Aud 725: Pediatric Audiology Final

Front 1

indicators of aud neuropathy

Back 1

pts have nml OAEs and cochlear microphonic with absent/severly abnormal ABR

Front 2

tone thresholds of aud neuropathy

Back 2

nml or near nml sensitivity to severe impairment

Front 3

aud neuro must have all these characteristics

Back 3

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)

Front 4

noisy enviornments for aud neuropathy pts

Back 4

impaired

Front 5

factors predisposing children to aud neuropathy

Back 5

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

Front 6

possible physiological causes

Back 6

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

Front 7

Treatment considerations (list possibilities)

Back 7

1. HAs
2. CI
3. directed perceptual training
4. formal training with visual cues

Front 8

Treatment of AN: HAs + considerations

Back 8

-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

Front 9

Treatment of AN: CIs + considerations

Back 9

-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

Front 10

Treatment of AN: Directed perceptual training

Back 10

- 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

Front 11

Treatment of AN: formal training with visual cues

Back 11

- 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)

Front 12

impact of neuro on speech perception

Back 12

-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

Front 13

mean onset age of AN

Back 13

9 years

Front 14

range of onset of AN

Back 14

birth-at least 60 years (largest group onset before 2)

Front 15

gender distribution of AN

Back 15

equal

Front 16

medical history of AN

Back 16

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

Front 17

audiograms of AN

Back 17

all degrees, all configurations, may change over time

Front 18

speech discrimination of AN

Back 18

-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

Front 19

acoustic reflexes of AN

Back 19

Absent in 93.5% of cases; 6.5% present or elevated

Front 20

OAEs of AN: presence

Back 20

80% of AN patients have clear OAE; 9% never had OAE during evaluations; 11% OAE disappeared over time

Front 21

ABR results of AN

Back 21

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)

Front 22

AN relationship between degree of HL and severity

Back 22

-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

Front 23

possible causes of abnormal ABR results

Back 23

1. demyeliniation of nerve fibers
2. slowing and/or loss of individual nerve fibers

Front 24

Peripheral neuropathy & AN

Back 24

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

Front 25

How do you diagnose peripheral neuropathy

Back 25

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

Front 26

Psychoacousics of speech perception in AN its

Back 26

-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

Front 27

What do patients with AN need in their HAs?

Back 27

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

Front 28

What is the key to treatment and management of HL?

Back 28

Early detection.

Front 29

Screening

Back 29

Preliminary acquisition of info for early detection of a condition

Front 30

total prevalence of HL

Back 30

5.7/1000

Front 31

Screening techniques

Back 31

most infant programs use either both ABR or OAEs

Front 32

Sensitivy and specificity of ABR as a screening tool

Back 32

>96%

Front 33

What must happen once an infant has been identified in screening process

Back 33

must have referral options for audiolgoic, medical, surgical, ed, psych, and other health/social services

Front 34

Cost of running universal newborn hearing screening program?

Back 34

$26.05/infant screened

Front 35

What should happen if no UNS (universal newborn screening) program in place?

Back 35

Newborns (birth – 28 days) should be screened if any condition on high risk register exists

Front 36

Infrastructure supporting infant hearing programs (9 components-list)

Back 36

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)

Front 37

Most common screening procedure

Back 37

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

Front 38

SSEP

Back 38

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

Front 39

Biggest difference between ABR and SSEP

Back 39

ABR determined by visual inspection vs SSEP is determined bys at indicator

Front 40

OAES for screening

Back 40

1. TEOAEs
2. DPOAES (sweep across several diff fq regions)
3. OAEs will not be present w/ HL >30 dB

Front 41

Most common technique for newborn screening

Back 41

Automated ABR and TEOAE
-sensitivy/specificity acceptable when trying to get losses

Front 42

Screening preschoolers

Back 42

- Kids not always in class
-Looking for HL that can be remediated with med attention (ex. otitis media)
-Pure tone play
-Immitance screening

Front 43

School screening ASHA recommendations

Back 43

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

Front 44

rational for identifying neonatal HL (6 reasons-list)

Back 44

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

Front 45

Evidence that support importance of aud input early in infancy

Back 45

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

Front 46

Average age of detection w/o screening

Back 46

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

Front 47

Prevalaence and cost effective considerations

Back 47

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

Front 48

WHO principles of screening

Back 48

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

Front 49

1960's and 1970's

Back 49

BOA, local use of high risk registers (HRR), studies of cardiovascular response

Front 50

JCIH 2000

Back 50

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

Front 51

EHDI programs provide data to

Back 51

Monitor quality, demonstrate compliance w/legislation & regulations, determine fiscal accountability & cost effectiveness, support reimbursement for services, & mobilize & maintain community support

Front 52

Conferences/consensus statements (list)

Back 52

Nova Scotia Conference 1974
Saskatton Conference 1978
Healthy People 2000
NIH Consensus Conference 1993

Front 53

1970-1980

Back 53

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)

Front 54

1980-1990

Back 54

ABR, AABR, TEOAE

Front 55

healthy people 2000

Back 55

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

Front 56

1990+

Back 56

AABR, screening ABR systems, DPOAE & TEOAE screening protocols

Front 57

Healthy people 2010

Back 57

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

Front 58

JCIH 1994 position statement

Back 58

Endorsed goal of universal detection of infants w/HL as early as possible – ID by 3 mos, intervention by 6 mos

Front 59

status of UNHS to date

Back 59

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

Front 60

current challenges for UNHS

Back 60

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

Front 61

JCIH recommendations

Back 61

physiological measures
ABR or EOAE

Front 62

JCIH ABR

Back 62

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

Front 63

JCIH EOAES

Back 63

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

Front 64

factors to consider for hospital

Back 64

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

Front 65

what do referral rates depend on

Back 65

1.expertise of screeners
2.environment
3.criteria chosen for “passing” test

Front 66

Duties as an educational audiologist

Back 66

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

Front 67

Qualities needed to work in schools

Back 67

-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

Front 68

OAEs of AN -amplitude

Back 68

Amplitude of OAEs = strong (not too surprising considering most patients in this group are children)

Front 69

Loss of OAEs in AN

Back 69

Reason for loss of emissions over time is unclear

Front 70

When does the cochlea have normal adult function?

Back 70

20 weeks gestational age

Front 71

Babies begin to hear at what gestational age?

Back 71

5th month of gestation

Front 72

It is easiest to test for startle and APR responses when a child is in what state?

Back 72

Light sleep or awake and quiet

Front 73

The moro reflex is a startle response where the knees and arms are drawn into the body

Back 73

True

Front 74

The auro-palpebral reflex is whole body movement in response to loud sound

Back 74

False: Auro-palpebral reflex is an eye blink response to loud sound

Front 75

Name three things that should be included in a case history

Back 75

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

Front 76

What is the auditory behavior in infants aged 0 to 6 weeks

Back 76

APR and startle, change in pre-stimulus state, eye-widening

Front 77

At what age do infants demonstrate sophisticated auditory perception skills?

Back 77

By 6 months of age

Front 78

What is auditory perception?

Back 78

An interpretation of sensory evidence-not about perception of sound itself, but about perception of objects and events that produce sound.

Front 79

Potential for _________ ______ in judging responses during BOA complicates the task

Back 79

Observer Bias

Front 80

Intensity levels necessary to elicit a response ________ as age increases.

Back 80

Decrease

Front 81

Types of behavioral responses to auditory stimuli change as a function of?

Back 81

Age

Front 82

Infants respond better to _______ than to other stimuli.

Back 82

Speech

Front 83

What is BOA?

Back 83

Behavioral Observation Audiometry = determine a response by reflexive behaviors, such as eye widening, blink, arousal

Front 84

What are the two general approaches for behavioral assessment of infants and young children?

Back 84

-Non-reinforced behavioral responses
-Operant procedures: reinforcement following desired behavioral response

Front 85

Which method obtains more variable responses, BOA or VRA?

Back 85

BOA is much more variable than VRA

Front 86

What type of reinforcement was determined to be most effective by Wilson and Thompson (1975)? Why?

Back 86

Complex reinforcer (animated toy) was most effective.
Continued to show a high rate of responses as the number of presentations increased.

Front 87

What is VRA?

Back 87

Visual Reinforcement Audiometry-head turn response to an auditory stimulus

Front 88

What is COR?

Back 88

Conditioned Orientation Reflex - Infants will reflexively turn their head toward a novel auditory or visual stimulus

Front 89

What are the response options for operant procedures?

Back 89

Leg swing, bar press, head turn

Front 90

What is the principle of operant psychology?

Back 90

Positive reinforcement increases the frequency of a behavior

Front 91

What structures contribute to mass reactance?

Back 91

The ossicles

Front 92

What is admittance?

Back 92

The ease of energy flow through a system, the amount of energy allowed through

Front 93

What are possible causes of a Type B tympanogram?

Back 93

otitis media
perforation
patent PE tubes (look at volume)
wax

Front 94

What structures contribute to stiffness reactance?

Back 94

tympanic membrane and middle ear tendons

Front 95

What is an abnormally low resonant frequency consistent with?

Back 95

Increased mass, decreased stiffness

Front 96

What are the two components of admittance?

Back 96

Susceptance (-Bs)
Conductance (G)

Front 97

How is immittance testing in infants different from immittance testing in adults?

Back 97

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.

Front 98

What are three general characteristics of infant ABRs?

Back 98

1. Smaller amplitudes
2. Longer latencies
3. Simpler waveform, waves other than I, III, and V are not easy to recognize

Front 99

What filter settings should be used for infant ABRs?

Back 99

30-3000 Hz in order to avoid filtering out most of the response

Front 100

What type of ABR stimulus is most commonly used for threshold estimation?

Back 100

Click stimulus

Front 101

What is a problem with using a click stimulus for threshold estimation?

Back 101

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)

Front 102

What is a common complaint of patients with auditory neuropathy?

Back 102

Difficulty understanding speech, even with near normal thresholds

Front 103

What does a pre-school screening consist of?

Back 103

Pure-tone screening using play audiometry. 1000, 2000, 4000 first at 50 dB, then screen at 20 dB

Front 104

To be considered as having auditory neuropathy, what 3 criteria must be met?

Back 104

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

Front 105

Immittance screenings should include what?

Back 105

History-ear pain, drainage etc. and actual immittance testing

Front 106

Educational Audiologists Terminology: FAPE

Back 106

Free and Appropriate Public Education

Front 107

Educational Audiologists Terminology: LRE

Back 107

Least Restrictive Environment

Front 108

Educational Audiologists Terminology: NCLB

Back 108

No Child Left Behind

Front 109

Educational Audiologists Terminology: SST

Back 109

Student Study Team

Front 110

Educational Audiologists Terminology: SAI

Back 110

Specialized Academic Instruction

Front 111

Educational Audiologists Terminology: Mainstreaming

Back 111

The process where a special education student attends the same class/academic track as their non-disabled peers

Front 112

Educational Audiologists Terminology: RTI

Back 112

Response to Intervention - This can slow the process down. Pych asks for 6 mo evaluation period, then another 6 mo adjustment and eval period.

Front 113

Educational Audiologists Terminology: Inclusion

Back 113

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.

Front 114

Educational Audiologists Terminology: Co-Teaching / Pushing in Services

Back 114

Resource teacher / speech therapist provide their services in the classroom instead of pulling the child out of class for individual direct service

Front 115

Educational Audiologists Terminology: Trace Program

Back 115

Ages 18-22, help with resumes, getting into community college, help until they get their high school diploma

Front 116

Educational Audiologists Terminology: IEP

Back 116

Individualized Education Program (or Plan). For ages 3-22, until they get their high school diploma

Front 117

Educational Audiologists Terminology: IFSP

Back 117

Individualized Family Service Plan. Under age 3

Front 118

Educational Audiologists Terminology: IDEA

Back 118

Individuals with Disabilities Education Act. Does not include paying for cochlear implants

Front 119

Educational Audiologists Terminology: California Ed Code

Back 119

California Special Education Programs: A composite of Laws-Revised Edition 2000. Does not specify type of hearing loss

Front 120

Educational Audiologists Terminology: Section 504

Back 120

The American Disabilities Act and Education Reform - provides accommodations and in some states equipment without special education services

Front 121

The high risk neonatal indicators for hearing loss (ten things)

Back 121

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