SHANZ – ENT 1.

03 Inner Ear: Hearing loss and aural rehabilitation

HEARING LOSS
INFANT CHILDREN ELDERLY
STAT • Foreign: 1-3/1000 VS PH: 5-6/1000 • Decreasing incidence with age • 3rd most commonly reported chronic problem of aged
• 50% incidence in high risk conditions: • Eustachian tube remodeling: population
o FMH (congenital/acquired) o short and horizontal → long and diagonal • Presbycusis in 8% (50% due to noise-induced if <50 yo)
o CNS infection (during pregnancy) • lesser acute infection due to nasal problem • 25% of 51-65 yo have hearing loss > 30dB in 1 ear
o ENT defects (cleft lip, cleft palate, microtia) • Prevalence: • 33% among >65 yo
o Ototoxic drugs <3 yr 12% • >50% among >85 yo
o Prematurity 4-5 yr 4-18% • starts as early as 4th decade
o HyperBILIRUBINEMIA 5-8 yr 5-7% •
o LBW < 1500 gr 6-8 yr 3-9%
Char • Silent, hidden handicap • Impacted cerumen: most common cause (improper cleaning) • Correlated with social and emotional isolation, clinical
• Stagnant reading comprehension scores o Losing resonant high frequency (4 kHz); net loss 25 dB depression, limited activity
Patho o only hear whisper 15 dB from normal speaking voice 40 dB • Atherosclerosis → diminish perfusion + hypoxia of
NOTE: • Otitis media (43-83%): acute/recurrent/chronic cochlea
Phonemic Sounds are differentiated by HIGH frequencies o 25 dB loss due to effusion • Noise exposure → accumulation → damage hair cells
o level of conversational speech upto 45 dB (if fluid is thicker) • Loop diuretics → ototoxic to stria vascularis (affect all
• Perforated eardrums: most damage hearing loss frequencies)
o lowest frequency • Aminoglycoside → damage cochlear hair cells
o bigger perforation = increase HL (depends on location) • Streptomycin, Gentamycin → Damage vestibule
o HL inversely proportional w/ air vol @ middle ear/ mastoid • Amikacin → affect cochlea
• Cisplatin → cochlear high tone loss
• Quinine → Reversible hearing loss (all frequency)
• Salicylate → mild/mod cochlear impair
• Diet & Metab → poor cochlear perfusion
• Uremia → direct labyrinth damage
• Genetic → early aging of auditory system
• Solvents: nitrobenzene, aminobenzene
• Heavy metal: Lead, mercury, arsenic
• Recreational drugs: alcohol, heroin, tobacco, cocaine
DX Behavioral Moro reflex: Startled on loud sound (normal) • Visual Re-inforcement audiometry • Pure tone audiometry (PTA)
Developmental milestones (check Hx) • Behavioral Audiometry: Puretone/ Speech audiometry • Speech discrimination score
Objective Otoacoustic Emission test (OAE) • High tone audiometry • Tympanometry
Auditory Brainstem Response (ABR)
TX • Early detection: • Impacted cerumen: Remove cerumen
o prevent delayed speech & language development • Acute/recurrent Otitis Media: Incise + Antibiotic & eardrop
o prevent social & emotional problems • Chronic otitis media: Operate
o prevent academic failure
• Joint committee on infant hearing:
o Language, cognitive & social dev. at par if fitted at 4 wk
o Intervention <6 months: language score ~ normal
o Intervention >6 months: language score < normal
o Early detection: minimize rehab during school years
• Goals in Universal Hearing Screening:
<1 mo Screen for hearing loss
<3 mo Specialist evaluation
<6 mo Intervention/ Rehab
 DEVELOPMENTAL HEARING MILESTONES
1-3 mo 3-6 mo 6-10 mo 10-16 mo 15-20 mo 20-24 mo
• Quiet when hearing • Lateralize head to • React to music: cooing • Know names of fav toys (points • Follow simple direction (go get • Combine simple words (daddy work)
familiar voice search source of voice • Understand common word when asked) your shoes) • Refer to self by name
• Startled upon nearby • Enjoys rattle & noise (no, bye, night night) • (+)respond to rhymes & jingles • Recognize names of body part • Show interest in sounds of radio/ Tv
loud sound making toys • Babble (da, ba, ma) (peek a boo) • Speak 10-20 words
• Imitate simple words and sounds (understandable)

INFANTS: OBJECTIVE SCREENING TEST

OAE TEST ABR
• Screen neonate/ infant • Electrophysiological testing among infants
• Non-invasive, quick, easy, bedside • GOLD STANDARD
• Plug with sensitive microphone probe @ ear canal • Drawbacks: requires well-trained operator, lengthy 45min-1hr. costly, child must not move
• PREP: Quiet & steady child; quiet room < 40 dB • Measure potentials from auditory nerve & brainstem with electrodes (10 ms latency)
• Test integrity: outer hair cells of cochlea (sound produced by instrument) • Indication: getting “REFER” from OAE
• Retrograde transmission of vibration from cochlea to TM • Accurate to determine hearing function
• Detect: otoacoustic emission (active cochlear vibration) • Sensi Spec: 97-100/96
• Sensi Spec: 84/92
• Agreement rate w/ ABR = 91%
• PASS: Presumed can hear but check dev. milestone •
• REFER: Hearing problem (not yet HL); repeat after 1 mo
• REFER AGAIN: do ABR

CHILDREN: SCREENING TEST

VISUAL RE-INFORCEMENT
AUDIOMETRY
• Younger children
• Test one ear at a time
1. Child sit on parent’s lap
2. Distract with toy
3. Suddenly put on a cartoon
with sound
4. Turn it off and play with the
child again
5. Repeat 2x
6. on the 3rd time: put the
sound without cartoon
7. CAN HEAR = associate
with/ look to cartoon
BEHAVIORAL AUDIOMETRY (PURETONE/SPEECH) HIGH TONE AUDIOMETRY
• Older children (can follow instruction) • measure threshold 8-16 kHz
• most common method • greater inter-individual variation than routine audiometry
• Measures hearing sensitivity • use headphones/ special insert phone
Pure tone Speech • Prevent cross-hearing by MASKING (shift cochlea sensitivity of non-
• std. for hearing acuity • recognition / tested ear)
• Audiometer: measure sensitivity to pure sine waves understanding of • Effective masking: intensity is barely masked by noise to ipsilateral ear
(determine hearing threshold 125 Hz – 8 kHz) speech • Masking dilemma: BC thresholds for both ears are within normal limit,
• Hearing threshold: lowest dB level; can hear 50% of the time • Enter booth, make AC thresholds equal/ exceed interaural attenuation (reduction in sound)
• Test AC (with headphone) and BC; normally equal a sound in bilateral conductive / mixed HL
• Determine and plot objective and reliable hearing threshold • Patient raises his • Vibrator pressed against mastoid/forehead: measure BC threshold
hand • Cranial bones will vibrate and eventually transmit test sound to inner ear
• lower limit of bone conduction testing: 0 dB across frequencies

CHILDREN: PURE TONE AUDIOMETRY RESULTS

NORMAL HEARING CONDUCTIVE HEARING LOSS SENSORINEURAL HEARING LOSS MIXED HEARING LOSS
• AC & BC = between 10-20 dB • AC threshold > BC threshold • AC & BC below normal, no air=bone gap (<10 dB) • AC & BC are down
• AC = BC • BC = normal; AC below normal; air-bone gap > 10 dB • Hearing threshold raised (higher frequency) • Air bone gap > 10 dB
• AC perception requires higher loudness level • Greater AC loss = impaired sound conduction
• Increased BC thershold
 CHILDREN: AUDIOMETRY TYPICAL PATTERN
NOISE INDUCED HEARING LOSS MENIERE DISEASE OTOSCLEROTIC STAPES FIXATION (Carhart notch)
• Notch at 4-6 kHz • Low frequency hearing loss at onset • Conductive loss + BC notch at immediate frequencies

ELDERLY: ETIOLOGY OF HEARING LOSS

CONDUCTIVE SENSORINEURAL (PRESBYCUSIS) – SPEECH DISCRIMINATION
• 95% Occlusion • Measures phonetically balanced words that patient hears and repeats correctly
• age related atrophy of • evaluate candidate for cochlear implant
apocrine gland → decreased Sensory Mechanical Strial (Metabolic) Neural
watery component → dry hard Most common Noise induced, trauma DM, HTN Tumors
wax Audiogram Down sloping Down sloping Flat Flat
• Tragal hairs are longer, thicker, High frequency loss
coarser → cerumen trap SD Score Good Poor Good Poor
• History Histo Rapid degeneration of organ corti 2nd to basilar membrane changes Slowly progressive atrophy of stria vascularis Loss of spiral ganglion cells

CONDITIONS RELATED TO SD SCORE Used 20 monosyllable words (cat, dog)

EXCELLENT REDUCED FURTHER REDUCED EXTREMELY REDUCED 90-100% correct = normal
• Conductive HL • Cochlear sensory HL • CN VIII Lesion • Cortical lesion 75-90% correct = slight difficulty
• Stimuli have Sufficient • Stimuli within audible • Normal auditory pure • Unable to understand 60-75% correct = moderate difficulty
loud level range tone threshold speech 50-60% correct = poor

ELDERLY: TYMPANOMETRY (Objective screening/ diagnosis of central/peripheral auditory disorder)

Measure status of middle ear cavity (measure compliance: admittance/immittance of ear drum); Test movement of TM
TYPE A TYPE B TYPE C TYPE D
• Normal, Pressure peak 0 daPa • Middle Ear fluid (Serous OME, space occupying • Eustachian tube dysfunction • Scarred eardrum
AD AS lesion of tympanic cavity, TM perforation • Early stages of OM without • Normal, hypermobile eardrums
• Pressure peak: high • Ossicular chain fixation/stiffness • Flat perfusion or perforated TM • notch in pressure peak
• TM hypermobility • Otosclerotic stapes footplate fixation • No point of max compliance (no movement) • Pressure: slight negative
• Ossicular chain (atrophic TM scar) • Stiff thickened TM • Bulging eardrum in AOM • Retracted TM
• Ossicular chain discontinuity • Moves a little when there is fluid
• Dislocation • Pressure peak 0
• Displaced chain • Reduced amplitude

PROBLEMS WITH HEARING IMPAIRMENT AURAL REHABILITATION

Decreased audibility • Mild, mod, severe, profound HHIE-S: Screening of Hearing Handicap Inventory for Elderly
• Inability to hear high pitched sounds (female: ss, sh, th, f) No referral, mild, severe handicap (hearing aids)
Decreased frequency selectivity • Difficulty to hear sound with background noise HEARING AIDS
Decreased dynamic range • Can’t distinguish soft VS loud • Management of choice for mod-severe HL
• Normally = wide • Newer aids: precise, tailored to pattern of HL
• Reaches pain threshold quickly (hate loud sound) • Significant improvement
• Cannot restore hearing to normal
• Miniature sound system (microphone + acoustic amplifier + speaker)
• Can adjust HL to audiogram part
• Binaural is better than monoaural:
o More natural hearing (balanced)
o Better speech understanding
o Better understanding in group/ noisy environment
o Better sound localization, quality, identification
 HEARING AIDS
ANALOG VS DIGITAL DIFFERENT KINDS FOR TYPICAL LOSS PATTERN
ANALOG DIGITAL BEHIND EAR IN THE EAR IN THE CANAL COMPLETELY IN THE CANAL
Basic tech Advanced signal processing • Mild-profound HL • Mild-severe HL • Mild-mod • Mild-mod sever HL
Good sound quality Better sound quality • Connected to plastic mold inside ear • Hard and plastic case severe HL • Largely concealed in ear canal
Loudness perception, noise reduction • Case behind ear (accommodate telecoil) • Customized to • Hard to adjust due to size
Less flexible, less precise Small computer: highly precise, more flexible • Poor fitting ear mold may cause • Can be damaged by fit size and • Not for children
Affordable Expensive feedback earwax or drainage shape • Most expensive
Just make sound louder Distinguish noise VS speech • Most powerful • Worn by children

HEARING AID FITTING COPING TECHNIQUES

• Fit binaurally
• Fit on better ear fist
• occlude external ear (prevent feedback)
• fit aid to individual (not individual to aid)
• check for impact socially (COSI)
• Motivation for success
• never force
• time for adjustment
• face to face – 14 inches away
• person’s attention
• better ear and talk to it
• speak slowly, clearly, distinctly
• don’t shout (painful)
• prepare to repeat
• gestures important
• make sure the person understands

OTHER SCREENING TECHNIQUES

TUNING FORK TEST STAPEDIAL REFLEX MEASURE ELECTROCOCHLEOGRAPHY
(ECOG)
• 512 Hz: best • Midline ear battery test • Measure neuroelectric events
• Low Hz: felt > heard • Measure change in TM compliance • Generated by cochlear structures
• High Hz: attenuate readily (dissipate) • Caused by contraction of stapedius ms and auditory nerve
• should not be struck too hard (overtone, false info) • Useful to differentiate cochlear and retrocochlear lesion sites • In response to acoustic stimulation
Acoustic reflex Acoustic reflex decay • Differential diagnosis of hydropic
WEBER RINNE threshold test cochlea (associated with meniere)
• Test lateralization • Measure AC/ BC • determine softest • Measure stapedius ms. ability to maintain sustained contraction
• Handle on midline of vertex/forehead • Fork near mastoid level of sound • Signal: 10 dB above acoustic reflex threshold for 10 sec
• Where sound is heard? bone then ear eliciting stapedial • Abnormal: response amplitude decreases to ½ or less of original
• Which is louder? ms. contraction amplitude within 5 sec
• Normal: midline • Combine with weber • use 500 or 1000 Hz
• Unilateral conductive HL: affected ear
• Unilateral sensorineural: better ear

AIR FLUID IMPEDANCE MATCHING DEVICE TYMPANIC MEMBRANE OSSICULAR AREAL LEVER MECHANISM (PHYSIOLOGY)
CHAIN (ANATOMY)
• Impedance: measure flow resistance • Function: impedance matching device • 99% sound waves bounce back when swimming & someone is talking
• if impedance of 2 materials are too different = sound will not easily pass thru • Compensate for impedance between air • THEORY: Sound waves hit water (endolymph in inner ear) → we lose all sounds
• Ear design: protect against high frequency loss and fluid when it reaches inner ear. When TM & ossicles are removed, sound waves hit
• Head underwater: difficult to hear • 97-99% (30dB) is reflected/ lost due to inner ear (it will cancel everything)
• When sound enters ear → hits TM (mechanical) → reaches cochlea (fluid) media difference • REALITY: 99% deflection = 30 dB, Normal speech is 40 dB, you remove 10 dB
• Impedance/ loss of air sound @ cochlea (murmur). Ear reverses the loss through mechanisms. so the loses are gained
• Ear function: provide gain to make up for loss back
• Lose 30 dB, Gain 30 dB
 AREAL MECHANISM LEVER MECHANISM AMPLIFICATION GAIN
• TM has large surface area 55 mm2 • dB deficit is gained • areal and lever mechanism = gain 29 dB (almost 30 dB)
• Stapes footplate has small surface area 3.2 mm2 • ossicular chain of malleus, incus and stapes • This is how middle ear tries to gain back fluid change from air to liquid
• Large surface area of eardrum transfers more energy to small stapes • Distance from malleus to incuse = 1.3 • TOTAL TRANSFORMER RATIO (ME) = 1.3 X 17 mm 2 = 22.1 mm2
footplate (hydraulic action) • Distance from incus to stapes = 1 • Roughly a pressure amplification gain of 27.5-29 dB
• 17:1 gain = 25 dB • Fulcrum is moved (more energy transferred)
• Disrupted by perforation of eardrum (you lose real surface in serous • Fulcrum = Longer malleolus & shorter incus
OM; because eardrum cannot move) • 1.3 pressure increase (2.5 or 3-4 dB)
• Disrupted by infection (stiffen ossicular chain)
• Total = 1 dB lost via impedance