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Disorders of the IE
Audiology Exam 4
| Question | Answer |
|---|---|
| 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 |