Term
A patient presents with type A tympanograms, normal ABRs, present acoustc reflexes, speech perception in accord with AI estimates regarding their hearing loss, and absent OAEs. What best describes the patient's auditory dysfunction? |
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Definition
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Term
A patient presents with type A tympanograms, no latency-intensity function from the ABR, absentor elevated acoustic reflexes, present OAEs, and speech perception poorer than would estimate via AI regarding their hearing loss. What best describes the patient's auditory dysfuntion? |
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Definition
Inner Hair Cell/auditory nerve loss |
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Term
Regarding imaging techniques, what is the best for obtaining detail of bone and what is best for obtaining soft tissue detail. |
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Definition
CT is best for bone.
MRI is best for soft tissue. |
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Term
What is the "gold standard" test for identifying vestibular schwannomas? |
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Definition
T-1 weighted MRI with gadolinium contrast |
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Term
What imaging techniques can provide physiologic information? |
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Definition
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Term
What research designs provides the best evidence for causality? |
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Definition
Randomized clinical trial |
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Term
A mutation in the gene SLC26A4 is responsile for what? |
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Definition
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Term
Using wide-band reflectance, a score of "0" means what and a score of "1" means what? |
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Definition
"0" means complete absorption
"1" means complete reflectance |
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Term
What syndrome includes craniofacial anomalies including maldevelopment of the maxilla and mandible, abnormal canthi, choanal atresia, and conductive hearing loss? |
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Definition
Treacher-Collins syndrome |
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Term
How long does it take for thalamic axons to mature? |
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Definition
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Term
In the case of two parents being heterozygous for a recessive gene for hearing loss (and assuming that they have the same recessive gene), what is the likelihood in each pregnancy of having a child with a hearing loss? |
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Definition
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Term
In the case of two parents being heterozygous for a recessive gene for hearing loss (and assuming that they have different recessive genes), what is the likelihood in each pregnancy of having a child with a hearing loss? |
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Definition
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Term
What syndromes may have progressive SNHL? |
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Definition
NF2, Stickler syndrome, & Usher syndrome |
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Term
In the case of X-linked dominant inheritance where only the dad has the affected gene, how many of his sons will express the disorder? |
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Definition
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Term
What are the 5 signs that are examined regarding ears during an ENT evaluation? |
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Definition
1. hearing loss
2. otalgia
3. otorrhea
4. tinnitus
5. dizziness/vertigo |
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Term
Why is CHARGE now referred to as a syndrome instead of a genetic association? |
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Definition
A mutation to the CDH7 gene on chromosome 8 is a common causal factor for CHARGE. |
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Term
What are some possible advantages of wide-band reflectance use in screening? |
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Definition
Allow for a more acurate assessment of the auditory system.
Allows for easier assessment of ME effusion
Can assess a wide frequency range
Assesses ME, inner ear, 8th neve |
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Term
What could be possible effects of a transplacental cytomegalovirus infection occuring during the first timester of fetal development? |
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Definition
Malformations of the inner ear
Microcephaly
Malformations of the auditory pathways in the brainstem |
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Term
Can an infant with asymptomiatic CMV at birth develop a hearing loss and/or mental retardation? |
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Definition
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Term
Maturation of the thalamic axons and intrinsic cortical axons likely affects what aspects of auditory system function? |
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Definition
Affect the cortex because they both are important for normal cortical function like auditory processing |
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Term
What is the difference between a syndrome and a sequence? |
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Definition
A syndrome is the presence of multiple anomalies from a single cause present in an indivudal whiela sequence is multiple anomalies without a known cause. |
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Term
The Eustachian tube embryologically is derived from... |
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Definition
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Term
The principal dilator of the Eustachian tube is |
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Definition
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Term
Which of the following would be characteristic of otitis media with effusion (OME)? |
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Definition
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Term
Which is the most likely sequence of events in the development of otitis media? |
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Definition
Eustachian tube closes, oxygen absorbed, creation of vacuum, fluid secreted |
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Term
Which of the following is a likely factor influencing otitis media? |
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Definition
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Term
Which of the following is a vaccine currently recommended by the American Academy of Pediatrics for otitis media? |
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Definition
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Term
Which of the following is now the usual recommendation for children with nonsevere AOM who are over the age of 2? |
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Definition
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Term
Children with OME should have their hearing tested at least how often? |
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Definition
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Term
Which of the following syndromes includes congenital hearing loss and a prolonged Q-T interval? |
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Definition
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Term
Which of the following syndromes is responsible for half of the cases of deaf-blindess in the US? |
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Definition
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Term
A cholesteatoma can possibly result from |
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Definition
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Term
The hearing loss associated with cholesteatoma is |
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Definition
Could be any of the above |
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Term
Carhart's notch is caused by |
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Definition
Restriction of inertial bone conduction path |
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Term
Which of the following is characterized by autosomal dominant hereditary pattern and spongy bone growth? |
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Definition
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Term
In its normal resting state, is the Eustachian tube open or closed? |
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Definition
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Term
What are the three functions of the Eustachian tube? |
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Definition
1. Pressure equalization
2. Mucociliary clearance
3. Middle ear protection |
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Term
Besides the classic theory regarding Eustachian tube dysfunction and OM, what are two other ways that ET dysfunction may result in OM? |
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Definition
1. A ET that stays open too often
2. Disturbance of mucosal cleaning |
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Term
What might you suspect if you saw a long-standing case of unilateral serous otitis media in an adult? What should you do? |
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Definition
Usually sign of lesion (neoplasm) in nasopharynx. Should refer to ENT or PCP |
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Term
Name one intratemporal and one intracranial complication of chronic OM |
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Definition
Intratemporal: hearing loss
Intracranial: meningitis |
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Term
What is an excellent, non-surgical treatment for conductive hearing loss resulting from atresia, Treacher-Collins syndrome, and a third option for otosclerosis? |
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Definition
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Term
What is a type As tympanogram indicative of? |
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Definition
Ossicular fixation (Ex: otosclerosis, tympanosclerosis, congenital stapes fixation, osteogenesis imperfecta) |
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Term
What might be a likely etiology of a congenital air-bone gap that included both low and high frequencies? |
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Definition
Superior semi-circular canal dehiscence syndrome or large vestibular aqueduct |
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Term
A patient with pulsatile tinnitus and a conductive hearing loss will have what kind of etiology? |
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Definition
Glomus jugulare tumor etiology. Refer to MRI or contrast CT |
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Term
If adult patient has bilateral otitis media that is surprisingly resistant to antibiotic Rx, what might you suspect? |
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Definition
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Term
The membranous labyrinth of the inner ear has its typical shape by... |
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Definition
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Term
Classic Mondini "dysplasia" specifically refers to... |
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Definition
Cochlea with only 1.5 turns |
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Term
General rules of thumb regarding malformations of the osseous and membranous labyrinths are: The ________ the interruption, the _________ the deformity and the _________ the hearing. |
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Definition
General rules of thumb regarding malformations of the osseous and membranous labyrinths are: The earlier the interruption, the more severe the deformity and the worse the hearing. |
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Term
Which of the following may initially present as bilateral otitis media resistant to antibiotic treatment? |
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Definition
Langerhans cell histiocytosis |
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Term
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Definition
is of little clinical value. |
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Term
After the inflammatory stage of bacterial labyrinthitis or meningitis, which of the following may likely occur? |
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Definition
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Term
When do most congenital inner ear malformations occur? |
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Definition
4th-8th week of gestation |
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Term
Which of the following disorders may have otologic characteristics virtually the same as Meniere's disease? |
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Definition
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Term
Which of the following can easily be identified using CT or MRI imaging? |
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Definition
osseous labyrinth malformation |
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Term
Which of the following might present with uveitis, cough, possible heart failure, and SNHL? |
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Definition
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Term
Systemic diseases that affect the temporal bone usually present with... |
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Definition
conductive hearing loss, sensorineural hearing loss, mixed hearing loss
answer: any of the above hearing losses |
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Term
Which of the following treatments for vertigo in Meniere's disease has the greater risk for hearing loss? |
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Definition
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Term
Which of the following syndromes typically has an inner ear malformation? |
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Definition
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Term
Which of the following describes incomplete development? |
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Definition
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Term
About how many children with congenital inner ear malformation have malformation of both the osseous and membranous labyrinth? |
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Definition
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Term
Which of the following is commonly found in children with a congenital inner ear malformation? |
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Definition
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Term
Noice-induced oxidative stress leading to cochlear injury may involve what 3 mechanisms? |
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Definition
impaired mitochondrial function, glutamate-induced excitotoxicity, & depletion of antioxidants, such as glutathione |
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Term
With sudden SNHL, what 2 factors yield a more positive prognosis? |
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Definition
younger age and less sensitivity of hearing loss |
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Term
What might a narrow internal auditory canal have to do with cochlear implantation? |
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Definition
The 8th nerve may be insufficiently developed and after cochlear implantation pt's have experienced facial pain, but no useful auditory function. |
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Term
What is an etiology of congenital unilateral profound SNHL? |
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Definition
Unilateral cochlear nerve aplasia |
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Term
A child with an inner ear malformation has much better auditory function than expected based on the PTA. What might be the source of this better-than-expected function? |
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Definition
ultra-high frequency hearing |
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Term
Hearing sensitivity in children with large vestibular aqueduct syndrome is... |
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Definition
usually normal or mild hearing loss w/ progressive hearing loss. Hearing sensitivity varies across individuals although 40% eventually have profound SNHL. |
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Term
Do you think there is a genetic susceptibility for NIHL in humans? |
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Definition
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Term
What do you think might account for the variability seen across patients with Meniere's symptoms? |
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Definition
Multifactorial; common end point for a variety of injuries or anatomical variables. |
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Term
What is true regarding autoimmune inner ear disease? |
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Definition
May recover within a 6-12 month period |
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Term
Ototoxicity of aminoglycoside antibiotics may be reduced when they are administered in conjunction with what? |
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Definition
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Term
In the case of a temporal bone fracture, presence of a conductive hearing loss after hemotympanum resolution likely means what? |
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Definition
ossicular chain disruption |
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Term
With regard to temporal bone fractures, a fracture to ______ capsule usually results in what? |
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Definition
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Term
What is the most likely result of the actual aging process? |
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Definition
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Term
Patient A, a 55 year old adult, presents with an asymmetric hearing loss. What should be suspected? |
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Definition
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Term
One finding in auditory neuropathy/dys-synchrony is that speech recognition is what? |
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Definition
worse than predicted based on the AI |
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Term
What SNHL etiology is characterized by CNS demyelination and relapses/remissions? |
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Definition
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Term
What causes large amounts of antibody or immune complexes to be produced in the blood and thereby cause ischemia and SNHL? |
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Definition
Leukemia, lymphomas, sickle cell anemia |
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Term
What is not possible using ENoG? |
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Definition
Degree of facial nerve degeneration |
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Term
An adult patient presents with unilateral tinnitus. What might you suspect? |
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Definition
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Term
What disorder likely presents with vertigo lasting for days? |
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Definition
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Term
What usually presents with a fluctuating hearing loss? |
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Definition
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Term
What tinnitus therapies is based on psychological techniques for treating phobias? |
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Definition
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Term
Progressive facial paralysis is likely ... |
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Definition
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Term
What is NOT likely in cases of auditory neuropathy/dys-synchrony? |
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Definition
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Term
Dysfunction of what two organs will likely potentiate the ototoxic effects of meds, particularly aminoglycoside antibiotics? |
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Definition
Liver and kidney dysfunction |
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Term
What are congenital fistulas usually associated with? What is a big risk? |
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Definition
Patients with inner ear malformations. They communicate with subarachnoid space, which leads to CSF leaks and meningitis |
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Term
What would likely happen if an aminoglycoside solution was placed into a healthy middle ear? |
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Definition
Most likely result in cochlear and vestibular toxicity |
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Term
How might auditory cortical reorganization lead to tinnitius? |
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Definition
Over representation of frequencies on the border of the damages cochlear region. Neurons that develop self perpetuating activity and areas of over representation may be too strong to be cancelled by inhibitory process. |
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Term
When a patient presents with a unilateral or asymmetric hearing loss, what etiology should be suspected? |
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Definition
Vestibular schwannoma or neoplasm |
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Term
What are three possible pathologic causes for auditory neuropathy/dyssynchrony? |
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Definition
1. dysfunction of IHCs
2. dysfunction of IHC/afferent nerve synapse
3. dysfunction of auditory nerve itself |
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Term
Is hearing loss a requisite for tinnitus to occur? |
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Definition
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Term
"Neoplasms of the CPA is always present with high-frequency hearing loss." True or false? Why or why not? |
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Definition
False. Growth could occur in the center or inside of 8th nerve. Would affect low or middle frequencies. Can result in any audiometric pattern, but typically high frequency HL. |
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