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Hearing Disorders
Hearing Disorders Test Questions
93
Audiology
Graduate
08/06/2014

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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?
Definition

Outer Hair Cell loss

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?
Definition
Inner Hair Cell/auditory nerve loss
Term
Regarding imaging techniques, what is the best for obtaining detail of bone and what is best for obtaining soft tissue detail.
Definition

CT is best for bone.

MRI is best for soft tissue.

Term
What is the "gold standard" test for identifying vestibular schwannomas?
Definition
T-1 weighted MRI with gadolinium contrast
Term
What imaging techniques can provide physiologic information?
Definition
PET
Term
What research designs provides the best evidence for causality?
Definition
Randomized clinical trial
Term
A mutation in the gene SLC26A4 is responsile for what?
Definition
Pendred syndrome
Term
Using wide-band reflectance, a score of "0" means what and a score of "1" means what?
Definition

"0" means complete absorption

"1" means complete reflectance

Term
What syndrome includes craniofacial anomalies including maldevelopment of the maxilla and mandible, abnormal canthi, choanal atresia, and conductive hearing loss?
Definition
Treacher-Collins syndrome
Term
How long does it take for thalamic axons to mature?
Definition
Early childhood
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?
Definition
25%
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?
Definition
Close to 0%
Term
What syndromes may have progressive SNHL?
Definition
NF2, Stickler syndrome, & Usher syndrome
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?
Definition
None
Term
What are the 5 signs that are examined regarding ears during an ENT evaluation?
Definition

1. hearing loss

2. otalgia

3. otorrhea

4. tinnitus

5. dizziness/vertigo

Term
Why is CHARGE now referred to as a syndrome instead of a genetic association?
Definition
A mutation to the CDH7 gene on chromosome 8 is a common causal factor for CHARGE.
Term
What are some possible advantages of wide-band reflectance use in screening?
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

Term
What could be possible effects of a transplacental cytomegalovirus infection occuring during the first timester of fetal development?
Definition

Malformations of the inner ear

Microcephaly

Malformations of the auditory pathways in the brainstem

Term
Can an infant with asymptomiatic CMV at birth develop a hearing loss and/or mental retardation?
Definition
Yes and Yes
Term
Maturation of the thalamic axons and intrinsic cortical axons likely affects what aspects of auditory system function?
Definition
Affect the cortex because they both are important for normal cortical function like auditory processing
Term
What is the difference between a syndrome and a sequence?
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.
Term
The Eustachian tube embryologically is derived from...
Definition
The 1st pharyngeal pouch
Term
The principal dilator of the Eustachian tube is
Definition
the tensor veli palatine
Term
Which of the following would be characteristic of otitis media with effusion (OME)?
Definition
Type B tympanogram
Term
Which is the most likely sequence of events in the development of otitis media?
Definition
Eustachian tube closes, oxygen absorbed, creation of vacuum, fluid secreted
Term
Which of the following is a likely factor influencing otitis media?
Definition
Craniofacial anomaly
Term
Which of the following is a vaccine currently recommended by the American Academy of Pediatrics for otitis media?
Definition
PVC7
Term
Which of the following is now the usual recommendation for children with nonsevere AOM who are over the age of 2?
Definition
Watchful waiting
Term
Children with OME should have their hearing tested at least how often?
Definition
Every 3-6 months
Term
Which of the following syndromes includes congenital hearing loss and a prolonged Q-T interval?
Definition
Jervell Lange-Nielson
Term
Which of the following syndromes is responsible for half of the cases of deaf-blindess in the US?
Definition
CHARGE
Term
A cholesteatoma can possibly result from
Definition
Acute otitis or OME
Term
The hearing loss associated with cholesteatoma is
Definition
Could be any of the above
Term
Carhart's notch is caused by
Definition
Restriction of inertial bone conduction path
Term
Which of the following is characterized by autosomal dominant hereditary pattern and spongy bone growth?
Definition
Otosclerosis
Term
In its normal resting state, is the Eustachian tube open or closed?
Definition
Closed
Term
What are the three functions of the Eustachian tube?
Definition

1. Pressure equalization

2. Mucociliary clearance

3. Middle ear protection

Term
Besides the classic theory regarding Eustachian tube dysfunction and OM, what are two other ways that ET dysfunction may result in OM?
Definition

1. A ET that stays open too often

2. Disturbance of mucosal cleaning

Term
What might you suspect if you saw a long-standing case of unilateral serous otitis media in an adult? What should you do?
Definition
Usually sign of lesion (neoplasm) in nasopharynx. Should refer to ENT or PCP
Term
Name one intratemporal and one intracranial complication of chronic OM
Definition

Intratemporal: hearing loss

Intracranial: meningitis

Term
What is an excellent, non-surgical treatment for conductive hearing loss resulting from atresia, Treacher-Collins syndrome, and a third option for otosclerosis?
Definition
Softband Baha
Term
What is a type As tympanogram indicative of?
Definition
Ossicular fixation (Ex: otosclerosis, tympanosclerosis, congenital stapes fixation, osteogenesis imperfecta)
Term
What might be a likely etiology of a congenital air-bone gap that included both low and high frequencies?
Definition
Superior semi-circular canal dehiscence syndrome or large vestibular aqueduct
Term
A patient with pulsatile tinnitus and a conductive hearing loss will have what kind of etiology?
Definition
Glomus jugulare tumor etiology. Refer to MRI or contrast CT
Term
If adult patient has bilateral otitis media that is surprisingly resistant to antibiotic Rx, what might you suspect?
Definition
Wegener's granulomatosis
Term
The membranous labyrinth of the inner ear has its typical shape by...
Definition
The 8th week gestation
Term
Classic Mondini "dysplasia" specifically refers to...
Definition
Cochlea with only 1.5 turns
Term
General rules of thumb regarding malformations of the osseous and membranous labyrinths are: The ________ the interruption, the _________ the deformity and the _________ the hearing.
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.
Term
Which of the following may initially present as bilateral otitis media resistant to antibiotic treatment?
Definition
Langerhans cell histiocytosis
Term
A SP/AP ratio of <0.4...
Definition
is of little clinical value.
Term
After the inflammatory stage of bacterial labyrinthitis or meningitis, which of the following may likely occur?
Definition
inner ear ossification
Term
When do most congenital inner ear malformations occur?
Definition
4th-8th week of gestation
Term
Which of the following disorders may have otologic characteristics virtually the same as Meniere's disease?
Definition
Syphilis
Term
Which of the following can easily be identified using CT or MRI imaging?
Definition
osseous labyrinth malformation
Term
Which of the following might present with uveitis, cough, possible heart failure, and SNHL?
Definition
Sarcoidosis
Term
Systemic diseases that affect the temporal bone usually present with...
Definition

conductive hearing loss, sensorineural hearing loss, mixed hearing loss

answer: any of the above hearing losses

Term
Which of the following treatments for vertigo in Meniere's disease has the greater risk for hearing loss?
Definition
Gentamicin injection
Term
Which of the following syndromes typically has an inner ear malformation?
Definition
Pendred
Term
Which of the following describes incomplete development?
Definition
Hypoplasia
Term
About how many children with congenital inner ear malformation have malformation of both the osseous and membranous labyrinth?
Definition
20%
Term
Which of the following is commonly found in children with a congenital inner ear malformation?
Definition
Progressive hearing loss
Term
Noice-induced oxidative stress leading to cochlear injury may involve what 3 mechanisms?
Definition
impaired mitochondrial function, glutamate-induced excitotoxicity, & depletion of antioxidants, such as glutathione
Term
With sudden SNHL, what 2 factors yield a more positive prognosis?
Definition
younger age and less sensitivity of hearing loss
Term
What might a narrow internal auditory canal have to do with cochlear implantation?
Definition
The 8th nerve may be insufficiently developed and after cochlear implantation pt's have experienced facial pain, but no useful auditory function.
Term
What is an etiology of congenital unilateral profound SNHL?
Definition
Unilateral cochlear nerve aplasia
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?
Definition
ultra-high frequency hearing
Term
Hearing sensitivity in children with large vestibular aqueduct syndrome is...
Definition
usually normal or mild hearing loss w/ progressive hearing loss. Hearing sensitivity varies across individuals although 40% eventually have profound SNHL.
Term
Do you think there is a genetic susceptibility for NIHL in humans?
Definition
yes
Term
What do you think might account for the variability seen across patients with Meniere's symptoms?
Definition
Multifactorial; common end point for a variety of injuries or anatomical variables.
Term
What is true regarding autoimmune inner ear disease?
Definition
May recover within a 6-12 month period
Term
Ototoxicity of aminoglycoside antibiotics may be reduced when they are administered in conjunction with what?
Definition
Salicylates
Term
In the case of a temporal bone fracture, presence of a conductive hearing loss after hemotympanum resolution likely means what?
Definition
ossicular chain disruption
Term
With regard to temporal bone fractures, a fracture to ______ capsule usually results in what?
Definition
Otic, SNHL
Term
What is the most likely result of the actual aging process?
Definition
Metabolic presbycusis
Term
Patient A, a 55 year old adult, presents with an asymmetric hearing loss. What should be suspected?
Definition
Neoplasm
Term
One finding in auditory neuropathy/dys-synchrony is that speech recognition is what?
Definition
worse than predicted based on the AI
Term
What SNHL etiology is characterized by CNS demyelination and relapses/remissions?
Definition
Multiple sclerosis
Term
What causes large amounts of antibody or immune complexes to be produced in the blood and thereby cause ischemia and SNHL?
Definition
Leukemia, lymphomas, sickle cell anemia
Term
What is not possible using ENoG?
Definition
Degree of facial nerve degeneration
Term
An adult patient presents with unilateral tinnitus. What might you suspect?
Definition
Neoplasm
Term
What disorder likely presents with vertigo lasting for days?
Definition
Labyrinthitis
Term
What usually presents with a fluctuating hearing loss?
Definition
Meniere's disease
Term
What tinnitus therapies is based on psychological techniques for treating phobias?
Definition
Acoustic desensitization
Term
Progressive facial paralysis is likely ...
Definition
caused by a neoplasm
Term
What is NOT likely in cases of auditory neuropathy/dys-synchrony?
Definition
Mass lesions on MRI scan
Term
Dysfunction of what two organs will likely potentiate the ototoxic effects of meds, particularly aminoglycoside antibiotics?
Definition
Liver and kidney dysfunction
Term
What are congenital fistulas usually associated with? What is a big risk?
Definition
Patients with inner ear malformations. They communicate with subarachnoid space, which leads to CSF leaks and meningitis
Term
What would likely happen if an aminoglycoside solution was placed into a healthy middle ear?
Definition
Most likely result in cochlear and vestibular toxicity
Term
How might auditory cortical reorganization lead to tinnitius?
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.
Term
When a patient presents with a unilateral or asymmetric hearing loss, what etiology should be suspected?
Definition
Vestibular schwannoma or neoplasm
Term
What are three possible pathologic causes for auditory neuropathy/dyssynchrony?
Definition

1. dysfunction of IHCs

2. dysfunction of IHC/afferent nerve synapse

3. dysfunction of auditory nerve itself

Term
Is hearing loss a requisite for tinnitus to occur?
Definition
No
Term
"Neoplasms of the CPA is always present with high-frequency hearing loss." True or false? Why or why not?
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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