Decreased Sound Tolerance

HYPERACUSIS
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

decrease
Loudness discomfort levels < 90 dBHL in 2 or more frequencies (0,25-8 kHz) q ( , )
Goldstein 96

Other authors < 100 dBHL in 2 or more frequencies (0,25-8 kHz)

Jastreboff 00

Dynamic range < de 55-60 dBHL

Goldstein 96, Jastreboff 00

tolerance
100

% Population highly bothered by noise

80

60
H A (% )

40

20

0 40

50

60
L
dn

70
(d BA)

80

90

Noise level
T.J. Shultz, 1978. Synthesis of social surveys on noise annoyance. JASA, 64, 377-405

sounds
All the sounds, although there is some variability , g y according to the sound frequency spectrum or intensity

When adverse response to certain sounds, even they are not very loud loud...

related concepts
Misophonia
Miso (hate): N Mi (h t ) Negative attitude t sound, ti ttit d to d
exaggerated reaction to the external sound
Jastreboff 2000

Includes

Phonophobia : fear to sound

It depends on - Type of sound - patients previous experiences - circumstances - patients psychological profile

HYPERACUSIS

MISOPHONIA PHONOPHOBIA

ABNORMAL ENHANCEMENT of sound evoked neural activity In AUDITORY PATHWAYS

REGULAR sound evoked neural activity In the AUDITHORY PATHWAYS

Secondary activation of the LYMBIC SYSTEM AND ANS

ABNORMAL EXAGERATED ACTIVATION of the LYMBIC SYSTEM and ANS

Patients can present a combination of both disorders

MECHANISMS OF HYPERACUSIS AND MISOPHONIA

HYPERACUSIS

Jastreboff 2004

MISOPHONIA

Recruitment

Stimulation and recruitment of the neighboring nerve fibers to the cochlear damage areas, when application of a high intense sound. It is a cochlear phenomenon, depending on the OHC loss

It produces a sensation of maximum intensity and distortion Breakdown of the correlation stimulus intensity / acoustic sensation intensity

ep de o ogy epidemiology
Fabijanska, 99 Symptom of hyperacusis 10349 by mail Andersson, 02 Symptom of hyperacusis y p yp 595 by internet 589 by mail

15,2 %

9% 8%
Tinnitus in hyperacusis patients

Hyperacusis in tinnitus patients

40 %

86 %
Jastreboff 00 Anari 99

Hyperacusis

Tinnitus

epidemiology

n=213 n 213
Parameter Percentage
47,4% , 52,6% 1,8 2,4 24 1 40,8% 59,2% In general, are you bothered by No g , y y the acoustic environment? Yes Number of activities affected by Average hyperacusis (0-11) (0 11) Standard St d d deviation Median Loudness discomfort level >90 dB. <90 dB.

Herraiz 04 H i

mechanisms ec a s s
What can start the hyperacusis?
Hazell 02 Study n=187

Stress

Previous tinnitus appearance pp Acute acoustic trauma Noise-induced hearing loss Ipsi / Contralateral sudden deafness Long term DST Drugs (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 Aetiology can be peripheral, central or both b i h l l b h Recruitment is always a cochlear disorder Misophonia / phonophobia is always a central disorder Mi h i h h bi i l t l di d A symmetrical hyperacusis in both ears use to be central
Jastreboff 99

Hyperacusis can be associated to recruitment and misophonia HA RC MP

pe p e a aet o ogy peripheral aetiology

mechanisms

MEDIAL OLIVOCOCLEAR EFFERENT SISTEM (MOC) DISORDER Abnormal sound enhancement from the OHC Overstimulation of IHC OAEs: high intensity on DPs in affected frequencies

The auditory thresholds where OHC amplification has to stop their function are increased
Sahley 97

Baguley 02

No changes in hyperacusis after vestibular neurectomy

pe p e a aet o ogy peripheral aetiology

mechanisms

LATERAL OLIVOCOCLEAR EFFERENT SISTEM (LOC) DISORDER LOC:

-Function: to adjust the binaural balance, necessary for precise sound localization dl li ti - It modulates the excitability of the cochlear nerve protection of the cochlea if acoustic trauma neural damage
Darrow K 2007

LOC impairment: Enhancement of the IPSILATERAL cochlear ABR Reduces the CONTRALATERAL ABR activity No changes on OAE-DPs Inner ear protection through DOPAMINE-mediated mechanism DOPAMINE mediated Cochlear perfusion of dopamine agonists ..... Impairment in acoustic trauma
DAldin 1995

mechanisms central aetiology

Disorders of the 5-HT mechanism 5 HT Fx: Auditory signal modulation and sound signification Also in: migraine depression Marriage 95 posttraumatic stress syndr. Endogenous endorphins Stress periods: endorphins in IHC synapses GLU potentiating: sound loudness
Sahley 01

st ess stress

end

glu

SUPER glu

mechanisms central aetiology

High dosage of systemic SALICILATES Reduction of the GABA inhibitory effect Reduction 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 hyperacusis Internal signal (TIN) or external (HyperA) Sub-cortical patterns detection Stimuli enhancement through connections to: Auditory cortex and prefrontal areas Limbic system ANS Hyperacusis: pretinnitus stage? Effects of auditory deprivation Auditory stimulation
TTT, S TTT Stapedectomy d

Tinnitus

Gain of CNS activity (DCN, IC)

CNS Hyper-excitability

Hyperacusis

causes of hyperacusis o ype acus s

peripheral causes

Cochlear disorders

Mnire D / EH Perilymphatic fistula Sudden deafness I / C Acoustic trauma Otosclerosis Post stapedectomy TTTs placement After wax removal Ramsay Hunt Sydr. Bells facial palsy Miastenia gravis

After a surgical procedure Stapedial R. alterations (HyperAc criteria?) Muscular disorders

causes of hyperacusis o ype acus s

central causes
Migraine Depression Posttraumatic stress sydr. Cranial-encephalic trauma Lyme D (Borrelia burgdorferi) D. Williams Sydr. (90%) BZD dependence sydr Chronic post viral fatigue sydr post-viral Serotonin dysfunction Tay-Sachs sydr (gangliosidosis 2) Multiple sclerosis Benign intracranial hypertension sydr Solomon 92 Carmen 73 Katzenell 01 Ceranic 98 Coyle 02 Van Borsel 97 Lader 84 Behan 91 Marriage 95 Gascon 92 Weber 02 Katzenell 01

c clinical sy pto s ca symptoms

HYPERACUSIS effects Social life disruption Labour life interruption p Refuse of social and family contact Avoid going out Avoid driving Obsessive tendencies, anxiety, depression Increase of the symptoms when associated phonophobia Tinnitus presence in 86% of the patients Hearing loss presence in 53% of the patients that seek for hyperacusis treatment.
Anari 99

Jastreboff 02

psyc oacoust c d ag os s psychoacoustic diagnosis

loudness discomfort level
Measurement of LDLs Pure tones/ narrow band noises Continuous / pulsed Start in hearing thresholds and increase intensity in 5 db (or 1 db) from 250 to 8000 hz Perform it TWICE and consider only the second measurement It can be found differences of 10-15dB between both
Jastreboff 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 authors Classification
Degree LDL Dynamic range

No hyperacusis Mild HA. Moderated HA HA. Severe HA.

95 dB in all the frequencies 80-90 in 2 or more freq. 65-75 65 75 in 2 or more freq freq. 60 in 2 or more freq.

60db or higher 50-55 in 1 freq 40-45 40 45 in 1 freq 35 or lower

diagnosis / tests otoacoustic emissions

Hyper-motility of the OHC: Enhancement of PDs loudness Alterations on DP GROW UP curves Alt ti DPs GROW-UP Evaluation of the MOC Efferent System
Collet 92 Lux-Wellenhof, 99 Hesse 99

auditory brain responses

Evaluation of the LOC system? Auditory neuropathy? Simulators?
Thornton 89

MOC efferent system disorder

No contralateral sound

40 dBHL

60 dBHL

FOTO PDS

diagnosis / handicap VAS

1 10

number of affected activities due to hyperacusis

Concerts C t Restaurants Cinema Shopping Social life S i l lif Going to church Working Driving Sport S t spectacles t l House keeping Taking care of the children Others

Score from 0 to 11

questionnaires
Khalfa 02: 14 items, sub scales items sub-scales Nelting 02: 27 items, sub scales sub-scales
Attention Social interaction Emotion Cognitive reaction Behavioural changes Emotion

diagnosis / handicap

sound hypersensitivity questionnaire THS / GF

Nelting 02 Herraiz 06 X X X

2
1- mild

points
1-10 11-17 18-25 18 25 26-45

2-moderate

Total i t T t l points / 45

Degrees / 4 D

3-severe 3 severe 4-very severe

t eat e t treatment
AVOID EAR PLUGS!!

INFORMATION for the patient INCREASE SOUND TOLERANCE

Progressive exposition to sounds

TRT

t tinnitus retraining t e apy TRT tus et a g therapy

Medical and audiologist counselling Hyperacusis mechanisms Association with other symptoms: tinnitus hearing loss tinnitus, Inappropriate / appropriate behaviour Treatment approaches Prognosis Progressive sound exposition Hyperacusis management program (PHA) from UAH-HUFA
Jastreboff 00, Vernon 98, Hazell 91, Valente 00 , , ,

Broad band noise generators Hearing aids

Hyperacusis management program UAH-HUFA

Indication: mild / moderate hyperacusis (VAS, THS, NACT) Method: Progressive sound exposition 2 hours / day, 3 a 6 m

Initially: tolerable intensity increase every week intensity, Type of sound: WTN Introduction of narrow band noises: 1-12 khz 4-8 khz, etc Also applicable sounds from the nature CDs on sale

Demonstrated efficacy on LDL improvement Knaster 88, Dominguez 01 (Auditory trainning) Reduction of the Recruitment coefficient

Unilateral disease: 59% cases Bilateral disease: 94% cases Reduction of the recruitment improvement of the intelligibility

Norea AJ, Chery Croze S Chery-Croze

Neuroreport 2007

Acoustic trainning for auditory hypersensibility

Hearing loss: Reduction of the INPUT from the Cochlea to the Cochlear Nuclei Central compensating activity Auditory hypersensibility

Treatment: Enrichment of the acoustic stimulation according to the damaged frequencies Narrow band noises should be used 1-12 khz 4-8 khz, etc The i t Th intensity of each stimulating frequency is calculated according to its hearing threshold it f h ti l ti f i l l t d di t it h i th h ld No progressive enhancement of the intensity of the stimulating sound Results: 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 db

30% of tinnitus patients received hyperacusis treatment previously

BBNG fitting Binaural BTE, ITE, ITC Sound intensity: progressive. Sometimes limited Wearing time 8 h /d g / Technology Analogical Digital:
Same intensity in all the frequencies when hearing loss Individualized sound therapies with different frequency spectrum Improve external canal resonance I l l

hearing aid fitting

Management of the hyperacusis Management of the recruitment - Stable or compensated SNHL - Fluctuant or decompensated SNHL

An incorrect fitting can: Increase the hyperacusis Increase the tinnitus loudness (rebound) 41% hyperacusis patients
Herraiz 03

hearing aid fitting

PROGRESSIVE FITTING: Increase 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 frequencies q - Avoid feed-back - Consider external ear canal resonance to avoid over-amplification even if we have to reduce the intelligibility h t d th i t lli ibilit

Increase the venting

- It will reduce the oclussive effect on lower frequencies but control the feed-back

hearing aid fitting

Band compression Increase the compression of louder sounds
Compression activation threshold Latency of activation Compression ratio p Duration of the compression even if we have to reduce the intelligibility

Auditory trainning

Knaster 88

Improves LDL and dynamic range

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 generators

management of recruitment
Management of recruitment in stable or compensated SNHL WTN, NBN stimulation Knaster Method (auditory training) h d ( di i i ) Management of recruitment in fluctuant or M t f it t i fl t t decompensate SNHL Corticosteroids orally / iv / it Diuretics Sulpiride Betahistine Histamine Pregabaline

results TRT
Gold G ld 97 n= 130 TRT
1 khz Improv.LDL (DS) 12,52* (11,8) (11 8) 2 khz 12,72* (14,9) (14 9) 4 khz 12,20* (14,1) (14 1) Total 12,48* (13,6) (13 6)

Hazell 02 n= 187 TRT

6m % patients with normalized LDL

15 m

25 m

45%

51%

61%

Num of affected activities: Initially: 3.5 15 months: 1.1

Treatment of phonophobia / misophonia

Psychologists
Active extinction method: Reinforcement to reduce the aversive stimulus Activities ith A ti iti with sound control and voluntary possibility to supress d t l d l t ibilit t them at any momento Positiviation Positivizar el sntoma

Sometimes we need a combined treatment por hyperacusis and phonophobia

Pharmacologic possibilities in hyperacusis g p yp

Hyperacusis in cochlear damage: Reduction in OHC activity: SALYCILATES
Mechanisms: Jastreboff 98, Sahley 99

Hyperacusis in central disorders Reduced serotonin (5HT) ??? R d d t i

Paroxetine, Fluoxetine, Sertraline
Marriage 95, Simpson 00

5HT Agonists Used in migraine: Sumatriptan, etc.

Junkel 97

GABA potentiating effect : Pregabaline Drugs for depression and anxiety: hyperacusis improvement g p y yp p

Thanks for your attention