Miscellaneous Flashcards
NF2 is an AD condition caused by a mutation in the ___ gene, and is characterized by:
- ___
- ___
- ___
Merlin (tumor suppressor) gene (c22)
- b/l vestibular schwannomas
- schwannomas of cranial, spinal and cutaneous n. (can be unilateral)
3.cranial and spinal meningiomas
A patient suffers a L-longitudinal temporal bone fracture after falling off a motorcycle. The fracture is otic capsule sparing and travels anterior to the geniculate ganglion. Facial strength is intact. Based on location of his fracture, what is the most likely sign or symptom he will have?
Dry eye.
(Ant to the geniculate ganglion the FN gives off the GSPN, which contains parasympathetic innervation to the lacrimal gland).
___ is the ideal audiologic testing in children 6-30mos of age.
Visual Reinforcement Audiometry.
(test creates a conditioned response to a sound stimulus by playing a visual reward (e.g., toy lights up or moves, etc.)
___ and ___ audiologic tests are useful in screening for hearing loss after a failed newborn hearing screen.
ABR (auditory brain stem response) and DPOAEs (distortion-product otoacoustic emissions).
___ is the audiometric testing of choice in children > 30mos.
Conditioned play audiometry.
(test pairs a sound stimulus w/game playing; e.g. dropping blocks in a bucket)
___ are solitary pedunculated osseous lesions that are smooth and round and originate on the tympanosquamous or tympanomastoidectomy suture line.
Osteomata.
(these contain cancellous bone that has marrow spaces + fibrovascular tissue)
___ are broad-based osseous lesions that occur around the circumference of the medial aspect of the EAC.
Exostoses.
(occur in multiples, often bilateral, and strongly correlated w/exposure to cold water, more difficult to remove than osteomata)
___ is an autoimmune condition that leads to fluctuating SNHL.
Cogan syndrome.
___ and ___ approaches to resection of a vestibular schwannoma preserve hearing.
Retrosigjmoid and Middle cranial fossa approach.
(Retrosig preferred for tumors >3cm)
The ___ approach is the safest for large vestibular schwannomas and has the highest rate of FN preservation.
Translabyrinthine approach.
____ can be offered as Tx for patients w/small vestibular schwannomas (<3 cm), and is intended to halt tumor growth.
Stereotactic radiosurgery.
The ___ approach to resection of vestibular schwannoma is best used for patients w/serviceable hearing and small intracanalicular tumors.
Middle Cranial Fossa.
(allows for good exposure to the IAC, but not the CPA).
What is the best screening audiologic test for an 8mo child?
Visual reinforcement audiometry.
(6mos - 3yrs)
Conventional audiometry cannot be performed until ages ___.
5-6yo.
____ describes a patient’s experience of loudness that increases disproportionally compared to someone with normal hearing.
Loudness recruitment.
____ is the phenomenon in which the perceived loudness of a stimulus on one side (eg. R ear) doubles when presented (both sides) binaurally.
Summation.
Speech Reception Threshold is the ___.
Level at which a pt can repeat back 50% of speech material.
Which inner ear structure terminates at the round window? (See image - P = promontory, Red arrow = round window niche)
Scalia Tympani (lower compartment of the cochlea ends at the round window).
Hearing loss as a result of bacterial meningitis is due to extension of infection from either the:
- _____
- _____
- Subarachnoid space to the scala tympani via a patent cochlear aqueduct
- Inflammation extending to the IAC
___ is the most common malformation of the cochlea.
Incomplete partition (Mondidi malformation).
(cochlea completes only 1-1.5 turns as compared to the normal 2.5 turns).
What is the most common complication of cochlear implantation in children?
Flap infection.
The FN is transected during cochlear implantation surgery. The frayed edges of the nerve are found to be intact. What is the best choice for repair of this FN injury?
Trim the frayed edges of the n and drill out the rest of the facial n canal as needed to relieve tension for reapproximation.
_____ are significant factors in the pathogenesis of OME (and prolonged pain after CI), and promote antimicrobial resistance.
Bacterial biofilms.
The Stenger test is used when a patient is suspected of ___ a hearing loss, stating:
Only the louder of 2 similar tones presented to both ears at the same time will be perceived.
Malingering (Pseudohypoacusis)
Negative Stenger test - patient will continue to respond to the tone when it is presented simultaneously, meaning they are perceiving it in the better ear and the threshold of the poorer ear is true. positive Stenger - patient will not respond to the simultaneous tones as they are perceiving the tone in the ear they are attempting to pass as poorer
A pt w/FN paralysis has obvious weakness and incomplete eye closure. There is normal symmetry at rest. What HB grade does this represent?
IV (incomplete eye closure w/normal symmetry).
A Type A Fisch infratemporal fossa approach involves anterior FN dissection and transposition to access the:
1) ___
2) ___
3) ___
1) jugular foramen (CN IX, X, XI, inf petrosal sinus, sigmoid sinus/IJV junction)
2) vertical ICA
3) infralabyrinthine regions
A Type B Fisch approach includes removing the:
1) ___
2) ___
retraction of the condyle and dissection of the horizontal ICA.
A Type B Fisch approach includes removing the:
1) zygomatic arch
2) temporalis m.
retraction of the condyle and dissection of the horizontal ICA.
* (additionally exposes petrous apex, horizontal ICA, and the clivus)
A Type C Fisch approach removes the ____ to expose the parasellar region, foramen rotundum, and cavernous sinus.
Pterygoid plates
A Type C Fisch approach removes the pterygoid plates to expose the ___, ___, and ___.
1) parasellar region
2) foramen rotundum
3) cavernous sinus.
A patient presenting with uni/bilat vestibular symptoms, uni/bilat SNHL, and uni/bilat interstitial keratitis (causing blurred vision or photophobia) has classic symptoms of _____?
Cogan’s syndrome
15yo has 3 episodes of facial swelling in the last year, particularly around the lip, w/temporary facial weakness. What other exam finding may be present, and what is the Dx?
(fissured tongue)
Melkersson-Rosenthal syndrome.
The Tullio Phenomenon is characterized by:
Vertigo/Nystagmus in response to loud sounds.
Both SSCD and Meneire’s disease demonstrate an ___ SP/AP ratio above 0.4 on ECOG.
Elevated SP/AP ratio.
What is the MOST common cause of neonatal facial paralysis?
Birth trauma.
In the Sunderland nerve injury classification, when does Wallerian degeneration first develop?
Grade 2 (axon is transected and there is subsequent Wallerian degeneration).
Cisplatin ototoxicity is related to the ___ of the patient, and ___ of cisplatin delivered.
- Age of patient (<5yo are more affected)
- Overall dose
Mnemonic “WANT CBS” to remember AD congenital HL inheritance:
W - waardenburg syndrome
A - apert syndrome
N - neurofibromatosis
T - treacher collins
C - crouzon disease
B - branchio-oto-renal syndrome
S - stickler syndrome
TMN Staging for Temporal Bone Neoplasms: (see image)
See image.
TMN Staging for Temporal Bone Neoplasms: (see image)
See image.
TMN Staging for Temporal Bone Neoplasms: (see image)
See image.
TMN Staging for Temporal Bone Neoplasms: (see image)
See image.
Neurofibromas + Flat hyper-pigmented macula’s + pigmented Iris hamartomas.
Diagnosis + chromosome?
NF1 + chromosome 17
___ HL is more common in patients w/vestibular schwannoma, and ___ HL is more common w/FN schwannomas.
Sensorineural (8th CN is not present w/in middle ear space).
Conductive (tympanic segment passes through middle ear space).
CN ___ and ___ palsies are core features of Mobius syndrome.
CN VI and VII.
Usher syndrome is an AR condition presenting with:
1.
2. decreased Vestibular function
3. Retinitis pigmentosa
Usher syndrome is an AR condition presenting with:
- Hearing loss
- decreased Vestibular function
- Retinitis pigmentosa
Usher syndrome is an AR condition presenting with:
- Hearing loss
2. - Retinitis pigmentosa
Usher syndrome is an AR condition presenting with:
- Hearing loss
- decreased Vestibular function
- Retinitis pigmentosa
Usher syndrome is an AR condition presenting with:
- Hearing loss
- decreased Vestibular function
3.
Usher syndrome is an AR condition presenting with:
- Hearing loss
- decreased Vestibular function
- Retinitis pigmentosa
______ disease is associated w/Endolymphatic Sac tumors.
von Hippel-Lindau disease (VHL)
List the CN sensory innervation to the external ear:
C2/C3
V3
X
VII
A painful, tender lesion on the auricle of a middle aged - elderly male w/a h/o irritation to that ear is a classic presentation for _____.
Chondrodermatitis nodularis helix is, or Winkler nodule.
(non-malignant, usually enlarges rapidly to its max size then remains stable).
___ units are a logarithmic measure of a sound relative to a reference level.
Decibel units.
___ level is the # of dB above a person’s threshold and requires a reference level.
Sensation level (SL)
(often used in speech testing to standardize sound to the patient, when they have an underlying HL - E.g. a 40dB HL presented with a 70dB HL stimulus is experiencing a 30dB SL).
What is the STRONGEST predictor of long-term serviceable hearing in patients w/vestibular schwannomas?
Speech discrimination.
The incidence of meningitis in patients w/temporal bone fractures w/o a CSF leak is ___%.
1% (very low).
Approximately _____ of patients w/Ramsay Hunt Syndrome will develop a HB score IV-VI facial palsy.
50%.
Which division(s) of the FN are affected by Ramsay-Hung Syndrome?
Motor and sensory.
What nerve provides secretomotor salivation (parasympathetic fibers) to the parotid gland?
Auriculotemporal (CN V3).
The tympanic plexus is composed of which nerves?
- Internal carotid plexus
- Tympanic n. (Jacobson’s n.)
Audiologic eval should be performed for patients w/unilateral tinnitus, or persistent tinnitus for greater than ____.
6 months.
In patients w/penetrating ear trauma, middle ear expoloration is indicated for:
1.
2. Progressive SNHL
3. Radiographic evidence of perilymphatic fistula or meningitis
In patients w/penetrating ear trauma, middle ear expoloration is indicated for:
- Intractable vertigo
- Progressive SNHL
- Radiographic evidence of perilymphatic fistula or meningitis
In patients w/penetrating ear trauma, middle ear expoloration is indicated for:
- Intractable vertigo
2. - Radiographic evidence of perilymphatic fistula or meningitis
In patients w/penetrating ear trauma, middle ear expoloration is indicated for:
- Intractable vertigo
- Progressive SNHL
- Radiographic evidence of perilymphatic fistula or meningitis
In patients w/penetrating ear trauma, middle ear expoloration is indicated for:
- Intractable vertigo
- Progressive SNHL
3.
In patients w/penetrating ear trauma, middle ear expoloration is indicated for:
- Intractable vertigo
- Progressive SNHL
- Radiographic evidence of perilymphatic fistula or meningitis
The loss of gain resulting from impedance mismatching between the air in the ear canal and fluid in the cochlea is about ___dB.
30dB.
___ is the most common infectious cause of congenital hearing loss?
CMV
The addition of a ___ to a fluoroquinolone otic agent slightly shortens the symptomatic period of otitis externa.
Steroid.
What is the most likely type of extra-axial CPA tumor in pediatric patients?
Vestibular schwannoma.
(Meningiomas and Epidermoids are also most common)
ENoG is of most value btwn ___ after onset of complete paralysis.
3-14 days.
A red hue (blush) seen on the promontory, is called…?
Schwartze sign.
_____ is a phenomenon in which patients w/HL experience a rapid increase in subjective loudness of a stimulus.
Loudness recruitment.
Hyperacusis is the presence of ___ w/sound stimuli, that others would only experience as being loud.
Discomfort w/sound stimuli.
____ is a phenomenon where individuals w/CHL (eg, otosclerosis) experience enhanced ability to understand speech in the presence of background noise.
Paracusis Willisii
____ is the subjective perception of more than 1 pitch in response to the same stimulus.
Diplacusis.
_____ is a phenomenon in which a sound stimulus presented to the test ear directly stimulates the contralateral (non-test) ear.
Crossover.
(B/c sound has to travel through skull, there is some degree of interaural attenuation, approx 40dB w/standard circumaural earphones).
Masking should be instituted whenever the air-conduction threshold in the test ear exceeds the non-test ear by ___dB for circumaural earphones or >70 dB for insert earphones.
Masking should be instituted whenever the air-conduction threshold in the test ear exceeds the non-test ear by 40_dB for circumaural earphones or >70 dB for insert earphones.
A patient with hypermobile joints, HL, cleft palate, and a flattened midface will likely be diagnosed with ___.
Stickler syndrome.
Delayed facial weakness following vestib schwannoma surgery carries a ___ prognosis than immediate facial weakness.
Delayed facial weakness following vestib schwannoma surgery carries a more favorable prognosis than immediate facial weakness (most patients acheive normal facial function)
Primary advantages of the Translab. approach:
1. reduced _____
2. direct access to the IAC and CPA
3. quick FN identification both in the mastoid segment + labyrinthine segment
Primary advantages of the Translab. approach:
1. reduced cerebellar retraction
2. direct access to the IAC and CPA
3. quick FN identification both in the mastoid segment + labyrinthine segment
Primary advantages of the Translab. approach:
1. reduced cerebellar retraction
2. direct access to the ___ and ___
3. quick FN identification both in the mastoid segment + labyrinthine segment
Primary advantages of the Translab. approach:
1. reduced cerebellar retraction
2. direct access to the IAC and CPA
3. quick FN identification both in the mastoid segment + labyrinthine segment
Primary advantages of the Translab. approach:
1. reduced cerebellar retraction
2. direct access to the IAC and CPA
3. quick ___ identification
Primary advantages of the Translab. approach:
1. reduced cerebellar retraction
2. direct access to the IAC and CPA
3. quick FN identification both in the mastoid segment + labyrinthine segment
Long term QOL outcomes in vestibular schwannoma patients show ___ differences btwn. stereotactic radiation, observation, and microsurgical intervention.
Long term QOL outcomes in vestibular schwannoma patients show no significant differences btwn. stereotactic radiation, observation, and microsurgical intervention.
