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HESP645 > Midterm > Flashcards

Midterm Flashcards

1
Q

Why do we care about the typically developing timeline for children?

A

1) to develop age appropriate diagnostic tests
2) to modify testing as needed
3) to recognize when a child has a delay

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2
Q

What are the three listening elements?

A

1) Comprehension
2) Retention
3) Response

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3
Q

What are three expressions of language?

A

1) speech
2) signing
3) writing

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4
Q

What is the cross-model regeneration?

A

Rewiring of the brain, Some of this can be reversed during early development, brain spurts begin to slow around 1o years (critical period)

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5
Q

Neuroplasticity & hearing

A

young brains are incredibly neuroplastic- if there’s a lack of auditory stimulation, the brain will try to make up for it with visual information, however, auditory neurons that are not reinforced will begin to atrophy (auditory deprivation)

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6
Q

/b/ and /g/ can be differentiated by ____

A

2 months of age

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7
Q

Incidental language

A

children “overhear” speech (reinforcement of language)- happens before oral motor movements for speech and before concepts of language have evolved- starts at birth

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8
Q

Early auditory responses

A

they are limited and reflexive; eye blinking/widening, sucking paradigms, startle response

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9
Q

which occurs first? localization or lateralization?

A

lateralization occurs first

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10
Q

Lateralization

A

right/left, horizontal plane; rudimentary development around 2-3 months, well developed by 6 months; seen in head turn, eye shifts

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11
Q

Localization

A

exact location in space- can occur in any plane

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12
Q

Auditory Feedback Loop

A

kids exposed to this at birth, “I hear it”-“I process it and modify it”- “I say it”, example- if you have a cold and you realize you sound different

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13
Q

Hoff 2003: “children who….”

A

children who hear longer utterances built more productive vocabularies at higher rates

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14
Q

Differences in language development

A

Socioeconomic status, maternal education- maternal education, aside from SES, is most strongly related to parental measures

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15
Q

Development: Birth-3 months

A

Auditory: startles to loud sounds, quiets (calms) when spoken to, recognize mom’s voice

Speech: Physiologic sounds (crying, cooing, gurgling), cries for different needs

Developmental: by 2 months baby can grasp objects, smiles, may have separation anxiety

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16
Q

Development 3-6 months

A

Auditory: responds to changes in tone, notices toys that make noise/sounds, pays attention to music, lateral eye tracking to sounds emerges, mimic vowels toward 6 months

Speech: true babbling begins, may use /p/, /b/, /m/ and vocalizes excitement and displeasure

Developmental: begin eating solid foods, begin sitting upright (~6months)

  • Circle of self gets bigger
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17
Q

Development: 6-10 months

A

Auditory: enjoys games like peak-a-boo, and pat-a-cake; turns and looks in the direction of sound (VRA!)

Speech: Babbling has long and short groups of sounds, canonical (reduplicated)- Uses speech or non-crying sounds to get and keep your attention, uses gestures (holding arms up)- deaf babies will stop reduplicated babbling due to a lack of reinforcement

Developmental: Head and neck muscles strengthen

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18
Q

Development: 9-12 months

A

Auditory: Listens when spoken to, recognizes words for common objects, begins to respond to simple requests like “come here”, localization instead of just lateralization, recognizes their own name

Speech: Understands “no”, tries to repeat words, may have 1-2 words (mama, dada, hi)

Developmental: first steps

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19
Q

Developmental: 12-17 months

A

Auditory: attends to toy or book for about 2 minutes

Speech: Follow simple directions accompanied by gestures, answers simple questions nonverbally, points to objects pictures, family members, says 2-3 words labeled to a person or object, tries to imitate simple words

How intelligible is he/she to others? Prime VRA age

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20
Q

Development 18-23 months

A

Speech/Language: follows simple commands without gestures, points to simple body parts (nose, mouth), understands simple verbs such as “eat”, “sleep”, correctly pronounces most vowels, /n/, /m/, /p/, /h/ especially in the initial position and is beginning to use other speech sounds, says 8-10 words and understands many more

Developmental: beginning to run, walks up and down stairs

*a normal 2 year old will want nothing to do with VRA

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21
Q

Development: 2-3 years

A

S/L: Knows about 50 words, some spatial concepts (in, on), pronouns such as “you” “me”, “her”, descriptive words such as “big”, “happy”, says about 40 words at 24 months, answers simple questions, speaks in 2-3 word phrases

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22
Q

Before the UNHS… average age of HL ID was…

A

average age of HL ID was 2.5 years, intervention around 3.5 years

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23
Q

When did JCIH and the UNHS come about

A

1994!

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24
Q

Main points from Yoshinaga-Itano (1998)

A

1) children who were early identified and received early intervention maintained language similar to their nonverbal cognitive quotient
2) Later identified kids showed >20 point discrepancies between nonverbal cognitive quotient and language development
* Early ID isn’t worth much w/o early intervention

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25
Q

Goal of the Colorado Home Intervention Program

A

optimal social-emotional, language, and communication strategies

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26
Q

Main findings from Moeller (2000)

A

negative correlation between age of enrollment and language outcomes- earlier enrollment = increased vocabulary & verbal reasoning skills, average time between identification and intervention = 3 months, outcomes affected by early enrollment in EI program and strong family involvement

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27
Q

Early Intervention

A

Broad term- applied to ALL areas of development

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28
Q

Early Intervention for HEARING LOSS

A

Fitting hearing aids, CI evaluation, Arranging home visits, Advice and training for parents

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29
Q

Goals of EHDI

A

1-3-6

1) All newborns will be screened for HL by 1 month of age
2) All infants who screen positive will have a diagnostic audiologic evaluation before 3 months of age
3) All infants identified with HL will receive appropriate early intervention services before 6 months of age
4) All infants and children with late onset, progressive, or acquired hearing loss will be identified at the earliest possible time
5) All infants with HL will have a medical home
6) Every state will have a complete EHDI tracking and surveillance system that will minimize loss to follow up

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30
Q

JCIH Goals for optimal outcomes (once HL is identified)

A
  1. children with HL should have access to timely and coordinated entry into early intervention programs
  2. children with HL should have access to culturally competent services with provision of the same quality and quantity of information given to families from the majority culture
  3. all children who are deaf/HOH should have their progress monitored from birth to 36 months
  4. all children identified with any degree/configuration of HL receive appropriate monitoring
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31
Q

Family centered intervention

A

all early intervention should be family centered, what is the family comfortable with? where are they at in their level of acceptance? it is the perrogative of the family to refuse intervention services

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32
Q

FDA cochlear implants in babies-

A

12 months old (minimum age) since 2000

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33
Q

You see a 2 year old in your clinic for a hearing evaluation and the parent reports that the kid says about 10 words but understands everything that is said to them- would you refer for a S/L eval?

A

YES

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34
Q

What is the general concept of a universal newborn screening?

A

the practice of testing every newborn for certain harmful or potentially fatal disorders that aren’t otherwise apparent at birth

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35
Q

What are babies (now a days) screened for at birth?

A

genetics, endocrine, metabolic function, critical congenital heart defects, hearing!

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36
Q

Prevalence vs. Incidence

A

prevalence: proportion of a population found to have a condition or abnormality expressed as a fraction or percent, how many people have this condition right now?
incidence: number of NEW cases arising in a given period of time (month, year, etc) incidence increases with age for HL b/c SNHL is permanent

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37
Q

Prevalence of HL

A

1-2/1000: severe to profound SNHL
4/1000: includes mild, moderate, and unilateral HL
Higher in NICU population

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38
Q

Screening Sensitivity

A

Ability of a test to correctly identify patients with disease, highest possible sensitivity to ID as many with the disease as possible

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39
Q

Screening Specificity

A

Ability of test to correctly ID those without disease, as test begins to fail normal hearing infants the specificity decreases

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40
Q

Decision Matrix for screenings

A

allows us to calculate validity of screening test:
True positive, refer
True negative, pass
False positive, refer with normal hearing
False negative, pass with hearing loss

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41
Q

Crib-o-gram

A

1970s - objective response to sounds, Simmons and colleagues- 90 dB SPL, 3k sounds- movements detected by photoelectric transducer/sensor placed under the crib mattress that can detect movements- 12% false positive rate, 5% false negative rate,

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42
Q

1960s Downs and Sterritt universal screening

A

first descried UNHS, city wide project for 1 year, 17k infants, 2500-3500 Hz pure tone at 90 dB SPL and white noise at 93 dB SPL
Eye blink or Moro’s reaction recorded by observers… problem? NOT OBJECTIVE

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43
Q

When did JCIH form?

A

1970

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44
Q

UNHS what happened with JCIH in the 1970s

A

formation of the high risk register, 5 risk factors part of the original criteria; Problem- only identified about 1/2 of babies with HL

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45
Q

What laid the groundworks for IDEA

A

EHCA: education of handicapped children act (1975)

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46
Q

Original high risk registery

A

1) history of childhood HL
2) Rubella or other fetal infection
3) Defect of the ear, nose, throat
4) Low birth weight
5) Billirubin level greater than 20 mg/100 ml

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47
Q

why is high bilirubin bad

A

it’s neurotoxic at high levels

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48
Q

When did OAEs/automated ABRs become widespread

A

1980s

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49
Q

When were ADA and IDEA passed

A

1990

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50
Q

When did JCIH state that infants should be screened with physiologic testing techniques and it should be universal?!

A

1994

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51
Q

Cost of screening factors

A

Factors- salary, screening rate, equipment costs, maintenance (calibration), lifetime of the equipment, number of children screened per year

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52
Q

OAE vs. ABR

A

Both physiologic measures, high sensitivity and specificity, Responses correlate with normal hearing, can be completed with automated equipment, pass/fail response, can be completed by trained volunteers, differences– OAE ok for well babies, ABR better for NICU babies (neural hearing risk)

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53
Q

Well Babies

A

majority of births- risk factors less likely- includes birthing centers and home births

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54
Q

NICU babies by level

A

Level 1- basic care
Level 2- premature/recovering from serious illness
Level 3- premature surgical or serious medical needs
Level 4- highest level often regional center

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55
Q

AABR

A

automated ABR- measuring at the level of the brainstem- screening abr at 30 or 35 dB- a mild HL could pass the screening- enough to tell a parent if their child’s hearing will be adequate for speech and language development - pass or fail- machine compares the response to a template

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56
Q

AABR advantages/disadvantages

A

Advantages: able to detect neural HL, ANSD will fail, automated (minimal training needed), non-invasive
Disadvantages: may take 10-20 minutes, cost of disposable equipment, requires sleeping baby, requires mature neurons

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57
Q

DPOAEs vs. TEOAEs

A

Can pass DP with hearing loss at 40dB; TE need at least 30 dB thresholds; TE are clicks (broadband); DP are more frequency specific

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58
Q

OAE advantages and disadvantages

A

Pro: fast (3-6 minutes), non-invasive
Con: need quiet area, affected by blockage in ear canal/ME status, will NOT detect ANSD/missing or hypoplastic nerve/retrocochlear pathology

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59
Q

How many screenings for WBN

A

2 then refer; re-screen should be before hospital discharge on the same equipment

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60
Q

How many screenings for NICU

A

1 - fail –> refer for diagnostic evaluation

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61
Q

Current JCIH risk factors

A

Caregiver concern, Family Hx of congenital/childhood HL, NICU stay >5 days, ECMO, ventilation, ototoxic med, hyperbilirubinemia requiring blood transfusion, Congenital infections (TORCH), craniofacial abnormalities associated with syndrome, syndromes associated with HL, neurodegenerative disorders, meningitis, head trauma, chemotherapy

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62
Q

TORCH:

A

toxoplasmosis, OTHER (syphilis), Rubella, CMV, Herpes

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63
Q

EHDI team members

A

birth hospital-family-pediatrician-audiologist-otolaryngologist-SLP-Deaf/HOH educators

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64
Q

Responsibilities of birth hospital

A

Complete screening, review results with parents, provide follow up info, report results to state

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65
Q

Responsibilities of the family

A

love and nurture the child, communication with infant, intervention appointments, support groups

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66
Q

Most common risk factors identified in the NICU

A

aminoglycoside medication for greater than 5 days then assisted ventilation, low apgar, very low birth weight

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67
Q

Risk Factors of WBN

A

family history of HL, craniofacial abnormalities, low apgar at 1/5 minutes, syndrome associated with HL

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68
Q

Not usually identified in WBN risk factors

A

Meningitis, VLBW, hyperbilirubinemia, ventilation

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69
Q

As of 2010 ____ states & DC have statutes or regulations governing newborn hearing screenings

A

41

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70
Q

___ states have EHDI programs

A

ALL

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71
Q

___% of infants in the US are screened by 1m month (current stat)

A

97

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72
Q

As of 2007, what was the USA loss to F/U rate

A

46% nationwide

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73
Q

Describe the barriers to follow up.

A

SES, maternal factors (age/education/prenatal care), Health, Availability of f/u facilities, Language

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74
Q

What are the risks of untreated childhood hearing loss?

A
  • Speech and language delays (or development altogether)
  • Social delays
  • Reading and educational delays
  • Psychological effects
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75
Q

What makes identifying hearing loss in children different from identifying hearing loss in adults?

A
  • Shorter attention span
  • Can’t speak for or express themselves
  • Smaller ears – affects tools, probe size, hearing aids, etc
  • Test procedures – VRA, CPA
  • Speech/language and Vocabulary – SRT, Word rec
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76
Q

What are the 3 stages of embryologic development?

A

Pre-embryonic, embryonic, fetal

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77
Q

The pre-embryonic stage is which weeks?

A

1-3

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78
Q

The embryonic stage is which weeks?

A

4-8

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79
Q

The fetal stage is which weeks?

A

9-full term

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80
Q

What are the highlights of the pre-embryonic stage?

A

formation of germ layers and the neural tube; otic pit becomes hallow

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81
Q

When (in development) would you expect there to be 1.5 turns of the cochlea?

A

week 8

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82
Q

What embryologic developmental stage is characterized by rapid growth?

A

Fetal stage

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83
Q

What is considered a pre-mature birth?

A

before 37 weeks

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84
Q

In what embryological development stage is development significantly susceptible to disruption from teratogens?

A

Embryonic Stage

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85
Q

In what stage of embryonic development would you expect to see gross development of major organs?

A

Embryonic Stage

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86
Q

In what stage are all auditory centers and pathways of the brainstem are identifiable?

A

Embryonic Stage

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87
Q

When in embryonic development would you expect to see 2.5 turns of the cochlea?

A

Week 10

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88
Q

When does the cochlea become adult in FORM?

A

Week 10

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89
Q

When does differentiation of the Organ of Corti & hair cells occur?

A

Weeks 11-12

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90
Q

When does the cochlear duct measure 33-37 mm (adult size)?

A

Week 16

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91
Q

When do supporting cells appear?

A

Week 17

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92
Q

At what point is the eardrum fully formed?

A

Week 30

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93
Q

At what point is the middle ear pneumatized?

A

Week 35

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94
Q

What is considered the perinatal period?

A

Birth-6 months

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95
Q

What are the highlights of the perinatal period?

A
  • Continued maturation of auditory cortex
  • Changes in latency responses to auditory stimuli
  • Greater sensitivity to speech than tones
  • Recognition of wider range of contrasts in phonemes until age 4 months
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96
Q

The layers of the TM and position of the layers

A
  • Outer layer – ectoderm
  • Middle fibrous layer – mesoderm
  • Inner layer – endoderm
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97
Q

When does the meatal plug disintegrate to form the ear canal?

A

Week 21

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98
Q

When are the ossicles formed?

A

Week 8

99
Q

What comes from Meckel’s cartilage

A
  • Associated with 1st arch
  • Head and neck of the malleus
  • Body and short arm of the incus
100
Q

What comes from Riechert’s cartilage

A
  • Associated with 2nd arch
  • Manubrium of malleus
  • Long arm of incus
  • Stapes
101
Q

What % of childhood HL is genetic?

A

50% (maybe a little more than..)

102
Q

What % of genetic childhood HL is syndromic/non-syndromic?

A
  • 30% Syndromic

* 70% Non-syndromic

103
Q

Prevalance of HL in children

A

~2-3/1000 (may be as high as 6/1000) HL

104
Q

% of childhood HL that is idiopathic

A

25%

105
Q

% of childhood HL that is environmental

A

25%

106
Q

What is the most common cause of genetic HL

A

Connexin 26

107
Q

What type of HL is mitochondrial HL?

A

Non-syndromic, genetic HL

108
Q

X-linked inheritance

A
  • Only occurs on X chromosome
  • Usually only seen in males (some females may exhibit mosaic characteristics)
  • Female children = 50% chance of being carrier
  • Male children = 50% chance of hearing loss
109
Q

Mitochondrial inheritance

A
  • Mitochondria passed by the mother
  • 100% chance of being affected
  • No mitochondria passed from father
110
Q

Why do we care about the causes of hearing loss?

A

to help:

1) prevent progressive hearing loss
2) diagnose co-morbidities
3) guide communication modes
4) hearing health of other family members

111
Q

What to look for on the outer ear (Causes of HL)

A

check for position of ears, check for lumps, ulcers, pre-auricular pits/tags, lesions on pinna etc.

112
Q

Causes of HL: Outer ear pathology

A

Microtia, Atresia, Stenosis, cerumen, foreign body, otitis media

113
Q

Microtia

A

Abnormally formed or absent pinna (1/20,000) births

114
Q

Atresia

A

Complete closure of the ear canal

115
Q

Stenosis

A

narrowing of ear canal

116
Q

If a kid has a conductive HL but an open (even narrow) canal…

A

Use a BTE! Kids love them

117
Q

Middle Ear Pathology (Causes of HL)

A

Otitis media (with effusion, w/o effusion, with perforation), cholesteatoma, trauma, foreign body, cleft lop/palate,

118
Q

Acute Otitis Media

A

0-21 days

119
Q

Sub-acute Otitis Media

A

22 days-8 weeks

120
Q

Chronic Otitis Media

A

lasting longer than 8 weeks

121
Q

Recurrent Otitis Media

A

three or more incidences of OM within 6 months- continuous problems during childhood, 71% by age 3

122
Q

___% of children have at least ear infection by 6 years old

A

96%

123
Q

What might cause unexplained clumsiness or balance issues?

A

Otitis Media

124
Q

Cleft Lip/Palate (Causes of HL)

A

considered common; 1 in every 700 births;w with cleft palate/lip- 97% chance of OM by 24 months

125
Q

Down Syndrome (Causes of HL)

A

Usually CHL due to effusion and/or cerumen impaction, although can be SNHL

126
Q

Pierre Robin (Causes of HL)

A

Cleft palate; tongue that is placed further back then normal (glossoptosis), smaller lower jaw, feeding problems and difficulty breathing, can occur on its own, also common with stickler syndrome

127
Q

Collapsing canals

A

HF CHL; when using supra-aural headphones; check by switching to inserts

128
Q

Endogenic causes of HL

A

Genetics…

129
Q

Exogenous causes of HL

A

TORCH, meningitis, anoxia, ototoxicity, trauma, noise induced HL

130
Q

Goldenhar’s Syndrome

A

autosomal dominant- head and facial abnormalities, kidney abnormalities, CHL most common, 15 % SNHL

131
Q

Waardenburg syndrome

A

autosomal dominant- white forlock in hair, wide set eyes, usually bilateral SNHL

132
Q

Treacher-Collin’s Syndrome

A

autosomal dominant- skeletal structure abnormalities in face, cleft palate, bilateral congenital microtia or atresia, CHL (but can be mixed or SNHL)

133
Q

Charge syndrome

A

CONSIDERED A DEAF-BLIND syndrome for educational purposes- autosomal dominant- coloboma, heart defect, atresia choanae, retarded growth and development, genital abnormality, ear abnormality, prone to autism or ANSD as co-morbidity

134
Q

Stickler Syndrome

A

eye abnormalities, hearing loss becomes more severe over time, SNHL or conductive, joint problems

135
Q

Hurler Syndrome

A

Moderate to severe SNHL, heart defects, dwarfism, severe intellectual disability

136
Q

Usher syndrome

A

3 progressive types (I, II, III); retinitis pigmentosa (tunnel vision), thyroid problems, balance issues, most common deaf-blind syndrome

137
Q

Alport syndrome

A

Definitely HA candidate, usually CI candidate- Mostly X linked- more often seen in males- kidney dysfunction and progressive SNHL

138
Q

Turner Syndrome

A

Monosomy X- spontaneous mutation; short stature, early loss of ovarian function

139
Q

Connexin 26 (DFNB1)

A

May account for 10-30% of AR cases, usually bilateral symmetrical SNHL, sometimes starts unilateral, often progressive

140
Q

Inner ear dysplasia

A

abnormal development of the inner ear

141
Q

4 types of inner ear dysplasia

A

Michel, Mondini, Scheibe, Alexander

142
Q

Problems with auditory nerve

A

1) hypoplasia

2) aplasia

143
Q

EVA

A 
enlarged vestibular aqueduct!
diagnosed through imaging
most common ear abnormality found on a CT scan
hearing loss is often progressive
often CI candidates
tends to run in families
144
Q

Auditory Processing Disorder

A

symptoms- listening complaints; use a test battery for diagnosis; treatment- accommodations (FM, notes); RULE OUT HL FIRST

145
Q

ANSD

A

auditory neuropathy spectrum disorder; dyssynchronous firing of nerves; variable audiogram, delayed speech/language development, absent or grossly abnormal ABR with the presence of normal OAEs and/or cochlear microphonic, OAEs may disappear over time, absent acoustic reflexes

146
Q

Maternal Rubella

A

Mild virus infection with low grade fever and rash, highly contagious, if contracted in pregnancy can cross placenta, typically flat severe to profound SNHL, no treatment only prevention

147
Q

CMV

A

cytomegalovirus; type of herpes; can cross the placenta; believed to be the biggest exogenous cause of childhood deafness; pregnant audiologists should not see CMV patients

148
Q

Meningitis

A

inflammation of the protective membranes covering the brain and spinal cord; can be viral or bacterial; SNHL is the most common complication- typically bilateral and symmetrical, ossification of the cochlea can happen

149
Q

Birth Anoxia

A

lack of oxygen to newborn at time of birth; cord strangulation, breeched delivery- symptoms= CP, HL (usually sharply sloping HF SNHL)

150
Q

Ototoxicity

A

medication poisonous to the auditory system or vestibular system; the drugs circulate in the bloodstream, endolymph composition is unique to the body, ~200 drugs listed as ototoxic or potentially ototoxic, 4 million people a year are at risk. More susceptible if you 1) have pre-existing kidney failure, 2) are taking more than 1 ototoxic drug, 3) have exposure to noise while on the med, 4) based on long duration and high dosage.

151
Q

Examples/types of ototoxic drugs

A

Aminoglycoside antibiotics, streptomycin (treats TB), Kanamycin (for bacterial infection), Neomycin (bacterial infections such as encephalitis), Gentamycin (pneumonia and meningitis), etc.

152
Q

Effects of chemotherapy

A

cisplating/carboplatin drugs; any drug containing platinum, used to treat cancer, can cause kidney damage and SNHL, Primarily basal turn of the cochlea affected (HF HL)

153
Q

Audiologic monitoring for ototoxicity

A

baseline audiogram, subsequent pure tone testing, HF testing, if on cisplatin daily monitoring, aminoglycisdes weekly monitoring, post treatment 3 month intervals for a year… if behavioral testing is not an option do OAEs or ABR

154
Q

Perilymphatic fistuala

A

hole in the oval window or round window; leak of perilymph; can be caused by head trauma or be spontaneous; usually HF SNHL and dizziness; repair- surgical…

155
Q

Noise induced HL

A

any hearing loss caused by exposure to noise; three types 1) acoustic trauma (sudden, usually noise > 120 dBA), 2) temporary threshold shift (2-20 minutes), 3) permanent (long term)

156
Q

Evaluations for all children with newly identified SNHL

A

all children should have an otologic (etiologic) work-up, ENT then maybe eyes, cardio, CT/MRI, genetics

157
Q

A mom of a patient of yours with newly dx’d Connexin 26 lets you know she is pregnant with her second child. She wants to know the risk of her baby also having Connexin 26. What do you tell her?

A

25% every single time

158
Q

A mom of a patient of yours with Down Syndrome informs you that he passed his newborn hearing screening and she has no concerns re: hearing loss. She is frustrated that her pediatrician keeps referring him for a hearing test every 6 months. What do you tell her?

A

prone to fluid- small ears-risk for wax; risk for SNHL; monitoring for mainly CHL, stenotic ear canals

159
Q

A mom of a patient of yours with cleft lip and palate is confused what his diagnosis has to do with hearing. How do you explain the connection/ reason for monitoring to mom?

A

eustachian tube might be underdeveloped leading to ME problems; association with CHL due to malformation-higher risk for effusion, infections

160
Q

You recently diagnosed a patient with SNHL. You refer to ENT and tell mom they will do a medical work-up, likely including an MRI or CT scan. Mom is very worried about the scan and wants to know what they could possibly be looking for. What do you tell her?

A

“checking to rule out other intervention needs before hearing aids”; other than NF2 tumors are very rare in kids; EVA, hypoplastic/aplastic nerve, abnormalities with the ossicles and/or any cochlear malformation

161
Q

A mom brings her son to the clinic and is convinced he has auditory processing disorder. She wants him evaluated right away. What is your first step?

A

comprehensive evaluation; make sure the kid has normal hearing; have to have normal hearing for an APD evaluation; kid has to be 6-7 years old at least- can’t do the APD eval
much younger than that (need expressive/receptive language)

162
Q

A mom comes in to the clinic with her 2 year old daughter and reports history of prematurity, blood transfusion, IV antibiotics, and speech language delay. She is convinced her daughter has something called auditory neuropathy that she read about online because her daughter is not talking and responds to sounds very inconsistently. Your evaluation yields MRL to speech at 50 dB HL in the SF, normal tymps AU, present and robust OAEs AU, and present reflexes AU. What do you tell mom re: ANSD?

A

if present reflexes, there’s no ANSD b/c reflexes test gross function of the auditory nerve- if you have a time locked response to an auditory stimulus, there can’t be dysynchrony. Need behavioral testing to figure out what’s going on. Might refer to early intervention-about how many words does she say? can she follow simple directions?

163
Q

When does the audiologic evaluation begin?

A

at the case history

164
Q

Questions for Case History

A 
Why are we doing a hearing test today?
Do you have any concerns about his/her hearing?
Do you have any concerns about his/her speech and language?
Birth History
Risk factors
Medical history
Major medical issues?
Developmental delays?
Ear infections?
Hearing history
Any past hearing tests?
165
Q

Observation Piece of the case history

A

pick up on attitudes and cultural differences, if you can have a normal conversation with the kid they probably dont have severe HL… do they sit still?

166
Q

conditioned oriented response

A

pre-cursor to VRA- loudspeaker and red flashing lights

167
Q

VRA

A

Visual Reinforcement Audiometry
Operant conditioning- child on the parent’s lap or high chair- present stimulus at moderate intensity- child localizes toward the sound- provide positive reinforcement - decrease stimulus

168
Q

Positive Reinforcement

A

Give something positive to make a behavior happen more often

169
Q

Negative Reinforcement

A

seat belt beeper stops when you buckle the seat belt - take something away to make a behavior happen more often

170
Q

Operant conditioning

A

changing behavior by reward - reinforcement punishment

171
Q

Classical conditioning

A

stimulus elicits response

172
Q

VRA is _____

A

continuous reinforcement

173
Q

Gestational Age

A

Out of 40 weeks, when you are born

174
Q

Chronological age

A

from moment of birth

175
Q

Adjusted age

A

difference between gestational age and chronological age

176
Q

What if the kid is not a candidate for VRA

A

Behavioral observation audiometry/startle to loud sounds/recommend they come back at a better age for VRA

177
Q

Prepping for VRA

A

establish rapport with the parent, let them know the goal, give them easy instructions (Sit here with them on your lap- I need you to try your best not to talk to the kid,
and not to react to the sounds) and tell them why, position the child, establish parent role, set up environment

178
Q

Conditioning phase of VRA

A

begin at a level where the child is expected to respond- if you suspect normal hearing maybe start at 40-50 dB

179
Q

Stimulus type/presentations

A

NBN, Fresh noise, Warble?
Puled NBN great for kids
start in the sound field
If the parent says there is a better ear, start with that ear

180
Q

Test frequency protocol

A

4/2/1/500
or 2/1/500/4
keep 8 in back pocket
speech

181
Q

Under 6 month audiometry

A

reflexive or attentive behaviors

cesation of activity

182
Q

audiometry 12-18 months

A

Prime VRA age

183
Q

audiometry 18-24 months

A

get bored from VRA quickly

184
Q

Role of a test assistant

A

Extra observer, centering and focusing the child, replacing headphones/inserts, helping parent stay on track

185
Q

Conditioned Play Audiometry

A

Best once children reach 30 months of age
Based on operant conditioning
Need good head and trunk support and fine motor skills to be able to do CPA
Need expressive and receptive behavior
Child must understand “if this then this” concept
Use case history for cognitive and developmental evaluation
The reinforcement is the game itself
blocks/bucket, peg board, simple puzzle- something with a lot of trials

186
Q

CPA Test Set Up Options

A

1) test team- 2 clinicians- someone else is focused on the kid and looking for a response
2) portable audiometer- probably supraural headphones
3) parent as test assist- kid might focus longer

187
Q

CPA Stimuli and Strategies

A 
start 40-50 dB above expected threshold
bracketing down 20 up 10 for some
start at 4kHz then 2 or 500
switch ears between frequency
Goal is 500-4000 Hz
188
Q

Masking with Kids

A 
yes it's possible
not always in portable audiometers
masking= kid more prone to fatigue
Using CPA with older kids
might get a bone line and then add 15 dB and see what happens
shortcut- skip the plateau as needed
189
Q

Tips for CPA

A

choices not questions

additional reinforcements- stickers bubble

190
Q

TROCA

A

tangible reinforcement operant conditioning audiometry - behavior produces a tangible reinforcement or reward such as candy- not really used anymore

191
Q

why do we do SRT

A

cross check principle

192
Q

Why do we do WR

A

assess clarity of speech understanding

193
Q

why do we do an audiogram

A

assess detection ability

194
Q

Auditory Skills Hierarchy

A

1) awareness
2) recognition/discrimination (differentiate between ling sounds)
3) identification
4) comprehension

195
Q

Speech Awareness Threshold

A

Used for young children or children with language delays- or elderly adults with poor discrimination- DO MLV- informal calling the child’s name, interesting/novel sounds (go go go), Goal = for them to look, correlation with responses to tonal stimuli- SAT should be significantly better than PTA

196
Q

Speech reception threshold

A

Spondees; “where’s mommy?” ideally used with spondees but if they cant do that …. try pointing to body parts or such. repeating #s is good for different body parts

197
Q

VRA and what are billed as the same thing

A

SAT

198
Q

If you do SRT first…

A

you have a good idea of what you are walking into for tones

199
Q

if you do CPA first, …

A

you get the more tiring thing done first

200
Q

NU Chips

A

50 monosyllabic words, 4 choices per page
normal hearing children 3+
most frequent english phonemes

201
Q

WIPI

A 
word intelligibility by picture identification
closed set
age 5-6 norms on kids with hearing loss
4/6 words rhyme, 2 distractors
ring, wing, king
202
Q

Which is harder? wipi or NU chips

A

WIPI

203
Q

PBK-50

A

Phonetically balanced Kindergarten
Misnomer- recommended for children 5-6+
Lists in other languages

204
Q

MLV vs. recorded speech

A

PBK and NU CHIPS are available as recorded speech - not wipi
Pros of MLV- allows for more flexibility, pausing between words, reinforcement when needed
Cons= can create problems with reliability

205
Q

Early speech perception test

A

For children with profound hearing loss and limited vocabulary/language skills- used for children 2 years old and up- must be able to chose between the two alternatives
Often used in CI candidacy
Scoring-
no pattern perception, consistent pattern perception, inconsistent word ID/consistent word ID, open set word ID?

206
Q

Ling 6

A

Discrimination /a/ /m/ /i/ /u/ /sh/ /s/

SUSHI MA

207
Q

What is common in 10-16 year olds

A

malingerers

208
Q

Purpose of hearing screening

A

detect those who demonstrate greater probability of having a disease or condition so they may be referred for further evaluation- results are pass/fail

209
Q

Who is missed in UNHS

A

ANSD and late onset HL

210
Q

Role of audiologist in screening

A

development, implementation, supervision, program evaluation

211
Q

Risks for screenings

A 
Infection control
Equipment function
Equipment calibration
Patient identification
Patient referral
Record keeping
212
Q

Screening for otitis media and outer/middle ear problems

A

debated, currently not universal

213
Q

OME risk factors

A

 First episode prior to 6 months of age
 Bottle feeding
 Children with craniofacial anomalies/syndromes
 Populations with higher incidence (Native American, Eskimo, Hispanic)
 Family history of chronic OME
 Daycare/crowded living situation
 Excessive cigarette smoke
 Children with SNHL, learning disabilities, behavior disroders

214
Q

School age screenings

A

AAA 2011 Guidelines: Preschool, Kindergarten, 1st, 3rd, 5th grade and 7th or 11th grade; k-3 for OME

215
Q

screening protocols

A

behavioral, 20/25 dB HL, 1-4 k, refer any no response, pass= all or nothing

216
Q

Audiologic Test Battery

A

Case History, Otoscopy, Behavioral Testing (BOA, VRA, CPA), Immittance, OAEs

217
Q

ME system - structures & purpose

A
  • TM, Ossicles, ligaments, muscles, eustachian tube
  • Transfer acoustic vibrations to the fluid filled cochlea (transducer/helps to overcome impedance + static admittance) = immittance
218
Q

Maximum conductive hearing loss

A

65-70 dB

219
Q

ME changes over time (as a child gets older)

A

Growth of the bony portion of the ear canal; increase in the overall size of the EAC; Decrease in density of ossicles (ossification and absorption of residual mesenchyme); changes in orientation of TM to be more vertical; progressive stiffening of the ossicular joints; De Chicchis et al. (2000) Trend for an increase in admittance and ear canal volume and a decrease in width with increasing age

220
Q

3 factors in immittance

A

Mass (TM and ossicles)
Friction (Viscosity of perilymph and mucous lining of the ME, TM, tendons, ligaments)
Elasticity (TM, air in the ear canal and ME)

221
Q

Measurements of Immittance

A

1) Tympanometric shape
2) Static Admittance
3) Tympanometric peak pressure
4) Ear canal volume
5) Tympanometric gradient (or width)

222
Q

Static Admittance

A

Measure of middle ear mobility measured in mmhos- low admittance associated with poor middle ear mobility- flat, almost always middle ear pathology; Roush et al (1995) was the first study to investigate admittance for children under 30 months- average range for 12-18 months: 0.2-0.6 mmhos, 18-24 months: 0.2-0.7 mmhos, 24-30 months: 0.3-0.8 mmho

223
Q

Peak Pressure

A

Large variability for normal ears; infants and young children (+50 to -150 daPa)

224
Q

Ear Canal Volume

A

measured from the probe tip to the TM; normative data for children age 1-7 years (0.3-0.9 cm3); Infants may be on the lower side as low as 0.3 cm3 - volume as a clinical tool- helps to clarify etiology for a flat tympanogram

225
Q

Gradient

A

Width and gradient; distance in daPa between sides of the tympanogram at 1/2 peak admittance; Type B is most associated with OME (no real gradient); Nozza and colleagues (1992-1994) TW best measure for separating middle ears with fluid vs. no fluid

226
Q

Why is 1000 Hz tympanometry more appropriate for children under 6 months of age?

(Neonates and Young Infants- immittance)

A

Cut-off for 226 Hz probe is usually 6 months - may be older depending on child age, size, disorder (i.e. down syndrome); impedance of neonatal ME is dominated more by mass and resistive elements at low frequencies; Resonant frequency of neonatal middle ear is lower than children/adults (350-450 Hz) -greater contribution of stiffness elements to overall impedance

227
Q

Type A tymps… reporting…

A

consistent with normal middle ear function

228
Q

Acoustic reflexes

A

Stapedius muscle

  • 1 mm in length
  • Stabilizes stapes

Acoustic stapedius reflex

  • Contraction that occurs in response to high intensity sound stimulus
  • Usually 500, 1000 and 2000 Hz
  • 4000 Hz is often absent even in normal hearing patients (not very diagnostically significant)
229
Q

TEOAES

A

clicks- broadband more sensitive- have to have HL worse than about 40 dB to lose TEOAEs; TEs about 10 dB greater in infants then adults; test usually 1-4 kHz; 6 dB noise floor; at least 4/5 present or 2-4 kHz

230
Q

DPOAES

A

Elicited by presentation of two tones resulting in 3rd tone (frequency) emitted; look at DP gram- criteria is 6 dB above the noise floor AND response greater than -10 to -6 dB SPL

231
Q

DP grams

A

overlap in responses between normal and HL ears is marked in an outline; responses that fall below the marked area is considered abnormal, responses that fall between the two lines (overlap) cannot be determined as normal or abnormal

232
Q

Physiologic Noise (OAEs)

A
  • Keep baby as calm as possible
  • Ensure you have the right tip size and you’re not up against the ear canal wall
  • Use a quiet room, a test booth if possible
  • Instruct parents to be quiet
  • Noise mainly concentrated below 1500 Hz
  • May include breathing, sucking, heartbeat, etc
233
Q

If you use a jerger type you must also do what?

A

say what that is consistent with

234
Q

Low admittance can be a sign of:

A

wax, otosclerosis

235
Q

Instructions for admittance to a child

A

we’re going to take a picture of your ear- look I’ll show you on mommys hand first

236
Q

what do present reflexes tell you

A

auditory nerve function is grossly present

237
Q

Describe 3 events (laws, research publications, etc) that led to the universal newborn hearing screening program.

A

1) Education of handicapped children act (1975) which would eventually become IDEA
2) Passing of the ADA in 1990
3) 1993 - NIDCD consensus statement recommended hearing screening in the first 3 months of life
4) 1994 JCIH stated that infants should be screened with physiologic test and it should be universal

238
Q

Describe the auditory and/speech language milestones for 6-9, 9-12, and 12-18 month olds.

A

** 6-9 months
Auditory: enjoys games like peak-a-boo and can turn and look in the direction of a sound (VRA!)
Speech: Babbling has long and short groups of sounds, canonical/reduplicated babble, uses speech or non-crying sounds to get and keep your attention, gestures for action (holding arms up to be picked up)
** 9-12 months
Auditory: Listens when spoken to and recognizes words for common items like cup or juice, begins to respond to simple requests like “come here”; localization more than just lateralization
Speech: understands “no”, tries to repeat words, may have 1-2 words like mama or hi
** 12-18 months
Auditory: attends to books or toys for about 2 minutes
Speech: Follows simple directions accompanied by gestures, answers simple questions nonverbally, points to objects/pictures/family members, says 2-3 words to label a person or object, tries to imitate simple words

239
Q

What are some adjustments to testing (including speech testing) that you can make for a language delayed or bilingual child?

A

numbers instead of words for spondees/srt, closed set/open set, pointing, visual cues, SAT instead of SRT

240
Q

Name 4 clinical uses of OAEs.

A
  1. screenings
  2. differential diagnosis of ANSD
  3. patient who cannot provide behavioral results (difficult to test patients)
  4. malingering
  5. Identify possible ME fluid
241
Q

Describe 3 symptoms that a parent might report that would make you suspicious of otitis media (with or without effusion).

A

1) the kid turns the tv volume up loud
2) they’re pulling on their ear(s)
3) they recently had a bad cold

242
Q

When might an SAT be more appropriate than an SRT? List as many reasons as you can think of.

A

 Used for young children
 Children with language delays (there are other options
as well)
 Children with language barriers (also other options)
 Elderly adults with poor discrimination
 Adults with cognitive impairments

243
Q

Describe 3 symptoms that a parent might report that would make you suspicious of otitis media (with or without effusion).

A

1) the kid turns the tv volume up loud
2) they’re pulling on their ear(s)
3) they recently had a bad cold

244
Q

Case history questions for school aged kid

A

Does he/she have an IEP? How is she/he doing in school? Has the teacher mentioned anything? Is she/he able to make friends? Can he/she use the phone with both ears?Does your child turn the TV or Computer volume up a lot?

HESP645 (2 decks)

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