APR and startle, change in pre-stimulus state, eye-widening
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At what age do infants demonstrate sophisticated auditory perception skills?
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By 6 months of age
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What is auditory perception?
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An interpretation of sensory evidence-not about perception of sound itself, but about perception of objects and events that produce sound.
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Potential for _________ ______ in judging responses during BOA complicates the task
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Observer Bias
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Intensity levels necessary to elicit a response ________ as age increases.
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Decrease
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Types of behavioral responses to auditory stimuli change as a function of?
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Age
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Infants respond better to _______ than to other stimuli.
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Speech
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What is BOA?
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Behavioral Observation Audiometry = determine a response by reflexive behaviors, such as eye widening, blink, arousal
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What are the two general approaches for behavioral assessment of infants and young children?
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-Non-reinforced behavioral responses
-Operant procedures: reinforcement following desired behavioral response |
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Which method obtains more variable responses, BOA or VRA?
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BOA is much more variable than VRA
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What type of reinforcement was determined to be most effective by Wilson and Thompson (1975)? Why?
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Complex reinforcer (animated toy) was most effective.
Continued to show a high rate of responses as the number of presentations increased. |
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What is VRA?
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Visual Reinforcement Audiometry-head turn response to an auditory stimulus
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What is COR?
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Conditioned Orientation Reflex - Infants will reflexively turn their head toward a novel auditory or visual stimulus
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What are the response options for operant procedures?
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Leg swing, bar press, head turn
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What is the principle of operant psychology?
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Positive reinforcement increases the frequency of a behavior
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What structures contribute to mass reactance?
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The ossicles
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What is admittance?
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The ease of energy flow through a system, the amount of energy allowed through
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What are possible causes of a Type B tympanogram?
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otitis media
perforation patent PE tubes (look at volume) wax |
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What structures contribute to stiffness reactance?
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tympanic membrane and middle ear tendons
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What is an abnormally low resonant frequency consistent with?
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Increased mass, decreased stiffness
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What are the two components of admittance?
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Susceptance (-Bs)
Conductance (G) |
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How is immittance testing in infants different from immittance testing in adults?
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Infants ME systems are mass and resistance dominant-mesenchyme in the ME
In adults, compliance (stiffness) dominates at 226 Hz. *for infants < 7 months old, use 800 Hz probe tone. |
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What are three general characteristics of infant ABRs?
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1. Smaller amplitudes
2. Longer latencies 3. Simpler waveform, waves other than I, III, and V are not easy to recognize |
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What filter settings should be used for infant ABRs?
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30-3000 Hz in order to avoid filtering out most of the response
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What type of ABR stimulus is most commonly used for threshold estimation?
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Click stimulus
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What is a problem with using a click stimulus for threshold estimation?
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Because of contributions from different regions click ABRs will often miss or underestimate the degree of loss if HL restricted to a particular frequency region. (e.g. HF losses, Low freq. losses, cookie-bite losses)
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What is a common complaint of patients with auditory neuropathy?
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Difficulty understanding speech, even with near normal thresholds
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What does a pre-school screening consist of?
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Pure-tone screening using play audiometry. 1000, 2000, 4000 first at 50 dB, then screen at 20 dB
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To be considered as having auditory neuropathy, what 3 criteria must be met?
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Evidence of poor auditory function- difficulty hearing in some situations
Evidence of poor neural function- elevated/absent reflexes and abnormal ABR Evidence of normal hair cell function – present OAEs or CM |
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Immittance screenings should include what?
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History-ear pain, drainage etc. and actual immittance testing
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Educational Audiologists Terminology: FAPE
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Free and Appropriate Public Education
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Educational Audiologists Terminology: LRE
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Least Restrictive Environment
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Educational Audiologists Terminology: NCLB
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No Child Left Behind
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Educational Audiologists Terminology: SST
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Student Study Team
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Educational Audiologists Terminology: SAI
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Specialized Academic Instruction
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Educational Audiologists Terminology: Mainstreaming
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The process where a special education student attends the same class/academic track as their non-disabled peers
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Educational Audiologists Terminology: RTI
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Response to Intervention - This can slow the process down. Pych asks for 6 mo evaluation period, then another 6 mo adjustment and eval period.
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Educational Audiologists Terminology: Inclusion
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Include both children who are special needs and their non-disabled peers. Usually an inclusion classroom is led by both “regular” and special ed teachers, and there may be paraprofessionals (classroom aides). The kids aren’t really doing the general education program, they are just included in the class.
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Educational Audiologists Terminology: Co-Teaching / Pushing in Services
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Resource teacher / speech therapist provide their services in the classroom instead of pulling the child out of class for individual direct service
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Educational Audiologists Terminology: Trace Program
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Ages 18-22, help with resumes, getting into community college, help until they get their high school diploma
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Educational Audiologists Terminology: IEP
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Individualized Education Program (or Plan). For ages 3-22, until they get their high school diploma
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Educational Audiologists Terminology: IFSP
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Individualized Family Service Plan. Under age 3
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Educational Audiologists Terminology: IDEA
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Individuals with Disabilities Education Act. Does not include paying for cochlear implants
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Educational Audiologists Terminology: California Ed Code
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California Special Education Programs: A composite of Laws-Revised Edition 2000. Does not specify type of hearing loss
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Educational Audiologists Terminology: Section 504
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The American Disabilities Act and Education Reform - provides accommodations and in some states equipment without special education services
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The high risk neonatal indicators for hearing loss (ten things)
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1. Family history of hereditary childhood sensorineural hearing loss
2. In utero infection such as TORCH (toxoplasmosis, rubella, cytomegalovirus, syphilis or herpes) 3. Craniofacial anomalies, including those with morphological abnormalities of the pinna and ear canal 4. Birth weight < 1500 g (3.3 lbs) 5. Hyperbilirubinemia requiring exchange transfusion 6. Ototoxic medications, including but not limited to the aminoglycosides, used in multiple course or in combination with loop diuretics. 7. Bacterial meningitis 8. Apgar score of 0-4 at 1 min. or 0-6 at 5 min. 9. Mechanical ventilation lasting 5 days or longer 10. Stigmata or other findings associated with a syndrome known to include a sensorineural or conductive hearing loss |