Transcript Hyperacusis : definitions

Hyperacusis:
mechanisms, assessment, treatments
David Baguley PhD
Head of Service: Audiology and Hearing Implants
Cambridge University Hospitals, UK
Visiting Professor,
Anglia Ruskin University, Cambridge
Objectives
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Definitions
Prevalence and incidence
Assessment
Mechanisms
Treatments
Hyperacusis : definitions
• “unusual tolerance to ordinary environmental
sounds” (Vernon, 1987 )
• “consistently exaggerated or inappropriate
responses or complaints to sounds that are
neither intrinsically threatening or uncomfortably
loud to a typical person”
(Klein et al, 1990)
• “abnormal reduced tolerance to environmental
sound”
(Baguley, 1990)
Terminology
• Hyperacusis : general over sensitivity to quiet sound
– Gk hyper (above) akousis (hearing)
– Perlman, 1938
– “hyperacusis dolorosa” Mathisen (1968)
• Phonophobia : very specific, association with migrane
– Gk phone (voice or sound) phobia (fear)
• Misophonia : ‘dislike of sound’ (Jastreboff, 2003)
– Gk misos (hatred) phone (voice or sound)
Terminology
• Collapsed sound
tolerance
– Hyperacusis Network
www.hyperacusis.net
• Recruitment
– abnormally steep
loudness growth
– Fr recruter (to recruit)
– OHC dysfunction
Hyperacusis in adults
• Point prevalence : 9% ( Andersson et al, 2002 )
• Internet survey: n= 595
• Postal survey: n= 589 ( 59.7% response rate )
• Incidence : unknown
Hyperacusis in childhood
• Prevalence : unknown
• Incidence : unknown
• Co-incidence with tinnitus : 38%
– (Baguley et al., 2012)
• Co-incidence with Auditory Processing Disorder : noted
• Association with Autistic spectrum disorder
Relationship with tinnitus
• 40% of tinnitus patients report
hyperacusis ( Jastreboff et al, 1996 )
• Other series : 20 - 60%
• 86% of hyperacusis patients report
tinnitus ( Anari et al, 1999 )
Severe hyperacusis
• 4-5% of population have severe tinnitus
• 40% of these have significant hyperacusis
• Severe hyperacusis : 2%
– Jastreboff, 2000
– Baguley and Andersson, 2007
Diagnosis
• History (onset, fear/distress, hearing)
• Loudness discomfort levels ?
– High within and inter subject variability
• (Stephens et al, 1977)
– Sensitive to instructions
• (Bronstein and Musiek, 1993)
• (Sherlock and Formby, 2005)
Quantification
• Urgent need for an instrument that is
– Well validated
– Low impact
– Sensitive to treatment effects
• Should an instrument measure:
– Functional impact
– Emotional impact / attrition / burden
– Avoidance (Blaesing and Kroner-Herwig, 2012)
Nelting et al (2002)
• 27 item self-report questionnaire
• German
• Validated on 226 subjects with hyperacusis
• Three factor solution
– Cognitive reaction
– Actional/somatic behaviour
– Emotional
• Blasing et al. (2010) English translation
Khalfa et al (2002)
• 14 item self-report questionnaire (HQ)
• Validated on 201 individuals from general population
• French, now translated
• Three factor solution
– Attentional
– Emotional
– Social
Aetiologies ( Katzenell and Segal, 2001 )
• Peripheral
– Bell’s Palsy
– Ramsey-Hunt
– Stapedectomy
– Perilymph Fistula
• Recruitment
– Cochlear hearing loss
• Central
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Migrane
Depression
Post Traumatic Stress
Head Injury
Multiple Sclerosis
Lyme Disease
Williams syndrome
Fibromyalgia
Idiopathic
Lyme Disease
• Bacterial infection
• Involves tick-borne spirochete
– Borrelia burgdoferi
• Peripheral and central neurological involvement
– (Neilds et al., 1999)
Williams Syndrome
• Incidence : 1 / 20,000
• Males = Females
• Elfin facial features
• Cardiac problems
• “failure to thrive”
• Distinctive behavioural phenotype
• Hyperacusis in 95 %
• 5-HT ( serotonin )
Mechanisms
• 5-HT ( Marriage and Barnes, 1995 )
• Auditory efferent dysfunction
• Changes in central auditory gain
Serotonin ( 5-HT )
• Auditory startle
• Dysfunction in
– migrane
– depression
– post-traumatic stress disorder
Serotonin ( 5-HT )
• Serotonergic fibres and terminal endings found
throughout central auditory pathway
• Postulated role of modulating sound perception or
determination of significance
– ( Thompson et al, 1994, Simpson and Barnes, 2000 )
Problems with 5-HT hypothesis
• Non-specific ( Phillips and Carr, 1998 )
• Possible role of SSRI medication
Efferent Auditory System
• Present in all mammals
• Medial efferents
– Arise from superior olive
– Synapse on OHC
• Possible role in auditory gain and hyperacusis
– Vernon et al ( 2002 )
• Auditory efferents run in the inferior vestibular nerve
Anastomosis of Oort
• 1300 fibres running from saccular branch of
inferior vestibular nerve to the cochlea
• 2-3mm long, 0.1 - 0.3mm wide in adult humans
• Contains efferent fibres from medial
olivocochlear system
• Oort ( 1918 )
Problems with efferent hypothesis
• Vestibular nerve section does not
– Influence tinnitus and hyperacusis
( Baguley et al, 2002 )
– Change psychoacoustical abilities
( Scharf et al, 1997 )
Changes in central auditory gain
• Central auditory gain is modulated by:
– Acoustic environment
– Mood
• Hyperacusis may be underpinned by chronic
increase in central auditory gain
– Maintained by anxiety
– Exacerbated by overprotection
Plasticity in central auditory system
• Plasticity first proposed by Ramon y
Cajal ( 1852 - 1934 )
• Caused by
– Injury and reorganisation
– Learning and experience
Formby et al., 2003
• Earplugs
– 2 weeks, 23 hours per day
– More sensitive to loudness
• Wide band sound generators
– 2 weeks, all waking day
– Reduced sensitivity to loudness
Relationship with mood
• Until recently, no quantitative data
• High levels of anxiety states
– Juris et al., 2012
– 47% anxiety disorder
• “emotional exhaustion”
– Wallen et al., 2012
– N=348 with EE, n=4 (1.1%) with hyperacusis
• Causality?
PTSD and sound tolerance
Tinnitus
Tinnitus and PTSD
Patient Rating
10
8
6
4
2
0
Tinnitus Loudness
Sound Tolerance Problems
Fagelson, (2007). The Association between tinnitus and PTSD; AJA.
31
Autistic spectrum disorder
– Normal peripheral hearing
• (Gravel et al. 2006, Tharpe et al., 2006)
– Hyper-reactivity to sound reported
• Rosenhall et al., 1999
– Hyper-reactivity to novel auditory targets
• Event related fMRI
• Gomot et al., 2008
– No evidence for efficacy of Auditory Integration
Training in ASD
• (Sinha et al., 2011)
Tensor Tympani
• Tensor tympani syndrome
– ( Klockhoff,1979 )
– Fluttering, beating
– Sensitivity to noise
– Diagnose with long time base
tympanometry
– Treat with relaxation or surgery
– (Bhimrao et al., 2012)
Acoustic Shock
• Acoustic Shock Programme definitions
– An Acoustic Incident is a sudden, unexpected,
noise event which is perceived as loud,
transmitted through a telephone or headset
– Acoustic Shock is an adverse response to an
acoustic incident resulting in alteration of auditory
function
Is hyperacusis treatable ?
• No RCT evidence
• BUT:
– Central auditory gain changes in ordinary life
– Significant influence of anxiety and attention
– Following grommet insertion, children with OME
normalise auditory gain
How not to treat hyperacusis
• Disorder of central gain, so :
– minimal use of earplugs or hearing protection
– no increase in fear and anxiety
– avoid loudness discomfort level tests
Treating hyperacusis in adults
• Explanation
• Counselling
• Relaxation
• Sound therapy
• www.hyperacusis.net
Sound therapy
• Wide-band noise, low
level, sound field or
behind the ear
• Binaural fitting
• Two approaches
– Increasing intensity
– Recalibration
• Role of sound at night?
Psychological input
• Co-morbidity with anxiety
• Patients will present to Audiologists
• Indicated if :
– debilitating anxiety
– screen with Hospital Anxiety and
Depression Scale
Other approaches
• Tinnitus Retraining Therapy
– Categorisation
– Strict protocol
• Neuromonics
– Filtered music
– Strict protocol
• Cognitive Behavioural Therapy
– Data imminent from RCT
In practice ….
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Normal hearing, rapid onset of DST
Varies with mood?
Accompanied by fear?
Medical input
Psychological crisis?
Summary
• Hyperacusis remains a scientific and
clinical enigma
• Facts are emerging from fiction
• Urgent need for clinical research !