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