Immittance Audiometry, ABRs, OAEs, Pediatric Audiology Flashcards

Question

Type As Tympanogram

Answer 1

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

Answer 2

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

Answer 3

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

Answer 4

* 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

Answer 5

* 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

Answer 6

* 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.

Answer 7

* 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

Answer 8

* 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

Answer 9

IPSILATERAL TESTING * Probe and stimulus in same ear CONTRALATERAL TESTING * Probe in test ear, stimulus in non test ear

Answer 10

* 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

Answer 11

*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

Answer 12

* 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

Answer 13

* Otoacoustic Emissions * Auditory Brainstem Response Test (ABR)

Answer 14

* 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.

Answer 15

* 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

Answer 16

* 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

Answer 17

* 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

Answer 18

* 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

Answer 19

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

Answer 20

* 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

Answer 21

* 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

Answer 22

* 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

Answer 23

* 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

Answer 24

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

Answer 25

* 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

Answer 26

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)

Answer 27

* 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

Answer 28

* Assess Neurologic Integrity * Latency and morphology of waves * Hearing Threshold Estimation * Wave V * Infant Screening

Answer 29

Waveform latency Waveform morphology--- Shape or definition of the wave

Answer 30

* Prolonged interpeak latencies * Wave V latency is significantly different between ears * Poor waveform morphology

Answer 31

* 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

Answer 32

* 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

Answer 33

* 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.

Answer 34

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

Answer 35

* 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.

Answer 36

* 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

Answer 37

* 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

Answer 38

* 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

Answer 39

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

Answer 40

 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

Answer 41

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

Answer 42

 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

Answer 43

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

Answer 44

 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

Answer 45

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)

Answer 46

 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

Answer 47

 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

Answer 48

 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)

Answer 49

 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

Answer 50

 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

Answer 51

 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

Answer 52

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

Answer 53

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

Answer 54

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

Answer 55

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

Answer 56

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

Answer 57

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

Answer 58

ASSOCIATED ANOMALIES: Craniofacial abnormalities ME abnormalities INHERITANCE: AD

Answer 59

ASSOCIATED ANOMALIES: Progressive blindness, Clumsy gait INHERITANCE: AR

Answer 60

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

Answer 61

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

Answer 62

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

Answer 63

 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

Answer 64

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

Answer 65

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

Answer 66

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

Answer 67

 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

Answer 68

 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 29 30

Answer 69

 Visual Impairment  Hearing Impairment  Cerebral Palsy  Epilepsy  Learning disabilities  Intellectual disabilities  Vestibular disorders 31 32

Answer 70

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,

Answer 71

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. 33 34

Answer 72

 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

Answer 73

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

Answer 74

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

Answer 75

 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

Answer 76

 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)

Answer 77

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

Answer 78

 BOA  Behavioral Observation Audiometry  COR/ VRA  Conditioning Orienting Reinforcement/ Visual Reinforcement Audiometry  CPA  Conditioned Play Audiometry  Conventional Audiometry

Answer 79

 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

Answer 80

 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

Answer 81

 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

Answer 82

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

Answer 83

 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)

Answer 84

 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

Answer 85

 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

Answer 86

 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 69 70

Answer 87

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

Answer 88

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

Answer 89

 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

Answer 90

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.

Answer 91

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

Answer 92

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

Answer 93

 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. 83 84

Answer 94

 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. 85