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Flashcards in Inner Ear Deck (355):
1

What % of patients with acoustic neuroma are asymptomatic at presentation

0.05

2

What is the incidence of congenital hearing loss

0.736111111111111

3

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

50

4

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

50

5

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

??

6

What is the definition of sudden SNHL

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

7

What % of these are hereditary

>60%.

8

What is the incidence of hearing loss after infection with mumps

0.5%.

9

What is the incidence of hearing loss after infection with mumps

0.5%.

10

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

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

11

What % of children with congenital CMV have hearing loss

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

12

What % of patients with unilateral SNHL have an acoustic neuroma

1 in 100,000

13

What is the incidence of Waardenburg's syndrome

1 in 4,000 births.

14

What % of children with congenital CMV have hearing loss

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

15

What are the 4 clinical subtypes of Waardenburg's syndrome

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

16

What are the 4 clinical subtypes of Waardenburg's syndrome

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

17

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

1/3.

18

What proportion of these patients will have an abnormal ABR

1/3.

19

What are the typical inheritance patterns of nonsyndromic hearing loss

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

20

What is the incidence of post-meningitic hearing loss

10- 20%.

21

What is the incidence of post-meningitic hearing loss

10- 20°/o.

22

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

10%.

23

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

10-25%.

24

What % of basilar skull fractures result in CSF leak

10-30%.

25

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

111 oooth

26

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

15- 20°/o.

27

What % of skull fractures involve the temporal bone

18%.

28

How small of a lesion can M Rl with gadolinium detect

2 mm.

29

How small of a lesion can MRI with gadolinium detect

2 mm.

30

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

20 - 40%.

31

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

20- 25°/o.

32

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

27%.

33

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

29%.

34

What % of CSF leaks are from non-traumatic causes

3- 4%.

35

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

30 - 40%.

36

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

30 - 40%.

37

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

30 - 50%.

38

What % the hereditary cases are syndromic

30%.

39

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

36 months.

40

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

36 months.

41

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

40- 50%.

42

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

40% risk.

43

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

40% risk.

44

What % of patients with RA have SNHL

44%.

45

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

4-5 mm.

46

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

4-5 mm.

47

What % of patients with neurofibromatosis type 1 have acoustic neuromas

5% and usually unilateral.

48

What % of patients with neurofibromatosis type 1 have acoustic neuromas

5% and usually unilateral.

49

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

50%.

50

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

50%.

51

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

58-64%.

52

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

6 to I 0 months.

53

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

6 to I 0 months.

54

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

60-70%

55

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

78%.

56

What % of these patients have hearing loss

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

57

What % of these patients have hearing loss

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

58

What % of CSF leaks are cranio-nasal

80%.

59

What % of patients with neurofibromatosis type 2 have acoustic neuromas

95% and usually bilateral.

60

What % of patients with neurofibromatosis type 2 have acoustic neuromas

95°/o and usually bilateral.

61

What is the reservoir sign

A rush of clear rhinorrhea occurs with sudden upright position.

62

What is the basic defect of this disease

Abnormal fibroblast growth factor (FGF) receptors.

63

What is the basic defect of this disease

Abnormal fibroblast growth factor (FGF) receptors.

64

What is the most common toxicity of methotrexate

Abnormal liver function.

65

What is the basic defect causing this syndrome

Abnormal potassium channels.

66

What is the basic defect causing this syndrome

Abnormal potassium channels.

67

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

Adenoid cystic carcinoma.

68

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

Alexander.

69

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

Along the tympanosquamous suture line (posterior and superior).

70

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

Aminoglycosides.

71

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

Aminoglycosides.

72

What is temporal bone myospherulosis

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

73

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

Anterior to the otic capsule.

74

What is the mechanism of type II immune reactions

Antibodies directed against a specific antigen within tissues activate complement.

75

What cell patterns are characteristic of acoustic neuromas

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

76

In which pattern are Verocay's bodies found

Antoni A.

77

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

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

78

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

At least 3 months after injury.

79

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

Atherosclerotic carotid artery disease.

80

What is the typical inheritance pattern of syndromic hearing loss

Autosomal dominant (AD).

81

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

Autosomal dominant.

82

What is the inheritance pattern of this disease

Autosomal dominant.

83

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

Autosomal dominant.

84

What is the inheritance pattern of this disease

Autosomal dominant.

85

What feature seen on electron microscopy is pathognomonic for Alport syndrome

Basket-weave configuration of the glomerular basement membrane.

86

What feature seen on electron microscopy is pathognomonic for Alport syndrome

Basket-weave configuration of the glomerular basement membrane.

87

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

Beh9et's dis•ease.

88

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

Behind-the-ear hearing aid.

89

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

Beta-2-transferrin assay.

90

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

Beta-2-transferrin.

91

What sort of hearing loss is typical after meningitis

Bilateral, severe to profound, and pennanent.

92

What sort of hearing loss is typical after meningitis

Bilateral, severe to profound, and permanent.

93

What is the habituation technique for the treatment of tinnitus

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%).

94

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

BPPV.

95

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

Branchio-oto-renal.

96

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

Branchio-oto-renal.

97

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

Breast, lung, and kidney.

98

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

Bullet trajectory lateral to the middle ear cavity.

99

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

c-ANCA.

100

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

Carbon dioxide.

101

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

Circulatory disturbance and inflammatory reaction (usually viral).

102

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

CMV.

103

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

CMV.

104

What laboratory studies are useful in the workup

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

105

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

Cogan's syndrome.

106

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

Combined transmastoid/middle fossa approach.

107

What approach is most often used for longitudinal fractures

Combined transmastoid/middle fossa.

108

Where do most basal cell carcinomas of the EAC arise

Concha.

109

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

Cookie-bite pattern.

110

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

Cookie-bite pattern.

111

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

Crouzon' s disease.

112

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

Crouzon's disease.

113

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

CT scan of the temporal bones.

114

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

CT scan.

115

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

CT scan.

116

What is residual inhibition

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

117

What is the mechanism of type Ill immune reactions

Deposition of immune complexes in the microcirculation.

118

What is the primary disadvantage of the translabyrinthine approach

Destroys hearing permanently.

119

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

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

120

What structures are resected in a subtotal temporal bone resection

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

121

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

Edema and intraneural hemorrhage.

122

What are the characteristics of CSF in the presence of meningitis

Elevated protein, WBC, and pressure~ decreased glucose.

123

What is the medical management of CSF leak

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

124

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

Endolymphatic sac.

125

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

Enlarged vestibular aqueduct.

126

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

Enlarged vestibular aqueduct.

127

How is the diagnosis of IIH syndrome made

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

128

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

External auditory canal (EAC).

129

What gene is responsible for this syndrome

EY A I on chromosome 8q I 3.3.

130

What gene is responsible for this syndrome

EY A I on chromosome 8q I3.3.

131

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

False.

132

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

False.

133

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

False.

134

T/F: Absence of papilledema excludes IIH syndrome

False.

135

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

False; outcome is independent of cell proportions.

136

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

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

137

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

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

138

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

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.

139

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

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.

140

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

Fast-spin echo MRI.

141

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

Fast-spin echo MRI.

142

What is the recommended treatment for AIED

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

143

What findings support the circulatory theory

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

What evidence supports the use of carbon dioxide for ISSNHL

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

When is surgical exploration indicated after temporal bone fracture

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

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

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

147

What is the function of connexin 26

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

148

What are the only antigen-specific tests for syphilis

FTA-ABS and MHA-TP.

149

What are the only antigen-specific tests for syphilis

FTA-ABS and MHA-TP.

150

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

Haemophilus injluenzae, Neisseria meningitides, Streptococcus pneumoniae.

151

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

Haemophilus injluenzae, Neisseria meningitides, Streptococcus pneumoniae.

152

What signs on physical exam are suggestive of CSF leak

Halo sign and reservoir sign.

153

How does hearing loss caused by mumps usually present

Hearing loss develops as the parotitis is resolving.

154

How does hearing loss caused by mumps usually present

Hearing loss develops as the parotitis is resolving.

155

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

Hearing loss.

156

The 68 kDa antigen is thought to represent what protein

Heat shock protein 70 (hsp 70).

157

What are the most common injuries encountered on surgical exploration

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

158

What are the potential side effects of cyclophosphamide

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

159

What are the 2 types of non-traumatic CSF leaks

High pressure and normal pressure.

160

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

High-frequency unilateral SNH L.

161

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

High-frequency unilateral SNHL.

162

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

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

163

What are the 3 clinical subtypes of Usher's syndrome

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

What are the 3 clinical subtypes of Usher's syndrome

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

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

I 0%.

166

What is the incidence of Waardenburg's syndrome

I in 4,000 births.

167

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

I OO%.

168

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

I OO%.

169

How is congenital CMV diagnosed in the newborn

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

170

How is congenital CMV diagnosed in the newborn

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

171

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

Idiopathic intracranial hypertension (IIH) syndrome.

172

What is the most common cause of venous pulsatile tinnitus

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

173

When is spontaneous recovery of hearing more likely

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

When is a middle ear exploration indicated

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

175

What is the predominant immunoglobulin in the endolymphatic sac

IgA.

176

What % of patients with unilateral tinnitus have retrocochlear pathology

II %.

177

What % of patients with unilateral tinnitus have retrocochlear pathology

II %.

178

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

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

179

What is the most common mechanism of CHL in longitudinal fractures

Incudostapedial joint dislocation.

180

What is the mechanism of action of methotrexate

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

181

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

Insomnia.

182

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

Internal auditory canal.

183

What manifestation of congenital syphilis is most commonly related to SNHL

Interstitial keratitis.

184

What manifestation of congenital syphilis is most commonly related to SNHL

Interstitial keratitis.

185

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

Intracranial injuries (53%).

186

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

Is the carotid artery or brain involved

187

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

Isolated lateral semicircular canal defects.

188

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

Isolated lateral semicircular canal defects.

189

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

It produces aflatoxin 8, a known carcinogen.

190

What are 5 other venous etiologies of pulsatile tinnitus

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

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

Lateral temporal bone resection.

192

What is the typical severity and pattern of AD hearing loss

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

193

What is the predominant immunoglobulin in the perilymph

lgG.

194

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

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

195

Which of these is most common

Longitudinal (80 - 90°/o).

196

What are the 3 types of temporal bone fractures

Longitudinal, transverse, and mixed.

197

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

Longitudinal.

198

Which of these accounts for the majority of facial nerve injuries

Longitudinal.

199

What is an early sign of relapse following treatment

Loud tinnitus.

200

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

Low frequency.

201

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

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

202

What laboratory tests can be used to diagnose CSF leak

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

203

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

Michel aplasia.

204

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

Michel aplasia.

205

Which approach is best in patients with tumors

Middle fossa.

206

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

Mondini aplasia and enlarged vestibular aqueduct.

207

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

Mondini aplasia and enlarged vestibular aqueduct.

208

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

Mondini aplasia.

209

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

Mumps.

210

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

Mumps.

211

What genetic mutation is responsible for Treacher Collins syndrome

Mutation of TCOF I on chromosome Sq.

212

What genetic mutation is responsible for Treacher Collins syndrome

Mutation of TCOF I on chromosome Sq.

213

What is the basic defect causing Alport syndrome

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

214

What is the basic defect causing Alport syndrome

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

215

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

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

216

What genetic mutation is responsible for neurofibromatosis type 1

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

217

What genetic mutation is responsible for neurofibromatosis type 1

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

218

What genetic mutation is responsible for neurofibromatosis type 2

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

219

What genetic mutation is responsible for neurofibromatosis type 2

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

220

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

Mutation of the PAX3 gene on chromosome 2q37.

221

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

Mutation of the PAX3 gene on chromosome 2q37.

222

What genetic mutations are responsible for most cases of Stickler syndrome

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

223

What genetic mutations are responsible for most cases of Stickler syndrome

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

224

What genetic mutations are thought to be responsible for osteogenesis imperfecta

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

225

What genetic mutations are thought to be responsible for osteogenesis imperfecta

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

226

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

Neck dissection, parotidectomy, and occasionally, partial mandibulectomy.

227

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

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

228

What nerve is involved in paroxysmal lacrimation

Nervus intermedius.

229

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

Neurofibromatosis type 2.

230

What evidence supports the use of antivirals for ISSN H L

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

231

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

Noise exposure.

232

What are the 2 main categories of tinnitus

Non-pulsatile and pulsatile.

233

Which is more common

Non-pulsatile.

234

What is the most common cause of CSF leak

Non-surgical trauma.

235

What is the treatment for relapsing polychondritis

NSAI Ds, steroids, dapsone.

236

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

Obliquely oriented fractures.

237

Is the facial nerve sacrificed during lateral temporal bone resection

Only if it is involved with tumor.

238

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

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

239

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

Otopalatodigital syndrome.

240

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

Otopalatodigital syndrome.

241

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

Oval window (especially in patients with Mondini dysplasia).

242

WhaK is the prognosis of I SSN H L

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

243

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

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

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

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

What factors lead to the best rate of recovery after ISSNHL

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

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

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

247

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

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

248

Mutation of what gene is associated with enlarged vestibular aqueduct

Pendrin on chromosome 7 q3 I.

249

What test assists in the diagnosis of Pendred syndrome

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

250

What test assists in the diagnosis of Pendred syndrome

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

251

Which part of the facial nerve is most often involved

Perigeniculate area.

252

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

Petrous apex.

253

What are the typical symptoms of interstitial keratitis

Photophobia, lacrimation, pain.

254

What auditory tests are performed in tinnitus analysis

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

255

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

Polyarteritis nodosa.

256

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

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

Which of these is associated with meningitis

Preformed bony pathway around the bony labyrinth.

258

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

Prelingual and more clinically diverse hearing loss.

259

How does the defect caused by arachnoid granulations usually present

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

260

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

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

261

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

Progressive to total deafness.

262

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

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

263

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

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

264

What other tests can be used to diagnose CSF leak

Radionuclide cisternography, CT cistemography, intrathecal fluorescein.

265

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

Ramsey-Hunt syndrome.

266

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

Ramsey-Hunt syndrome.

267

What physical exam findings are classic for measles

Rash, conjunctivitis, and Koplik 's spots.

268

What physical exam findings are classic for measles

Rash, conjunctivitis, and Koplik's spots.

269

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

Retrosigmoid.

270

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

Rhabdomyosarcoma, chondrosarcoma, and osteosarcoma.

271

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

Rheumatoid factor.

272

What is the most common form of inner ear aplasia

Scheibe aplasia ( cochleosaccular dysplasia or pars inferior dysplasia).

273

What temporal bone malformation is classic for rubella

Scheibe malformation.

274

What temporal bone malformation is classic for rubella

Scheibe malformation.

275

What evidence refutes the circulatory theory

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

276

What is the differential diagnosis of a CPA tumor

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

277

What are the clinical features of Alport syndrome

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

278

What are the clinical features of Alport syndrome

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

279

What is the most common otologic manifestation of Wegener's

Serous otitis media.

280

What is dystopia canthorum

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

281

What is dystopia canthorum

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

282

What factor is most related to hearing outcome after surgery

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

283

What other test can be useful in diagnosing Alport syndrome

Skin biopsy.

284

What other test can be useful in diagnosing Alport syndrome

Skin biopsy.

285

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

Sleeve resection.

286

What are the 3 classic findings of congenital rubella syndrome

SNHL, cataracts, heart malformations.

287

What are the 3 classic findings of congenital rubella syndrome

SNHL, cataracts, heart malformations.

288

What are the most common complications of acoustic neuroma resection

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

289

Which pattern has the worst prognosis

Solid patten1.

290

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

Squamous cell carcinoma (SCCA).

291

What histologic finding distinguishes cholesteatoma from cholesterol granuloma

Squamous epithelium is only present in cholesteatomas.

292

What evidence supports the use of corticosteroids for ISSNHL

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

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

Stickler.

294

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

Stickler.

295

Which organism most commonly causes post-meningitic hearing loss

Streptococcus pneumoniae.

296

Which organism most commonly causes post-meningitic hearing loss

Streptococcus pneumoniae.

297

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

Subtotal temporal bone resection.

298

Which semicircular canal forms first? Last

Superior canal forms first; lateral canal forms last.

299

Which semicircular canal forms first? Last

Superior canal forms first; lateral canal forms last.

300

What is the mechanism of type IV immune reactions

T -cell mediated delayed hypersensitivity.

301

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

Taste.

302

What is a serious complication of lumbar drainage

Tension pneumocephalus.

303

Why is skull-based surgery more difficult in elderly patients

The dura is more fragile and prone to tearing.

304

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

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

305

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

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

306

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

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

307

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

Through the fissures of Santorini.

308

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

Total temporal bone resection.

309

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

Trans labyrinthine.

310

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

Translabyrinthine, middle fossa, and retrosigmoid.

311

Which approach offers the best exposure

Translabyrinthine.

312

Which approach results in the best facial nerve outcome

Translabyrinthine.

313

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

Transverse.

314

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

Transverse.

315

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

Treacher Collins.

316

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

Treacher Collins.

317

What protein does this gene produce

Treacle.

318

What protein does this gene produce

Treacle.

319

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

True.

320

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

True.

321

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

True.

322

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

True.

323

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

True.

324

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

True.

325

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

True.

326

Which histologic pattern of adenoid cystic carcinoma has the best prognosis

Tubular pattern.

327

Inhalant allergy and anaphylaxis are what type of immune reactions

Type I, medi.:1ted by IgE.

328

Which type of neurofibromatosis is characterized by cutaneous neurofibromas

Type I.

329

Which type of neurofibromatosis is characterized by cutaneous neurofibromas

Type I.

330

Which of these subtypes is primarily found in Norwegians

Type III.

331

Which of these subtypes is primarily found in Norwegians

Type III.

332

What are the indications for MRI in a patient with tinnitus

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

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

Unlikely to detect other retrocochlear etiologies of SNH L.

334

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

Unlikely to detect other retrocochlear etiologies of SNHL.

335

What is the most common presentation of tumors of the EAC

Unremitting pain and serosanguinous otorrhea.

336

What findings support the inflammatory theory

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

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

Up to 4%.

338

What syndrome is characterized by SNHL and retinitis pigmentosa

Usher's syndrome.

339

What syndrome is characterized by SNHL and retinitis pigmentosa

Usher's syndrome.

340

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

Van der Hoeve's syndrome.

341

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

Van der Hoeve's syndrome.

342

What treatments are used to try to optimize cochlear blood flow

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

343

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

Via the spiral modiolar vein.

344

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

Viennatone maskers.

345

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

Vogt-Koyanagi-Harada syndrome.

346

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

W ildervanck syndrome.

347

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

Waardenburg's syndrome.

348

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

Waardenburg's syndrome.

349

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

Wegener's granulomatosis.

350

What is the treatment for II H

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

What is the most definitive test for AIED

Western blot immunoassay to 68 kDa antigen.

352

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

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

353

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

Wildervanck syndrome.

354

What is Norrie syndrome

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

355

What is Norrie syndrome

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