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Neuro II > Clinical Hearing Disorders > Flashcards

Clinical Hearing Disorders Flashcards

1
Q

What are the 3 possible classifications of hearing loss?

A

conductive
sensorineural
mixed

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2
Q

What are the 2 types of congenital hearing loss? What percent do each make up of the total?

A

environmental (50%) & genetic (50%)

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3
Q

What are the “ABCD’s” or congenital hearing loss?

A
  • A- affected family member
  • B- bilirubin
  • C- congenital intrauterine causes/CMV
  • D- synDromes,
  • ’s - small (1/50 in NICU)
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4
Q

What is the most common cause of non-syndromic congenital sensorineural hearing loss (SNHL)?

A

CMV

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5
Q

What is the median age of identification of hearing loss in an infant born with asymptomatic CMV?

A

18 months - most are not detected on newborn screening

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6
Q

35-50% of patients with cytomegalic inclusion disease will have what condition?

A

bilateral deafness

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7
Q

What is a newborn screening for hearing loss & how does it work?

A

most states obligate hospitals to perform hearing screenings at birth

every newborn gets an otoaccousticemission (OAE) - device that pics up the response to clicking sounds

OAE clicks & then detects depolarizations in the inner ear - if this is normal → implies the presence of normal anatomy

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8
Q

What are the symptoms seen in congenital rubella?

A
  • congenital cataract
  • cardiovascular anomalies
  • intellectual disabilities
  • retinitis
  • deafness
    • 5-10% of mothers with rubella in first trimester
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9
Q

What is the underlying mechanism of deafness in congenital rubella?

A

degeneration of the oran of Corti

adhesion between the organ of Corti & Reissner’s membrane, rolled up tectorial membrane, partial or complete stria atrophy

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10
Q

Why do 20% of babies with kernicterus have severe deafness?

A

damage to the dorsal & ventral cochlear nuclei and superior & inferior collculi nuclei

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11
Q

What type of hearing loss is seen in kernicterus babies?

A

high frequency hearing loss

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12
Q

What bilirubin level is indicative of exchange transfusion?

A

serum level > 20mg/dL

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13
Q

What are the two types of hearing loss seen in congenital syphilis? Which is more severe?

A

early (infantile)- often severe & bilateral - usually fatal

late (tardive)- progressive hearing loss w/ varying severity & onset

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14
Q

75-80% of genetic deafness is due to genes with what type of inheritance pattern?

A

autosomal recessive

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15
Q

What is term for congenital non-syndromic hearing loss that has an autosomal recessive loci? How many loci & how many genes?

autosomal dominant loci? How many loci & how many genes?

x-linked loci?

A
  • Recessive:
    • DFNB (DeaFness Neurologic Autosomal recessive)
    • 21 loci & 19 genes
  • Dominant:
    • DFNA
    • 38 loci & 11 genes
  • X-inked
    • DFN
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16
Q

What is the most common cause of genetic non-syndromic deafness (~49%)? What is the prevalence of carrier state in US?

A

DFNB1 locus

chromosome 13 Gap Junction B2 gene mutation (GJB2) for connexin 26

1:31 carriers

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17
Q

What is the most common cause of autosomal dominant hearing loss?

A

DFNA6/14–WS1

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18
Q

What is the gene mutated in X-linked non-syndromic hearing loss? Mechanism & type of hearing loss?

A

DFN3 locus

POUF4 transcription factor

mixed hearing impairment w/ fixation of stapes (conductive) w/ perilymphatic gusher (sensorineural)

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19
Q

What is the rare X-linked disorder that affects the collagen of the basement membranes of the kidneys & the inner ear?a

A

Alport Syndrome

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20
Q

What are the symptoms/clinical picture seen in a patient with Alport Syndrome?

A
  • renal failure
    • hematuria in infancy
  • progressive sensorineural hearing loss
    • may not be evident until 2nd decade of life
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21
Q

What autosomal recessive disorder presents with a thyroid goiter & profound sensorineural hearing loss?

A

Pendred Syndrome

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22
Q

What is the cause of hearing loss in patients with Pendred Syndrome?

A

abnormal iodine metabolism

Mondini aplasia or enlarged vestibular aqueduct

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23
Q

What test is performed to diagnose Pendred Syndrome?

A

Perchlorate discharge test

(+) abnormal organification of nonorganic iodine

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24
Q

What is the most common type of autosomal recessive syndromic hearing loss?

A

Usher syndrome

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25
Q

What syndrome is characteristic of senorineural hearing loss & retinitis pigmentosa? It accounts for what fraction of all deaf & blind persons in the US?

A

Usher syndrome

½ of death & blind persons

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26
Q

What is the most common form of autosomal dominant syndromic hearing loss?

A

Waardenburg syndrome

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27
Q

What syndrome is characterized by unilateal or bilateal SNHL, pigmentary anomalies (white forelock, vitiligo, heterochromia irides) & craniofacial features (broad nasal root, synophrys, dystopia canthorum)

A

Waardenburg syndrome

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28
Q

What is dystopia canthorum?

A

wide-set eyes

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29
Q

What is synophrys?

A

unibrow

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30
Q

At what time point in gestation does the cochlea reach adult size?

A

9 weeks

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31
Q

Are congenital inner ear malformations more likely to be unilateral or bilateral?

A

bilateral (65%)

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32
Q

What condition is characterized by complete agenesis of the petrous portion of the temporal bone?

A

michel aplasia

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33
Q

When does the insult responsible for Michel Aplasia occur? What is the malformation? Why does this lead to hearing loss?

A

prior to the end of the 3rd gestational week

complete agenesis of petrous portion of the temporal bone - no inner ear structures → anacusis

34
Q

What is anacusis?

A

total deafness

35
Q

Cochlear implants or conventional amplification are not useful in what type of hearing loss?

A

anacusis

total deafness

36
Q

What condition is characterized by only the basal coil of the cochlea developing? This is often associated with other physiologic feature?

A

Mondini Aplasia

endolymphatic duct is usually enlarged

37
Q

Mondini Aplasia is described in what 5 conditions?

A

Pendred

Waarenburg

Treacher Collins

Wildervanck

CMV

38
Q

What is the presentation of a patient with Mondini Aplasia?

A

presentation ranges from deafness → normal hearing

usually asymmetric, but affects both ears

39
Q

What condition is characterized by poorly differentiated organ of Corti with deformed tectorial membrane & collapsed Reissner’s membrane - but normal bony labyrinth & superior portion of membranous labrynth?

A

Scheibe Aplasia

a.k.a.

Cochlearsaccular Dysplasia / Pars Inferior Dysplasia

40
Q

What is the most common form of inner ear aplasia?

A

Scheibe Aplasia

41
Q

Scheibe Aplasia is describe in what 5 conditions?

A

Jervell Lange-Nielsen

Refsum

Usher

Waardenburg

congenital rubella

42
Q

Treatment for Scheibe Aplasia?

A

conventional amplification with rehab intervention is beneficial

43
Q

Treatment for Michel aplasia?

A

no treatment

cochlear implants and conventional amplification are not useful

44
Q

What condition is characterized by cochlear duct differentiation that is limited at the level of the basal coil? This results in what type of hearing loss?

A

Alexander Aplasia

high-frequency hearing loss w/ adequate residual hearing in low frequencies

45
Q

Treatment for Alexander Aplasia?

A

conventional amplification is beneficial

46
Q

What condition is characterized by vestibular aqueduct 1.5 mm or greater? Cause?

A

Enlarged Vestibular Aqueduct Syndrome

hydrodynamic changes & labyrinthine membrane disruption

47
Q

Symptoms seen in Enlarged Vestibular Aqueduct Syndrome? Treatment?

A

early onset SNHL

usually bilateral & progressive

vertigo or incoordination

Treatment: avoid head trauma/contact sports

48
Q

Performing a CT/MRI is most beneficial in diagnosing what general type of hearing loss?

A

unilateral

49
Q

Performing a Connexin 26/GJB2 assay is most beneficial in diagnosing what general type of hearing loss?

A

bilateral

looking for DFNB1

50
Q

What are the common causes of non-congenital conductive hearing loss?

A

diseases interrupting sound transmission to the inner ear

  • cerumen (wax) impaction
  • infection (OM, OE)
  • external canal pathology
  • tympanic membrane pathology
  • middle ear pathology
51
Q

What condition is characterized by vascular, spongy bone to fibrosis & sclerotic bone? Where in the ear is this the most common?

A

otosclerosis

usually near anterior oval window (antefenestrum)

52
Q

What are the symptoms seen in otosclerosis?

Treatment?

A
  • Symptoms
    • progressive conductive or mixed hearing loss
    • 30-50yr
    • type A tympanogram
    • (+) Schwartze’s sign
  • Treatment
    • observation
    • hearing aid
    • stapedectomy
53
Q

What is Schwartze’s sign?

A

promontory hyperemia/redness

54
Q

What condition is suggestive by conductive hearing loss with hypermobile TM & malleus on pneumatic otoscopy? This leads to what type of hearing loss?

A

Ossicular discontinuity

conductive hearing loss with a deep tympanogram

55
Q

What are the common causes of ossicular discontinuity? Treament?

A
  • Cause
    • trauma
    • chronic otitis media
    • eustachian tube dysfunction
    • previous surgery
  • Treatment
    • tympanoplasty with ossicular chain reconstruction
56
Q

What are the different exam findings you would expect to see in patients with congenital cholesteatoma in age ranges 2-6yr, 4-12yr, and 12-30yr?

A
  • 2-6: tympanic membrane white mass only
  • 4-12: middle ear white mass, white TM
  • 12-30: mastoid white mass, hearing loss, vertigo
57
Q

What types of cells are responsible for the development of a congenital cholesteatoma?

A

epithelial cells in the middle ear adjacent to the malleus & posterior mesotympanum

58
Q

Treatment for congenital cholesteatoma?

A

surgical

59
Q

What is the cause of acquired cholesteatoma? Frequently associated conditions?

A

middle ear epithelium & keratoma formation with secondary enzymatic erosion - usually through TM perforation / epitympanic (attic) retraction pocket

frequently has chronic otitis media/otorrhea

60
Q

Treatment for acquired cholesteatoma?

A

surgical

61
Q

What is the most common cause of non-congenital sensorineural hearing loss?

A

noise induced

62
Q

What is the term for age-related non-congenital sensorineural hearing loss? How is this hearing loss described?

A

prebycusis

progressive high frequency hearing loss with discrimination loss

63
Q

What condition is characterized by aural fullness, roaring tinnitus, fluctuating hearing loss, severe episodic whirling vertigo?

A

Meniere Disease

a.k.a. endolymphatic hydrops

64
Q

What is Lermoyez Syndrome?

A

variant of Meniere Disease with increasing fullness & hearing loss relieved with vertigo attack

65
Q

What is Crisis of Tumarkin?

A

variant of Meniere Disease that has otolithic crisis w/ drop attacks

66
Q

What are the systemic autoimmune diseases that can lead to non-congenital hearing loss?

A

polyarteritis nodosa

wegener granulomatosis

SLE

RA

ulcerative colitis

67
Q

Whta is Cogan Syndrome?

A

nonsyphilitic interstitial keratitis & vertigo → bilateral progressive SNHL & bilateral Meniere’s Diseaer *15-20%)

68
Q

What is the treatment for autoimmune SNHL?

A

high dose steroids

cytotoxic medications

transtympanic steroids

69
Q

What is the clinical picture of a patient with idiopathic sudden SNHL?

A

abrupt or rapidly progressing hearing loss over minutes/days

40-54yr

70
Q

Most common causes of idiopathic sudden SNHL?

A
  • Causes
    • viral
    • immune complex mediated
    • vascular
  • Treatment
    • High dose steroids
    • antivirals
    • antibiotics
    • diuretics
    • histamine
    • anticoagulants
71
Q

What are the symptoms of a perilymphatic fistula?

A

sudden or progressive SNHL associated with roaring tinnitus, dysacusis, dysequilibrium

72
Q

What symptoms are seen with a Superior Semicircular canal fistula? Cause?

A

vertigo induced by lifting, valsalva or sound

dehiscence of superior semicircular canal – middle fossa (body labyrinth has eroded)

73
Q

Why do aminoglycosides lead to ototoxicity & therefore SNHL?

A

loss of outer hair cell

74
Q

Why do macrolides lead to ototixicity & therefore SNHL?

A

affects stria vascularis

75
Q

Which diuretics lead to ototixicity & therefore SNHL? Mechanism?

A

loop diuretics, ethacrynic acid, furosemide

stria vascularis

76
Q

Why do salicylates lead to ototixicity & therefore SNHL?

A

affect outer hair cells & spiral ganglion

77
Q

Why do antidepressants lead to ototixicity & therefore SNHL?

A

increase serotonin with disinhibition of dorsal cochlear nucleus

78
Q

Antineoplastic drugs & radiation lead to what type of hearing loss?

A

ototoxicity - SNHL

79
Q

What are the tumors of the middle ear?

A
  • multiple myeloma
  • leukemia
  • neorofibroma
  • fibrous dysplasia
  • eosinophilic granuloma
  • metastatic carcinoma
  • glomus tumors/paraganglioma
80
Q

What is the most common type of cerebellopontine angle tumors?

other type?

A

acoustic neuroma/vestibular schwannoma (78%)

meningioma (3%)

81
Q

What test should you do if you suspect a cerebellopontine angle tumor (CPA)?

Treatment?

A

MRI with & without gadolinium

Treatment: observation vs. surgery vs. radiation

Neuro II (30 decks)

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