Inner Ear Flashcards
1
Q
What % of patients with acoustic neuroma are asymptomatic at presentation
A
0.05
2
Q
What is the incidence of congenital hearing loss
A
0.736111111111111
3
Q
How many words should a child age 24 - 36 months be able to say
A
50
4
Q
How many words should a child age 24 - 36 months be able to say
A
50
5
Q
What is the 5-year survival for SCCA confined to the lateral EAC
A
??
6
Q
What is the definition of sudden SNHL
A
>20d8 hearing loss over at least three contiguous frequencies occurring within 3 days.
7
Q
What % of these are hereditary
A
>60%.
8
Q
What is the incidence of hearing loss after infection with mumps
A
0.5%.
9
Q
What is the incidence of hearing loss after infection with mumps
A
0.5%.
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.
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.
12
Q
What % of patients with unilateral SNHL have an acoustic neuroma
A
1 in 100,000
13
Q
What is the incidence of Waardenburg’s syndrome
A
1 in 4,000 births.
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.
15
Q
What are the 4 clinical subtypes of Waardenburg’s syndrome
A
- 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
Q
What are the 4 clinical subtypes of Waardenburg’s syndrome
A
- 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
Q
What proportion of patients with AIED will not have any vestibular symptoms
A
1/3.
18
Q
What proportion of these patients will have an abnormal ABR
A
1/3.
19
Q
What are the typical inheritance patterns of nonsyndromic hearing loss
A
10 - 20% AD, 75% autosomal recessive (AR), 2 - 3% X-linked,
20
Q
What is the incidence of post-meningitic hearing loss
A
10- 20%.
21
Q
What is the incidence of post-meningitic hearing loss
A
10- 20°/o.
22
Q
In what % of these cases can a definite cause be determined
A
10%.
23
Q
What % of patients with CSF leak secondary to non-surgical trauma will develop meningitis
A
10-25%.
24
Q
What % of basilar skull fractures result in CSF leak
A
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.
