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HYPERACUSISHYPERACUSISDecreased Sound Tolerance
Carlos Herraiz
¿what is hyperacusis?
Abnormal decrease of the tolerance to environmental sounds (ATA)
Exaggerated or inappropriate response to sounds that healthy population are not bothered by.
Disorder of the sound amplification process
decreasedecreaseLoudness discomfort levels < 90 dBHLin 2 or more frequencies (0,25-8 kHz)q ( , )
Goldstein 96
Other authors < 100 dBHLOther authors < 100 dBHLin 2 or more frequencies (0,25-8 kHz)
Jastreboff 00
Dynamic range < de 55-60 dBHLDynamic range < de 55-60 dBHL
Goldstein 96, Jastreboff 00
tolerancetolerance100
% Population highlybothered by noise
60
80bothered by noise
40
HA
(%
)
20
40 50 60 70 80 900
Ldn
(dBA)
Noise level
T.J. Shultz, 1978. “Synthesis of social surveys on noise annoyance”. JASA, 64, 377-405
soundssounds
All the sounds, although there is some variability, g yaccording to the sound frequency spectrum or intensity
When adverse response to certain sounds, even they are not very loudthey are not very loud...
related conceptsrelated concepts
Misophonia
Mi (h t ) N ti ttit d t dMiso (hate): Negative attitude to sound, exaggerated reaction to the external sound
Jastreboff 2000
PhonophobiaIncludes : fear to sound
It depends onIt depends on- Type of sound- patient’s previous experiences- circumstances- patient´s psychological profile
HYPERACUSIS MISOPHONIAPHONOPHOBIAPHONOPHOBIA
ABNORMAL ENHANCEMENT of sound evoked neural
REGULAR sound evoked neural activity
of sound evoked neural activity
In the AUDITHORY PATHWAYS
In AUDITORY PATHWAYSPATHWAYS
Secondary activation of the ABNORMAL EXAGERATED Secondary activation of theLYMBIC SYSTEM AND ANS ACTIVATION of the
LYMBIC SYSTEM and ANS
Patients can present a combination of both disorders
MECHANISMS OF HYPERACUSIS AND MISOPHONIA
HYPERACUSIS
Jastreboff 2004 MISOPHONIA
RecruitmentRecruitment
Stimulation and recruitment of the neighboring nerve fibers to the cochlear damage areas, when application of a high intense sound.sound.
It is a cochlear phenomenon, depending on the OHC loss
It produces a sensation of maximum intensity and distortion
Breakdown of the correlationstimulus intensity / acoustic sensation intensity
epidemiologyFabijanska, 99
Symptom of hyperacusis
ep de o ogy
Symptom of hyperacusis10349 by mail 15,2 %
Andersson, 02
Symptom of hyperacusisy p yp595 by internet
589 by mail9 %
8 %
Hyperacusis in tinnitus patients Tinnitus in hyperacusis patients
40 % 86 %
Hyperacusis TinnitusJastreboff 00 Anari 99
epidemiology
n 213
Parameter PercentageIn general, are you bothered by No 47,4%
n=213
g , y ythe acoustic environment?
,
Yes 52,6%
Number of activities affected byhyperacusis (0-11)
Average 1,8
St d d 2 4hyperacusis (0 11) Standarddeviation
2,4
Median 1
Loudness discomfort level >90 dB. 40,8%
<90 dB. 59,2%
H i 04Herraiz 04
mechanismsec a s sWhat can start the hyperacusis?
Hazell 02Study n=187
StressPrevious tinnitus appearanceppAcute acoustic traumaNoise-induced hearing lossIpsi / Contralateral sudden deafnessLong term DSTDrugs (Psycho-drugs, ototoxicity)
69% non-auditory etiologic factors
Professionals on risk: teachers, musicians, hunters
mechanisms
To consider
• Hyperacusis can be associated to normal hearing or hearing loss
A i l b i h l l b h• Aetiology can be peripheral, central or both
Recruitment is always a cochlear disorderMi h i / h h bi i l t l di dMisophonia / phonophobia is always a central disorderA symmetrical hyperacusis in both ears use to be central
Jastreboff 99
• Hyperacusis can be associated to recruitment and misophonia
RC
HA
MPRC MP
peripheral aetiologymechanisms
MEDIAL OLIVOCOCLEAR EFFERENT SISTEM (MOC) DISORDER
pe p e a aet o ogy
Abnormal sound enhancement from the OHC
Overstimulation of IHCOverstimulation of IHC
OAEs: high intensity on DPsOAEs: high intensity on DPs in affected frequencies
The auditory thresholds where OHC amplification has to stop their function are increasedhas to stop their function are increased
Sahley 97
No changes in hyperacusis after vestibular neurectomyBaguley 02
peripheral aetiologymechanisms
LATERAL OLIVOCOCLEAR EFFERENT SISTEM (LOC) DISORDER
pe p e a aet o ogy
LOC:-Function: to adjust the binaural balance, necessary for precise
d l li tisound localization- It modulates the excitability of the cochlear nerve
protection of the cochlea if acoustic trauma neural damageprotection of the cochlea if acoustic trauma neural damage
Darrow K 2007
LOC impairment: Enhancement of the IPSILATERAL cochlear ABRReduces the CONTRALATERAL ABR activity
No changes on OAE-DPs
Inner ear protection through DOPAMINE-mediated mechanismInner ear protection through DOPAMINE mediated mechanismCochlear perfusion of dopamine agonists ..... Impairment in acoustic trauma
D’Aldin 1995
mechanisms
Disorders of the 5 HT mechanism
central aetiology
Disorders of the 5-HT mechanismFx: Auditory signal modulation and sound significationAlso in: migraine
depressiondepressionposttraumatic stress syndr.
Endogenous endorphins
Marriage 95
Endogenous endorphinsStress periods: endorphins in IHC synapsesGLU potentiating: sound loudness
Sahley 01
stress endst ess
SUPERglu glu
mechanisms
central aetiology
High dosage of systemic SALICILATESReduction of the GABA inhibitory effect Reduction of the 5HT effect Reduction of the GABA effectReduction of the 5HT effect Reduction of the GABA effect
AUDITORY CORTEX ACTIVITY
Enhancement of the startle acoustic reflex
Behavioural response that correspond to HYPERACUSIS
Sun 09
mechanisms
the NPM and hyperacusis
Parallelism in the mechanism between tinnitus and hyperacusisInternal signal (TIN) or external (HyperA)Sub-cortical patterns detectionStimuli enhancement through connections to:
Auditory cortex and prefrontal areasLimbic systemANS
Hyperacusis: “pretinnitus stage?”
Effects of auditory deprivation Tinnitus
Auditorystimulation
Gain of CNS activity (DCN, IC)CNS Hyper-excitability
TTT S dTTT, StapedectomyHyperacusis
causes of hyperacusiscauses o ype acus speripheral causes
Cochlear disorders Ménière D / EHPerilymphatic fistula Sudden deafness I / CAcoustic traumaOtosclerosis
After a surgical Post stapedectomyprocedure TTTs placement
After wax removal Stapedial R. Ramsay Hunt Sydr.Stapedial R. alterations(HyperAc criteria?)
Ramsay Hunt Sydr. Bell´s facial palsy
Muscular disorders Miastenia gravisMuscular disorders Miastenia gravis
causes of hyperacusiscauses o ype acus scentral causes
MigraineDepression
Solomon 92Carmen 73Depression
Posttraumatic stress sydr.Cranial-encephalic traumaLyme D (Borrelia burgdorferi)
Carmen 73Katzenell 01Ceranic 98Coyle 02Lyme D. (Borrelia burgdorferi)
Williams Sydr. (90%)BZD dependence sydrChronic post viral fatigue sydr
Coyle 02Van Borsel 97Lader 84Behan 91Chronic post-viral fatigue sydr
Serotonin dysfunctionTay-Sachs sydr (gangliosidosis 2)Multiple sclerosis
Behan 91Marriage 95Gascon 92Weber 02Multiple sclerosis
Benign intracranial hypertension sydrWeber 02Katzenell 01
clinical symptoms
HYPERACUSIS effects
c ca sy pto s
HYPERACUSIS effects
Social life disruptionLabour life interruptionpRefuse of social and family contact Avoid going outAvoid driving
Obsessive tendencies, anxiety, depressionIncrease of the symptoms when associated phonophobia
Tinnitus presence in 86% of the patients Anari 99
Hearing loss presence in 53% of the patients that seek for hyperacusis treatment. Jastreboff 02
psychoacoustic diagnosispsyc oacoust c d ag os sloudness discomfort level
Measurement of LDLs
Pure tones/ narrow band noisesContinuous / pulsedStart in hearing thresholds and increase intensity in
5 db (or 1 db) from 250 to 8000 hzPerform it TWICE and consider only the second measurement
It can be found differences of 10-15dB between bothJastreboff 04
Patient has to have the possibility to stop the test in any moment
Psychoacoustic diagnosis
loudness discomfort level
Normal values: OVER 90 – 100 dB according to different authorsClassification
Degree LDL Dynamic range
No hyperacusis ≥95 dB in all the frequencies 60db or higherNo hyperacusis ≥95 dB in all the frequencies 60db or higher
Mild HA. 80-90 in 2 or more freq. 50-55 in 1 freq
Moderated HA 65 75 in 2 or more freq 40 45 in 1 freqModerated HA. 65-75 in 2 or more freq. 40-45 in 1 freq
Severe HA. ≤60 in 2 or more freq. 35 or lower
diagnosis / tests
otoacoustic emissionsHyper-motility of the OHC:
Enhancement of PDs loudness
Alt ti DP GROW UPAlterations on DPs GROW-UP curves
Evaluation of the MOC Efferent System
Collet 92Lux-Wellenhof, 99Hesse 99
Evaluation of the LOC system?
Hesse 99auditory brain responses
Thornton 89
Evaluation of the LOC system?Auditory neuropathy?Simulators?
Thornton 89
MOC efferent system disorderMOC efferent system disorder
No contralateralsound
40 dBHL 60 dBHL
FOTO PDS
diagnosis / handicap
1 10VAS
C t S i l lif S t t l
number of affected activities due to hyperacusis
Concerts Social life Sport spectacles
Restaurants Going to church House keeping
Cinema Working Taking care of the childrenCinema Working Taking care of the childrenShopping Driving Others
Score from 0 to 11
Khalfa 02: 14 items sub-scalesAttentionSocial interaction
questionnaires
Khalfa 02: 14 items, sub scales
Nelting 02: 27 items, sub-scales
Social interactionEmotion
Cognitive reactionNelting 02: 27 items, sub scalesBehavioural changesEmotion
diagnosis / handicap
sound hypersensitivity questionnaire THS / GÜF
X
Nelting 02Herraiz 06
XX
XX
0 1 2 3 points
T t l i t / 45 D / 4 18 253 severe11-172-moderate1-101- mild
Total points / 45 Degrees / 426-454-very severe18-253-severe
treatment
AVOID EAR PLUGS!!
t eat e t
AVOID EAR PLUGS!!
INFORMATION for the patientINFORMATION for the patient
INCREASE SOUND TOLERANCE TRTProgressive exposition to sounds
tinnitus retraining therapy TRT
Medical and audiologist counselling
t tus et a g t e apy
Medical and audiologist counselling
Hyperacusis mechanismsAssociation with other symptoms: tinnitus hearing lossAssociation with other symptoms: tinnitus, hearing lossInappropriate / appropriate behaviourTreatment approaches PrognosisPrognosis
Progressive sound expositionProgressive sound exposition
Hyperacusis management program (PHA) from UAH-HUFAJastreboff 00, Vernon 98, Hazell 91, Valente 00, , ,
Broad band noise generatorsHearing aids
Hyperacusis management program UAH-HUFA
Indication: mild / moderate hyperacusis (VAS, THS, NACT)
Method:Progressive sound exposition 2 hours / day, 3 a 6 mInitially: tolerable intensity increase every weekInitially: tolerable intensity, increase every weekType of sound: WTN
Introduction of narrow band noises:1-12 khz4-8 khz, etc…
Also applicable sounds from the natureCDs on sale
Demonstrated efficacy on LDL improvement
Knaster 88, Dominguez 01 (Auditory trainning)Knaster 88, Dominguez 01 (Auditory trainning)Reduction of the Recruitment coefficient
Unilateral disease: 59% casesBilateral disease: 94% cases
Reduction of the recruitment improvement of the intelligibility
Noreña AJ, Chery-Croze SNoreña AJ, Chery Croze SAcoustic trainning for auditory hypersensibilityNeuroreport 2007
Hearing loss: Reduction of the INPUT from the Cochlea to the Cochlear Nuclei
Central compensating activityCentral compensating activity
Auditory hypersensibility
Treatment: Enrichment of the acoustic stimulation according to the damaged frequenciesNarrow band noises should be usedNarrow band noises should be used
1-12 khz4-8 khz, etc…
Th i t it f h ti l ti f i l l t d di t it h i th h ldThe intensity of each stimulating frequency is calculated according to its hearing thresholdNo progressive enhancement of the intensity of the stimulating sound
Results: significant reduction of the LDL after 15 weeks of treatmentResults: significant reduction of the LDL after 15 weeks of treatment
broad band noise generators
Indication: Severe hyperacusis (VAS, THS, NACT)yp ( , , )Jastreboff 04: LDL< 80-85 db30% of tinnitus patients received hyperacusis treatment previously
BBNG fittingBinauralBTE, ITE, ITCSound intensity: progressive. Sometimes limitedWearing time 8 h /dg /Technology
AnalogicalDigital:Digital:
Same intensity in all the frequencies when hearing lossIndividualized sound therapies with different frequency spectrumI l lImprove external canal resonance
hearing aid fittinghearing aid fitting
Management of the hyperacusis
Management of the recruitment
- Stable or compensated SNHL
- Fluctuant or decompensated SNHL
An incorrect fitting can:
Increase the hyperacusisIncrease the hyperacusis
Increase the tinnitus loudness (rebound)41% hyperacusis patients41% hyperacusis patients
Herraiz 03
hearing aid fittinghearing aid fitting
PROGRESSIVE FITTING:
• Increase slowly the time of use of the hearing aidIncrease slowly the time of use of the hearing aid • Adjust the maximum output (no more than LDL) • Limit the gain of the hearing aid
- Beware with the lower frequenciesq- Avoid feed-back- Consider external ear canal resonance to avoid over-amplification
if h t d th i t lli ibiliteven if we have to reduce the intelligibility
• Increase the “venting”It will reduce the oclussive effect on lower frequencies- It will reduce the oclussive effect on lower frequencies
but control the feed-back
hearing aid fitting
Band compression
Increase the compression of louder soundsIncrease the compression of louder sounds Compression activation thresholdLatency of activationCompression ratiopDuration of the compression
even if we have to reduce the intelligibility
Auditory trainningImproves LDL and dynamic range
Hyperacusis improvement
Knaster 88
Hyperacusis improvement
Possitive effect on intelligibility Dominguez 01
Some patients require management for hyperacusis before fitting- Hyperacusis management program (PHA) UAH-HUFA- Broad band noise generatorsBroad band noise generators
management of recruitmentmanagement of recruitment
Management of recruitment in stable or compensated SNHL
WTN, NBN stimulationh d ( di i i )Knaster Method (auditory training)
M t f it t i fl t tManagement of recruitment in fluctuant or decompensate SNHL
Corticosteroids orally / iv / itCorticosteroids orally / iv / itDiureticsSulpirideBetahistineHistamine
Pregabaline
results TRT
G ld 97
results TRT
Gold 97n= 130TRT
1 khz 2 khz 4 khz Total
Improv.LDL(DS)
12,52*(11 8)
12,72*(14 9)
12,20*(14 1)
12,48*(13 6)
6 m 15 m 25 m
(DS) (11,8) (14,9) (14,1) (13,6)
Hazell 02n= 187TRT
6 m 15 m 25 m
% patients with normalized LDL
45% 51% 61%
Num of affected activities:Num of affected activities: Initially: 3.515 months: 1.1
Treatment of phonophobia / misophoniaTreatment of phonophobia / misophonia
Psychologists
Active extinction method: Reinforcement to reduce the aversive stimulusA ti iti ith d t l d l t ibilit tActivities with sound control and voluntary possibility to supress
them at any momento PositiviationPositivizar el síntoma
Sometimes we need a combined treatment por hyperacusisSometimes we need a combined treatment por hyperacusis and phonophobia
Pharmacologic possibilities in hyperacusis
Hyperacusis in cochlear damage:
g p yp
Reduction in OHC activity: SALYCILATES Mechanisms: Jastreboff 98, Sahley 99
Hyperacusis in central disorders
R d d t i (5HT) ???Reduced serotonin (5HT) ???Paroxetine, Fluoxetine, Sertraline
Marriage 95, Simpson 00
5HT AgonistsUsed in migraine: Sumatriptan, etc.
Junkel 97
GABA potentiating effect : Pregabaline
Drugs for depression and anxiety: hyperacusis improvementg p y yp p
Thanks for your attention