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Audiology > Immittance Audiometry, ABRs, OAEs, Pediatric Audiology > Flashcards

Immittance Audiometry, ABRs, OAEs, Pediatric Audiology Flashcards

1
Q

Purpose of Immittance Testing

A
  • Primary:
  • Evaluates health and function of the middle ear system
  • Secondary:
  • Evaluates the acoustic reflex pathway which includes the 7th and
    8th cranial nerves and brainstem
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2
Q

Advantages

A

▪Objective
▪Not time consuming
▪Noninvasive
▪Easy to administer
▪Used to detect presence of ___Conductive_________ component
3
4

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3
Q

Immittance Concepts

A

Measurement of how easily a system can be set into vibration by a driving
force
▪ IMPEDANCE
❖Measures ___Blockage_______ of energy flow through a system
▪ ADMITTANCE
❖Measures___Easy_______ of energy flow through a system (compliance)
* Admittance and impedance are reciprocal terms

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4
Q

Impedance Matching Transformer

A

Back to Anatomy and Physiology!
Impedance Matching Transformer
* Matches __Low___acoustical impedance of air to
__High___ acoustical impedance of cochlear fluid.
* Without a middle ear, most of the incoming signal.
would be reflected back due to the much
higher impedance of the cochlear fluids.
Much less sound energy would be
transmitted to the cochlea.

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5
Q

Middle Ear Anatomy
The air pressure

A

Air pressure must be equal
on both sides of the ear
drum in order to optimize
mobility of the whole
system

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6
Q

Immittance Audiometry

A

Assesses how well sound energy
flows through the __Outer_____and __Middle_____ ear to the
cochlea.
❖Assesses how well the ear is performing its ___IMPEDANCE____
matching function.

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7
Q

Instrumentation
What does the probe do?

A

Electro-acoustic Device
➢Compares probe signal introduced into
ear canal with the resultant signal
reflected off the TM
➢Measurement of the signal reflected
back to the probe provides information
about efficiency of the middle ear
system.

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8
Q

Probe Tube Assembly

A
  • Three holes
  • Speaker – generates a fixed tone of 85
    dB SPL at 226 Hz.
  • Microphone – picks up sound
    reflecting off TM
  • Air pump – changes air pressure from
    positive to room or atmospheric air
    pressure to negative air pressure
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9
Q

Tympanometry

A

Tympanometry reflects change in the physical properties of the middle ear
system and tympanic membrane as air pressure in the external ear canal is
varied

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10
Q

Tympanometry Prodecure

A
  • A probe tip seals the external auditory canal.
  • A change in air pressure will be presented
    going from positive to negative
  • A known signal (220 Hz at 85 dB SPL) will be
    presented through the probe tip
  • A microphone will record the amount of the
    signal that is reflected back
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11
Q

Tympanogram
X and Y axis

A

A plot of middle ear admittance as a
function of ear canal pressure.
* X axis: Air Pressure
* Swept from +200 – 400 daPA
* Y axis: Compliance
* Measured in cm3 or ml
* Peak is the point where air pressure
is equal on both sides of the TM

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12
Q

Tympanometry
What we get from it

A

Middle Ear Pressure (TPP)
٭Static Acoustic Compliance (SAC)or (Ytm)
٭Ear Canal Volume (ECV or PVT or Veq)
٭Tympanometric Width (TW) / Gradient

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13
Q

Middle Air Pressure

Tympanic Peak Pressure (TPP)

A

Tympanic Peak Pressure (TPP)
* Decapascals (daPa)
* Referenced to normal atmospheric
pressure
* Provides information regarding the
functioning of the Eustachian tube

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14
Q

Tympanic Peak Pressure (TPP)

A

Maximum tympanic membrane mobility at TPP
* Air pressure is equal on both sides of the tympanic membrane

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15
Q

Abnormal Tympanic Peak Pressure (TPP)
Why and what will the probe do

A

If eustachian tube is not functioning properly, negative pressure will build up in the
ME space.
* When the probe tone in the ear canal reaches negative pressure, matching the
negative air pressure in the ME, the TM will vibrate most efficiently at a negative air
pressure.
* This is represented by the negative peak on the tympanogram

Type C

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16
Q

Static Acoustic Compliance (SAC)

A
  • Height of the tympanogram peak
  • How compliant or mobile is the TM /
    ME System
  • Provides information about
  • The ossicular chain
  • Tympanic membrane health
  • Middle ear pathology
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17
Q

Static Acoustic Compliance (SAC) Norms

A
  • Normal
  • .3 – 2.5 ml
  • Pathology – Decreased TM Mobility
  • <.29ml Fixation of the ossicles
  • Fluid in the middle ear
  • Pathology – Increased TM Mobility
  • > 2.5ml
  • Increased TM mobility
  • Ossicular disarticulation
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18
Q

Ear Canal Volume (ECV)
What info does it give us?

A

Ear Canal Volume (ECV) or (Veq)
* Measurement of the physical volume of the ear
canal
* Provides information regarding
* Cerumen impaction or ear canal blockage
* Tympanic membrane perforation
* Pressure equalization tube patency.
21

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19
Q

Ear Canal Volume (ECV

A

Equivalent volume of air space from end of
probe tip to the TM
* Measured at +200daPa when TM is __stiff____ and
more/ sound is reflected back to TM
* Equivalent volume of air space is predicted by
intensity of reflected sound

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20
Q

Ear Canal Volume – Too Small, Too Large or Just Right

A

Normal Ear Canal Volume
* Adult: .5 – 2.5 cc
* Pediatric : .3 – 1.5 cc
* Too Small – Ear Canal Blockage
* Adult: < .5 cc
* Pediatric: <.3 cc
* Too Large – TM Perf or Patent PE Tubes
* Adult: > 2.5 cc
* Pediatric: > 1.5 cc

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21
Q

Tympanometric Width (TW)
or Gradient

A

❖Describes shape of tympanogram
in region of the peak
❖Tympanometric width at 50% of
peak static admittance
❖Expressed in daPa
Pathology:
* Middle Ear Fluid - TW >200daPa

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22
Q

Key Points

A
  • Tympanometry is not dependent on hearing
  • It is an indirect measurement of middle ear function, based on the
    movement of the tympanic membrane in response to air pressure
    change.
  • Integrity of the tympanic membrane is essential to immittance testing
  • Pathology of the tympanic membrane will interfere with obtaining
    reliable measurements
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23
Q

Tympanogram Classification

A
  • Based on tympanometric
    shape
  • Each shape is consistent
    with a specific disorder
  • Main types: A, As, Ad, B, C
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24
Q

Type A Tympanogram

A

Normal Middle Ear Pressure and Compliance
ECV
* Adult .5 – 2.5 ml or cm3
* Pediatric .3 – 1.5 ml or cm3
SAC
* .3 – 2.5 ml or cm3
Pressure
* -199 to +50daPa
* Normal ____________
* ___Sensorineural________Hearing Loss

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25
Q

Type As Tympanogram

A
  • ECV: Normal
  • SAC: .1 – 2.9
  • ME Pressure: Normal
  • Pathologies:
    Otosclerosis
    Thickened or scarred TM
    Tympanosclerosis
  • ____________ Hearing Loss
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26
Q

Type Ad Tympanogram

A

Type Ad Tympanogram
Increased Tympanic Mobility
* ECV: Normal
* SAC: >2.5
* ME Pressure: Normal
* Pathologies:
Ossicular Disarticulation/Discontinuity
Thinly healed tympanic membrane
* ____________ Hearing Loss

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27
Q

Type C Tympanogram

A

Type C Tympanogram
Negative Middle Ear Pressure
* ECV: Normal
* SAC: Normal
* ME Pressure: >-199 daPa
* Pathologies:
Eustachian tube dysfunction
* ____________ Hearing
Loss

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28
Q

Type B Tympanogram

A
  • SAC: No Peak
  • Pressure: No Peak Pressure
    Pathologies
  • Normal ECV: Middle ear effusion
  • Reduced ECV: <.3 Child <.5 Adult
    Wax obstruction, foreign body, or
    improper probe position
  • Large ECV: >2.5
    TM perforation, Patent PE Tube
  • _____________ Hearing Loss
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29
Q

Acoustic (Stapedial) Reflex Measurement

A
  • Tympanometry uses air pressure to stiffen the tympanic membrane
  • Acoustic Reflexes uses loud sounds to stiffen the tympanic membrane
  • Acoustic (Stapedial) Reflex Threshold
  • Lowest high-intensity stimulus level to elicit contraction of the stapedial
    muscle which stiffens the ME system
  • Immittance instrument measures this change in admittance
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30
Q

Stapedial Muscle/Reflex

A
  • Stapedial muscle is attached to the stapes and
    contracts to loud sounds
  • Contraction of the stapes stiffens the middle ear
    ossicles which limits the movement of the
    tympanic membrane.
  • Stiffening of the middle ear system reduces
    efficiency of sound transmission to the cochlea.
  • The probe microphone measures the greater
    reflection of the signal.
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31
Q

Acoustic Reflex is a Bilateral Phenomenon

A
  • Stimulation of one ear results in
    contraction of both ears
  • Evaluates the acoustic reflex pathway
  • Presence or absence of the acoustic
    reflex can provide additional diagnostic
    information for CHL or SNHL
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32
Q

The Acoustic Reflex Arc

A
  • Reflex arc:
  • Peripheral ear,
  • VIIIth n.
  • Cochlear nucleus
  • Superior Olivary Complexes
  • Motor Nuclei of VII CN
  • VIIth nerves to stapedial
    muscle of both middle ears
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33
Q

Set-Up for Acoustic Reflex Measurement
2 ways of doing it

A

IPSILATERAL TESTING
* Probe and stimulus in same ear

CONTRALATERAL TESTING
* Probe in test ear, stimulus in
non test ear

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34
Q

Acoustic Reflex Threshold Measurement
What frequencies are the reflexes tested?

A
  • Reflexes are typically tested at: 500, 1000,
    2000, AND 4000 Hz
  • Lowest level in dB HL at which change in
    admittance can be read
  • The probe tip measures the increased
    reflection of the signal and records it as
    needle deflection
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35
Q

Acoustic Reflex Elicitation
Depends on

A

*Depends on the integrity of:
* The hearing in the stimulus ear
* The middle ear status of the probe ear
* The integrity of the central reflex arc

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36
Q

Interpretaton
Acoustic reflex

A
  • Interpretation: Compare the ART to the PT threshold at the frequency tested and determine the SL.
  • Normal Hearing: 70-100 dB HL or SL
  • Cochlear Pathology: <65dB dB SL (Reflex present at normal dB HL
  • Significant SNHL: Absent (moderately-severe and above)
  • Conductive HL: Absent
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37
Q

Electrophysiological
Measures
The 2 types of

A
  • Otoacoustic Emissions
  • Auditory Brainstem Response
    Test (ABR)
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38
Q

What
are
OAE’s

A
  • Sounds produced in a healthy cochlea that
    can be measured in the external ear canal.
  • Generated by the outer hair cells of a
    healthy cochlea.
  • Objective measure that evaluates outer
    hair cell (cochlear) integrity.
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39
Q

Otoacoustic
Emissions
What can they tell us, when does it happens and in what HL are they present

A
  • Pre-neural phenomenum
  • Differentiates between sensory and
    neural
  • Present in ears with normal hearing
  • If present may rule out mild or
    greater sensory impairment.
  • Absent in ears with conductive loss
  • Absent in ears with SN hearing loss
  • Does not quantify degree of HL
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40
Q

Back to A& P of the
Cochlea!

A
  • In a healthy cochlea, sound stimulates
    movement of the outer hair cell.
  • The mechanical movement or motility of
    the OHCs amplifies soft sounds for
    transmission to the cochlea and
    sharpens and improves frequency
    resolution
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41
Q

Outer Hair Cell Motility
How the ear generates the OAE

A
  • Mechanical movement or motility of
    the OHC generates a byproduct
    called otoacoustic emissions
  • These emissions travel from the
    cochlea through the OW, across the
    ossicular chain and vibrate the
    tympanic membrane
  • The OAE is converted to an acoustic
    signal that can be recorded in the ear
    canal
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42
Q

RECORDING OAEs

A
  • Probe tip containing microphone
    and speaker seals the ear canal
  • Speaker delivers signal and the
    microphone records sound present
    in the ear canal
  • Signal averaging separates emission
    response from the noise floor
  • Can be obtained in sleep or
    sedated states
  • Objective
  • Ear specific
  • Frequency Specific
    (somewhat)
    9
    10
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43
Q

Types of Evoked OAES

A
  • Evoked OAE’s
  • Transient (TEOAE’s)
    * Elicited with click stimuli
  • Distortion Product (DPOAE’s)
    * Elicited with pairs of tones
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44
Q

Distortion Product Otoacoustic Emissions

A
  • A pair of tones at 2 different frequencies are sent into the
    ear.
  • The frequencies are separated by a ratio of 1.2
    (Ex. F1=1000Hz and F2=1200Hz)
  • Outer hair cells generate a third tone, the distortion product,
    resulting from the formula
    (2f1-f2) (2X1000 – 1200) = (2000-1200)
    DP = 800Hz
  • Results plotted as DP- gram
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45
Q

Interpretation
of OAE’s

A
  • Present Otoacoustic Emissions
  • Hearing is 30-35 dB HL or better
  • Partially Present Otoacoustic
    Emissions
    * May have HL at particular
    frequencies
  • Absent Otoacoustic Emissions
    • Cochlear disorder, Conductive
      pathology
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46
Q

APPLICATION
OF OAEs

A
  • Pediatric assessment and the difficult-to-test
    population
  • Monitor ototoxiciy
  • Differential DIagnosis– Use with ABR to
    separate cochlear and neural components of
    SNHL (Auditory Neuropathy)
  • Hearing screenings
  • Newborn hearing screening
  • Identify functional or non-organic hearing loss
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47
Q

Auditory
brainstem
response
(ABR)

A
  • Auditory evoked potential (AEP)
    that measures electrical
    response to sound as it travels
    to the brainstem.
  • Noninvasive
  • Ear specific
  • Performed with AC and / or BC
  • Frequency specific stimuli
    23
    24
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48
Q

Auditory
evoked
potentials
(AEP)

A

Auditory system
* Converts acoustic stimuli into electrical signals (voltages or
potentials) to be processed by brainstem structures and the
brain

  • AEPs measure electrical activity in the auditory system
  • Provide information about hearing and the integrity of the
    structures carrying the electrical signals.
  • Signal Averaging
  • AEPs are only a small part of the electrical activity generated
    by brain and environment
  • Signal averaging averages out random background
    activity and amplifies the time locked amplified
    auditory signal
49
Q

Auditory Brainstem Response
The waves
The time and if it’s affected by anything

A
  • Consists of a waveform with a series of 5
    positive peaks
  • The waves correspond to different structures
    of the brainstem
  • Occurs within the first 10 msec following
    signal onset
  • Unaffected by sleep and pharmacotherapy
  • Relates to behavioral threshold
  • May be about 10 to 20 dB poorer
    than behavioral measures
50
Q

Auditory Brainstem Response Waves

A

Wave I: Afferent activity of the CN VIII fibers as they leave the
cochlea and enter the internal auditory canal.
(latency of 1.5 msec)
Wave II: Auditory N / Cochlear Nucleus
(latency of 2.5 msec)
Wave III: CN and Superior Olivary Complex
(latency of 3.5 msec)
Wave IV: Lateral Lemniscus
(latency of 4.5 msec))
Wave V: Inferior Colliculus.
(latency of 5.5 msec)

51
Q

Recording the ABR

A
  • Electrode placement
  • Vertex
  • Forehead
  • Earlobe/mastoid
  • Earphones placement
  • Delivers a series of tones/clicks to
    each ear separately
  • Patient lying still or asleep
  • 30 minutes or longer
52
Q

APPLICATION
OF ABRs

A
  • Assess Neurologic Integrity
  • Latency and morphology of waves
  • Hearing Threshold Estimation
  • Wave V
  • Infant Screening
53
Q

Neurologic
integrity

A

Waveform latency

Waveform morphology— Shape or definition of the
wave

54
Q

ABR Indicators for
Retrocochlear Pathology

A
  • Prolonged interpeak latencies
  • Wave V latency is significantly
    different between ears
  • Poor waveform morphology
55
Q

ABR Sensitivity in diagnosing
VIIIth Nerve (Retrocochlear) Tumors

A
  • In a 2001 report by Schmidt, Sataloff, Newman, Spiegel, and Myers, the sensitivity
    was 58% for tumors smaller than 1 cm, 94% for tumors 1.1-1.5 cm, and 100% for
    tumors larger than 1.5 cm. The overall sensitivity was 90%.
  • CONCLUSION: Auditory brainstem response testing cannot be relied on for
    detection of small acoustic neuromas
56
Q

Hearing Threshold Estimation
Wave V

A
  • Wave V is the most robust
    component
  • Can be observed close to
    behavioral thresholds.
  • Used to estimate hearing
    sensitivity in infants, young
    children and difficult-to-test
    patients
57
Q

Infant Hearing Loss

A
  • 3 infants out of every 1,000 have a hearing loss
  • HL is one of the most common congenital disorders
  • Only 50% have an identified risk factor
  • 90% of infants born with HL have 2 hearing parents.
  • Early intervention (programs and services for families before 6
    months of age provides improved communication outcomes.
58
Q

The Case For
Early
Identification
and
Intervention

A

Children whose hearing loss was identified
and habilitated before 6 months of age
achieved better receptive and expressive
language skills than children whose
hearing loss was identified after 6 months
of age.
* Early identification advantage persists into
the School Years

59
Q

Longitudinal Outcomes of children with
hearing impairment (LOCHI) Study

A
  • The earlier hearing aids or cochlear implants were fitted, the better the
    speech, language and functional performance outcomes.
  • Better speech perception was also associated with better language and
    higher cognitive abilities.
  • Better psychosocial development was associated with better language and
    functional performance.
  • Higher maternal education level was also associated with better outcomes.
  • Qualitative analyses of parental perspectives revealed the multiple facets of
    their involvement in intervention.
60
Q

Early Hearing Detection and Intervention (EHDI)

A
  • First three years are critical period for
    development of speech and language
  • Goals 1 – 3 - 6:
  • Hearing screening by 1st month of life
  • Diagnostic evaluation before 3 months
  • Treatment before 6 months of age
  • Amplification
  • Early intervention services
61
Q

SLP Role
in the
EHDI
Process

A
  • SLP role includes but is not limited to:
  • Administration of hearing screening to
    newborns and toddlers
  • Provision of speech-language therapy
    for children exhibiting communication
    delays as a consequence of pediatric
    hearing loss
  • Parent counseling regarding the
    importance of hearing screening and
    follow-up.
    51
    52
62
Q

Otoacoustic Emissions
Vs

Auditory Brainstem Response

A
  • Sounds are presented to the ear canal
  • Small microphone measures the
    cochlear response in the ear canal
  • Average test time: 5 – 15 min/baby

Auditory Brainstem Response

Sounds are presented and surface electordes
measure brainstem activity.
• Average test time: 20 min/baby

63
Q

Definitions of Pediatrics

A

Congenital – Present at birth
 Prenatal – Occurs to fetus before birth
 Perinatal – Occurs in period shortly before or after
birth (8 wks before to 4 wks after)
 Postnatal - Occurs after birth

64
Q

Three germ layers in embryonic development

A

 Ectoderm
 Epidermis (skin, hair, nails) and nervous system structures
 Ear: Outer ear skin and inner ear sense organs such as the hair cells
and innervating nerves

 Mesoderm
 skeletal, circulatory, reproductive organs and kidneys
 Ear: Ossicles and temporal bone and cartilage

 Endoderm
 Epithelial linings of respiratory tract and digestive tract
 Ear: Forms middle ear lining, aerated mastoid cavities and
eustachian tube

65
Q

External Ear Development

A

External Ear Development
 Ectodermal Tissue
 Begins to form at 4th week of fetal
development
 By 20th week of pregnancy, the auricle is
fully formed and open
 External ear continues to grown in size
until 9 years of age

66
Q

Middle Ear Development

A

 Mesodermal and Endodermal Tissue
 Begins developing at 3rd fetal week
 By 8th week, malleus and incus are present in cartilage and
stapes by week 15.
 By 9th week, 3 tissue layers of the TM are present
 Development of ME continues through 37th week of
gestation

67
Q

Inner Ear Development

A

Ectodermal Tissue
 3rd week – IE begins to develop
 6 weeks - vestibular structures
 11th week - Presence of 2 ½ cochlear coils
 Development of the inner ear is mainly in the first trimester and
mature by the 20th week
 Some changes are still occurring between the 5th month (i.e. 20th week)
and the 8th month

68
Q

KEY POINTS

A

 Knowledge of the origins of auditory structures can be diagnostically
significant.
 Ectoderm is responsible for development of outer skin layers and the inner
ear
 Skin disorder and deafness - Keratitis Ichthyosis Deafness Syndrome
 During the formation of the embryo, an abnormal formation of one organ
will many times indicate the abnormal formation of another.
 Microtia, atresia and preauricular pits raise concerns about associated
IE disorders

69
Q

More KEY POINT

A

Time of disruption of fetal development determines degree of
developmental malformations and susceptibility to hearing loss
 For example, since kidneys and ear develop around the 5th to 8th week
of pregnancy, and because of their similar embryonic structure,
infection occurring at that time may affect development of both organs
at the same time.
 Malformations occurring late in fetal development are simple while
those occurring early on are more complex and severe (ex. Rubella)

70
Q

Additional Testing and Treatment

A

 CT Scan Imaging – abnormally formed OC
 Rule out syndromes, kidney disorders, facial defects
 Interdisciplinary team for best coordinated care
 ENT
 Plastic Surgeon – surgical reconstruction
 Audiologist- Amplification device to maximize hearing for speech and
language development
 Speech Pathologist for possible communication delays
 Pediatrician
 Genetic counselor

71
Q

Genetics and Hearing Loss

A

 Genetic factors are responsible for over 50% of hearing
loss.
 Autosomal Recessive – 80% and more severe HL
 Autosomal Dominant – 18% and less severe HL
 X Linked - 1 to 3%
 Mitochondrial - <1%
 2/3 of hereditary HL is non-syndromic and 1/3 is syndromic

72
Q

Hereditary Hearing Loss
Chromosomes

A

 All hereditary material, in the form of DNA, is carried as genes on the
chromosomes
 All human body cells contain 23 pairs of
chromosomes (46 total)
 22 pairs (44) autosomes
 2 sex chromosomes
 Female – two X chromosomes (46,XX)
 Male – one X and one Y (46,XY)

73
Q

Autosomal Dominant Inheritance
Vs Autosomal Recessive Inheritance

A

 One parent carries the trait
 Child needs to inherit one copy of
dominant trait to have disorder

 50% chance that the offspring will
have the trait
 Accounts for 20% of genetic HL

Autosomal Recessive Inheritance

 2 parents carry recessive gene
 Child receives both copies of the genes

 25% chance that fetus will inherit both genes
 50% chance that child will be a carrier
 25% chance child is genetically normal

 Accounts for 80% of childhood deafness

74
Q

X-linked Recessive Inheritance

A

 Affects only males
 Trait is on the Mother’s X chromosome
 Males infected through carrier female
 50% chance of son expressing trait
 50% chance that daughter is a carrier
 Accounts for 2-3% of deafness

75
Q

Syndromic vs Non-syndromic HL

A

 NON-SYNDROMIC
 No associated abnormalities

 SYNDROMIC
 Presence of other abnormalities
 external ear, skull, facial deformities, cleft palate,
 optic disorders, changes in eye, hair and skin pigmentation,
 thyroid disease,
 disorders of the heart,
 musculoskeletal anomalies,
 mental retardation,
 difficulty with balance

76
Q

Alport

A

ASSOCIATED ANOMALIES: Kidney problems
INHERITANCE: X Linked, AR, AD

77
Q

Branchio-oto-renal (BOR)

A

ASSOCIATED ANOMALIES: Neck cysts and/or ear tags and kidney problems
INHERITANCE: AD

78
Q

Jervell and Lange-Nielsen

A

ASSOCIATED ANOMALIES: Cardiac problem. Prolonged QT intervals
INHERITANCE: AR

79
Q

Neurofibromatosis II

A

ASSOCIATED ANOMALIES: Tumors on Auditory Nerve
Balance, tinnitus, ABI candidates
INHERITANCE: AD

80
Q

Pendred

A

ASSOCIATED ANOMALIES: Thyroid enlargement or low thyroid function Associated with EVA or mondini dysplasia
INHERITANCE: AR

81
Q

Stickler

A

ASSOCIATED ANOMALIES: Connective tissue disorder with craniofacial abnormalities, cleft
palate, eye problems (myopia, retinal detachment, glaucoma,
cataracts), arthritis, over-flexible joints
INHERITANCE: AD

82
Q

Treacher Collins

A

ASSOCIATED ANOMALIES: Craniofacial abnormalities
ME abnormalities
INHERITANCE: AD

83
Q

Usher

A

ASSOCIATED ANOMALIES: Progressive blindness, Clumsy gait
INHERITANCE: AR

84
Q

Waardenburg

A

ASSOCIATED ANOMALIES: White patch of hair or light-colored skin patches;, different color
eyes, widely spaced eyes, flattened nose bridge. Can show some,
one, all, o none of typical features. do well with CI.
INHERITANCE: AD

85
Q

Non-Syndromic Hearing Loss

Connexin 26 HL

A

Connexin 26 HL
 Autosomal Recessive
 Mutation in GJB2 (gap junction beta 2) gene that provides instructions for
making a protein called connexin 26
 Connexin 26 is important for the proper flow of potassium in the cochlea
 Most common cause of congenital hearing SNHL
 70% with severe to profound HL (>75dB HL)
 Normal inner ear structures

86
Q

CHARGE Association

A

C – Coloboma of the eye
H – Heart defects
A – Atretic choanae
R – Retarded postnatal growth
G – Genital and or urinary abnormalities
E – Ear anomalies and deafness
CHL – Common
SNHL – 90% Malformed cochlea

87
Q

Cochlear Malformations

A

 Mondini – incomplete formation
 1.5 turns to the cochlea instead of 2.5.

 Scheibe Dysplasia
 Organ of corti is completely missing
 SCCs and utricle are normal

 Michel Deformity
 Complete absence of IE

88
Q

Enlarged Vestibular Aquaduct (EVA) Syndrome

A

The vestibular aqueduct, is a fluid filled tube that connects
the inner ear to the endolymphatic sac, is enlarged.
 Leading cause of congenital progressive SNHL
 Head trauma can cause fluctuation and progression of HL
can occur following head trauma
 May be mixed component
 Can occur alone or be associated with Pendred and other
syndromes
 Detected with CT/MRI scan and history
 Counseling – Children should avoid activities that can lead to
head injuries, wear protective head gear

89
Q

TORCH Infections

A

Toxoplasmosis
Other infections
Syphilis, Hepatitis B
Herpes Zoster
Rubella
Cytomegalovirus (CMV)
Herpes Simplex Virus

90
Q

Perinatal Birth Trauma

A

 Anoxia
 Prematurity
 High Forceps Delivery
 Hyperbilirubinemia
 Kernicterus

91
Q

What is Cytomegalovirus (CMV)

A

 Very common typically harmless virus
 Member of the herpes virus family
 Can cause cold-like symptoms
 Most US adults have been exposed
 CMV can be transmitted from person to person in bodily fluids

92
Q

Congenital Cytomegalovirus (cCMV)

A

 When a pregnant women or woman about to be pregnant has CMV cold
virus circulating in their system, it can pass through placenta to growing
fetus.
 Leading cause of intrauterine fetal demise, stillbirth and pregnancy loss
 Most common congenital viral infection and the leading cause of non-
genetic hearing loss in newborns
 1 out of 200 babies is born with cCMV
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93
Q

Possible Neurodevelopmental Outcomes

A

 Visual
Impairment
 Hearing
Impairment
 Cerebral
Palsy
 Epilepsy
 Learning disabilities
 Intellectual disabilities
 Vestibular disorders

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94
Q

Presentation with cCMV

A

10 % are born symptomatic
* Small for gestational age
* Microcephaly
* Petechiae/purpura
* Hepatosplenomegaly
* Seizures
* Intracranial abnormalities
* Laboratory abnormalities
90% are born asymptomatic
* Up to 25% of children with asymptomatic cCMV, will develop progressive or late onset
hearing loss in childhood,

95
Q

Testing for cCMV

A

Diagnosis is only possible when the infant is <21 days
 Difficult to distinguish cCMV vs post natal CMV after 3 weeks of
age
 Test by urine, saliva, or blood

cCMV Newborn Screening
 ALBANY, N.Y. (September 29, 2023) – The New York State
Department of Health announced that effective October 2, 2023,
all babies will be screened for Congenital Cytomegalovirus
(cCMV), making New York the second state in the nation, after
Minnesota, to screen all babies for the virus.
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34

96
Q

CMV Risk Reduction

A

 Avoid kissing toddlers on the lips
 Don’t put a pacifier in your mouth
 Wash hands well after a diaper change
 Don’t share food, straw, or utensils

97
Q

Postnatal Infections

A

Viral Infections
 Mumps – unilateral HL
 Measles
 Pertussis
 Rubella
 Chicken Pox

98
Q

Postnatal Bacterial Infections

A

Meningitis
 Sequelae to otitis media
 Inflammation of the meninges
of the brain
 Bilateral, symmetrical and
irreversible SNHL
 HL may range from mild to
profound

Labyrinthitis
 Infections of the labyrinth
 Affects auditory and vestibular
system
 Hearing loss and vertigo
 Cause often unknown
37
38

99
Q

Ototoxic Medications

A

 Aminoglycoside Antibiotics
 Gentamycin, tobramycin, kanamycin, streptomycin
 Diuretics
 Furosemide (Lasix)
 Chemotherapy
 Cisplatin
 Chemotherapy drug puts young children with cancer at high risk of hearing loss
 75% of patients five years old and younger had experienced cisplatin-related hearing
loss three years after starting therapy
 www.sciencedaily.com/releases/2021/09/210907110725.htm

CASE HISTORY

100
Q

Comprehensive Audiological Evaluation

A

 Case history
 Interview and observation
 Otoscopic examination
 Audiometry
 Type and degree of hearing loss, speech discrimination, and auditory
perception
 Objective measures
 Immittance, otoacoustic emissions (OAEs), evoked auditory potentials (EAPs)

101
Q

Pediatric Case History

A

Information derived from a comprehensive case history
includes:
 Factors that increase the possibility of HL
 Clues about the etiology of HL
 Other developmental concerns or issues
 How the family views HL

102
Q

Pediatric Assessment - Test Techniques

A

 BOA
 Behavioral Observation Audiometry

 COR/ VRA
 Conditioning Orienting Reinforcement/ Visual Reinforcement
Audiometry

 CPA
 Conditioned Play Audiometry

 Conventional Audiometry

103
Q

Behavioral Observation Audiometry (BOA)

A

 Birth to 6 months (developmental)
 Unconditioned response
 Sudden novel sound presented
 Observe baby’s response to sound
 Minimum response level (MRL)
 Softest level that elicits a change in behavior
 Typically, louder than threshold
 Variables
 Infant’s behavioral state and responses
 Method of stimulus presentation
 Observer bias

104
Q

Visual Reinforcement Audiometry (VRA)

A

 Chronological or cognitive age of 6 months - 2 ½ years
 Conditioned response
 Child conditioned to look or turn towards a sound source and
is reinforced by a lighted animal or video
 Signal presented through speaker in soundfield or
through earphones
 Threshold responses
 Child faces midline when not
responding to sound
 Role of assistant is to distract
the child usually with a toy
 Distraction toy should not be
too interesting that it interferes
with responsiveness to signal

105
Q

Conditioned play audiometry (CPA)

A

 Child performs a specific
play activity in response to
sound (i.e. throwing a block
in a bucket)
 Chronological or cognitive
age of 2-4 years
 Threshold test

106
Q

Speech Recognition / Awareness Thresholds

A

SDT/SAT
 Can be 10-15dB lower than SRT
 Stimuli used are child’s name, continuous discourse, CV syllabi or short
phrases

 SRT
 Gain information about child’s hearing sensitivity in the frequency range
of 500-2000Hz
 Spondee picture card or pointing to objects representing the spondee
words can be used for children with expressive or receptive speech and
language deficits

107
Q

Word Recognition Scores (WRS)

A

 Impact of HL on speech understanding abilities of the
child
 Helps determine type and severity of HL
 Provides valuable information for AR recommendations
(HA, ALD, CI)

108
Q

Word Lists

A

 Phonetically Balanced Kindergarten (PB-K)
 Age 4 and older who can provide verbal response

 Northwestern University Children with Hearing
Impairment (NU-CHIPS)
 4 item closed response task
 Age 2 ½ and older

 Word Intelligibility by Picture Identification (WIPI)
 6 item closed response picture identification task
 Age 5 and older

109
Q

Objective test measures

A

 Immittance Measures
 Presence of middle ear pathology
 Auditory Brainstem Response (ABR) Test
 Close to behavioral thresholds
 Can obtain bone conduction thresholds
 Sedated vs non sedated
 Otoacoustic Emissions
 Normal or near normal level of hearing
 Can be obtained within first several hours after birth and are not dependent on maturation of
the auditory system
 Useful in monitoring effects of ototoxic medications in cases of premature or ill infants

110
Q

Otitis Media in Children

A

 Number 1 reason for children’s visits to a pediatrician in
the US
 Before the age of 6 years, approximately 85-90% of
children will have at least one bout of OM
 Nearly 20% of children with recurrent OM will require
the placement of PE tubes.

 Underlying cause of OM is related to ET dysfunction
which leads to the production of fluid by the
mucosal lining of the ME
 ET in children is more horizontal, shorter and
composed of more flaccid cartilage compared to
adults
 Typically, ET is fully developed by 7-8 years
 Chronic condition may be related to allergies or
some other underlying medical condition (URI)
eust_tube_compare_sm
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111
Q

Risk Factors for Otitis Media

A

Family history of OM
 Low socioeconomic environments
 Exposure to second hand smoke
 Day care attendance
 Children with Down Syndrome and craniofacial
anomalies

112
Q

Audiological f/u Otitis Media

A

Ongoing audiological f/u for unresolved OM
 Monitor for conductive HL and or other complications such as
cholesteatoma or perforated TM
 Monitor for long-term effects such as speech and language
development and /or APD
 Consider mild gain FM system in the classroom or Bone-
anchored HA (BAHA) for children with chronic OM

113
Q

Unilateral HL in Children

A

 2 out of every 1000 young children have permanent unilateral hearing loss.
 Children with unilateral hearing loss are at 10 times the risk for learning problems
compared to children with 2 normal hearing ears
 Difficulty with sound localization
 Difficulty with speech recognition in noise
 Exhibit educational and behavioral problems in school
 May benefit from HA or ALD
 Candidate for BAHA or CI
 Use of communication strategies training to prevent communication breakdown

114
Q

Auditory Neuropathy Spectrum Disorder (ANSD)

A

Audiometric profile:
 SNHL that can range from mild to profound and may fluctuate
 Normal OAEs
 Absent or severely abnormal ABR
 Absent ipsi and contra AR
 Believed to be function of a lesion central to the cochlea and may
be result of lack of synchrony of neuronal firing.

115
Q

Auditory Neuropathy Spectrum Disorder (ANSD)
Causes

A

 Possible Causes
 Prematurity
 Hyperbilirubinemia
 Hypoxia
 Metabolic disorders
 Genetic
 Comorbid disabilities
 Variability in function

116
Q

Auditory Neuropathy Spectrum Disorder (ANSD)
 Management options

A

 Management options
 Amplification (hearing aids)
 Personal FM devices
 Cochlear Implants
 Sign Language
 Visual cues and support
 Lip reading
 Cued speech

117
Q

Teaching Strategies

A

 Provide visuals
Written outline
Boards and projectors
Visual prompts and nonverbal cues
Flashing or turning off lights and pointing can be
used to indicate a transition to a new activity.

Teaching Strategies
 Use the word ‘Listen: to indicate to students that they need to pay attention to the
information that will follow.
 Repetition allows students to hear and process what has been said.
 Repeat what other students in the class have said so student with HL does not miss
out on questions, comments, or class discussion
 Listening buddy can repeat or clarify directions, as well as take notes for student
with HL
 Students with HL should meet with teacher or a tutor before and after lessons to
verify comprehension and provide student with more practice and familiarity with
topics.
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118
Q

More Teaching Strategies

A

 Use secret or nonverbal signal between teacher and student with HL to
indicated comprehension of materials or a breakdown of understanding.
 Make eye contact with students with HL before making important
announcement or giving directions to ensure that the students are attention
and prepared to listen
 Face student when speaking to better project the voice as well as give
speechreading cues. Good lighting is important.
 Speak at measured pace to allow student time to process what is being
said.
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Audiology (6 decks)

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