Disorders Flashcards

Question

Otosclerosis Audiogram findings

Answer 1

Audiogram **Early stage:** normal – mild conductive, rising configuration **Middle stage:** conductive/mixed HL, rising or flat configuration **Late stage:** flattening of the previously rising conductive or mixed HL * Poorer at 2000 Hz, narrowing ABG --- Carhart’s notch

Answer 2

* Bilateral conductive or mixed hearing loss with a rising configuration * Carharts Notch * Increased after hormonal event pregnancy or menopasue * Roaring, hissing, or pulsatile tinnitus * CT Scan

Answer 3

**Surgery** * stapedotomy * Partial Stapedectomy * Total Stapedectomy * Prothesis Contraindications: dead contralateral ear, active OM or TM perforation, Meneire’s **Amplification**

Answer 4

* WRS sometimes worsens if there is cochlear involvement * Oval window otosclerosis cannot be easily managed * Round window can cause permanent CHL * Hyperacusis * Facial paralysis * Chorda Tympani nerve may be sacrificed * Perilymphatic fistula * Labyrithitis * Disarticulation of the incus * SNHL * Immediate or delayed CHL

Answer 5

**Meniere’s disease** * dizziness/vertigo * Roaring Tinnitus * LF rising HL Meniere- SNHL Oto - CHL **Osteogenesis imperfecta** **SSCD** Superior semicircular canal dehiscence * LF HL 250-1,000 CHL, ABG * SSCD- ARTs present, tymps normal * Oto - ART absent, normal tymps

Answer 6

* Blow to the side of the head/falls * Sports injuries such as in racquetball, football, boxing * Blast injuries (e.g., bombs/improvised explosive device – IEDs) * Motor vehicular accidents * Foreign body insertion

Answer 7

TM perf Ossicular Disarticulation Hemotympanum (blood in tymp cavity)

Answer 8

* trauma to head or face * Cholesteatoma * Untreated ME infection

Answer 9

CT Scan – “ice cream cone” sign = normal appearance **Incudomalleolar Disarticulation**

Answer 10

**Otoscopy: ** perforation in TM bleeding in the EAC w/TM perf rarely TM appear normal **Immittance: ** Type Ad abnormal reflexes **Pure Tone** conductive/mixed HL **Speech**

Answer 11

most common cause is from motor vehicle accidents Also caused by * falls * assaults * etc

Answer 12

Blunt Trauma Fracture * Longitudinal fractures (direct blow to temporal/parietal aspect) * Transverse fractures (commonly due to a blow to the occiput)

Answer 13

**Longitudinal** (direct blow to temporal/parietal aspect) * Most common **Transverse** (commonly due to a blow to the occiput) * Less common * affect oval or round window

Answer 14

* CHL or SNHL * TM perf * Blood filled ME * Ossicular disarticulartion * BPPV * Perlymphatic Fistula

Answer 15

**Paraganglioma (glomus tumor)** - Most common & divided into two categroies * Glomus Tympanicum * Glomus Jugulare Both benign, slow progressive growth

Answer 16

* Sporadic or AD inheritance

Answer 17

Paraganglioma (aka Glomus tumor) * Most common benign tumor of ME * Arise from paraganglia cells * Genetic inheritance - AD * More common in females after 5th decade of life

Answer 18

**Glomus Tympanicum** * arises along the course of the **Jacobson nerve** in the tympanicum cavity * Smaller Signs/Symptoms: * Symptoms Arise ealier tham GJ * Pulsatile tinnitus * Red TM * Lateral growth of the TM * Growth of the tumor can inhibit ossicular movement = CHL * Medial growth can cause SNHL, facial nerve dysfunction, vertigo * Treatment = Surgery **Glomus Jugulare** * arises from the dome of the **internal jugular vein** * Larger * More common Signs and Symptoms: * Symptoms Arise later tham GT * HL * Ear pain * Aural Fullness * Vertigo -- if 8N involvement Audiological Findings: * Otoscopy: Red mass filling ME cavity may be visible Puretones: depends on size, involvement of ossicles & neural * CHL - osscular involvement * MHL- neural involvement Tymps: * As - tumor on TM * B - tumor significantly on TM stopping movement * **Tymps will show jagged edges** corresponding to patients pulse! * Treatment: surgery or radiation

Answer 19

**Glomus Jugulare** * arises from the dome of the **internal jugular vein**

Answer 20

**Glomus Tympanicum** * arises along the course of the **Jacobson nerve** in the tympanicum cavity

Answer 21

**Glomus Jugulare**

Answer 22

Inner Ear Homeostasis: The process by which chemical equilibrium of IE fluids and tissues is maintained.

Answer 23

Ions involved in IE homeostasis **Sodium Na and Potassium K+** as well as Chloride CL- and Calcium Ca

Answer 24

Endolymph and perilymph must maintain their specific ion concentration for **maximum sensitivity of hair cells** * Endolymph and perilymph are the support system for hair cell function * HL occurs when this support system fails

Answer 25

**intracellular** potential of -80mV (Na) relative to surrounding perilymph * Endocochlear Potential high K+ outside of cell +80mV w/ -80mv intracellular potential = +160 differential **Differential** potential of +160 mv

Answer 26

Disorders by Ion Homeostasis can be permanent or temporary Increased K+, Increased endolymph = **Endolymphatic Hydrops** * Menieres * Cogans Decrased K+, Decreased endolymph = **Endolymphatic Xerosis** * Connexin 26 * Majorty caused by xerosis are permanent

Answer 27

Otosclerosis * Cartharts Notch at 2,000hz * ART abnormal SSCD * enlarged ABG 250-500hz (increased BC sensitivity due to bone osccilator vibration of skull and increased perilymph movement) * Normal ART

Answer 28

**Connexin 26** * Endolymphanic Xerosis, decreased endolymph * permanent ion disorder due to abnormal connexin gap junction proteins Symptoms * Constant SNHL

Answer 29

**Menieres Disease** Sudden - Onset HL Autoimmune Labyrinthitis Disorders manifest temporary hearing loss and recovery indicates that the damage is not to the hair cells but to the ion homeostatic process * Fluctuating SNHL (any severity) * Abnormal loudness recruitment * Tinnitus

Answer 30

Viral or Bacterial Viral is divided into two * RNA * DNA

Answer 31

Rubella Virus (german measles) * Congenital HL * Cardiovasular problems * intillecular disabiltes Paramyxovirus (mumps) * Acquired permanent SNHL * Typically unilateral

Answer 32

* CMV (Cytomegalovirus) * AIDS * Meningitis

Answer 33

CMV (Cytomegalovirus)

Answer 34

* Urine polymerase chain reaction (PCR) is the gold standard * CT scan -CMV can also show intracranial calcification

Answer 35

CMV infection can result in decreased life expectancy Signs: * hepatomegaly & splenomegaly (enlarged liver or spleen) * rash – “blueberry muffin” (reddish blue to magenta) * decreased immunity, such as decreased immunity to the ‘flu vaccine * Infants born with it can have delayed onset SNHL * It passes through the placenta and is teratogenic to the fetus * Infected mothers may only exhibit symptoms of a common cold/mild ‘flu

Answer 36

* Infants born with it can have delayed onset SNHL * CMV causes a progressive profound permanent SNHL with the final stage generally being reached by ~ 3 to 5 years

Answer 37

CMV (Cytomegalovirus)

Answer 38

* Cardiovascular problems * Neurological and motor deficits * Blindness * Intellectual disability

Answer 39

AIDS For populations around risk for HIV the condition must be considered in all cases of sudden bilaterial or unilateral HL

Answer 40

* OME with or without CHL; SNHL (during later disease stages) * Otalgia * Vertigo * Tinnitus * Aural fullness * Reduced OAEs (OHCs affected) * Delayed ABR interwave latencies (central effects) Auditory symptoms also may be secondary to * Ototoxic effects of the AIDS drugs * Recurrent/chronic OME and other opportunistic infections that attack the ear due to the suppressed immune system

Answer 41

Inflammation of the meninges surrounding the brain and spinal cord

Answer 42

Various viruses - more common Bacteria, including mycobacterium tuberculosis

Answer 43

Primary meningitis * The disease originates in the meninges Secondary meningitis * OME leading to mastoiditis/labyrinthitis and then meningitis * Cholesteatoma that spreads to the meninges

Answer 44

Initial symptoms include * High fever * Neck rigidity (because meningitis covers the spinal cord too) * Malaise (feeling blah) * Nausea/vomiting * In severe cases, coma and death Severe/untreated infections also can lead to * Blindness * Paralysis due to damage of the motor centers of the brain * Hearing loss/deafness * One of the most significant causes of acquired sensorineural hearing loss * Meningitis can cause permanent abnormal cochlear bone formation - calcification * Vertigo and balance problems

Answer 45

**Antibiotics** **Mastoidectomy** * If the infection is related to middle ear disease and is not resolved by antibiotics **Amplification or cochlear implant** * if a severe/profound hearing loss has occurred * post-meningitis CI candidates, make sure cochlea hasnt ossified

Answer 46

An abnormal connection in either or both the oval and round window that separate the air filled ME and the fluid filled perilymphatic space of the inner ear.

Answer 47

Idiopathic

Answer 48

* Episodic vertigo without hearing loss * Hearing loss without vertigo * Symptoms maybe virtually indistinguishable from Meniere’s disease, i.e., tinnitus, SNHL, vertigo, aural fullness * Miscellaneous symptoms with disequilibrium but not episodic vertigo

Answer 49

Surgical repair

Answer 50

Noise induced HL is casued by both **dose** (level of noise) as well as **duration** amount of time to exposure of sound

Answer 51

Noise Notch * greatest threshold shift is between 3,000 to 6,000. hz * Peak loss at 4,000 and 6,000hz * SNHL

Answer 52

* Typically Symmetrical Bilateral SNHL (w/shooters opposite of shoot side is worse) * LF 40db HL or betetr * HF 75dbHL or better * Noise Notch HL worse btwn 3,000 to 6,000. Peak loss at 4,000 and 6,000hz

Answer 53

* Prevention from more NIHL * Amplification

Answer 54

* Loss of hair cells (initially OHCs) * Loss of secondary or supporting cells * Secondary neural degeneration * Threshold elevations of compound action potentials of the auditory nerve, ABR, and higher level evoked responses * loss of cochlear nonlinearities * The endocochlear potential (EP) is generally unaffected in NIHL * Degeneration of the stria vascularis is not typical in NIHL

Answer 55

Acoustic Trauma is a distinct form of NIHL * sudden permanent HL from a single event or expsoure * bombs * firearms * industrial blasts TM perf Noise notch, Flat or HF slope Type Ad & absent reflex w/ OD

Answer 56

Presbycusis/ ARHL * HF slope

Answer 57

NIHL * Noise Notch and HF slope

Answer 58

NIHL + Presbycusis * HL Slope * Noise Notch * progressing with age scale

Answer 59

**NIHL**: * Loss of hair cells (initially OHCs) * Loss of secondary or supporting cells * Secondary neural degeneration **ARHL**: * degeneration of the stria vascularis and lateral cochlear wall * Compromised blood supply correlated with the extent of strial degeneration * mostly normal sensory cells except in the most basal and apical turns of the cochlea * Significant degeneration of spiral ganglion in the presence of sensory cells

Answer 60

**NIHL**: * Thresholds worsen * ABR's mids and need louder sound to be triggered. due to Electrical signals from the auditory nerve are weakened * loss of cochlear nonlinearities (Doesn’t amplify soft sounds well, Can’t compress loud sounds & reduced resolution) * endocochlear potential (EP) & Stria vascularis is generally unaffected in NIHL **ARHL**: * Reduction of endocochlear potential (EP) * ARHL is, a vascular, metabolic, and neural disorder * Early NIHL can exacerbate ARHL - (remember, NIHL has notch and with age, the notch may disappear) * presbycusis can cause worse thresholds in the HF's decreasing/eliminating the noise notch

Answer 61

NIHL * Typically Symmetrical Bilateral SNHL (w/shooters opposite of shoot side is worse) * LF 40db HL or betetr * HF 75dbHL or better * Noise Notch HL worse btwn 3,000 to 6,000. Peak loss at 4,000 and 6,000hz * Profound SNHL is rare * Can be temporay (TTS temporary threshold shift) * Normal Tymps Acoustic Trauma * Sudden Permanent HL from a single event * Direct injury to cochlea * 140 SPL or louder * TM perf * Noise Notch * HF slope or Flat configuration (MC) * Ad Tymp

Answer 62

Cochlear amplifiers are the OHC's * The cochlear amplifier is the active process located in the OHC that amplify sound vibrations inside the cochlea * Loss of just OHC can reduce sensitivity by ~40 to 50 db HL

Answer 63

* Extracellular resting potential * The EP is a **+80 to +100 mV** electrical potential found in the **endolymph of the scala media.** * **Generated mainly by the stria vascularis**, the EP is essential for driving K⁺ influx into hair cells during mechanoelectrical transduction. * This high positive potential makes the hair cells more responsive to stereocilia deflection, **ensuring efficient sound transduction**. * EP acts as a battery to drive current though the hair cells when they move in response to sound

Answer 64

* EP is generated by the Stria Vascularis * With age, the stria vascularis can degenerate = ↓ in EP * A decline in EP leads to: Reduced driving force for K⁺ entry into hair cells. **Decreased hair cell responsiveness** Global loss of cochlear sensitivity, across all frequencies—not just high ones **Loss of EP affects HF's more than LF's = HF slope seen in Presbycusis**

Answer 65

Age-related loss of OHCs leads to: * Decreased amplification of sound. * Reduced ability to distinguish between frequencies. * Elevated hearing thresholds (especially at high frequencies) * This contributes to the classic symptoms of presbycusis: difficulty hearing soft sounds, poor speech discrimination, and trouble hearing in noisy environments.

Answer 66

Osteopetrosis Mobius * children

Answer 67

Mobius Syndrome is a rare congenital facial nerve disorder associated with hypoplasia of 6th and 7th CN nuclei

Answer 68

* Genetic- multifactorial * Exposure in utero to teratogens such as Cocaine, Ergotamine or Misoprostol

Answer 69

* Congenital facial diplegia (bilateral facial paralysis) * Associated CN VI unilateral or bilateral paralysis * Other cranial nerve deficits * Deformities of extremities * Musculoskeletal deformities * Intellectual disability

Answer 70

* Ophthalmologic consultation * Nerve reconstructive surgery

Answer 71

Bell's Palsy * Adults

Answer 72

* most common cause of acute unilateral facial paralysis * Bilateral can occur but rare * Pregnancy can increase risk

Answer 73

Right side

Answer 74

* **Idiopathic** - Most common * Herpes Simplex virus * Rare: a otitis media, CPA,partoid gland or skull based tumor.

Answer 75

BP is an **exclusionary diagnosis**, but misdiagnosis is rare * Onset of partial/total unilateral facial paralysis during a 48-hour period * Fever and neck stiffness at the onset * *No* hearing loss or vertigo * No other cranial neuropathy * Normal head and neck examination * Drying of eye due to decreased eye closure and lack of lacrimation * Rare cases of recurrence * Some spontaneous recovery should be noted **within 3 to 6 months** in all patient

Answer 76

* Otoscopy: normal * PTA - Normal, HL is rare * Tymps: normal * ART: Determined by distance from the stapedius nerve Abnormal ART lesion *proximal* close to the stapedius nerve Present ART lesion *distal* far to the stapedius nerve

Answer 77

* ENoG to assess degeneration of nerve fibers * CT scan & MRI (differential diagnosis to rule out 7CN tumors)

Answer 78

* decompression of the nerve when more than 90% degeneration occurs within 2 weeks of onset * Steroids ro reduce inflammation * Antiviral w/steroids * Eye care to prevent permannt damge from dry eyes

Answer 79

* CPA or skull-based tumors * Vestibular schwannoma * Otitis media * Parotid gland tumors

Answer 80

For Bell's Palsy majority of patients recover function within **3 to 6 mos** w/no medical or surgical intervertion.

Answer 81

* young * partial paralysis & recovery wi/2mos * intact ART (distal lesion) * ENoG shows less than 90% nerve degeneration

Answer 82

* 65 + * greater then 90% nerve degeneration wi/ first 2 weeks * Diabetic

Answer 83

* Otitis externa (untreated) * Otitis Media (Chronic or Acute) * Herpes Zoster Oticus

Answer 84

Facial Neuromas or schwannnomas are rare benign neoplasms of schwann cells

Answer 85

* Facial weakness or paralysis (gradual) * Hearing loss (SNHL,CHL or MHL) location based Other symptoms * Tinnitus * otorrhea * Ear canal mass * otalgia * vestibular symptoms

Answer 86

If tumor is confined to IAC or CPA, no facial symptoms maybe present * ART are helpful for DD

Answer 87

* PTA: **SNHL** Due to cochlear nerve compression * Tymps: normal * Abnormal ART lesion *proximal* close to the stapedius nerve * Present ART lesion *distal* far to the stapedius nerve * ENoG: Can be normal is located in CPA * CT & MRI: used to DD from CN 8 tumors * ABR: determine if tumor is acoustic or facial neuroma

Answer 88

* ART's are abnormal on the affected side (right) on the side measured Right Cn7 neuroma * Right ipsilateral = Absent * Left Contrlateral = Absent * Right contr = Present * Left Ipsi = Present

Answer 89

* Surgical removal contoversy * Radiotherapy * decompression of facial nerve

Answer 90

* Otitis Media w/CHL (w/ or wo/ cholsteatoma) * Cholesteatoma * Glomus Tumor * Meningiomas * Acoustic Neuroma (vestibular schwannoma)

Answer 91

Facial Neuroma * ART Absent --> Lesion proximal to stapedius nerve

Answer 92

**Intact OAE’s but absent or abnormal ABR’s** * OAE's present bc OHC function are intact * ABR Abnormal/Abent w/CM CM present since OHC functioning ABR absent due to 8th CN & lower brainstem patheays are affectd by ANSD

Answer 93

* OAE's Normal * ABR Abnormal * ECochG Absent (bc 8th n involvement) * ART's Absent (bc 8th n involvement) * No OAE Suppression - (abnormal) * Audio: Typically normal (range from mild to profound) * Poor WRS Even Worse QuickSIN * Tymps Normal

Answer 94

Age realted Hearing loss * progressive loss or degeneration of endocohlear potential, sensory hair cells, snyapses and others * Slowly Progressive sloping HF SNHL

Answer 95

Cochlea - primary site for age related HL

Answer 96

* Sloping HF SNHL - Most common * Flat SNHL - rare in late stages * Poor WRS/Speech * Abnormal Loudness recruitment * Tymps: Normal * ART: Agreeance w/HL * OAE's Agreeance w/HL

Answer 97

* Benign & Malignant tumior * Tumor in the meninges * Arise from neuroglial cells

Answer 98

Benign * Slow Growing * do no spread across other areas of the body * not typically life threating * Rare if kills Malignant * Fast Growing * Spreads throughout body = death * Life threatening

Answer 99

Intra-axial or intrinstic tumor

Answer 100

Extra Axial Tumors or extrinsic tumors * Do NOT come from neurons or glial cells * Instead, they come from supporting or surrounding structures of the brain. such as Tissue that encases or supports the brain (meninges) Pineal and pituitary gland tumors These are located near but not within the brain tissue itself. Cranial nerve sheath tumors Cerebral Metastatic tumors

Answer 101

Tumors originating within the brain tissue; Example: Astrocytoma and glioblastoma

Answer 102

Do NOT come from neurons or glial cells Instead, they come from supporting or surrounding structures of the brain. * All tissue that encase or support the brain substance, e.g., meningioma * Pineal and pituitary gland tumors * Cranial nerve sheaths (VII and VIII N tumors included) * Tumor metastases to the brain from any organ/structure

Answer 103

Schwannoma

Answer 104

Schwannoma

Answer 105

**Internal Auditory canal (IAC) from the CN 8 - Most common** Jugular foramen from CN 9 & 10, superior to jugular bulb Fallopian canal of the CN 7

Answer 106

Almost always unilateral * except for NF2 where bilateral Vestibular schwannomas are common

Answer 107

* Benign Slow growing tumor * Extra - Axial * Almost always unilateral Except for NF2 where bilateral Vestibular schwannomas are common * Arise from Schwann Cells * Typically occurs between 40 to 60 yrs

Answer 108

Signs and symptoms almost always occur on the **same side to the lesion (tumor)**

Answer 109

Between 40 to 60 yrs * rare before 30yrs

Answer 110

* Hearing loss * Headache * Tinnitus * Unsteady Gait * Imbalance/dizziness * Facial Paralysis - rare * Nystagmus

Answer 111

* Unilateral HF SNHL - CN8 * Brainstem lesion show a flat unilateral SNHL * Positive Tone Decay * OAEs: Normal if HL is not significant * Decreased OAE suppression * Speech: WRS- worse then you would expect for type of loss - Not in agreement with PTA * ART: Abnormal/absent 8N lesion - Abnormal Ipsi & Contra on the affected side * Brainstem lesion - Normal Ipsi and absent contras or all absesnt * Reflex decay: positive for CN 8 VS, @ 500 & 1,00hz * ABR: Abnormal ↑ absolute latency Wave 5 & ↑ interpeak latency wave 3-5

Answer 112

* MRI T1 w/ gadolinium contrast - Gold standard * CT Scan - not as sensitive for small Tumors

Answer 113

Meningioma

Answer 114

Schwannoma

Answer 115

Meningioma

Answer 116

* Most common benign tumor of the CNS * Arises from meninges * almost always singly (one, individually) * Clear association with NF2 - Multiple meningiomas are common * Aggressive and locally invasive * Large and grow in CPA

Answer 117

* Progressive Unilateral SNHL * Tinnitus -- Pulsatile Tinnitus if tumor involves jugular foramen * CN 8 involvement Abnormal ARTs on affected side if unilateral normal Tymps Positive Acoustic reflex decay * Poorer WRS in noise * Abnormal ABR * Normal OAE

Answer 118

* Otitis media: if it gains access to the ME; i can mimic OM (Red TM, Granulation tissue formation, CHL) * **Paraganglioma (Glomus Tumor)** : Meningiomas appear highly vascular on an otoscopic exam. May also be confused for a glomus tumor on MRI, May present with a neck mass similar to a glomus jugulare tumor * **Facial CN VII nerve involvement** : Mimics CN VII neuron, Facial numbness * **Vestibular Schwannoma**: Especially If tumor is confined to the IAC (rare), Difficult to differentiate diagnosis even on MRI

Answer 119

**CN 8** * Absent ipsi & contras for the ear side affected (right VS) * Right Ipsi - Absent, Left Ipsi Normal, Right Contra Absent, Left contra Present **Brainstem** Size Dependant * Small: Both Contras Absent only * Large: All Reflexes Absent **CN 7** * Absent Ipsi & contra of the measured side affected (RIght) * Right Ipsi Absent, left Ipsi Present, Right Ipsi Present, Left Contra Absent.

Answer 120

Autoimmunity: Immune system attacks itself; not longer protecting from external pathogens = cause autoimmune disease * can be limited to a single organ such as thyroid (Hashimoto's ) or can cause systemic damage- MS, Rheumatoid Arthritis

Answer 121

* **Autoimmune** disorders - MS, sudden onset SNHL * **Vasculature** disorders - Wegener granulomatosis * **Metabolic** disorders - diabetes, thyroid disorders

Answer 122

* blood-labyrinthe barrier controls movement of circulating inflammatory and other cells * Protects IE from systemic immune response

Answer 123

Steroids * first line of treatment

Answer 124

Characterized by * Progressive bilateral SNHL - is reversible * Responsive to immunosuppressive agents (corticalsteroids Classified in 2 types * Primary AEID - disease restricted the the ear * Secondary AEID - multisystemic that also involve ear (cogan,wegener granulomatosis)

Answer 125

* ***Bilateral* SNHL** - progressing over weeks to months * Aural fullness/tinnitus * Vestibular symptoms

Answer 126

* Physical examination * There may be manifestations of systemic autoimmune disease such as --- Effusion in ME and EAC and cough, skin lesions, vision loss * Lab tests/imaging may be helpful to dx

Answer 127

* Corticosteroids * Begin as soon as possible - irreversible damage within 3 months of onset

Answer 128

* **Sudden SNHL** - except this is usually unilateral, AEID bilateral & is progressive SSNHL is not * Menieres- both have fluctuating SNHL, tinnitus, aural fullness, vertigo

Answer 129

* 30 or greater dB SNHL occurring in at least three adjoining frequencies within 3 days * Over 3 days is called rapidly progressive * typically unilateral - bi (rare) * Always Sensorinenural & sudden * 1/2 SNHL is permanent even with treatment * Even with recovery of thresholds, WRS may not get better

Answer 130

* idiopathic * Viral Infections * Ototoxic * Trauma * Tumor * Autoimmune

Answer 131

* 3,3,3 →**30 dB + SNHL occurring in at least 3 contiguous frequencies within 3 days** * **Unilateral sudden SNHL** in any severity * Typically in high frequencies or flat mild to profound SNHL * Deteriorating speech understanding abilities (WRS are worse) * Vestibular system is often spared but may be affected - Dizziness, vertigo - early stages * Followed by long symptom free periods, eventually resulting in oscillopsia (bobbing eyes) and ataxia * **Normal tymps** * ARTs consistent with the degree of HL * If less than 60 dB, ARTs can be normal

Answer 132

* Medical urgency - treatment should begin < 1 week of onset * Corticosteroid ~4 weeks * Long term steroid treatment can cause - Increased appetite, weight gain, organ damage, bone loss

Answer 133

* Evaluation - case hx, audiological evaluation * MRI to rule out other disorders - trauma, tumors, MS * Lab tests Useful if related to hormone levels Autoimmune conditions (increased antibodies) Diabetes (increased glucose)

Answer 134

damage/demyelination of the myelin sheath that surrounds nerve fibers in brain and spinal cord (no cure) * Progressive * Causes impaired sensation, movement, and cognition * MS - Most commmon demylinating disease * ~30 yrs

Answer 135

* Genetic * Idiopathic * Autoimmune

Answer 136

MS is a **Progressive** neurological **autoimmune** disorder

Answer 137

* **Plaques** - Hallmark of MS * Remyelination can occur = remission phases * 1st symptoms = eye muscle paralysis, horizontal nystagmus, diplopia, dizziness * Fatigue * Abnormal reflexes, poor coordination/control of muscles * Numbness, tingling, itching * As MS progresses = dysarthria, ataxia, HL, vestibular problems

Answer 138

Audiological Findings * SNHL- not an initial symptom or complaint * Audio - Varies **Bilateral HF SNHL** **LF Rising SNHL** Rare: sudden SNHL reported or Fluctuating HL * ARTs vary (normal to abnormal w/HL) * Poorer WRS (central neural pathways affected) - than expected * Poorer WRS in noise - than expected

Answer 139

Case history disease progression and remission stages High levels of immunolgobulin **CT & MRI** * 2 or more plaques

Answer 140

* No cure * Immunosuppressive agents/corticosteroids are recommended * Symptomatic treatment * Treatment varies

Answer 141

* Susac's syndrome or Schilder's disease = autoimmune Has white matter defects but only on the corpus callosum and similar symptoms to Menieres * Diabetes -- HL, Visual Probs, poor WRS * Stroke --- Poor WRS, visual problems, cognitive/mem issues, poor motor skills * Sudden SNHL

Answer 142

* autoimmune * Often misdiagnosed for MS * Plaques present on Corpus Callosum

Answer 143

* Plasques on Corpus Callosum * Asymmetric SNHL (LF) * Dizziness/vertigo * Tinnitus

Answer 144

* rare demyelinating disorder * motor, sensory and cognitive problems

Answer 145

* persoanlity changes & poor attention --- diff from MS * Progressive loss of intellectual fucntion -- diff from MS * vision & hearing loss * headaches vommiting & sezires

Answer 146

* rare chronic autoimmune disorder * Endolympahatic Hydrops (similar menieres) * Degenertive changes in organ of corti * Demyelination 8n * Hallmark **Vestibular dysfunction and interstitial keratitis** * Normal EcochG * Vertigo * ataxia * Oscillopsia * **Unilateral or Bilateral HF SNHL**

Answer 147

* Corticosteroids * HA * CI * Vestib rehab

Answer 148

* Menieres * Labyrinthinitis/Neuronitis * Wegener's Granulomatosis

Answer 149

* Auto immune vasculitis affected upper and lower respritory tracts, ears & kidneys * CHL w/ OM (becuase of obstruction of nasopharynx) * Sudden unexplained SNHL - WG shoudl be considered Treatment * Corticosteroids DD * SSNHL

Answer 150

chronic, incurable, but manageable carbohydrate metabolism disorder * HL in diabetics can be caused by impairment of vasculature or metabolic pathways, or peripheral neuropathy * Hearing loss with poor WRS due to neuropathy, especially in noise, may be seen with or without systemic symptoms of neuropathy

Answer 151

manifestation of vascular changes may affect the stria vascularis and the spiral ganglion * LF HL -common * HF SNHL and fluctuating HL based on blood glucose levels also are reported * WRS scores generally consistent with the SNHL, unless neuropathy is present * Microvasculature diseases in diabetes may increase risk of NIHL and/or reduce chance of recovery from TTS from noise

Answer 152

* Imbalance * Dizziness/Vertigo * Nystagmus

Answer 153

* Meniere's * Recurrent Vestibular neuritis * BPPV * Migrain Associated vertigo * SSCD

Answer 154

Vestibular **Labyrinthitis** - *Inflammation* of inner ear **labyrinth** Vestibular **neuritis** - *inflammation* of vestibular **nerve**

Answer 155

Preceded by * cold/flu/OM * measles/mumps * Meningitis * Infectious mononucleosis

Answer 156

Labyrinthitis & Neuritis Vestibular Symptoms * Acute vertigo * Nausea/vomiting * Nystagmus Labyrinthitis ONLY Cochlear symptoms * Aural fullness * Tinnitus * HF SNHL - resolves completely or partially ~ 50% | Neuritis - Vesti ONLY -------- Labyrinthitis = Vestib & Cochlear

Answer 157

Antibiotics/Antiviral Symptomatic treatment for vertigo (vestibular suppressant) Steroids

Answer 158

* OME * Perilymphatic fistula * BPPV - no HL

Answer 159

* dizziness/vertigo is the aura of the headache ~⅔ patients present with true vertigo might not have headache * **migraine symptoms in at least 50%** of the vertigo episodes * Always have Vertigo, Some have Migraine * lasting from minutes to 24 hrs (typically MAV) * Headache, nausea, vomiting, and pallor * Photophobia * Motion intolerance and noise sensitivity * Ataxia and numbness/weakness of the extremities * HL is UNCOMMON

Answer 160

* Mirgaine medications * Diet - avoid triggers * Vetib rehab

Answer 161

Brief episodes of mild to intense dizziness/vertigo, Involved posterior semicircular canal * otoconia from utricle or saccule get disloged into SCC

Answer 162

* Can occur at any age ; average is ~55 yrs at onset * Rare in children * **Idiopathic (most common)** * **Head trauma** * Vestibular neuritis * Following stapes surgery * Meniere’s Disease * Migraine * Diabetes * Osteoporosis

Answer 163

* Brief episodes of mild to intense dizziness/vertigo Sometimes nausea/vomiting * Triggered when turning head to get in and out of bed * Worse in the morning and evening * semicircular canal

Answer 164

posterior semicircular canal * Rarely involves Horizontal SCC

Answer 165

Acute: resolves spontaneously over 3 months Intermittent: active and inactive periods over several years Chronic: continuous symptoms over long durations

Answer 166

* Audiogram and MRI are normal (unless they already have HL) * Dix-Hallpike test - Nystagmus can be observed and assessed * Nystagmus present = bppv

Answer 167

* Epley Maneuver

Answer 168

Idiopathic syndrome characterized by by endolymphatic hydrops * multifactoral or autoimmune

Answer 169

Characterized by 4 symptoms * **Intermittent** episodes of vertigo lasting from minutes to hours * **Fluctuating** SNHL * **Tinnitus** (low frequency roaring) * **Aural fullness**/pressure

Answer 170

1. Two or more episodes lasting 20 min or longer 2. At least two of the four characteristic symptoms are present (present during episodes)

Answer 171

* Sudden with unilateral aural fullness, vertigo, tinnitus, and SNHL * Vertigo - *2 to 4 hours* can last 12-24 hours * Vertigo of long duration & other symptoms = Meniere’s Disease * **Nystagmus** always present with vertigo * Patients can lose balance and fall injuring themselves * After acute attack - hearing returns normal * **Attacks last couple hours to a day** * Occur in **clusters** - patient symptoms free for months or years * Remissions may confuse clinical picture and make diagnosis difficult

Answer 172

* Attacks are **frequent and severe** * HL does not return normal - **SNHL is permanent** * Vertigo stops but patient may feel dizzy frequently * WRS deteriorate * Diplacusis, tinnitus, and recruitment - permanent

Answer 173

* Low frequency (rising) SNHL * Worse from 250 to 1000 Hz - normal in high frequencies * Flat; moderate to moderately severe SNHL * Bilateral disease; asymmetry of > 25 dB HL reported * ECochG - Abnormally large ECochG SP component, increases the SP/AP amplitude ratio * Tymps normal

Answer 174

* reverse cookie-bite configuration * ECochG - Abnormally large ECochG SP component, increases the SP/AP amplitude ratio * Tymps Normal

Answer 175

* Hearing stabilizes with flat severe SNHL - peaks at 1000 and 2000 Hz * Unaidable because of distortion of speech/sound * Rarely SNHL progress to profound in Meniere’s * ECochG - Abnormally large ECochG SP component, increases the SP/AP amplitude ratio * tymps Normal

Answer 176

During Acute attack * Symptomatic treatment to control vertigo and nausea with sedatives and tranquilizers Long-term * Decreasing endolymph - **low sodium diet & diuretics** * Increase vascular circulation of inner ear * Alter immune activity causing Meniere’s * If endolymphatic duct is blocked a shunt may be placed to direct extra fluid to the CSF Cochlear implant * Mostly significant improvement in hearing performance, compared to the gains experiences by patient w/o Meniere’s * CI electrode had no negative effect on fullness, tinnitus, or vestibular symptoms * successful hearing rehab with CI after surgical labyrinthectomy CI is an excellent option who meet criteria

Answer 177

**Acoustic neuroma** * Non-episodic symptoms; Progressive unilateral SNHL, poor WRS, tinnitus, occasional aural fullness **Labyrinthine** viral infections * SNHL, history of ear surgery or viral illness; progression disequilibrium, symptoms may resolve **Idiopathic vertigo** * nausea/vomiting **Perilymphatic fistula** * Sudden severe or fluctuating SNHL w/ disequilibrium - only occasional vertigo **Cogan’s syndrome** * Episodic vertigo, SNHL, and eye symptoms (not in Meniere’s)

Answer 178

SSCD creates a third mobile window into the inner ear that alters the normal fluid mechanics of the vestibulocochlear system

Answer 179

* Developmental abnormality to the temporal bone **congenital** - Most common * Head trauma such as skull fractures or major pressure altering events * Idiopathic

Answer 180

Signs * Vestibular symptoms * Auditory symptoms * Or both auditory and vestibular symptoms * No symptoms (observe patient)

Answer 181

SSCD can mimic several auditory and vestibular disorders. * **Patulous ET** -- Aural fullness/pressure and autophone are common in both * **Vestibular pathology** - SSCD Can be evoked by loud noises, maneuvers that change ME/intracranial pressure (coughing, sneezing, straining) * **CHL and/or fluctuating HL** * mimic otosclerosis or Meniere’s)

Answer 182

Acoustic Reflexes * SSCD = Normal ART * Otosclerosis = Absent ART

Answer 183

* Vestibular assessment * High resolution temporal bone **CT scans ** * MRI useful for differential diagnosis * ECochG - Abnormal High Sp/AP ratio *

Answer 184

* ear plugs * Surgical repair

Answer 185

* Sickness of disembarkement * illusion of movement after long travel on boat * Typically resolves within 24 hrs * is symptoms presist for longer than 12 mos chances of spontanious recovery decrases Symptoms * rocking, swaying or disequallibrium * Symptoms worse in enclosed spaces * Symptoms improve or disappear during movement (driving)

Answer 186

* CHARGE * EVA * BOR * waardenburgs * Ushers

Disorders Flashcards

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