Disorders Final Flashcards by Nicole Ballestas

What is hearing loss resulting from overexposure to loud noise?

This is the 2nd most common type of acquired hearing loss
30 million Americans are exposed daily to dangerous noise conditions (includes children)
often occupational; regulation of workplace noise by Occupational Safety & Health Association (OSHA)

Noise Induced Hearing Loss

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What are the three types of NIHL?

Noise Induced Permanent Threshold Shift (NIPTS), Acoustic trauma, Noise Induced Temporary Threshold Shift (TTS)

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Repeated exposure (over the years) to SPLs lower than those that produce acoustic trauma

-Long-term; Usually occupational (ONIHL)

Noise Induced Permanent Threshold Shift (NIPTS)

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Permanent cochlear damage after one exposure to very high SPLs (firecracker, gunfire, jackhammer)
Physical trauma & acoustic have similar and may occur together
Hearing loss “muffled”
Tinnitus
Disequilibrium (rare)
Hemotympanum
Perforation, otorrhea
Ossicular disarticulation

Acoustic Trauma

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Exposure to loud sounds for a few hours

-“buzzy” ears, muffled hearing, decline in thresholds but recovery within hours or days
- not temporary, IHC most affected; long-term effects are initially hidden but progress over time
- the greater the SPL the greater the shift
- max threshold shift occurs 0.5 - 1 octave above the center frequency exposure
- when the max shift in this reaches its maximum, it will lead to a maximum permanent threshold shift

Noise Induced Temporary Threshold Shift (TTS)

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No stereocilia disarray, but decrease in stiffness
Strial swelling – Excitotoxicity
Loss of some spiral ligament fibrocytes
Space of Nuel Collapse (then recovers)
Synaptic terminal swelling/retraction
Steriocelia often damages regardless of hair cell survival
Threshold recovery, ABR & DPOAE thresholds recover from initial shift, but ABR amplitudes decline
Loss of synapses, terminals after noise
Delayed loss of afferents

This pathophysiology is indicative of…

TTS

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The condition where nerve fibers become permanently disconnected from IHC. Not revelaved by any tests of threshold sensitivity

Hidden Hearing Loss (HHL)

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What is the formula for noise exposure?

For every 3 dB increase cut exposure time by half (ex: 85 dBA: 8 hr exposure limit, 88 dBA 4 hr exposure limit, 91 dBA: 2 hr exposure limit…)

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Definition: Equal amounts of sound energy will produce equal amounts of hearing impairment regardless of how sound energy is distributed in time

Equal Energy Hypothesis

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How much (loudness, annoyance)
Duration (continuous, intermittent, impulse, impact, for how long)

These define…

Characteristics of Noise

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Duration of exposure, level of exposure, frequency spectrum of noise, distance from source

These are…

Contributing factors of NIHL

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Dehydration, heart disease, smoking, alcohol, diabetes

Health and Lifestyle risk factors that may interact with noise

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Public health issues, educate parents and families, discuss hearing loss prevention versus hearing conservation to help patients identify sources of noise in his/her environment, provide appropriate hearing protection

The role of an audiologist

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Measure noise exposure levels, document compliance with OSHA guidelines, obtain baseline hearing measures, administer ongoing testing of employees, educate, provide, and monitor the use of hearing protection, may be involved in litigation

the role of an audiologist in the industry

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Information about what sounds can cause damage
Wear hearing protection
Be alert to hazardous noise in the workplace
Protect children (who can’t protect themselves)
Baseline audiogram
Public Awareness

NIHL Prevention

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Typically attenuates high frequencies to greater extent than low frequencies
Rated approx.. 10 dB more effective than reality (likely due to usage)
Flat attenuation earplugs
Special Earplugs for musicians, specific needs

Hearing Protection

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140 dBA impulse (Impulse/impact noise – less than 0.2 seconds)
Immediate Hearing loss; Both temporary & permanent conditions
Often accompanied by head trauma
May be otoscopic evidence of damage to TM
May be ossicular damage
May be oval/round window fistula
Pure Tone thresholds range from mild to severe (in rare case – complete loss)
May have poor speech discrimination
May have spontaneous nystagmus and/or reduced caloric response
Hearing will often improve for a few days/weeks after acute trauma
Complete recovery depends on the extent of damage

This is indicative of…

Acoustic Trauma

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Direct Damage to stereocilia/hair cells
Mixing of perilymph and endolymph
Casacade
ROS
If recovery, usually see change within 1 month or 3-6 mos

Pathophysiology of Acoustic Trauma

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What is indicative of central & peripheral auditory system changes

Age-related hearing loss (Presbycusis)

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65 – 75 years: 30 -35%
75 years +: 40 – 50%
90 years +: > 90%

Incidence/Prevalence of Presbycusis

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Bilateral, symmetric, SNHL (high frequencies most affected)
Progressive hearing loss with age
Progressively poorer word recognition scores
Gradual onset
Negative history of noise exposure
Normal otologic exam

Clinical Characteristics of Presbycusis

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DPOAES: decline independently from thresholds in aging
SRT: increase w/age; greater difficulty understanding comfortably loud speech (esp. in noise)
Tymps: usually normal (woman – decreased compliance)

Test results for Presbycusis

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Loss of tissue elasticity of pinna and ear canal
Stiffening of tympanic membrane
Degeneration of middle ear musculature
Calcification of ligaments
Ossification of ossicles
Inner Ear: Major structures degenerate independently

Pathophysiology of aging on the Outer and Middle ear

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According to the Schuknecht Study, impact to this part will result in a bilateral precipitous high-freq SNHL and will initially commensurate speech discrimination ability

Sensory

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- Degeneration of hair cells & supporting cells @ base of the cochlea (more OHC) - Atrophy of the organ of Corti (affects auditory nerve) - Abruptly sloping, high-frequency loss - Starts at middle age & progresses slowly

Pathophysiology of Cochlear damage (sensory - Schuknecht)

According to the Schuknecht Study, impact to this part will result in a high-freq hearing loss; mild; poor speech discrim scores (early on); progresses rapidly with aging

Neural

- Loss of dendrites & then cochlear neurons - Audiogram: loss of neurons prior to loss of IHC may not affect hearing sensitivity until up to 90% of radial afferent neurons have degenerated (Expect speech understanding to be worse than anticipated from audiogram) - Primary: neuropathy - Secondary: hair cells die, no input, nerve cells die - Can cut ½ nerve with out changing thresholds - Can lose up to 90% without threshold change

Pathophysiology of Neural damage (neural - Schuknecht)

According to the Schuknecht Study, impact to this part will result in flat SNHL with good preservation of speech understanding

Strial/Metabolic

- Degeneration of stria vascularis (Disruption of nutrient supply & changes in cochlear electrical potentials) - Audiogram: flat; reasonable speech discrim scores - Decrease in endocochlear potential correlated with poorer thresholds - Larger genetic effect for this pattern

Pathophysiology of Strial/Metabolic damage (Strial/Metabolic - Schuknecht)

According to the Schuknecht Study, impact to this part will result in a sloping SNHL, including low freq, speech understanding good

Mechanical (Cochlear Conductive)

- Changes due to mass/stiffness changes/atrophy of the spiral ligament

Pathophysiology of Strial/Metabolic damage (Mechanic - Schuknecht)

Not Schuknecht category - Hearing loss has a significant effect but may occur in the absence of hearing loss - “Can’t hear in background noise”

Central damage

Age overall impacts...

Working memory, speed of processing, attention & the brain, senses (touch, vision as well as hearing, central auditory processing)

* Diabetes * Hypertension * Hypercholesterolaemia * Surgeries (anaesthesia) * Prescription drug ototoxicity * Hormones, sex * Cardio/microvascular * Diet (antioxidants good) These are all ___________

Risk factors for hearing

This ability deteriorates with age, especially from the seventh decade on

Speech-in-noise

This increasingly affects speech understanding as listeners age

Reverberation

This ability is highly correlated with their ability to understand speech in babble and reverberation

The ability of elderly listeners to segregate non-speech streams using only envelope information

hearing loss matters a lot....

low context

hearing loss matters less....

high context

- Changes with aging - Estrogen seems to be protective (thresholds, word rec decline with menopause) - Women aon Hormone replacement therapy do better postmenopausal than those not on HRT - Aldosterone levels associated with better thresholds

Hormones

- Early identification is important (social, psychological, economic consequences) - Increased success with HAs - Disuse atrophy/plasticity (Cortical reorganization)

Presbycusis Identification

A screening tool for Presbycusis

Hearing Handicap Inventory for the Elderly – screening version (HHIE-S)

- Depression - Confusion - Inattentiveness - Increased tension - Negativism Is often confused with....

Hearing Impairment in Elderly

- Poor general health - Reduced mobility - Reduced interpersonal communication Is often confused with....

Hearing Impairment in Elderly

Counseling, alleviation of symptoms, HAs, ALDs, and Group Rehab are all ....

Presbycusis Treatment & Rehabilitation Options

- Lifestyle - Hearing aids and/or assistive listening devices - Financial constraints - Measuring hearing aid benefit - Quality of life - Family involvement Should all be considerations for....

Rehab

Anything beyon the cochlea is defined as...

Retrocochlear

- 80 -85% of CPA tumors (MOST COMMON) - 5 – 10% of intracranial tumors - 3:2 female to male - About 50 yrs old, mostly White (black, Hispanic, & Asian populations were more likely to present with large tumors) - Affects schwann cells on vestibular part of VIII nerve - Cellular transformation/proliferation of schwann cells This is the epidemiology of....

Vestibular Schwannoma

Where is the nerve of origin for Vestibular Schwannoma?

Obersteiner – Redlich Zone (where Schwann cells & oligodendrocytes meet)

- Nerves 5 (trigeminal), 7(facial), 8 - AICA artery - Contains CSF This describes....

Cerebellopontine Angle (CPA)

- 8.5mm in length - Lined w/dura & filled w/spinal fluid - CN7 & CN8 - Where the peripheral & central systems meet This describes....

Internal auditory Canal (IAC)

- Benign; Often characterized by slow growth - 5% are NF2 patients (less than 30 yrs, bilateral) - 95% sporadic - Idiopathic This is...

The natural history of Vestibular Schwannoma

- Unilateral/Asymmetric hearing loss (most common symptom) - Usually high freq/can be mid-freq - Sudden/fluctuating 5-15% - Unilateral Tinnitus - Headache - Balance disturbance/imbalance/dizziness - Episodic vertigo - Facial weakness/facial numbness/facial palsy - Aural fullness - Ataxia (loss of control of bodily movements) - Otalgia/jaw pain - Hyperacusis These are...

Signs and symptoms of Vestibular Schwannoma

- Pure tone: asymmetry - Speech Discrim tests: asymmetry - Poor discrim for thresholds  Will decline even if no/little tumor growth - PI/PB function (rollover) - Immittance audiometry - AR ~60% sensitivity if present - Tone decay – rarely - ABR, stacked ABR I-III & I –V intervals, Wave V latency difference, ABR peaks absent, misses small tumors - Vestibular testing (ENG) - tells about superior vestibular nerve (SSC, utricle) - VEMPS - tell about inferior vestibular nerve (saccule) - DPOAEs decreased in some cases This is...

Diagnostic Results for Vestibular Schwannoma

- Potential effect of tumor on nerve – compression - Effect on blood flow - Change in gene expression secretion This is...

The pathophysiology of a Vestibular Schwannoma

- MRI for diagnosis (gold standard) - Observation - Surgery - Gamma knife treatment - radiosurgery noninvasive treatment option (delayed hearing loss) - Wait & see - for elderly patients, small tumors, refusal of treatment, tumor on side of only hearing ear - If you take it out ---> higher likelihood of preserving hearing This is...

Management for Vestibular Schwannoma

This is a surgical approach for VS where hearing is sacrificed but it is a good identification of CN VII and there is less risk of CSF leak

Translabyrinthine

This is a surgical approach for VS where hearing preservation is possible; it's excellent for intracanalicular tumors but there is lack of access to CPA (need to retract temporal bone)

Middle Fossa Approach

This is a surgical approach for VS where hearing preservation possible; there's access to CPA, limited access to IAC, difficulty repairing CN VII

Retrosigmoid/Suboccipital

Advantages: Decreased length of stay, decreased cost, rapid return to full employment, lower immediate post-treatment morbidity & mortality Disadvantages: Necessity for regular monitor & frequency re-scanning, does not eliminate the tumor & has higher recurrence rates, higher incidence of trigeminal nerve injury

Gamma Knife Intervention

- Hearing loss, Facial nerve injury, Cerebellar damage, Arterial occlusion/hemorrhage, Meningitis, CSF leaks, headache

Complications of surgery

- Medical Referral & support - Monitoring - Rehabilitation HA, CROS, BAHA, CI, ABI, Vestibular Rehab

Audiologist's Role

- Nothing to do with VS - Peripheral tumors - Café au lait spots >6 - AD

Neurofibromatosis 1 (NF1)

- 15% of Intracranial tumors & 3% of CPA tumors - Benign & do not metastasize - Locally aggressive because they invade bone - Put pressure on nerves, brain vascular Has a characteristic shape on imaging: close to brain tissue with a “tail” Which CPA tumor is this?

Meningiomas

- If bilateral VS/young (<30 yrs.) - This gene on chromosome 22 (22q11-13.1) Autosomal dominant Bilateral VS or Eye tumors Genetic testing to detect mutations Café au lait spots < 6 -Faster growing vs. VS What does this describe?

Neurofibromatosis 2 (NF2)

- ~20% of all vertigo, 2% lifetime prevalence - Brief mild to intense vertigo  Triggered by changes in head position (getting out of bed, reaching up to a high shelf) - Episodes usually less than 30 seconds (Otoconia/otoliths out of the utricle) - Peak incidence in the 50s; Rare < 35 yrs unless head injury - Associated with head injury & vestibular neuronitis - May be present with VS*** - Nystagmus with Dix/Hallpike diagnostic - Treated with Epley, Semont Do maneuver to get everything right in back semi-circular canal - HEARING LOSS MIGHT NOT BE PRESENT

BPPV: Benign Paroxysmal positional vertigo

a disorder of the inner ear characterized by dysfunction in the fluid balance regulating system

MENIERE DISEASE

- Incidence 0.2 – 2% - Slightly more common in those of European descent - Women > Men - Incidence highest in 40s and 50s (has been reported in children) - Associated with a history of allergies, smoking, virus, or respiratory infection, aspirin, stress, ETOH, fatigue, autonomic nerve disease What is this?

Epidemiology of Meniere Disease

* Stage 1: <=25 * Stage 2: 26 – 40 dB * Stage 3: 41 – 70 dB * Stage 4: > 70 dB What does this desribe?

Stages of hearing loss in Definite/Certain Meniere’s

- Endolymphatic hydrops  leads to distortion of scala media (membranous labyrinth) - Idiopathic What does this describe?

Etiology of Meniere's Disease

* Fluid imbalance - Perilymph ---> similar to CSF, High Na+, Low K+ - Endolymph (in stria vascularis) ---> similar to Intracellular fluid (ICF), Low Na+, High K+ - Membranous labyrinth separates the two (difference of 80 mV in charge & no pressure difference) * Hydrops - Obstruction of endolymphatic duct/sac? (No evidence in animals) - Alteration of endolymph absorption? - Autoimmune? - Small endolymphatic aqueduct or sac? * Build-up in pressure may lead to micro-ruptures of membranous labyrinth - May explain episodic nature of attacks - Healing of ruptures may account for return of hearing - Apex wound more rightly than base, bigger effect? * Hydrops alone do not seem to cause this...

Pathophysiology of Meniere's Disease

may include symptoms of tinnitus, fullness & fluctuating hearing loss WITHOUT vertigo

“Cochlear hydrops”

- hearing loss fluctuates, low freq SN initially, but declines with time across frequencies - May not be most disturbing symptom unless interferes w/work - Reduced discrimination, tinny - Recruitment, dipaclusis (ears perceive different pitch) - Usually begins unilaterally, but 40% will become bilateral - Distortion & fluctuation - Tinnitus, - Episodes of vertigo ---> you or your environment is moving/ illusion of movement - Subjective: patient moving - Objective: environment moving - Drop attacks (vertigo so disabling they collapse) - Can be resolution of vestibular symptoms (burn out) - Aural Fullness, - Fluctuation - Symptom-free periods - VARIABILITY What are these symptoms of?

Meniere's Disease

vertigo at least 20 min, usually last 1-2 hours, can be up to 24 hours - nausea, vomiting - feel tired, unsteady & nauseated for hours – days - Interim- symptom free, but may deteriorate - timing & freq variable, may be able to predict Triggers: diet, menstrual cycle, stress What does this describe?

Vertigo attack associated with MD

- Episodic vertigo w/out documented hearing loss - SNHL fluctuating or fixed with disequilibrium but w/out definitive episodes - Other causes ruled out ---> always there What does this describe?

Possible MD

- One definitive episode of vertigo - Audiometrically documented hearing loss on at least one occasion - Tinnitus or aural fullness in the treated ear - Other causes excluded What does this describe?

Probable MD

- 2+ spontaneous episodes of vertigo 20mins or longer - Audiometrically documented hearing loss on at least one occasion - Tinnitus or fullness in the affected ear What does this describe?

Definite MD

Definite MD + histopathologic confirmation (Temporal bone pathology) What does this describe?

Certain MD

- Can have resolution of vestibular symptoms with no surgical treatment (8-10 years = burn out) - Study found: Long term PTA in affected ear ~50 dB; Speech discrim 53%; Caloric response reduction 50% - Hearing loss: Early stage  unilateral SNHL low freq rising to normal - Hearing loss: Late stage  bilateral HL, one worse than other, flat across freq - Hearing loss doesn’t get worse with vertigo What does this describe?

The natural history of MD

- Case history - Complete hearing test including LDLs (reduced dynamic range) - Vestibular, especially for bilateral * C-VEMP – (Saccule, ipsi, IVN)  likely absent in symptomatic ears * O-VEMP – (utricle, SVN, complex)  likely absent in symptomatic * ENG – SVN * Dix-Hallpike BPPV - Possibly, ECOG, ABR< EEG, DPOAES, glycerol (rare) * ECOG --->SP/AP ratio > 0.5 * Glycerol reduces hydrops - RULE OUT tests: blood tests, allergy tests, MRI This describes...

Diagnostic testing for MD

- Conservative treatment – acceptable results (control of vertigo) - Lifestyle changes * Regular meals; avoid triggers * Low salt, No MSG, caffeine, smoking, ETOH - Medical treatment: decreasing the amount of fluid in the inner ear * Low salt diet  salt believed to alter fluid balance in inner ear leading to depletion of endolymph * Diuretic  not great research suggesting this works * Vasodilators ---> allow the blood to flow more freely - Acute therapy ---> Meclizine, Benzodiazepines, Corticosteroids - Maintenance therapy ---> no conclusive studies show efficacy of drugs intended to alter disease course of Meniere’s - Other management: placebo effect? * PE tubes, Antiviral drugs, Anti-nausa, Homeopathy, Herbal  gingko, Chiropractic, Acupuncture ---> positive case studies What does this describe?

Management of MD

Dietary modification

1st Level Management of MD

Meniett, Intratympanic corticosteroids, Endolymphatic shunt, Intratympanic gentamycin, Endolymphatic sac vein decompression

2nd Level Management of MD

pressure pulses delivered via probe in ear canal, portable low intensity alternating generator – “ promotes the flow of endolymph out the cochlea, alleviating the hydrops & relieving the symptoms”

Menniett

preferred because of gentamicin’s vestibuloselectivity, side effects = temporary imbalance or nystagmus, & hearing loss; relieves symptoms but not always

Intratympanic gentamycin

supported to address the site of obstruction causing hydrops. Types = decompression, shunting, drainage, removal, damage; debate on if effective or not

Endolymphatic sac vein decompression

- Vertigo is usually the initial concern * Short and midterm very disruptive * Control is usually successful long term - Hearing loss and tinnitus * No treatment shown success for preventing decline of hearing & decreasing tinnitus - Long term expect far more effect on QOL - Functional Level Scale (completed by the patient) * How Meniere’s disease function is assessed - Rehabilitation *Physical/audiological issues -Fluctuating HL --->hearing aids w/multiple memories (best hearing & worst hearing) -Emotional Issues * Stress of illness * Restriction of activities - Vestibular rehabilitation What does this describe?

Audiologic Management of MD

- Etiology - Diagnosis - Role of genetics, autoimmunity, viruses, environment - Fluid dynamics - Changes at the molecular, cellular and organ level What does this describe?

What remains unresolved about MD

* Recognize emotional & social aspects * Reasonable expectations * Support for identifying triggers * Use of earplugs to protect from noxious noise * Support use of amplification * Support groups What does this describe?

The essentials of counseling for MD

* Recurrent episodes of headache with light/sound sensitivity * Nausea, vomiting * Unilateral What does this describe?

Migraine

* Intense rocking swaying sensation * Usually after cruise * More common in women * Etiology unknown * May not resolve * May resolve with vestibulo-ocular reflex reset What does this describe?

MAL DE DEBARQUEMENT

* Vertigo, imbalance, dizziness, unsteadiness, motion sensitivity (may last > 24 hours) * Muffled hearing, tinnitus, fullness * Hearing loss  very uncommon, if present usually low freq, rarely progressive What does this describe?

Vestibular Mirgaine

vertigo lasts up to 24 hrs, SNHL nearly always progressive/most often unilateral/fluctuation common, tinnitus may be unilateral or bilateral and often significant intensity, photophobia never present The main differentials for...

vertigo may last longer than 24 hrs., SNHL very uncommon, tinnitus may be unilateral or bilateral but rarely obtrusive, photophobia is often present and may be associated with dizziness

Vestibular Migraine

* 1/1000 Clinically: when cerebellar tonsils extend through foreman magnum * Type 1, may not be diagnosed * Dizziness, muscle weakness, numbness, vision problems, headache, progressive SNHL, tinnitus & problems w/coordination * Occipital headache with valsalva * Post surgery tinnitus What does this describe?

CHIARI MALFORMATION

* Abnormal connection between inner and middle ear that allows leakage of perilymph * Like MD also a fluid imbalance (in both too much endolymph relative to perilymph theory) * Prevalence, diagnosis, treatment all controversial What does this describe?

PERILYMPHATIC FISTULA

- Hearing loss (83% - 90%) - Fluctuating SNHL, sudden or progressive and/or fluctuating word discrimination - Vestibular symptoms * Vertigo with or without head position changes * Dysequilibrium (off-balance), nausea, and vomiting * Tullio & Hennebert signs - Tinnitus  may be roaring - Aural fullness What does this describe?

Signs & Symptoms of Perilymph Fistula

* Physical trauma (including temporal bone fracture) * Acoustic trauma (18 inches from fire engine siren) * Barotrauma * Spontaneous * Ear surgery, esp. stapes surgeries * Neoplasms, cholestomas * Congenital especially cochlear abnormalities What does this describe?

Etiology of Perilymph Fistula

sensation of self-motion when no self-motion is occurring or the sensation of distorted self-motion during an otherwise normal head movement

Vertigo

"sensation of disturbed spatial orientation without false or distorted sense of motion”

Dizziness

* Case history * Battery of test: * Fistula test – pressure to ear * Look for nystagmus * Tymp * ECOG – elevated SP/AP * ENG * VEMP * Exploratory surgery * MRI w/intrathecal gadolinium (AIR IN COCHLEA BAD!) * NO GOLD STANDARD What does this describe?

Diagnostics & Testing for Perilymph Fistula

* Bed rest * Avoidance of straining * Reoccurs 21 – 47% of patients * Exploratory tympanostomy & repair What does this describe?

Treatment for Perilymph Fistula

* Vestibular neuritits * Vestibular, vision & concentration * Viral/bacterial infection of the vestibular nerve *May have long recovery or be chronic * BPPV * Ototoxicity What does this describe?

Episodic vertigo WITHOUT hearing loss

* Labyrinthitis * Hearing loss, tinnitus, vestibular * Viral/bacterial infection of the labyrinth * Serous or Suppurative * Gradual recovery or chronic What does this describe?

Episodic Vertigo WITH hearing loss

Clinical syndrome characterized by electrophysiological evidence of normal or near normal cochlear function and absent or abnormal auditory pathway transduction *Some sort of disconnect between OHCs and nerve itself; not necessarily one site of lesion; people will present differently*

Auditory Neuropathy

* Normal outer hair cell function as measured by present OAEs or the presence of a cochlear microphonic * OAEs may be present initially but disappear over time, or be absent at time of diagnosis * Abnormal auditory nerve response as observed by absent or markedly abnormal ABR * Acoustic reflexes are absent in most cases What does this describe?

Audiological Findings of Auditory Neuropathy

* PT thresholds ranging from normal to profound * Disproportionately poor speech recognition abilities for the degree of hearing loss * Difficulty hearing in noise * Impaired temporal processing * Hearing fluctuation * Some individuals have little or no communication difficulties while others are functionally deaf * abnormal ABR with present CM * NOT ALL INDIVIDUALS DIAGNOSED WITH THIS EXPERIENCE THE SAME PROBLEMS OR TO THE SAME DEGREE What does this describe?

Clinical Characteristics Reported for ANSD

* Disorder initially thought to be rare * Many published reports since late 90's describing patients with similar audiologic test findings. * Estimates range from 7 -10% of children diagnosed with permanent hearing loss What does this describe?

Prevalence of ANSD

* Genetic: *Syndromes  Charcot-Marie-Tooth disease; Friedrich’s Ataxia; Hereditary motor and sensory neuropathy (HSMN) * Non-syndromic  Recessive genetic mutations: Otoferlin (OTOF), Pejvakin (PJVK)  Autosomal dominant mutations: AUNA1 (onset of auditory symptoms in late teens) * Perinatal Conditions * Hyperbilirubinemia * Hypoxia * Low birth weight * More common in premature infants * Congenital Condition: Cochlear nerve deficiency (Small nerve or absent nerve) * Infectious Processes: Viral infections (e.g. mumps, meningitis * Head injury: E.g. Shaken baby syndrom

Possible Etiologies and Associations for ANSD

* ABR * Immittance - Tymps - AR * OAEs * Behavioral Audiometry * Speech Recognition testing What does this describe?

Recommended Audiologic Test battery for ANSD

* Pre-neural response (occurs before wave 1 in the ABR) * Unlike the ABR, this shows a direct phase relationship to the acoustic waveform. When the polarity of the stimulus is changed there is a reversal of this waveform as well * this is the only one that changes * Considered to have limited clinical use in past; renewed interest in diagnosis of ANSD * Can be recorded in normal ears, ears with “typical SNHL” and ears with ANSD * Significance in ANSD is this is present when neural response is absent or markedly abnormal * Have to have good recording conditions * Use single polarity clicks at high-intensity levels * Must use insert earphones What is being described?

Cochlear Microphonic

If response remains with sound tube disconnected from transducer, response obtained is __________ and not ________

Stimulus artifact; CM

* Medical history * Ear exam * Etiology * Evaluate for other associated problems *Seizures, Motor delays, Visual problems, Ear canal problems, Otitis Media * Radiologic Studies (MRI/CT) * Inner ear malformations, Cochlear nerve integrity * Other studies as needed What does this describe?

Otologic Examination

* Child has an auditory disorder but its difficult to know prognosis at time of ABR eval * Degree of deficit may be mild or severe or even normal hearing thresholds * Results of behavioral testing are necessary before specific recommendations can be made * HA use is helpful in some cases but not in others * CI may be necessary * Frequency follow up needed * Enrolled in early intervention * Communication strategy will need to be determined * Work together as a team to find a solution What is being described here?

ANSD counseling for families

- Amplification should be fitted as soon as ear specific elevated pure-tone and speech detection thresholds are demonstrated by conditioned test procedures - Hearing aid fitting strategies… should follow established guidelines for the fitting of amplification in infants and toddlers

Amplification Guidelines for ANSD

- Evidence of auditory nerve sufficiency should be obtained prior to surgery using appropriate imaging technology - Children with ANSD who do not demonstrate good progress…

ANSD Guidelines (COMO 2008): Special Considerations for CI

- Parent questionnaires (e.g. PEACH, IT_MAIS, or MAIS) - Early speech perception test battery * Standard *Low verbal - MLNT/LNT words and phonemes - PB-K words and phonemes - CNC words - HINT sentences in quiet and in noise conditions What is this?

Evaluation of Speech Perception Following Hearing Aid Fitting or Cochlear Implantation

* Needs to have child awake but quiet * The shorter the gap detection, the higher the PBK word score (research study) * evaluates outer ear to auditory cortex

CAEP (Cortical Auditory Evoked Potential)

evaluates outer ear to lower brainstem

ABR

* Not Conductive! Duh * > 30 dB loss at 3 contiguous frequencies occurring w/in 24 – 72 hours * Usually unilateral (Bilateral 1-5% of cases) * Mild – profound, may be upsloping * Tinnitus ~50% * 32 – 65% spontaneous recovery *Trend for older age to have poorer recovery * Fullness, much less common * Vertigo (less common) ~25% * Age: typically 40 -54 years (can have kids)

SYMPTOMS OF SUDDEN SENSORINEURAL HEARING LOSS

Which disorder excludes * Pre-existing hearing loss * Bilateral hearing loss * Low freq hearing loss (M’S D) * Oscillopsia * Signs of neurological/optical dysfunction * Signs of stroke * Head trauma * Acoustic Trauma

Sudden Sensorineural HL

* Idiopathic * Blood supply – vasospasm * Autoimmune: Cogan syndrome, systemic lupus erythematosus, and other autoimmune rheumatologic disorders * Intracochlear membrane rupture * Viral infection (bacterial) What is described?

Pathophysiology of SSNHL

* Viral (syphilis, meningitis, encephalitis) * Other infection – toxoplasmosis etc * Meniere D * Neoplasms (CPA tumor) * Vascular abnormalities/ Endocrine abnormalities (Hyothyroidism, Diabetes mellitus) * Multiple Sclerosis * Congenital anomalies (Mondini, EVA) * Trauma, PLF * Ototoxicity * Lyme * Virus ---> immune/autoimmune response  HL?

Differential Diagnosis for SSNHL

* Genetic polymorphisms * Endothelial function * Migraine * Statin use * Cardiovascular risk factors * Periodontal disease? * Chronic otitis media? * Smoking, alcohol use * Diabetes Are all _____________ for this....

Risk factors for SSNHL

* PT, Speech, immittance including AR * OAEs (tell you if cochlear or retrocochlear cause) * ABR * Vestibular tests usually only if vestibular symptoms * MRI  only if suspicion of tumor This is what?

Testing for SSNHL

* Considered otologic emergency ---> ENT more likely to treat w/corticosteroids & for long time (may add in antivirals) * High spontaneous recovery rate so hard to demonstrate significant efficacy * Counseling * Communication strategies; may become HA candidate What is being described?

Referral for SSNHL

* Anti-inflammatories  steroids * Hyperbaric oxygen * Both combined * Enhanced sound (prevent cortical reorganization) What is being described?

Tx for SSNHL

* Age younger than 15 yrs or older than 65 yrs * Elevated ESR inflammation measure * Vertigo of vestibular change evident on ENG * Hearing loss in opposite ear * Severe/profound hearing loss * Late presentation * Family history What is being described here?

Negative prognostic factors for SSNHL

more likely profound, 50% no recovery, more commonly associated with life threatening conditions Pharmacological ----> speedballing, Imatinib, heroin, cocaine, methadone overdose What is being described?

Bilateral SSNHL

Bilateral SNHL of at least 30 dB at any frequency with progression in at least one ear, defined as a threshold shift that is greater than 15 dB at any frequency or 10 dB at 2 or more consecutive frequencies or a significant change in discrimination score.

AUTOIMMUNE INNER EAR DISEASE (AIED)

* Progressive, often fluctuating, bilateral (79%) SNHL over a period of weeks to months * Poor speech discrim * Vertigo, generalized imbalance, and ataxia * Tinnitus (roaring, ringing, hissing) * Aural fullness * Excludes SSNHL occurring in less than 24 hours * Existing AI disease ----> lupus, ulcerative colitis, rheumatoid arthritis, scleroderma

Signs & Symptoms of AIED

* 20 – 50 years * Possibly more woman than men

Epidemiology of AIED

* Immune reaction to inner ear antigens * Circulating immune complexes

Pathophysiology of AIED

PLF, Meniere’s disease, SSNHL are all ....

Differentials of AIED

* Audiometry, possibly vestibular * Antigen nonspecific: * Antigen specific tests

Testing for autoimmune

* Medical emergency ----> refer * IF under treatment: * Monitor hearing, balance * Possible amplification * Communication strategies * Counseling What is being described?

Audiologist's role in AIED

- Corticosteriods (response is diagnostic, positive response ~60%) Others - Cyclophosphamide – anti-tumor - Methotrexate –anti-inflammatory - Plasmapheresis What are these?

Tx for AIED

autoimmune disorder * Rare – 200 cases in literature * Interstititial keratitis (eye inflammation) * Hearing loss & vestibular impairment * Hearing loss ---> biilateral and permanent * Meniere like symptoms, starts with vestibular than hearing loss

Cogan's Syndrome

- Genetic, hemoglobin change * Some protection from malaria * 1/500 African – Americans - Vaso-occlusive episodes (pain, esp. joints), organ damage - Hemolytic anemia (blood cells fall apart) - Longer life  seeing more ontological complications - Hearing loss: EVERYTHING – bilateral, unilateral, gradual, sudden - Other tinnitus and vertigo possible

Sickle Cell Anemia

- Sickled RBCs ----> impaired oxygen supply - Compression of the auditory canal and auditory nerve by the hyperactive bone marrow in the petrous part of the temporal bone - More susceptible to bacterial meningitis due to decrease immune function

Pathophysiology of Sickle Cell Anemia

(in womb is damaging to auditory system) - Brain damage, MR, growth retardation, hearing loss in babies - Pendred is euthyroid - Common in adults * 1.4-2% of women; 0.1-0.2% of men * Increases with age * Changes body temperature ----> will affect evoked potential

Hypothyroidism

mitochondrial genetic syndrome - 2/3 of adults have hearing loss (~2x greater risk of HL) * Metabolic disease * Type 1: lack of insulin, often identified in children ----> could cause neuropathy * Type 2: hyperglycemia, insulin resistance, lifestyle

DIABETES: MELAS

* Microvascular * Inflammation * Hyperglycemia: smoking increases risk * Glucose control - Energy source - Hair cells & spiral ganglion cells (spiral lamina most affected) - Chronic hyperglycemia can disrupt function * Microangiopathy: the thickening of basement membrane of the capillaries within the stria vascularis * Neuropathy * ROS What is being described?

The Diabetes Mechanism

* Thresholds * More difficulty in noise * Epithelial changes may affect earmold comfort * Danger for malignant otitis externa after EMI or cerumen removal What is being described?

Audiological Effects of Diabetes

* Cardiac health risk factors ---> smoking, waist circumference, cholesterol, BP * This is related to heart attacks * Association studies ----> good heart health = good hearing * Coronary artery disease linked to poorer hearing * Hypertension Proposed Pathophysiology: Decreased vascular supply to stria vascularis * Hypothesis: low freq. HL as a predictor of cardiovascular status not the other way around * Shuknect: strial HL

Hypertension/Cardiovascular

- SSNHL - Treatment: apheresis (clean out the blood)

Hypercholesterolaemia/hyperlpproteinemia (basically high cholesterol)

- SSNHL - Microembolisms (particles from ateriosclerotic plaques, fat or air) - PLF - Tears in scala - Risk factors: contributors to presbycusis, factor in decreased hearing in adults

Cardiopulmonary surgery

* Acute (24-48hr), idiopathic, unilateral, peripheral, lower-motor neuron facial nerve paralysis * Resolves over time in 80 – 90% of cases (especially if mild case, not elderly) * 20-30/100000 * More common in diabetics, pregnant women * Treat with corticosteroids & antivirals What is being described?

Bell's Palsy (CN VII)

* Facial muscle weakness * Eyelid closure * Aching of the ear or mastoid * Alteration of taste * Hyperacusis, tinnitus * Tingling or numbness of cheek/mouth * Tearing * Visual signs * Rarely SSNHL or progressive HL, may be low frequency * May show up in AR * Bilateral palsy: Guillain-Barre Syndrome- ---> REFER: autoimmune response What is being described?

Symptoms of Bell's Palsy (CN VII)

* Twitching (myoclonus) * Irritation of VII or its nucleus * 40+, may be a sign of MS in younger age

Hemifacial Spasm

* Neuropathic pain * 50+

Trigeminal neuralgia V---in Ramsay Hunt Syndrome

* In kids especially – significant symptom: Facial paralysis * SSNHL – take history look for test for Borrelia burgdorferi * Not much on gradual hearing loss, but some reports of HF hearing loss * Tinnitus common

Lyme Disease

* Anterior inferior cerebellar artery (AICA) stroke (2nd most common brainstem stroke: * Vertigo, tinnitus, hearing loss, alone or with facial weakness, hemiataxia, hypalgesia * Retrocochlear: middle cerebellar peduncle, cerebellar flocculus, inferolateral tementum of the pons or cochlear * Vascular loops * Association of stroke with both increased hearing loss and increased risk of SSNHL * Ischemic and hemmoragic

Stroke

* Association between CKD and HL * Dialysis patients especially at risk

Chronic Kidney Disease

* Inflammatory, demyelinating disease of the central nervous system * Diagnosis is described as definite, probable or possible _____ * Chronic progressive, relapsing remitting * New treatments may change * Disease of young adults ---> occurs in 20s

Multiple Sclerosis (MS)

* Hearing loss rarely the first sign * Sudden, usually fluctuates, recovers * Retrocochlear ---> testing * Vertigo relatively common, initial symptom * Imbalance and disequilibrium more commong * Testing: PT, speech (may be poorer) AR pattern, ABR, vestibular, MRI

The auditory system of MS