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ENT Specialties MAIN > Inner Ear > Flashcards

Inner Ear Flashcards

1
Q

What % of patients with acoustic neuroma are asymptomatic at presentation

A

0.05

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2
Q

What is the incidence of congenital hearing loss

A

0.736111111111111

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3
Q

How many words should a child age 24 - 36 months be able to say

A

50

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4
Q

How many words should a child age 24 - 36 months be able to say

A

50

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5
Q

What is the 5-year survival for SCCA confined to the lateral EAC

A

??

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6
Q

What is the definition of sudden SNHL

A

>20d8 hearing loss over at least three contiguous frequencies occurring within 3 days.

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7
Q

What % of these are hereditary

A

>60%.

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8
Q

What is the incidence of hearing loss after infection with mumps

A

0.5%.

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9
Q

What is the incidence of hearing loss after infection with mumps

A

0.5%.

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10
Q

What is a typical word discrimination score in a patient with acoustic neuroma

A

0-30% in >50% of patients with an acoustic neuroma.

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11
Q

What % of children with congenital CMV have hearing loss

A

1 0% are born with hearing loss, I 0 - 15% eventually develop hearing loss.

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12
Q

What % of patients with unilateral SNHL have an acoustic neuroma

A

1 in 100,000

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13
Q

What is the incidence of Waardenburg’s syndrome

A

1 in 4,000 births.

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14
Q

What % of children with congenital CMV have hearing loss

A

1 O% are born with hearing loss, I 0 - 15% eventually develop hearing loss.

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15
Q

What are the 4 clinical subtypes of Waardenburg’s syndrome

A
  1. SNHL (20%), heterochromia irides, pigment anomalies, dystopia canthorum. 2. As above, without dystopia canthorum (SNHL in >50%) 3. Klein-Waardenburg’s syndrome: microcephaly, mental retardation, limb and skeletal abnormalities, in addition to signs of # 1. 4. Shah-Waardenburg’s syndrome: #2 + Hirschsprung’s disease
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16
Q

What are the 4 clinical subtypes of Waardenburg’s syndrome

A
  1. SNHL (20%), heterochromia irides, pigment anomalies, dystopia canthorum. 2. As above, without dystopia canthorum (SNHL in >50%) 3. Klein-Waardenburg’s syndrome: microcephaly, mental retardation, limb and skeletal abnormalities, in addition to signs of # I. 4. Shah-Waardenburg’s syndrome: #2 + Hirschsprung’s disease
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17
Q

What proportion of patients with AIED will not have any vestibular symptoms

A

1/3.

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18
Q

What proportion of these patients will have an abnormal ABR

A

1/3.

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19
Q

What are the typical inheritance patterns of nonsyndromic hearing loss

A

10 - 20% AD, 75% autosomal recessive (AR), 2 - 3% X-linked,

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20
Q

What is the incidence of post-meningitic hearing loss

A

10- 20%.

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21
Q

What is the incidence of post-meningitic hearing loss

A

10- 20°/o.

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22
Q

In what % of these cases can a definite cause be determined

A

10%.

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23
Q

What % of patients with CSF leak secondary to non-surgical trauma will develop meningitis

A

10-25%.

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24
Q

What % of basilar skull fractures result in CSF leak

A

10-30%.

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25
Q

What is the concentration of immunoglobulins in the perilymph compared to the serum

A

111 oooth

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26
Q

What is the 5-year survival for SCCA extending beyond the lateral EAC

A

15- 20°/o.

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27
Q

What % of skull fractures involve the temporal bone

A

18%.

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28
Q

How small of a lesion can M Rl with gadolinium detect

A

2 mm.

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29
Q

How small of a lesion can MRI with gadolinium detect

A

2 mm.

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30
Q

What % of patients with a tumor in the middle ear will present with facial nerve palsy

A

20 - 40%.

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31
Q

What % of patients with longitudinal temporal bone fractures have facial nerve paralysis

A

20- 25°/o.

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32
Q

What % of sporadic cases of congenital HL are caused by this mutation

A

27%.

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33
Q

What % of patients with AIED will also have a systemic autoimmune disease

A

29%.

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34
Q

What % of CSF leaks are from non-traumatic causes

A

3- 4%.

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35
Q

Diligent workup of congenital hearing loss with state-of-the-art techniques is inconclusive what % of the time

A

30 - 40%.

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36
Q

Diligent workup of congenital hearing loss with state-of-the-art techniques is inconclusive what % of the time

A

30 - 40%.

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37
Q

What % of patients with immune-mediated Meniere’s disease will have a positive anti-68-kD Western blot test

A

30 - 50%.

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38
Q

What % the hereditary cases are syndromic

A

30%.

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39
Q

At what age should children be able to say multi-word sentences

A

36 months.

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40
Q

At what age should children be able to say multi-word sentences

A

36 months.

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41
Q

What % of patients have facial nerve injury after transverse fracture of the temporal bone

A

40- 50%.

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42
Q

If one parent and one sibling are deaf, what is the risk of hearing loss for subsequent offspring

A

40% risk.

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43
Q

If one parent and one sibling are deaf, what is the risk of hearing loss for subsequent offspring

A

40% risk.

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44
Q

What % of patients with RA have SNHL

A

44%.

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45
Q

How small of a lesion can fast-spin echo MRI detect

A

4-5 mm.

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46
Q

How small of a lesion can fast-spin echo MRI detect

A

4-5 mm.

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47
Q

What % of patients with neurofibromatosis type 1 have acoustic neuromas

A

5% and usually unilateral.

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48
Q

What % of patients with neurofibromatosis type 1 have acoustic neuromas

A

5% and usually unilateral.

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49
Q

What % of infants with significant congenital hearing loss will not have risk factors

A

50%.

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50
Q

What % of infants with significant congenital hearing loss will not have risk factors

A

50%.

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51
Q

What % of patients with severe tinnitus are successfully treated with masking devices

A

58-64%.

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52
Q

At what age should children be able to respond to their name and understand simple words

A

6 to I 0 months.

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53
Q

At what age should children be able to respond to their name and understand simple words

A

6 to I 0 months.

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54
Q

What is a typical word discrimination score in a patient with acoustic neuroma

A

60-70%

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55
Q

What % of cerebellopontine angle (CPA) tumors are acoustic neuromas

A

78%.

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56
Q

What % of these patients have hearing loss

A

80% (50% mixed, 30% conductive, 20°/o sensorineural).

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57
Q

What % of these patients have hearing loss

A

80% (50°/o mixed, 30% conductive, 20°/o sensorineural).

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58
Q

What % of CSF leaks are cranio-nasal

A

80%.

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59
Q

What % of patients with neurofibromatosis type 2 have acoustic neuromas

A

95% and usually bilateral.

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60
Q

What % of patients with neurofibromatosis type 2 have acoustic neuromas

A

95°/o and usually bilateral.

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61
Q

What is the reservoir sign

A

A rush of clear rhinorrhea occurs with sudden upright position.

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62
Q

What is the basic defect of this disease

A

Abnormal fibroblast growth factor (FGF) receptors.

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63
Q

What is the basic defect of this disease

A

Abnormal fibroblast growth factor (FGF) receptors.

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64
Q

What is the most common toxicity of methotrexate

A

Abnormal liver function.

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65
Q

What is the basic defect causing this syndrome

A

Abnormal potassium channels.

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66
Q

What is the basic defect causing this syndrome

A

Abnormal potassium channels.

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67
Q

What is the most common tumor of glandular origin to involve the EAC or middle ear

A

Adenoid cystic carcinoma.

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68
Q

Which inner ear aplasia is characterized by high frequency hearing loss with normal low frequency hearing

A

Alexander.

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69
Q

Where are the laceration and bony disruption in the EAC most often found after longitudinal temporal bone fracture

A

Along the tympanosquamous suture line (posterior and superior).

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70
Q

Mitochondrial mutations have been found to produce enhanced sensitivity to the ototoxic effects of which medications

A

Aminoglycosides.

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71
Q

Mitochondrial mutations have been found to produce enhanced sensitivity to the ototoxic effects of which medications

A

Aminoglycosides.

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72
Q

What is temporal bone myospherulosis

A

An unusual foreign-body reaction occurring in tissues exposed to petrolatum-based products.

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73
Q

Where does the fracture line typically course in relation to the otic capsule

A

Anterior to the otic capsule.

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74
Q

What is the mechanism of type II immune reactions

A

Antibodies directed against a specific antigen within tissues activate complement.

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75
Q

What cell patterns are characteristic of acoustic neuromas

A

Antoni A (tightly arranged) and Antoni B (loosely arranged).

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76
Q

In which pattern are Verocay’s bodies found

A

Antoni A.

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77
Q

Why does spontaneous CSF otorrhea present late when caused by arachnoid granulations

A

Arachnoid granulations become larger with time; the normal pulsation of CSF pressure can cause bony erosion.

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78
Q

When should middle ear exploration and ossicular reconstruction be performed after temporal bone fracture

A

At least 3 months after injury.

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79
Q

What is the most common cause of pulsatile tinnitus in patients older than 50

A

Atherosclerotic carotid artery disease.

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80
Q

What is the typical inheritance pattern of syndromic hearing loss

A

Autosomal dominant (AD).

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81
Q

What is the inheritance pattern of branchio-oto-renal syndrome

A

Autosomal dominant.

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82
Q

What is the inheritance pattern of this disease

A

Autosomal dominant.

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83
Q

What is the inheritance pattern of branchio-oto-renal syndrome

A

Autosomal dominant.

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84
Q

What is the inheritance pattern of this disease

A

Autosomal dominant.

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85
Q

What feature seen on electron microscopy is pathognomonic for Alport syndrome

A

Basket-weave configuration of the glomerular basement membrane.

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86
Q

What feature seen on electron microscopy is pathognomonic for Alport syndrome

A

Basket-weave configuration of the glomerular basement membrane.

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87
Q

What disease is characterized by recurrent aphthous ulcers, ocular inflammation, cutaneous vasculitis, and SN H L

A

Beh9et’s dis•ease.

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88
Q

What type of masking device is recommended for patients with hearing loss

A

Behind-the-ear hearing aid.

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89
Q

What is the most accurate method of determining if otorrhea is CSF

A

Beta-2-transferrin assay.

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90
Q

What substance is unique to CSF, perilymph, and vitreous humor

A

Beta-2-transferrin.

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91
Q

What sort of hearing loss is typical after meningitis

A

Bilateral, severe to profound, and pennanent.

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92
Q

What sort of hearing loss is typical after meningitis

A

Bilateral, severe to profound, and permanent.

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93
Q

What is the habituation technique for the treatment of tinnitus

A

Binaural broad-band noise generators are worn for at least 6 hours everyday for at least 12 months; in a study by Mattox et al., tinnitus was significantly improved in 84%).

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94
Q

What is the most common etiology of dizziness after longitudinal temporal bone fracture

A

BPPV.

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95
Q

What syndrome is characterized by hearing loss, renal defects, and cervical fistula

A

Branchio-oto-renal.

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96
Q

What syndrome is characterized by hearing loss, renal defects, and cervical fistula

A

Branchio-oto-renal.

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97
Q

What are the most common sites of origin of metastatic tumors of the temporal bone

A

Breast, lung, and kidney.

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98
Q

What is associated with a decreased risk of intracranial and vascular injuries after gunshot wounds to the temporal bone

A

Bullet trajectory lateral to the middle ear cavity.

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99
Q

What test has greater than 90°/o specificity for the diagnosis of Wegener’s

A

c-ANCA.

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100
Q

Which of these vasodilators can override the intracranial autoregulatory mechanism of blood flow

A

Carbon dioxide.

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101
Q

What are two common theories on the etiology of idiopathic sudden sensorineural hearing loss (ISSNHL)

A

Circulatory disturbance and inflammatory reaction (usually viral).

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102
Q

What organism is most commonly associated with virus-induced congenital deafness

A

CMV.

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103
Q

What organism is most commonly associated with virus-induced congenital deafness

A

CMV.

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104
Q

What laboratory studies are useful in the workup

A

Coagulation profile (CBC, PT, PTT), viral studies, ESR.

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105
Q

What syndrome is characterized by vestibuloauditory symptoms in association with non-syphilitic interstitial keratitis, mostly in young adults

A

Cogan’s syndrome.

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106
Q

What is the best surgical approach for facial nerve exploration in a patient with a temporal bone fracture distal to the geniculate ganglion with intact hearing

A

Combined transmastoid/middle fossa approach.

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107
Q

What approach is most often used for longitudinal fractures

A

Combined transmastoid/middle fossa.

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108
Q

Where do most basal cell carcinomas of the EAC arise

A

Concha.

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109
Q

What does the audiogram typically look like in a child with SNHL secondary to rubella

A

Cookie-bite pattern.

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110
Q

What does the audiogram typically look like in a child with SNHL secondary to rubella

A

Cookie-bite pattern.

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111
Q

What disease is characterized by cranial synostosis, exophthalmos, parrot-beaked nose, and hypoplastic mandible

A

Crouzon’ s disease.

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112
Q

What disease is characterized by cranial synostosis, exophthalmos, parrot-beaked nose, and hypoplastic mandible

A

Crouzon’s disease.

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113
Q

What is the initial test of choice in patients with pulsatile tinnitus and a retrotympanic mass

A

CT scan of the temporal bones.

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114
Q

In the workup of congenital hearing loss, what test has the highest diagnostic yield

A

CT scan.

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115
Q

In the workup of congenital hearing loss, what test has the highest diagnostic yield

A

CT scan.

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116
Q

What is residual inhibition

A

Decreased or absent tinnitus following exposure to MML plus 10 dB for I minute.

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117
Q

What is the mechanism of type Ill immune reactions

A

Deposition of immune complexes in the microcirculation.

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118
Q

What is the primary disadvantage of the translabyrinthine approach

A

Destroys hearing permanently.

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119
Q

What is the initial test of choice in patients with pulsatile tinnitus and normal otoscopy

A

Duplex carotid ultrasound and echocardiogram in patients suspected of AC AD; otherwise, M RIIM RA/M R V.

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120
Q

What structures are resected in a subtotal temporal bone resection

A

EAC, middk ear, petrous bone, TMJ, parotid gland with facial nerve.

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121
Q

What are the most common etiologies of nerve dysfunction after longitudinal temporal bone fracture

A

Edema and intraneural hemorrhage.

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122
Q

What are the characteristics of CSF in the presence of meningitis

A

Elevated protein, WBC, and pressure~ decreased glucose.

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123
Q

What is the medical management of CSF leak

A

Elevation of the head of bed, antitussives, laxatives, anti-hypertensives, analgesics, bedrest, lumbar drain.

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124
Q

In which area of the inner ear are immunologically active structures most commonly found

A

Endolymphatic sac.

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125
Q

What inner ear malformation is associated with early onset SNHL, usually bilateral and progressive, and vertigo

A

Enlarged vestibular aqueduct.

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126
Q

What inner ear malformation is associated with early onset SNHL, usually bilateral and progressive, and vertigo

A

Enlarged vestibular aqueduct.

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127
Q

How is the diagnosis of IIH syndrome made

A

Exclusion of lesions producing intracranial hypertension, lumbar puncture with CSF pressure of more than 200 mmH,Q and normal CSF constituents.

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128
Q

What is the most common site of ear and temporal bone tumors

A

External auditory canal (EAC).

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129
Q

What gene is responsible for this syndrome

A

EY A I on chromosome 8q I 3.3.

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130
Q

What gene is responsible for this syndrome

A

EY A I on chromosome 8q I3.3.

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131
Q

T/F: SNHL associated with mumps usually causes vestibular dysfunction

A

False.

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132
Q

T/F: SNHL associated with mumps usually causes vestibular dysfunction

A

False.

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133
Q

T /F: The majority of patients with pulsatile tinnitus do not have a treatable underlying cause.

A

False.

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134
Q

T/F: Absence of papilledema excludes IIH syndrome

A

False.

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135
Q

T/F: Tumors with a high % of Antoni A cells relative to Antoni B cells have a better prognostic outcome

A

False; outcome is independent of cell proportions.

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136
Q

What are the indications for hearing evaluation every 6 months until age 3

A

Family history of hereditary childhood hearing loss. In utero infection (TORCH). Neurodegenerative disorders.

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137
Q

What are the indications for hearing evaluation every 6 months until age 3

A

Family history of hereditary childhood hearing loss. In utero infection (TORCH). Neurodegenerative disorders.

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138
Q

What are the indications for performing hearing screening in neonates if universal screening is not available

A

Family history of hereditary childhood SNHL. Congenital perinatal infection (TORCH). Head or neck malformation. Birth weight 20). Bacterial meningitis. Apgar 0 - 4 at I minute or 0 - 6 at 5 minutes. Prolonged ventilation (>5 days). Ototoxic medications.

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139
Q

What are the indications for performing hearing screening in neonates if universal screening is not available

A

Family history of hereditary childhood SNHL. Congenital perinatal infection (TORCH). Head or neck malformation. Birth weight 20). Bacterial meningitis. Apgar 0 - 4 at I minute or 0 - 6 at 5 minutes. Prolonged ventilation (>5 days). Ototoxic medications.

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140
Q

Which is less expensive - MRI with gadolinium or fast-spin echo MRI

A

Fast-spin echo MRI.

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141
Q

Which is less expensive - MRI with gadolinium or fast-spin echo MRI

A

Fast-spin echo MRI.

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142
Q

What is the recommended treatment for AIED

A

First line treatment is high-dose prednisone, then methotrexate, then cyclophosphamide.

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143
Q

What findings support the circulatory theory

A

Fisch et al ( 1976) showed that the perilymphatic oxygen tension was 30°/o lower in patients with ISSNHL versus normal subjects. Ciuffetti et al (1991) found disturbances in microcirculatory blood flow in 16 patients with ISSNHL.

144
Q

What evidence supports the use of carbon dioxide for ISSNHL

A

Fisch et al ( 1983) compared carbogen (95% oxygen and 5% carbon dioxide) inhalation therapy daily for 5 days to papverine and low-molecular-weight dextran for 5 days and found a statistically significant improvement in hearing levels with carbogen therapy. These findings have not been replicated.

145
Q

When is surgical exploration indicated after temporal bone fracture

A

For massively displaced fractures with compromise of the carotid artery or VII; or for Vllth nerve paralysis with >90% degeneration documented on ENoG within 14 days of the injury.

146
Q

What is the typical course of the fracture line in transverse temporal bone fractures

A

Foramen magnum across the petrous apex, across the lAC and otic capsule, to the foramen spinosum or lacerum.

147
Q

What is the function of connexin 26

A

Formation of gap junctions in the stria vascularis, basement membrane, limbus, and spiral prominence of the cochlea.

148
Q

What are the only antigen-specific tests for syphilis

A

FTA-ABS and MHA-TP.

149
Q

What are the only antigen-specific tests for syphilis

A

FTA-ABS and MHA-TP.

150
Q

What are the most common organisms causing nonfatal bacterial meningitis in children >2.5 years

A

Haemophilus injluenzae, Neisseria meningitides, Streptococcus pneumoniae.

151
Q

What are the most common organisms causing nonfatal bacterial meningitis in children >2.5 years

A

Haemophilus injluenzae, Neisseria meningitides, Streptococcus pneumoniae.

152
Q

What signs on physical exam are suggestive of CSF leak

A

Halo sign and reservoir sign.

153
Q

How does hearing loss caused by mumps usually present

A

Hearing loss develops as the parotitis is resolving.

154
Q

How does hearing loss caused by mumps usually present

A

Hearing loss develops as the parotitis is resolving.

155
Q

Following acoustic neuroma resection, what problem do patients perceive as most troublesome

A

Hearing loss.

156
Q

The 68 kDa antigen is thought to represent what protein

A

Heat shock protein 70 (hsp 70).

157
Q

What are the most common injuries encountered on surgical exploration

A

Hematoma and contusion with bony spicules impinging on the nerve sheath.

158
Q

What are the potential side effects of cyclophosphamide

A

Hemorrhagic cystitis, leukopenia, sterility, and malignancies of the urinary tract.

159
Q

What are the 2 types of non-traumatic CSF leaks

A

High pressure and normal pressure.

160
Q

What is the most common type of hearing loss from acoustic neuroma

A

High-frequency unilateral SNH L.

161
Q

What is the most common type of hearing loss from acoustic neuroma

A

High-frequency unilateral SNHL.

162
Q

What are other risk factors for development of ear and temporal bone tumors

A

History of radiation to the head and neck, chronic chromate burns secondary to using matchsticks to clean the ear canal.

163
Q

What are the 3 clinical subtypes of Usher’s syndrome

A

I - severe-profound H L, absent vestibular function, pre-pubertal retinitis pigmentosa. II - moderate-severe H L, normal vestibular function, post-pubertal retinitis pigmentosa. III - progressive hearing loss.

164
Q

What are the 3 clinical subtypes of Usher’s syndrome

A

I - severe-profound H L, absent vestibular function, pre-pubertal retinitis pigmentosa. II - moderate-severe H L, normal vestibular function, post-pubertal retinitis pigmentosa. III - progressive hearing loss.

165
Q

What % of patients with a tumor in the EAC will present with cervical metastases

A

I 0%.

166
Q

What is the incidence of Waardenburg’s syndrome

A

I in 4,000 births.

167
Q

What is the sensitivity of stacked derived-band ABR in detecting an acoustic neuroma

A

I OO%.

168
Q

What is the sensitivity of stacked derived-band ABR in detecting an acoustic neuroma

A

I OO%.

169
Q

How is congenital CMV diagnosed in the newborn

A

Identification of serum anti-CMV lgM, “owl eye” bodies in the urinary sediment, and intracerebral calcifications on radiographs.

170
Q

How is congenital CMV diagnosed in the newborn

A

Identification of serum anti-CMV lgM, “owl eye” bodies in the urinary sediment, and intracerebral calcifications on radiographs.

171
Q

What is the most common cause of pulsatile tinnitus in young female patients

A

Idiopathic intracranial hypertension (IIH) syndrome.

172
Q

What is the most common cause of venous pulsatile tinnitus

A

Idiopathic intracranial hypertension syndrome (pseudotumor cerebri. benign intracranial hypertension).

173
Q

When is spontaneous recovery of hearing more likely

A

If patient is without vestibular symptoms and suffers only partial hearing loss, particularly low-frequency (better prognosis if apex of the cochlea is involved).

174
Q

When is a middle ear exploration indicated

A

If the loss occurs in an only-hearing ear… to rule out fistula.

175
Q

What is the predominant immunoglobulin in the endolymphatic sac

A

IgA.

176
Q

What % of patients with unilateral tinnitus have retrocochlear pathology

A

II %.

177
Q

What % of patients with unilateral tinnitus have retrocochlear pathology

A

II %.

178
Q

What is the strongest predictor of poor recovery of facial nerve function following temporal bone trauma

A

Immediate onset of facial paralysis in a patient with a closed head injury.

179
Q

What is the most common mechanism of CHL in longitudinal fractures

A

Incudostapedial joint dislocation.

180
Q

What is the mechanism of action of methotrexate

A

Inhibits dihydrofolate reductase, interfering with DNA synthesis, repair, and replication.

181
Q

Tricyclic antidepressants are most likely to benefit patients with tinnitus who have what other problem

A

Insomnia.

182
Q

Tumors that metastasize to the temporal bone via the meninges most often traverse what structure

A

Internal auditory canal.

183
Q

What manifestation of congenital syphilis is most commonly related to SNHL

A

Interstitial keratitis.

184
Q

What manifestation of congenital syphilis is most commonly related to SNHL

A

Interstitial keratitis.

185
Q

What are the most common associated injuries following gunshot wounds of the temporal bone

A

Intracranial injuries (53%).

186
Q

What are the most important questions to answer in the preoperative evaluation of a temporal bone tumor

A

Is the carotid artery or brain involved

187
Q

What is the most commonly identified inner ear malformation on temporal bone imaging studies

A

Isolated lateral semicircular canal defects.

188
Q

What is the most commonly identified inner ear malformation on temporal bone imaging studies

A

Isolated lateral semicircular canal defects.

189
Q

Why J[s aspergillus infection a risk factor for ear and temporal bone tumors

A

It produces aflatoxin 8, a known carcinogen.

190
Q

What are 5 other venous etiologies of pulsatile tinnitus

A

Jugular bulb abnormalities; hydrocephalus associated with stenosis of the sylvian aqueduct; increased intracranial pressure associated with Amold-Chiari syndrome; abnormal condylar and mastoid emissary veins; idiopathic or essential tinnitus.

191
Q

What surgical approach is used for tumors that involve both the cartilaginous and bony ear canal without extension into the middle ear

A

Lateral temporal bone resection.

192
Q

What is the typical severity and pattern of AD hearing loss

A

Less severe, delayed-onset, high frequency hearing loss.

193
Q

What is the predominant immunoglobulin in the perilymph

A

lgG.

194
Q

What maneuvers on physical exam will decrease or completely eliminate pulsatile tinnitus of venous origin

A

Light digital pressure over the ipsilateral internal jugular vein and head turning towards the ipsilateral side.

195
Q

Which of these is most common

A

Longitudinal (80 - 90°/o).

196
Q

What are the 3 types of temporal bone fractures

A

Longitudinal, transverse, and mixed.

197
Q

Which of these is associated with conductive hearing loss (CHL)

A

Longitudinal.

198
Q

Which of these accounts for the majority of facial nerve injuries

A

Longitudinal.

199
Q

What is an early sign of relapse following treatment

A

Loud tinnitus.

200
Q

In patients with IIH, what is the usual pitch of the tinnitus

A

Low frequency.

201
Q

How can one determine if maskers will be effective in the treatment of tinnitus

A

Measure the MML and loudness matching; if the MML is lower or equal to the loudness matching, maskers will likely be effective.

202
Q

What laboratory tests can be used to diagnose CSF leak

A

Measurement of glucose (nasal secretions are devoid of glucose), beta-2-transferrin.

203
Q

What is the term for complete agenesis of the petrous portion of the temporal bone

A

Michel aplasia.

204
Q

Which inner ear aplasia will not allow cochlear implant or amplification aids

A

Michel aplasia.

205
Q

Which approach is best in patients with tumors

A

Middle fossa.

206
Q

What inner ear malformations are more common in patients with Pendred syndrome

A

Mondini aplasia and enlarged vestibular aqueduct.

207
Q

What inner ear malformations are more common in patients with Pendred syndrome

A

Mondini aplasia and enlarged vestibular aqueduct.

208
Q

What is the term for a developmentally deformed cochlea where only the basal coil can be identified

A

Mondini aplasia.

209
Q

Of all the viruses associated with hearing loss, which one is most likely to be associated with unilateral hearing loss

A

Mumps.

210
Q

Of all the viruses associated with hearing loss, which one is most likely to be associated with unilateral hearing loss

A

Mumps.

211
Q

What genetic mutation is responsible for Treacher Collins syndrome

A

Mutation of TCOF I on chromosome Sq.

212
Q

What genetic mutation is responsible for Treacher Collins syndrome

A

Mutation of TCOF I on chromosome Sq.

213
Q

What is the basic defect causing Alport syndrome

A

Mutation of the COL4A5 gene producing the alpha chain of type IV collagen in basement membranes.

214
Q

What is the basic defect causing Alport syndrome

A

Mutation of the COL4A5 gene producing the alpha chain of type IV collagen in basement membranes.

215
Q

What genetic mutation is thought to be responsible for 50 - 80°/o of all AR hearing loss

A

Mutation of the DFNB 1 gene on chromosome I 3q encoding for connexin 26.

216
Q

What genetic mutation is responsible for neurofibromatosis type 1

A

Mutation of the NF I gene (nerve growth factor gene) on chromosome 17q 11.2.

217
Q

What genetic mutation is responsible for neurofibromatosis type 1

A

Mutation of the NF I gene (nerve growth factor gene) on chromosome I7qll.2.

218
Q

What genetic mutation is responsible for neurofibromatosis type 2

A

Mutation of the NF2 gene (tumor suppressor gene) on chromosome 22ql2.2.

219
Q

What genetic mutation is responsible for neurofibromatosis type 2

A

Mutation of the NF2 gene (tumor suppressor gene) on chromosome 22ql2.2.

220
Q

What genetic mutation is responsible for most cases of types 1 and 3 of Waardenburg’s syndrome

A

Mutation of the PAX3 gene on chromosome 2q37.

221
Q

What genetic mutation is responsible for most cases of types 1 and 3 of Waardenburg’s syndrome

A

Mutation of the PAX3 gene on chromosome 2q37.

222
Q

What genetic mutations are responsible for most cases of Stickler syndrome

A

Mutations in the COL2Al gene on chromosome 12 or the COLIIA2 gene on chromosome 6.

223
Q

What genetic mutations are responsible for most cases of Stickler syndrome

A

Mutations in the COL2Al gene on chromosome 12 or the COLIIA2 gene on chromosome 6.

224
Q

What genetic mutations are thought to be responsible for osteogenesis imperfecta

A

Mutations of the COLlA I gene on chromosome 17q and the COLIA2 gene on chromosome 7 q.

225
Q

What genetic mutations are thought to be responsible for osteogenesis imperfecta

A

Mutations of the COLlA I gene on chromosome 17q and the COLlA2 gene on chromosome 7 q.

226
Q

What procedures are often performed in conjunction with a lateral temporal bone resection

A

Neck dissection, parotidectomy, and occasionally, partial mandibulectomy.

227
Q

What other procedures are routinely performed with a subtotal temporal bone resection

A

Neck dissection, temporal craniotomy to rule-out transdural extension.

228
Q

What nerve is involved in paroxysmal lacrimation

A

Nervus intermedius.

229
Q

An 18-year-old man with unilateral hearing loss has an enhancing lesion in the CPA and a meningioma in the occipital region. He has no skin lesions or subcutaneous nodules. What disease does he most likely have

A

Neurofibromatosis type 2.

230
Q

What evidence supports the use of antivirals for ISSN H L

A

No randomized, prospective studies have demonstrated this therapy to be effective.

231
Q

What is the most common cause of hearing loss and associated tinnitus

A

Noise exposure.

232
Q

What are the 2 main categories of tinnitus

A

Non-pulsatile and pulsatile.

233
Q

Which is more common

A

Non-pulsatile.

234
Q

What is the most common cause of CSF leak

A

Non-surgical trauma.

235
Q

What is the treatment for relapsing polychondritis

A

NSAI Ds, steroids, dapsone.

236
Q

What is the most common type of temporal bone fracture in children

A

Obliquely oriented fractures.

237
Q

Is the facial nerve sacrificed during lateral temporal bone resection

A

Only if it is involved with tumor.

238
Q

What is the current standard of care for the workup and treatment of ISSNHL

A

Otologic exam, audiogram and rule-out retrocochlear pathology… treatment with steroids, +/antivirals, +/diuretics.

239
Q

What syndrome is characterized by hypertelorism, short stature, broad fingers and toes, cleft palate, and conductive hearing loss

A

Otopalatodigital syndrome.

240
Q

What syndrome is characterized by hypertelorism, short stature, broad fingers and toes, cleft palate, and conductive hearing loss

A

Otopalatodigital syndrome.

241
Q

What is the most common site of CSF leakage from the inner ear into the middle ear in children

A

Oval window (especially in patients with Mondini dysplasia).

242
Q

WhaK is the prognosis of I SSN H L

A

Overall recovery to functional hearing levels in 65-69%; no conclusive evidence that outcome is improved by medical treatment.

243
Q

What are the indications for performing hearing screening in infants 29 days to 2 years

A

Parent concern. Developmental delay. Bacterial meningitis. Head trauma associated with loss of consciousness or skull fracture. Ototoxic medications. Recurrent or persistent otitis media with effusion for at least 3 months.

244
Q

What are the indications for performing hearing screening in infants 29 days to 2 years

A

Parent concern. Developmental delay. Bacterial meningitis. Head trauma associated with loss of consciousness or skull fracture. Ototoxic medications. Recurrent or persistent otitis media with effusion for at least 3 months.

245
Q

What factors lead to the best rate of recovery after ISSNHL

A

Patients treated with steroids and vasodilators, with worse initial PTA and SDS, younger age, and greater number of treatments are most likely to improve ( Fetterman et al 1 996).

246
Q

What gene is associated with both Pendred syndrome and enlarged vestibular aqueduct

A

PDS gene, encoding for pendrin protein, on chromosome 7 q31.

247
Q

What gene is associated with both Pendred syndrome and enlarged vestibular aqueduct

A

PDS gene, encoding for pendrin protein, on chromosome 7 q31.

248
Q

Mutation of what gene is associated with enlarged vestibular aqueduct

A

Pendrin on chromosome 7 q3 I.

249
Q

What test assists in the diagnosis of Pendred syndrome

A

Perchlorate challenge test… perchlorate will displace more iodine than normal from the thyroid gland in these patients.

250
Q

What test assists in the diagnosis of Pendred syndrome

A

Perchlorate challenge test… perchlorate will displace more iodine than normal from the thyroid gland in these patients.

251
Q

Which part of the facial nerve is most often involved

A

Perigeniculate area.

252
Q

Tumors that metastasize to the temporal bone hematogenously most often involve which area of the temporal bone

A

Petrous apex.

253
Q

What are the typical symptoms of interstitial keratitis

A

Photophobia, lacrimation, pain.

254
Q

What auditory tests are performed in tinnitus analysis

A

Pitch matching, loudness matching, minimum masking level (MML), and residual inhibition.

255
Q

What disease is a necrotizing vasculitis of small and medium-sized muscular arteries, most commonly involving the renal and visceral vessels, and is a potential cause of hearing loss

A

Polyarteritis nodosa.

256
Q

What are the two types of congenital defects that lead to spontaneous CSF otorrhea

A

Preformed bony pathway around the bony labyrinth, often associated with a meningocele and aberrant arachnoid granulations located over a pneumatized area of the skull.

257
Q

Which of these is associated with meningitis

A

Preformed bony pathway around the bony labyrinth.

258
Q

What is the typical severity and pattern of X-linked hearing loss

A

Prelingual and more clinically diverse hearing loss.

259
Q

How does the defect caused by arachnoid granulations usually present

A

Presents after age 50 as unilateral serous otitis which is at first recurrent and then persistent.

260
Q

What is the usual presentation of autoimmune inner ear disease (AIED)

A

Progressive SNHL over weeks to months in middle-aged women, occasionally with a serous middle ear effusion.

261
Q

What sort of hearing loss is most common in patients with Cogan’s syndrome

A

Progressive to total deafness.

262
Q

What are the clinical features of Jervell and Lange-Nielsen’s syndrome

A

Prolonged QT interval, syncope, sudden death, and hearing loss.

263
Q

What are the clinical features of Jervell and Lange-Nielsen’s syndrome

A

Prolonged QT interval, syncope, sudden death, and hearing loss.

264
Q

What other tests can be used to diagnose CSF leak

A

Radionuclide cisternography, CT cistemography, intrathecal fluorescein.

265
Q

What is the likely diagnosis for someone who presents with vesicles on the pinna and EAC, facial nerve weakness, and SNHL

A

Ramsey-Hunt syndrome.

266
Q

What is the likely diagnosis for someone who presents with vesicles on the pinna and EAC, facial nerve weakness, and SNHL

A

Ramsey-Hunt syndrome.

267
Q

What physical exam findings are classic for measles

A

Rash, conjunctivitis, and Koplik ‘s spots.

268
Q

What physical exam findings are classic for measles

A

Rash, conjunctivitis, and Koplik’s spots.

269
Q

Which approach is best in patients with tumors > 2.5 em with good hearing

A

Retrosigmoid.

270
Q

What: are the most common types of sarcoma of the temporal bone

A

Rhabdomyosarcoma, chondrosarcoma, and osteosarcoma.

271
Q

What autoantibody is present in 75°/o of patients with rheumatoid arthritis (RA)

A

Rheumatoid factor.

272
Q

What is the most common form of inner ear aplasia

A

Scheibe aplasia ( cochleosaccular dysplasia or pars inferior dysplasia).

273
Q

What temporal bone malformation is classic for rubella

A

Scheibe malformation.

274
Q

What temporal bone malformation is classic for rubella

A

Scheibe malformation.

275
Q

What evidence refutes the circulatory theory

A

Schuknecht et al ( 1973) reports no histologic evidence of vascular compromise to the organ of Corti in these patients.

276
Q

What is the differential diagnosis of a CPA tumor

A

Schwannoma, meningioma, epidermoid, lipoma, arachnoid cyst, cholesterol granuloma.

277
Q

What are the clinical features of Alport syndrome

A

Sensorineural hearing loss (SNHL) and renal failure (presenting as hematuria).

278
Q

What are the clinical features of Alport syndrome

A

Sensorineural hearing loss (SNHL) and renal failure (presenting as hematuria).

279
Q

What is the most common otologic manifestation of Wegener’s

A

Serous otitis media.

280
Q

What is dystopia canthorum

A

Shortened and fused medial eyelids resulting in small medial sclera, lateral displacement of the inferior puncta, and hypertelorism.

281
Q

What is dystopia canthorum

A

Shortened and fused medial eyelids resulting in small medial sclera, lateral displacement of the inferior puncta, and hypertelorism.

282
Q

What factor is most related to hearing outcome after surgery

A

Size of tumor; significantly more likely to have preservation of hearing if

283
Q

What other test can be useful in diagnosing Alport syndrome

A

Skin biopsy.

284
Q

What other test can be useful in diagnosing Alport syndrome

A

Skin biopsy.

285
Q

What surgical approach is used for small, localized tumors of the cartilaginous ear canal that have not invaded deep structures

A

Sleeve resection.

286
Q

What are the 3 classic findings of congenital rubella syndrome

A

SNHL, cataracts, heart malformations.

287
Q

What are the 3 classic findings of congenital rubella syndrome

A

SNHL, cataracts, heart malformations.

288
Q

What are the most common complications of acoustic neuroma resection

A

SNHL, paralysis of VII, CSF leak (10-35%), meningitis (1-10(%), intracranial hemorrhage (0.5-2%).

289
Q

Which pattern has the worst prognosis

A

Solid patten1.

290
Q

What is the most common histologic type of tumor involving the EAC or middle ear

A

Squamous cell carcinoma (SCCA).

291
Q

What histologic finding distinguishes cholesteatoma from cholesterol granuloma

A

Squamous epithelium is only present in cholesteatomas.

292
Q

What evidence supports the use of corticosteroids for ISSNHL

A

Steroid therapy is among the few treatment methods in ISSNHL to have single modality, randomized, prospective studies demonstrating effectiveness (Wilson et al 1980, Moschowitz et al 1984).

293
Q

What syndrome is characterized by cleft palate, micrognathia, severe myopia, retinal detachments, cataracts, marfanoid habitus, and hearing loss

A

Stickler.

294
Q

What syndrome is characterized by cleft palate, micrognathia, severe myopia, retinal detachments, cataracts, marfanoid habitus, and hearing loss

A

Stickler.

295
Q

Which organism most commonly causes post-meningitic hearing loss

A

Streptococcus pneumoniae.

296
Q

Which organism most commonly causes post-meningitic hearing loss

A

Streptococcus pneumoniae.

297
Q

What surgical approach is used for tumors involving the middle ear that appear confined to the temporal bone

A

Subtotal temporal bone resection.

298
Q

Which semicircular canal forms first? Last

A

Superior canal forms first; lateral canal forms last.

299
Q

Which semicircular canal forms first? Last

A

Superior canal forms first; lateral canal forms last.

300
Q

What is the mechanism of type IV immune reactions

A

T -cell mediated delayed hypersensitivity.

301
Q

Of the disorders of lacrimation, taste, and salivation, which is the first to return after injury to the nervus intermedius

A

Taste.

302
Q

What is a serious complication of lumbar drainage

A

Tension pneumocephalus.

303
Q

Why is skull-based surgery more difficult in elderly patients

A

The dura is more fragile and prone to tearing.

304
Q

What do Keams-Sayre, MELAS, MERRF, and Leber’s hereditary optic neuropathy all have in common

A

They are all mitochondrial disorders with varying degrees of hearing loss.

305
Q

What do Keams-Sayre, MELAS, MERRF, and Leber’s hereditary optic neuropathy all have in common

A

They are all mitochondrial disorders with varying degrees of hearing loss.

306
Q

Why are in-the-ear hearing aids not recommended in patients with tinnitus

A

They can produce too much occlusion effect and amplification of the lower frequencies, resulting in exacerbation of tinnitus.

307
Q

What is the most common route of spread of tumors in the cartilaginous portion of the EAC

A

Through the fissures of Santorini.

308
Q

What operation is performed for tumors that involve the medial aspect of the temporal bone in the region of the petrous apex

A

Total temporal bone resection.

309
Q

Which approach is best in the high-risk surgical patient, regardless of tumor size

A

Trans labyrinthine.

310
Q

What are the three surgical approaches to resection of an acoustic neuroma

A

Translabyrinthine, middle fossa, and retrosigmoid.

311
Q

Which approach offers the best exposure

A

Translabyrinthine.

312
Q

Which approach results in the best facial nerve outcome

A

Translabyrinthine.

313
Q

Which of these fractures is most likely to result in facial nerve paralysis

A

Transverse.

314
Q

Which of these is most likely to occur from a blow to the occiput

A

Transverse.

315
Q

What disease is characterized by lower lid colobomas, downward slanting palpebral fissures, hypoplastic mandible, malformations of the external ear, cleft palate, and hearing loss

A

Treacher Collins.

316
Q

What disease is characterized by lower lid colobomas, downward slanting palpebral fissures, hypoplastic mandible, malformations of the external ear, cleft palate, and hearing loss

A

Treacher Collins.

317
Q

What protein does this gene produce

A

Treacle.

318
Q

What protein does this gene produce

A

Treacle.

319
Q

T/F: 1 in 31 people are carriers for the connexin 26 mutation

A

True.

320
Q

T/F: Superior SCC deformities are always accompanied by lateral SCC deformities

A

True.

321
Q

T/F: Superior SCC deformities are always accompanied by lateral SCC deformities

A

True.

322
Q

T/F: After subtotal temporal bone resection, all patients will have facial nerve paralysis and a dead ear

A

True.

323
Q

T/F: Cranio-aural CSF leaks are more likely to spontaneously close than cranio-nasal CSF leaks

A

True.

324
Q

T/F: The pitch of the tinnitus usually corresponds to the frequency of hearing loss

A

True.

325
Q

T/F: Cochlear implantation has been shown to relieve tinnitus in a large % of profoundly deaf individuals

A

True.

326
Q

Which histologic pattern of adenoid cystic carcinoma has the best prognosis

A

Tubular pattern.

327
Q

Inhalant allergy and anaphylaxis are what type of immune reactions

A

Type I, medi.:1ted by IgE.

328
Q

Which type of neurofibromatosis is characterized by cutaneous neurofibromas

A

Type I.

329
Q

Which type of neurofibromatosis is characterized by cutaneous neurofibromas

A

Type I.

330
Q

Which of these subtypes is primarily found in Norwegians

A

Type III.

331
Q

Which of these subtypes is primarily found in Norwegians

A

Type III.

332
Q

What are the indications for MRI in a patient with tinnitus

A

Unilateral unexplained tinnitus with or without hearing loss; bilateral symmetrical or asymmetrical hearing loss suspicious for retrocochlear etiology (poor discrimination, absent acoustic reflexes, acoustic reflex decay, abnormal ABR).

333
Q

What is the primary disadvantage of fast-spin echo M RI

A

Unlikely to detect other retrocochlear etiologies of SNH L.

334
Q

What is the primary disadvantage of fast-spin echo M RI

A

Unlikely to detect other retrocochlear etiologies of SNHL.

335
Q

What is the most common presentation of tumors of the EAC

A

Unremitting pain and serosanguinous otorrhea.

336
Q

What findings support the inflammatory theory

A

Up to 1/3 of patients report URI symptoms preceding SNHL (Mattox 1977, Jaffe 1973); patients have been shown to seroconvert to a variety of viruses (Wilson et al 1983 ); histologic evidence consistent with viral infection (Schuknecht et al 1973 ).

337
Q

What % of these cases will turn out to have a vestibular schwannoma

A

Up to 4%.

338
Q

What syndrome is characterized by SNHL and retinitis pigmentosa

A

Usher’s syndrome.

339
Q

What syndrome is characterized by SNHL and retinitis pigmentosa

A

Usher’s syndrome.

340
Q

What is the name for the subtype of osteogenesis imperfecta in which progressive hearing loss begins in early childhood

A

Van der Hoeve’s syndrome.

341
Q

What is the name for the subtype of osteogenesis imperfecta in which progressive hearing loss begins in early childhood

A

Van der Hoeve’s syndrome.

342
Q

What treatments are used to try to optimize cochlear blood flow

A

Vasodilators (histamine, papaverine, verapamil, carbon dioxide) and blood thinners ( defibrinogenation therapy, dextran, papaverine).

343
Q

How do lymphocytes responding to antigenic stimulation in the inner ear enter from the systemic circulation

A

Via the spiral modiolar vein.

344
Q

What devices are used in the habituation technique for the treatment of tinnitus

A

Viennatone maskers.

345
Q

What syndrome is characterized by vestibuloauditory symptoms in association with uveitis, depigmentation of periorbital hair and skin, loss of eyelashes, and aseptic meningitis

A

Vogt-Koyanagi-Harada syndrome.

346
Q

What X-linked syndrome is associated with the Klippei-Feil syndrome, SNHL, and cranial nerve VI paralysis

A

W ildervanck syndrome.

347
Q

What syndrome accounts for the most common form of hereditary congenital deafness

A

Waardenburg’s syndrome.

348
Q

What syndrome accounts for the most common form of hereditary congenital deafness

A

Waardenburg’s syndrome.

349
Q

What disease is characterized by necrotizing granulomas with vasculitis in one or more organs and focal necrotizing glomerulonephritis

A

Wegener’s granulomatosis.

350
Q

What is the treatment for II H

A

Weight reduction and acetazolamide (250 mg TID) or furosemide (20 mg BID)~ lumbar-peritoneal shunt for patients with visual deterioration, persistent headaches or disabling tinnitus.

351
Q

What is the most definitive test for AIED

A

Western blot immunoassay to 68 kDa antigen.

352
Q

When is surgical exploration indicated for facial nerve paralysis after gunshot injuries

A

When >90°/o degeneration is documented on ENoG within 14 days of the injury.

353
Q

What X-linked syndrome is associated with the Klippei-Feil syndrome, SNHL, and cranial nerve VI paralysis

A

Wildervanck syndrome.

354
Q

What is Norrie syndrome

A

X-linked disease characterized by blindness, progressive mental retardation, and hearing loss.

355
Q

What is Norrie syndrome

A

X-linked disease characterized by blindness, progressive mental retardation, and hearing loss.

ENT Specialties MAIN (17 decks)

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