Aud & Vestib Disorders Flashcards
What is an ABR? What does it assess? What is one clinical app of it?
It is used to assess the neural auditory pathway. The ABR tests and assesses the electrical potentials generated by the auditory neural pathway and it is an electrophysiologic test called an auditory evoked potential. We clinically use waves 1, 3, and 5 and as intensity decreases latency increases.
To diagnose ANSD
To confirm results of behavioral tests & establish site of lesion
How do we know OAEs are a measure of OHC fxn? What are different stimuli used for TEOAE and DPOAE?
Absence or damage of OHCs is associated with absence of OAEs further supporting the hypothesis that OAEs are generated by OHCs
TEOAE: brief acoustic stimulus (click or tone burst)
DPOAE: Simultaneously presented pure-tones of two appropriate frequencies (fi & f2) presented at two intensity levels (L1 & L2)
2f1-f2 elicits the best DPOAEs in humans
Baby failed screening and the audiologist did ABR and diagnosed sensorineural HL. 3 months later had normal hearing and passed.
What could have been done to cross check the loss?
What could have been the cause of the fail?
OAEs
Neuromaturation
What disorder should you not use alternating clicks to determine?
ANSD
A decrease in intensity should also cause a decrease in latency
f
CM are produced by OHC
t
Actual hearing threshold is typically ~10 to 15 dB HL better than the ABR threshold
t
normative Peak Latency Values at 80dB nHL
Wave I: 1.5 ms (mean)
(SD = + 0.25 ms)
Wave II: 2.6 ms (mean)
(SD = + 0.25 ms)
Wave III: 3.7 ms (mean)
(SD = + 0.25 ms)
Wave IV: 4.7 ms (mean)
(SD = + 0.5 ms)
Wave V: 5.5 ms (mean)
(SD = + 0.5 ms)
Inter-peak values at ~80 dB nHL presentation level
I - III IPL: 2.25 ms
(SD = + 0.5 ms)
III - V IPL: 2.0 ms
(SD = + 0.5 ms)
I - V IPL: 4.0 ms
(SD = + 0.5 ms)
abr transducer
insert
abr stim
click, chirp, tone bursta
abr rate
> 20/s
90/s useful for neurodiagnosis
abr intensity
variable in db nHL
10-90 dB
Relationship BW latency and Intensity
As intensity decreases the latency of the waves (wave V) increases
As intensity decreases the morphology of the ABR response deteriorates
abr & CHL
absolute latencies are pushed out but relative interwave latencies were retained within normal limits
abr & SNHL
wave 1 is prolonged (~ 2 ms)
wave 5 has normal latency (~ 6 ms)
Latency-intensity function shows a wave V not repeatable at 45 dB nHL – a higher hearing threshold
retro path & abr (vestib schwan
diminished wave I and absent wave III & V
What is congenital aural atresia? What is a condition associated with it? What is the FDA approved device for it and at what age?
failure of canalization results in this
associated with microtia & ME anomalies
spontaneous, can occur with Treacher collins, trisomy 22, crouzon’s & hemifacial microsomia
BAHA, after age 5 years old
man with bilateral mixed HL from 500-4000 in the high frequencies. What could be the cause?
Collapsing canals
What is the external canal condition caused by radiation to the head and neck?
ORN
OSTEORADIONECROSIS
what are the signs of impacted cerumen?
Mild CHL
tymps <.02 ml
Coughing
Dizziness
All the above
all
What is not the sign of it?
Otalgia
Tymps >.03 ml?
Complaints of fullness
Mild CHL
Dizziness
Tymps >.03 ml?
most common viral infection
accompanies VII N paralysis
earliest symptoms = painful rash in ear canal, concha, or below/behind auricle
rash caused by virus localizing in skin & results in painful vesicular (blister) eruptions
herpes zoster oticus (shingles)/ramsay hunt syndrome
outward scar tissue growth
follows ear trauma, piercings, viral infections (like herpes)
can spread
keloid
what is the most common cause of ear canal atresia
treacher collins
problem for younger children - cartilage is not fully developed
older adults - cartilage is deteriorating
collapsing canals
irregular & multiple
bony growth
most common
exostosis
less common
smooth and regular
osteomas
ALL PT PRESENTING WITH NON HEALING GRANULATION TISSUE IN EAC SHOULD GET BIOPSY
t
most serious complication that happens because of radiation that was used at the base of the skull bones due to cancer
can occur years after radiation
Osteoradionecrosis
List 2 surgical complications of otosclerosis & discuss one of them
OTSC surgery can result in a perilymphatic fistula and a decrease in WRS. Regarding PLF, if implanted prosthesis gets misplaced after surgery it can puncture a hole into the inner ear space allowing perilymph to escape into the ME cavity
Another complication is
Cholesteatomas are pseudotumors. Justify this. What are tymps findings of one that caused ossicular chain disarticulation? What is preferred treatment.
they are this because they are not considered tumors but they have same mannerisms like bone erosion, growth, and vasculature. They will take over and invade all structures that they contact, similarly to a tumor. In the R ear, tymps would show a type Ad with high static admittance. Surgery is the preferred treatment and antibiotics can be given beforehand to reduce inflammation and bleeding for surgery.
Define paraganglioma and give an example
paragangliomas arise from the paraganglia cells. it is a tumor along the jacobsons or arnolds nerves and an example is the glomus tympanicum. they can also arise from the bulb of the internal jugular and an example would be a glomus jugulare
What is inner ear homeostasis? What as an example of a condition/agent that can disrupt it
it is the balance of the ions and fluids in the inner ear and the maintenance of keeping it. An example is endolymphatic hydrops which is when there is an excess amount of endolymph as seen in Meniere’s disease.
What is TTS and PTS
both occur as a result of loud noise exposure. TTS happens when exposed to noise exposure and it can recover from 15 mins-14 hours and the threshold shift can return to baseline. PTS however doesn’t return to baseline due to irreversible damage to the cochlea. It usually doesn’t recover after 14 hours of noise exposure. HHL (need to look up to add)
What demographic group is most prone to otosclerosis
white women bw 20-40 yrs
What is correct for ossicular disarticulation?
only caused by temporal bone fractures
typically self correcting condition
hemotympanum is never reported with this
can be caused by a single exposure to very high intensity sound
ARTs are normal if tympanic membrane is intact
can be caused by a single exposure to very high intensity sound
Otosclerosis is a focal disease of
otic capsule
For NIHL, what is wrong
is caused by both dose and duration of disease
daily dose of noise exposure that doesn’t cause TTS usually doesn’t cause a PTS
consistent exposure to loud industrial noise can cause a NIHL but consistent exposure to loud music does not
noise notch in 3 and 6 kHz range is characteristic of NIHL
primarily NIHL will always progress to profound SNHL
consistent exposure to loud industrial noise can cause a NIHL but consistent exposure to loud music does not
primarily NIHL will always progress to profound SNHL
SSCD is differential diagnosis for otosclerosis. Clinically, they are differentiated by
purulent OM
tinnitus
normal ARTs
low static admittance (type C) or normal tymps
LF (rising) SNHL
normal arts
Jerger type Ad can be clinical finding in which disorders
ossicular disarticulation
cholesteatoa
Jerger type b HV can be clinical finding in which disorders
cholesteatoma
Glomus jugulare tumor can present with
unilateral CHL
unilateral mixed HL
tymp with jagged edges corresponding to PT pulse
intact but inflamed looking ™
all
all
FN weakness/paralysis potential complication for what conditions EXCEPT
glomus tympanicum
chronic suppurative OM
cholesteatoma
NIHL
gunshot wound to temporal bone
NIHL
which conditions are contraindications to surgery for OTSC
abg >/= 20 dB HL in affected ear
OTSC involvement of endolymphatic duct with meniere’s disease like s/s
SNHL in contralateral ear
SSCD in contralateral ear
none
OTSC involvement of endolymphatic duct with meniere’s disease like s/s
which infectious disease affect mother that results in congenital HL in fetus
CMV infection
rubella (german measles)
AIDS
All
a and b
all
17 yr old female has chronic bilateral OM since 6 mos old. Has had plenty of PE tubes but none for the last 3 years. Has significant allergies and frequency neg ME pressure (type c) bilaterally. Last ME infection was reported 3 yrs ago. She noticed hearing in right ear is worse for the last year. Copious foul smelling discharge noted on otoscopy in R ear. Pure tones show mild - moderate CHL in R and normal in L. Type b HV in R and type A tymp in left.
She most likely developed which condition due to chronic OM and/or negative ME pressure in R ear
what is the best treatment for this?
cholesteatoma
surgery
what is incorrect for glomus tumors
can arise from paraganglia cells on dome of internal jugular vein, arnold & jacobsons
glomus tympanicum can commonly grow to large size and cause hoarseness and dysphagia
several genes are associated with thee but most are sporadic
involvement of XII N indicates extensive growth of jugulare tumor
glomus tympanicum can commonly grow to large size and cause hoarseness and dysphagia
inner ear homeostasis disruption can be from
cochlear disorder disrupting stria vascularis & K+ transport
cochlear disorder reducing EP
genetic mutation resulting in abnormal gap junction or tight junction proteins
viral infections that affect aquaporins & water transport in cochlea
all
all
causes profound permanent SNHL visual problems and neurological and motor deficits in affected babies
CMV
HL secondary to ____ can result in cochlear ossification
meningitis
an incudomalleolar disarticulation appears as an ______ sign on CT scans
broken ice cream cone
What is synaptopathy or hidden hearing loss? Name one possible cause of hidden hearing loss.
What is a cochlear microphonic? Why are OAEs normal and ABRs are abnormal in ANSD? What are 2 diagnostic ABR findings for ANSD
Cochlea can be divided into 3 systems that provide its extraordinary sensitivity to sound. List and describe fxn of each of these.
Describe presbycusis and the primary audiologic characteristics. What are 2 anatomic and 2 physiologic differences between age-related and NIHL
Which auditory assessment will be normal for someone diagnosed with ANSD
oae
Common site for facial nerve entrapment
Meatus and fallopian tube?
60 yr old complains of L sided facial weakness, SNHL, & tinnitus. What would reflexes look like for proximal VII N lesion?
Abnormal left ispilateral (sound and probe in left ear) and abnormal right contralateral (sound right, probe left)
Differential diagnosis for a facial nerve schwannoma includes
Vestibular schwannoma
Cholesteatoma
Glomus tumor
Meningioma
Poor prognostic indicator for Bells palsy
Intact ART during disease process
<90% nerve degeneration after 2 weeks of onset as measured on electroneurography (ENoG)
Older diabetic PT
Partial paralysis of affected nerve
Younger PT w/ partial recovery w/in 2 mos
Older diabetic PT
33 yr old with history of combat-related noise exposure and concussion injury complaining of bilateral high pitched tinnitus & extreme difficulty hearing. Pure tones had slight to mild symmetrical hl. Tymps were normal. ARTs are elevated/abs bilaterally. Word in quiety was 80% and in noise was 40%. This case history and results are consistent with
NIHL
What is not a characteristic of ANSD
Poor performance on WRS
Abnormal ME ARTs
increasing latency as intensity decreases of the stimulus
Something else
increasing latency as intensity decreases of the stimulus
Endocochlear potential
Shows equal voltage in endolymph of both cochlea and vestibular system
Produced by organ of corti
loss/decrease affects hf more than lf
Intracellular potential with small negative voltage
Is not affected by aging
loss/decrease affects hf more than lf
Which of the following are TRUE for ANSD
ANSD may be caused by abnormal ribbon synapses mediating IHC NT release
ANSD ______ of the VIII N
ANSD ______ acoustic reflex thresholds
________
Recent clinical trials suggest that gene therapy may restore hearing for some types of ANSD
ANSD may be caused by abnormal ribbon synapses mediating IHC NT release
ANSD ______ acoustic reflex thresholds
________
Recent clinical trials suggest that gene therapy may restore hearing for some types of ANSD
According to killion and fikret-pasa’s classification of presbycusis, type 1 HL is consistent with
Loss of primarily OHC and normal loudness sensation
Pediatric disorder that affects facial as well as CN VI (abducens)
Mobius syndrome
Based on review of presbycusis, which is correct?
Genetic plays no role in development of presbycusis
NIHL is recognized easy on audio for older adults with presbycusis
According to killion individuals with type 3 will have no issues understanding speech in noise with amplification
In PT with hidden hearing loss due to aging speech in noise testing would be beneficial diagnostic auditory treatment
Presbycusis is peripheral auditory phenomenon caused only by OHC loss
Which nerve is involved in eye movements assessed during vestibular testing?
CN III - Occulomotor
Which branch of the FN innervates the outer ⅔ of the tongue & can be sacrificed during ME surgery such as removal of cholesteatoma?
chorda tympani`
a left FN schwannoma will show an abnormal left ispilateral response and an abnormal _____ contralateral response
right
Osteopetrosis (bony dysplasia) is a disorder of aging that results in hardening of the cranial bones
f
______ syndrome is a rare congenital disorder with possible multifactorial inheritance resulting in hypoplasia of CNs VI & CN VII
mobius
In the case of Bell’s palsy, MEAR will be present if the lesion is ______ to the stapedius nerve
distal
otalgia, HL, vertigo, facial paralysis (usually more severe), skin rash w/ fluid filled blisters on concha and/or external canal & along sensory parts of the 7th N (rash along the nerve pathway), severe degeneration of the FN
herpes zoster oticus → Ramsay-Hunt Syndrome
high fever, stiff neck, malaise, nausea, vomiting
-Untreated→ blindness, paralysis, HL/deafness (acquired SNHL) & vertigo
meningitis
feeling of sudden “pop” after lifting something heavy
Vertigo w/out HL
-HL w/out vertigo
-Symptoms not determined from Meneries (SNHL, vertigo, tinnitus, aural fullness)
acquired
perilymphatic fistula
Noise notch noted from 3-4 sometimes 6 kHz
accompanied by tinnitus
-symmetrical
-profound SNHL is rare
-LF thresholds better than 40 dB HF thresholds rarely better than 75 dB
-Presbycusis can flatten noise notch
NIHL
normal tymps
ARTs abn w/ lesion PROXIMAL to stapedius n & ARTs present w/ lesion DISTAL to stapedius n
SNHL
gradual facial weakness
Others→tinnitus, otorrhea, ear canal mass, otalgia, vestibular symptoms
facial schwan
d/d for facial schwan
-OM w/ CHL (w/ or w/out cholesteatoma),
cholesteatoma paragangliomas, meningiomas,
acoustic neuroma (vestib schwannoma →because of SNHL that can be present in facial neuromas
-Slow, progressive HF sloping SNHL
-speech perception especially w/ noise & reverberation
-Recruitment
presbycusis
only one where there are bilateral cases of vestib schwans
NF2
-T1 w/ gadolimium contrast-enhanced MRI →gold standard
diagnostic feature for these on MRI is increased lumen size of IAC
vestib schwan
Present around 40-60 yrs
-More common in females
Always unilateral & singular tumor & slow growing
-Asymmetrical unilateral HF SNHL (can be as low as 10 dB difference) or bs lesion showing flat unilateral SNHL
-+TD in more frequencies & doesn’t diminish w/ high intensity
-WRS worse than thresholds w/ +rollover >.45
-Normal tymps
- abn/abs or normal ARTs
-Normal OAEs if HL not significant with decreased contra suppression
vestib schwan
HF asymmetry, R ipsi & contra abn, & word rec drops in R ear w/ noise
vestib schwan
Appears later in life
-More common in females→ associated w/ progesterone levels & breast cancer
Progressive unilateral SNHL
vertigo
tinnitus
abn art on affected side (mimic 8 neuroma)
normal tymp
+RD, +rollover & poorer scores in noise
-Usually ABR abn
-Normal OAEs w/ appropriate loss
meningiomas
d/d for meningiomas
OM→occurs when meningiomas reach ME , difference is red ™, granulation tissue formation & CHL (*Adults don’t get OM unless there is an underlying issue→REFER)
-Paragangliomas→meningiomas are more vascular on otoscopy, look similar on MRI & present w/ neck mass similar to glomus jugulare
-FN Tumor→both can cause facial numbness/weakness w/ CN VII involvement
-Vestib Schwannoma→mostly when in IAC, difficult to differentiate
Progressive bilateral SNHL happening weeks to months (QUICK)→ some have unilateral fluctuating SNHL
aural fullness, tinnitus, vestib symptoms
SNHL is reversible
Rare, females more, symptoms bw 20-50 yrs
Autoimmune Inner Ear Disease (AIED)
d/d for AIED
-SSHL→ most common, SSHL is usually unilateral and is sudden but not progressive but here it is bilateral and progressive
-Meniere’s→ mimics due to fluctuating SNHL, tinnitus, aural fullness & vertigo
-Others to rule out→ vestib schwan, MS & otosyphilis
> 30 dB HL SNHL occurring in at least three contiguous frequencies within < 3 days
-HL always sudden & SN & can exist w/ pre-existing SNHL w/ any severity
-unilateral & rapid deteriorating speech understanding
-Normal tymps & ARTs w/ appropriate HL
-May have hx of cold or viral infection 1-3 wks prior to symptoms
sudden SNHL
SSHL
If one ear has been affected, the risk of the other ear being affected by SSHL during a patient’s lifetime rises by 4 to 17% (same or the other ear)
t
-Progressive neurological autoimmune disorder
Plaques are the hallmark in white matter
-~ 85% have bilateral HF SNHL but can be any audio pattern
-Some LF rising SNHL
multiple sclerosis
ms
> /=2 plaques in white pathways w/ hx, clinical presentation provides diagnosis
MS
-young adults, females, caucasian and they have fluctuating SNHL - MS should be looked at especially in northern areas
yes
d/d for MS
-Susac’s & Schilder’s (other ai)
-Diabetes→HL, visual problems, disequilibrium, poor WRS
-Stroke→poor wrs, visual problems, cognitive/memory issues, poor motor skills
-SSHL
what is susac syndrome
triad:
cognitive issues, vision, asymmetric lf hl
women 20-40 yrs
why is susac d/d for ms
-MS→ difference is there are no psychological issues in MS unlike here early, MS gets worse & this self limiting, HL happens here but not always in MS, MS has 2 or more lesions & here not as many
childhood variant of mS
schilder’s disease
-motor, sensory & cognitive issues
-personality changes, poor attention, progressive loss of intellectual fxn, vision, hearing and balance problems, muscle weakness, headaches, seizure
Adrenal glands & myelinated nerves and their axons are affected
bilateral
schilders
HL is fluctuating but progresses to profound SNHL (Meniere’s will not end in profound HL)
vestibular auditory symptoms and interstitial keratitis is the hallmark characteristics
red painful light sensitive eyes, blurred & decreased vision
they have cardiovascular issues or they have inflammation of the blood vessels
cogan syndrome
d/d for cogan
-Menieres- must show eye symptoms, imbalance & ataxia that is not vertigo, & systemic manifestations not seen in Menieres
-Labyrinthitis/neuritis - HL, vertigo, tiniitus, nausea but no eyes
-Wegener’s - SNHL & vertigo but no eyes
-Inner ear labyrinth inflammation
-aural fullness, tinnitus, HF SNHL
-acute vertigo, nausea/vomiting, nystagmus
vestibular labyrinthitis
d/d for vestibular labyrinthitis & neuritis
-OME
-Perilymphatic fistula → sudden HL & vestib
-BPPV→ no HL, mimics neuritis more but vertigo is sudden
-acute vertigo, nausea/vomiting, nystagmus
vestibular neuritis
nervous system problem that causes repeated dizziness (or vertigo) in people who have a history of migraine symptoms. Unlike traditional migraines, you may not always have a headache
Migraine Associated Vertigo/Vestibular Migraine
headache, nausea, vomiting, photophobia, loss of peripherals, motion intolerance, noise sensitivity, ataxia, numbness of extremities
Migraine Associated Vertigo/Vestibular Migraine
idiopathic
Brief episodes of mild-intense vertigo that last for 1 min & may have nausea/vomiting
-Triggered by head position
BPPV
otoconia dislodge & migrate into SCCs & move fluid when it shouldn’t causing false signals to the brain resulting in vertigo
BPPV
multifactorial chronic IE disorder
vertigo, tinnitus, HL & fullness
causes distortion
BPPV
what are the 4 symptoms needed for Meniere’s disease
SNHL that comes & goes (LF SNHL like MS), LF/roaring tinnitus, intermittent vertigo lasting a long time, & aural fullness/pressure
what is needed for a definitive diagnosis of Meniere’s
2 or more episodes lasting >/= 20 mins & 2/4 characteristic symptoms present
waht is tullio’s phenomenon
sound induced vertigo
what can an audio show for SSCD
mimics OTSC or Meniere’s, CHL/fluctuating HL, most have LF ABG worse from 250-1000
what are vestib systoms that can be seen in SSCD
vertigo/dizziness, nystagmus, Tullio’s phenomenon (sound induced vertigo), oscillopsia
rare, usually unilateral and affects all ages
SSCD
what is a d/d for SSCD
-patulous ET→ aural fullness/pressure & autophony common in both
-OTSC→ abn arts in OTSC but normal in SSCD because it is not ME pathology and not a true ABG
how to diagnose SSCD
-vestib assessment
-ct
ECohG
-phantom perception of self motion
mal de debarquement
rocking/swaying,disequilibrium rarely accompanied w/ true vertigo
-anxiety & depression
-worsen when lying down or with stress/fatigue
-improve/disappear during continuous movement like driving
mal de debarquement
how to diagnose mal de debarquement
-subjective hx of when it began
-rule out other causes w/ objective measures like vestib testing & imaging
SP/AP ratio of > 0.42 or > 42% is considered significant/positive for
Ménière’s disease
when will you see + echog or abnormality?
seen during an active episode of menieres
when they have some type of symptom - hL or fullness
