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Disorders of the IE

Audiology Exam 4

QuestionAnswer
Most common type of hearing disorder includes all damage occurring to the ______________ structures Inner ear
Most common type of hearing disorder Includes all damage occurring to inner ear structures. What are five? • Sensory Cells (hair cells) • Cochlear partitioning membranes • Cochlear fluids • Synaptic connections with auditory nerve • Malformations
What are three common congenital inner ear malformations? Cochlear aplasia, common cavity, and cochlear hypoplasia (severe)
When it comes to inner ear malformations, similar to some outer ear disorders, the structures of the ______ can develop ___________ Cochlea, abnormally prenatally
Most inner ear malformations have a _______ associated with them Genetic link
Some inner ear malformations can be cause by _______ factors Teratogenic
Aplasia The failure of an organ or tissue to develop normally
Michel Aplasia Characterized by a COMLPETE absence of inner ear structures
In Michel Aplasia, what is not present? The cochlea or vestibular systems
Michel Aplasia needs ________ to be diagnosed CT scan
Michel Aplasia results in? Complete deafness
Why does Michel Aplasia result in complete deafness? The lack of cochlea prevents the ability to hear any sound
What is the treatment for Michel Aplasia? Possible brainstem implant
Cochlear Aplasia Malformation which results in an absence of bone and membranous portion of the cochlea, but vestibule and semi-circular canals develop normally
Cochlear Aplasia typically results in? Profound hearing loss
Common Cavity Malformation When both bone and membranous structures of the cochlea and vestibule share one common cavity
Common Cavity Malformation typically results in? Profound Sensorineural hearing loss
Common Cavity Malformation may also experience what? Significant balance problems
Cochlear Hypoplasia Underdeveloped boney and membranous cochlea with only approximately 1 turn
Mondini Malformation Underdeveloped boney and membranous cochlea with approximately 1.5 cochlear turns
In both Cochlear Hypoplasia and Mondini Malformation, hearing loss is more ________ depending on the patient, but often severity is _______________ Variable, severe to profound
Presbycusis Age related hearing loss
What is the most common form of acquired sensorineural hearing loss? Presbycusis
Age related hearing loss begins at ~ age _____ 50
_______ of adults have presbycusis by age _______ 1/3, 65
________ of adults have presbycusis by age ______ 2/3, 75
Presbycusis typically have ___________ then slope to a high frequency sensorineural hearing loss Normal hearing in low frequencies
__________ are absent in frequencies with hearing loss DPOAEs
_________-_ may be absent depending on severity of hearing loss Acoustic Reflexes
If purely a SNHL, __________ expected to be normal. Which one? Tympanometry, Type A Tympanogram
Presbycusis SNHL most common complaints are? 1. I can hear but can’t understand 2. hearing in background noise 3. "Sounds like people are mumbling"
What is the second most common cause of acquired SNHL (after Presbycusis) Noise Trauma
Excessive exposure to loud sounds damage what two things? What are they responsible for? Stereocilia and the tip links responsible for the mechanically gated ion channels
Temporary Threshold Shift (TTS) Pure tone threshold shift from noise exposure is only temporary
Permanent Threshold Shift (PTS) Pure tone threshold shift from noise exposure is permanent
Safe level of noise depends on the ______ and _______ Frequency and Duration
A ________ noise is considered "safe for ____ hours of exposure according to OSHA standards. Every time the duration is _______, the acceptable "safe" noise limit rises by _________ 90 dBA, 8, cut in half, 5 dB
Noise-Induced Hearing loss is characterized by a ________ in pure tone audiometry 4 kHZ notch
What classes of drugs are ototoxic to the inner ear? Antibiotics, Quinine, Chemotherapy Agents, Diuretics,
Antibiotics include? • ‘mycins’: • Gentamycin • Neomycin
Quinine includes? Anti-malaria drug
Diuretics are taken for __________ or _______ problems Kidney, heart
Diuretics are the only ototoxic drugs that induce a _______ hearing loss Temporary
Ototoxic drugs cause sensorineural hearing loss first in __________then progress to ________ over time High frequencies, lower frequencies
Ototoxicity monitoring utilizes __________ up to _______ to detect first signs of damage Testing pure tone thresholds, 20 kHz
Menier's Disease Caused by endolymphatic hydrops, results in fluctuating hearing loss and bouts of vertigo that last hours
Endolymphatic Hydrops Over production of endolymph fluid in the cochlea
Menier's Disease is diagnosed based on? Symptoms
What are the four symptoms of Meniere's Disease? • Roaring tinnitus • Aural Fullness • Low-frequency fluctuating SNHL • Vertigo lasting for hours
Is there a cure for Meniere's Disease? No
Many times, _______ is prescribed to deal with vertigo for Meniere's Disease Meclizine
The ECochG and aid in diagnosis of Meniere's Disease is? AP/SP Ratio: >.45 = Meniere’s Sensitivity only 60-70%
What is the case history for Meniere's Disease? • Two episodes of vertigo, > 20 min but < 24 hours • Roaring quality tinnitus and aural fullness
Fluctuating Hearing loss Initial presentation as low-frequency loss
Superior Canal Deheciense Thinning of superior semi-circular canal bone with base of cranial cavity
Deheciense "The splitting or bursting open of a pod or wound”
Superior Canal Deheciense is unique becuase it presents as a _____________ even though lesion is located within _________ Low-frequency conductive loss, inner ear
What are the common reports of Superior Canal Deheciense? • Tulio Phenomena • Autophony
Superior Canal Deheciense diagnosis can be aided with? Which two specifically? VEMP testing. 1. cVEMP 2. oVEMP
What are the symptoms and signs of Superior Canal Dehiscence? - Autophony - Hear pulsing or Eye movements - Sound-induced vertigo/nystagmus
Auditory Neuropathy _______ is not fully understood - thought to be the result of poor connection of _______ with __________ Lesion, auditory nerve, cochlear IHCs
What are IHC's primarily responsible for? Sending auditory information to the brain
What are OHCs primarily responsible for? Amplification of signal
Auditory Neuropathy is diagnosed by a combination of results, what are they? • No response ABR or poor waveform morphology • Present DPOAE or CM response
In Auditory Neuropathy, pure tone thresholds can be anywhere from? Normal to severe
With Auditory Neuropathy, we can lose a significant number of IHCs without? Affecting thresholds
With Auditory Neuropathy, patients most often perform very poorly on __________ Speech testing
With Auditory Neuropathy, ______ is much poorer than expected for ______________ WRS, audiogram thresholds
Sudden Idiopathic Hearing Loss Hearing loss occurs suddenly with no known cause
Sudden Idiopathic Hearing Loss is typically ________ Unilateral – only occurs in one ear
What is the prevalence of Sudden Idiopathic Hearing Loss? 5-20/100,000 persons each year
With Sudden Idiopathic Hearing Loss, ____% of patients also report ______ problems 50%, vestibular
What are the treatments and timeframes for Sudden Idiopathic Hearing Loss? • If treated within 2-4 weeks with oral corticosteroids • If not treated within 4 weeks – very unlikely to resolve on its own
Sudden Idiopathic Hearing Loss is an __________! Otologic Emergency
Created by: RachelJClark
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