1. health and fitness
2. disorders

audiology matters
In this issue...
Hyperacusis
Definitions, Epidemiology, and Possible Mechanisms of
Hyperacusis
The Hyperacusis Network
Sound Tolerance in Autism Spectrum Disorder
New Findings on Hyperacusis in Williams Syndrome
Military Trauma and its Influence on Loudness Perception
Dr David Baguley, PhD, MBA,
Complaints about Low Frequency Environmental Noise
The Treatment of Hyperacusis with Cognitive Behaviour Therapy
Using TRT to Treat Hyperacusis, Misophonia and Phonophobia
An Interview with James W Hall III
Cambridge University Hospitals NHS
Foundation Trust, Hills Road,
Cambridge, CB2 2QQ, UK.
E: [email protected]
In this issue...
Hyperacusis and Decreased
Sound Tolerance
Dr David Baguley,
PhD, MBA,
Cambridge University
Hospitals NHS
Foundation Trust,
Hills Road, Cambridge,
CB2 2QQ, UK.
E: [email protected]
nterest in the fascinating symptom of hyperacusis
(decreased sound tolerance) continues to grow around
the world and this is extremely heartening for patients,
clinicians and researchers alike. Within the last few months,
both the British Society of Audiology and the Association for
Research in Otolaryngology have held symposia specifically
concerned with hyperacusis, and sound tolerance issues have
been the focus of featured sessions at the American Academy
of Audiology in the recent past. Knowledge in the area is
growing at a very encouraging rate and the field is benefiting
from a multidisciplinary approach. With this in mind, I have
drawn together commissioned articles for this edition of
Audiology Matters that I hope will contribute to the debate
and research endeavour regarding hyperacusis around the
world. I am very grateful to contributors who have given of
their valuable time and shared their insights and in particular
am especially pleased that a good number of the articles
present data and ideas that have not previously been
published. The intention was not to comprehensively
describe the whole topography of sound tolerance problems
– rather to consider the issues from particular perspectives, to
challenge and inspire interested clinicians and researchers.
Additionally the interview with Prof Jay Hall III gives some
useful and interesting reflections on a career spent developing
the science of Audiology (including services for people with
hyperacusis) and sharing that knowledge.
To set the scene, I have given an introduction which
describes what is presently known about the terminology
used to describe sound tolerance problems and the
epidemiology of such problems. In doing so, I necessarily
raise more questions than provide answers and it is clear that
further research and some consensus building is needed in
this area. Important insights in to the patient experience of
decreased (or collapsed) sound tolerance are given by Dan
Malcore. The important work of the Hyperacusis Network
(www.hyperacusis.net) is described and for many patients
this has been an invaluable resource in managing their lives
and recovering quality of life.
Dr Teresa Tavassoli describes the sound tolerance challenges experienced by individuals with autistic spectrum
I
disorder and provides some fascinating insights. Prof Joseph
Attias considers the specific situation of individuals with
Williams Syndrome, a neurodevelopmental disorder in which
hyperacusis has been frequently reported. Traditionally there
had been an assumption that cochlear function was normal
in such persons but recent investigations have indicated that
this is commonly not the case and these findings cast a new
light upon the experiences of such people. An entirely
different perspective comes from Prof Mark Fagelson, who
writes about experiences of hyperacusis in combat veterans
and the association with post traumatic stress disorder. Given
the large number of such persons in the health care systems
of the developed world, and particularly the United States of
America, this is of some considerable topical importance.
Writing from the United Kingdom, Prof Andy Moorhouse
and Tim Husband consider the situation of individuals who
have a complaint of low frequency environmental sound.
Such complaints are more common than might at first be
considered and puzzlingly, in many cases no specific low
frequency signal can be identified in their environment. The
work described considered whether such complaints can be
adequately addressed within an Audiology Clinic setting and I
do hope that this will be of interest to the reader.
Two different perspectives on treatment are given by Prof
Gerhard Andersson, and by Prof Pawel Jastreboff and Dr
Margaret Jastreboff respectively. Professor Andersson
describes how hyperacusis can be treated using Cognitive
Behavioural Therapy, and how specifically the fear, anxiety and
avoidance behaviours that some hyperacusis patients exhibit
can be ameliorated. Professor and Dr Jastreboff share with us
their latest thoughts on how decreased sound tolerance can
be treated using protocols derived initially from Tinnitus
Retraining Therapy (TRT), and it is hoped that this will be of
interest to clinicians working within the TRT community.
In summary I do hope that this series of short articles will
provoke discussion and challenge and inspire both clinicians
and researchers to address the concerns and experiences of
patients with hyperacusis. This is an area replete with opportunities for research and clinical trials; further developments
are awaited with great anticipation.
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
69
Definitions, Epidemiology and
Possible Mechanisms of
Hyperacusis
Dr David Baguley,
PhD, Head of Service:
Audiology / Hearing
Implants, Cambridge
University Hospitals and
Visiting Professor,
Anglia Ruskin University,
Cambridge, UK.
T
he experience of becoming troubled by decreased sound tolerance can be catastrophic for a patient and their family, and represents a real challenge for the audiologist. Whilst the symptom has been described for many years, it is only in the
last decade that protocols for diagnosis and treatment have been formulated, and that an
evidence base for treatment efficacy has begun to build. In this piece I seek to introduce the
Correspondence
E: [email protected]
Declaration of
Competing Interests
None declared.
reader to modern understandings of hyperacusis, describing the terminology used, present
information regarding epidemiology, and potential mechanisms.
Definitions
There are many words in current use to indicate a decrease in the ability to tolerate sound
(see Baguley and McFerran for comprehensive
review) [1]. The word hyperacusis seems first
to have been used by Perlman [2], and a
suggested later modification to hyperacusis
dolorosa [3] was not widely adopted. A definition of hyperacusis is ‘an abnormal, lowered
tolerance to sound’ [4]. In practice this refers
to individuals who have developed a sense of
discomfort when exposed to external sound
of an intensity that would not trouble most
others. For some the discomfort depends on
whether they make the sound, or some
external source – in such circumstances it is
usually the case that self made sound is tolerated better. For others however the source
may be irrelevant. Similarly, for some it is
specific sound that is bothersome, such as
that of an unruly child, whereas for others it is
all sound that evokes discomfort.
The term phonophobia has been applied to
these situations, especially when it is a specific
sound that is bothersome, but there are two
issues with that. The first is the implication
that as a ‘phobia’ this is essentially a psychological phenomenon, and optimally treatable
by psychological therapy – but whilst there
may be psychological features to a case, this
may not be true. Second, phonophobia is
used very specifically by neurologists to
describe the sound intolerance symptoms
experienced by some migraine sufferers, and it
is far from clear that hyperacusis in general is
similar to that.
Proponents of tinnitus retraining therapy
(TRT) have introduced a new word to
describe hyperacusis: misophonia. This derives
from the Greek for ‘dislike of sound’, and so
resonates with the aversion experienced by
patients. It is yet to be seen whether this term
is adopted outside the TRT community.
Sometimes it is a challenge to disentangle
70
hyperacusis from recruitment, the abnormal
growth of loudness associated with Outer
Hair Cell dysfunction. One differentiating
factor is that recruitment is not modulated by
emotional state, whereas hyperacusis can be
(specifically by anxiety), but this is essentially
subjective and hard to quantify.
It is interesting to note that patients
develop their own terms to describe their situation. The Hyperacusis Network (www.hyperacusis.net) patient community uses the
phrases reduced or collapsed sound tolerance
to describe their experiences and these words
are very meaningful for patients in communicating their situation.
Epidemiology
At the present time firm evidence about the
epidemiology of hyperacusis is sparse. A study
in Sweden [5], combined internet and postal
survey techniques and identified a figure of 8%
of the adult population who reported loudness tolerance issues, but this probably does
not represent the number of people for whom
that is clinically significant. A more realistic estimate is of 2% of the adult population [1].
Even less is known about the prevalence of
hyperacusis in childhood. In my clinic I see a
steady but small stream of children with a
primary complaint of hyperacusis, and
reduced sound tolerance can be especially
problematic in a school context. Figure 1 is a
self-portrait by an eight-year-old boy with
hyperacusis, and it demonstrates his
response to sound in his environment. There
is some evidence regarding an association
between sound tolerance issues and children
identified with Autism Spectrum Disorders
(ASD), but there is much work to be done in
this area. There are indications that hyperacusis in ASD may be a form of auditory
hyper-vigilance, and as such phenomenologically different from general adult hyperacusis
experiences [6].
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
feature
Pathophysiology
Whilst hyperacusis may be idiopathic, in
some individuals there are indications that it
is associated with other symptoms or conditions. The link with migraine was mentioned
above, and both depression and post-traumatic stress disorder have been linked with
hyperacusis. Interestingly these three conditions may share a serotonergic basis, and this
has been proposed as a mechanism underlying hyperacusis [7].
A number of specific medical conditions
can also give rise to sound tolerance symptoms [8]. An absent stapedial reflex, such as
seen in some patients with facial palsy, may
result in an apparent increase in the
perceived intensity of sound. Some patients
report hyperacusis post head injury.
Reduced tolerance to sound is a recognised
feature of Lyme Disease, and has also been
reported in Addison’s Disease, Multiple
Sclerosis, and Fibromyalgia. As such, the clinician who assesses a patient with hyperacusis needs to be mindful of these (and
other) differential diagnoses.
The relationship between hyperacusis
and tinnitus is complex. Many persons with
troublesome tinnitus report hyperacusis
(perhaps as many as 40%), and it does seem
that the overwhelming majority of hyperacusis patients report tinnitus. For some
people sound tolerance was reduced first,
and then tinnitus developed: for others this
was the other way around. This is an area
where more research is needed.
What then are the potential mechanisms
of idiopathic hyperacusis? There are several
theories, and whilst none of them is entirely
satisfactory, they all propose that hyperacusis is a manifestation of increased central
auditory gain. Some have proposed medial
auditory efferent system dysfunction as
underlying this, but evidence regarding the
lack of impact upon loudness tolerance
when efferent modulation of hearing is
ablated (in surgical vestibular nerve section
for example)[9] argues against this. The
possibility of disturbance of serotonin function has been mentioned above, but this is a
very broad proposal, not linking hyperacusis
with the disruption of a particular form of
serotonin or concentration thereof. Sahley
and Nodar [10] considered that glutamate
function in the primary auditory synapses
may be potentiated by opiod peptides
released during stress, leading to the overrepresentation of the intensity of sound. It is
difficult to see how this proposal might be
empirically tested, and in any case the relationship between hyperacusis and stress is
more complex than this implies. In a sense
we are starting from the wrong place in
Figure 1: Self-portrait by an eight year old boy
with hyperacusis. Reproduced with permission
from Tinnitus: a multidisciplinary approach
(Baguley et al. in press).
making these and other suggestions: the fact
is that the specifics of how loudness is
encoded and perceived in the human auditory brain are yet to be fully established [11],
and so it should not be a surprise that the
mechanisms of loudness perception disorders remain obscure.
Work by Craig Formby and colleagues
[12] provides emergent evidence that loudness perception can change, and that it is
influenced by the presence / absence of
sound stimulation. Experiments where
volunteers consistently wore earplugs, or
wideband sound generators (at a stable
intensity) have respectively demonstrated
reduction or increase in the ability to
tolerate intense sound. Work by Munro and
Blount [13] corroborates these findings by
indicating that stapedial reflex thresholds
can reduce when earplugs are consistently
worn. The idea that the human auditory
brain exhibits adaptive plasticity, and can
adapt on the basis of learning, injury and
sound stimulation is now widespread.
Hyperacusis may well be a consequence of a
set of circumstances that influence loudness
perception.
Whilst treatment for hyperacusis is
outside the scope of the present article, one
can note that the concept that adaptive
plasticity underpins the development of
hyperacusis carries also the implication that
the situation can potentially be reversed. As
discussed by other authors in this edition of
Audiology Matters, present treatment
involves education, counselling and sound
therapies, and future work is needed to
optimise such interventions.
Summary
Whilst hyperacusis is not common, there are
significant numbers of people who face
challenges with their reduced sound tolerance. The mechanisms behind the phenom-
enon are presently obscure, and the heterogeneity within the hyperacusis patient
population may be indicative of multiple
mechanisms at work. Evidence regarding a
role for adaptive plasticity is gathering, and
this carries an implied hope that a decrease
in sound tolerance may be reversed.
References
1. Baguley DM, McFerran DJ. Hyperacusis and disorders
of loudness perception. In: Textbook of Tinnitus.
Edited by Møller AR, Langguth B, deRidder D,
Kleinjung T. Springer: New York; 2011.
2. Perlman HB. Hyperacusis. Ann Otol Rhinol Laryngol
1938;47:947-53.
3. Mathisen H. Phonophobia after stapedectomy. Acta
Otolaryngol 1969;68(1):73-7.
4. Baguley DM. Hyperacusis. J R Soc Med
2003;96(12):582-5.
5. Andersson G, Lindvall N, Hursti T, Carlbring P.
Hypersensitivity to sound (hyperacusis): a prevalence
study conducted by the Internet and post. Int J
Audiol 2002;41(8):545-54.
6. Gomot M, Belmote MK, Bullmore ET, Bernard FA,
Baron-Cohen SA. Brain hyper-reactivity to auditory
novel targets in children with high functioning
autism. Brain 2008;131(Pt 9):2479-88.
7. Marriage JE, Barnes NM. Is central hyperacusis a
symptom of 5 hydroxytrytamine (5-HT) dysfunction? J Laryngol Otol 1995;109(10):915-21.
8. Baguley DM, Andersson G, McKenna L, McFerran D.
Tinnitus: a multi-disciplinary approach. WileyBlackwell: London 2012; (in press).
9. Baguley DM, Axon P, Winter IM, Moffat DA. The
effect of vestibular nerve section upon tinnitus. Clin
Otolaryngol Allied Sci 2003;27(4):219-26.
10. Sahley Tl, Nodar RH. A biochemical model of
peripheral tinnitus. Hear Res 2001;152(1-2):43-54.
11. Loudness. Springer Handbook of Auditory Research.
Edited by: Florentine M, Popper AN, Fay RR.
Springer: New York; 2011.
12. Formby C, Sherlock LP, Gold SL. Adaptive plasticity
of loudness induced by chronic attenuation and
enhancement of the acoustic background. J Acoust
Soc Am 2003;114(1):55-8.
13. Munro KJ, Blount J. Adaptive plasticity in brainstem
of adult listeners following earplug-induced deprivation. J Acoust Soc Am 2009;126(2):568-71.
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
71
The Hyperacusis Network
Dan Malcore,
The Hyperacusis
Network,
Post Office Box 8007,
Green Bay,
Wisconsin 54308
H
Correspondence
E: [email protected]
W: www.hyperacusis.net/
yperacusis is a decreased sound tolerance (DST) to normal environmental noise. In many cases it is the unfortunate by-product of living in
a world of excess noise. Other causes of hyperacusis reported by our
community include drug interaction, wax removal (irrigation) of the ear, Lyme
disease, Meniere’s Syndrome, Temporomandibular Joint Syndrome (TMJ), head
Declaration of
Competing Interests
None declared.
injury, Superior Canal Dehiscence Syndrome (SCDS), acoustic neuroma, autism or
Down Syndrome. I will briefly explain my experience.
The Hyperacusis
Network offers a
support network
where people can share
their experiences,
frustrations, and
treatment with one
another
72
In 1991 our local college basketball team
was playing at our local arena. The PA
system was broadcast through only two
Bose speakers 50 feet away from our seats.
It was brutally loud. We registered a
concern with the arena management to no
avail. Weeks later our family flew to Toronto
and was dining at an old fashioned singalong restaurant. We were seated right next
to some tower speakers. Shouting was the
only way to talk. One session featured
chimes. Little did I realise that the entire
chime board was mounted directly above
our table. Returning to Green Bay I met
with my local doctor. He had no answers
and suggested that my hearing sensitivity
would go away after a couple of weeks
convinced that the airplane flight caused
the problem. A few nights later my wife and
I attended a movie at our local cinema.
Alternatively putting my finger in each ear I
noticed that the volume of sound was
nearly three times louder in my left ear over
my right ear. My symptoms were severe and
my life was collapsing around me. In a
matter of only few days I could no longer
tolerate the normal sounds of life (conversation, telephone, television). Even my
young children whispering to me or the
sound of turning my head on my pillow at
night was distressing. I drove six hours to
Mayo Clinic with earmuffs on. The clinician
ordered an MRI and ABR test to rule out
any pathological problems (for example,
acoustic neuroma). These tests were the
final straw for my hearing tolerances. Before
I left Mayo a nurse came into the room and
said, “I am going to put a pocket watch
next to your ear. Each time I take a step
back I will ask you if you can still hear the
ticking of the watch.” She was in disbelief to
witness that after travelling the full length
of the room I could still hear the watch
perfectly. Most patients with hyperacusis
cannot produce an audiogram with minus
decibel hearing levels. This is not the benchmark for diagnosing hyperacusis. Patients
who have loudness discomfort levels below
65 decibels are in distress because they
cannot even tolerate the sound of their
own voice. In my case, I even altered the
loudness and tone of my voice to accommodate my tolerances. I quickly became
hoarse and was on the verge of losing my
voice completely. I left Mayo clinic with no
answers and no way to recover.
After googling ‘noise sensitivity’, I discovered the American Tinnitus Association
and asked them if they had anyone in their
network with my kind of hearing sensitivity.
They introduced me to the term hyperacusis and gave me 75 names of people
around the world who, like me, were
desperate for answers. I wrote to everyone
on the list. Ultimately this gave birth to The
Hyperacusis Network.
To this day, no one understands what
causes hyperacusis. No one knows for sure
what has been affected in a patient’s auditory pathways or sound processing centre
of the brain. Many hearing clinicians know
little about hyperacusis, and often perform
tests on patients which further collapse
their tolerance to sound and give the
patient little hope, leaving them with the
suggestion that they must ‘learn to live with
it.’ This defines the need for our network.
Most patients arrive at our cyber doorstep
hopeless with no idea where to turn. What
is the correct pathway?
First the patient must find a clinician
trained to treat hyperacusis. The best test
to perform on a patient experiencing a
decreased sound tolerance is a Loudness
Discomfort Level (LDL) test. Without this
test it is difficult for a qualified clinician to
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
feature
track the progress of the patient
throughout the course of treatment. Some
patients are so sensitive to sound they are
barely able to function in the world as we
know it. Phonophobia, or fear of sound
further complicates their world. Why? Our
survival instinct suggests to the hyperacusis
patient that if excessive sound is the likely
culprit then sound (noise) must be eliminated from their life to recover. Some
patients are so fearful of noise they flee
from all noise and live in near total isolation. All too often hyperacusis patients
overprotect their ears with earplugs and /
or earmuffs. Tragically this further collapses
their tolerances to sound. One of the few
things we know about hyperacusis is that
silence makes our condition worse. When I
came down with sudden severe hyperacusis in 1991 there was no protocol to
rehabilitate sound tolerances. I was
desperate for help, owned my own business and had a wife and five young children
at home to feed.
In the early 1990s Dr Pawel Jastreboff
from the University of Maryland and Dr
Jack Vernon from the Oregon Health and
Science University were two of the first
clinicians to formulate a retraining therapy
that kept the patient’s hearing active with
carefully delivered broadband noise. The
broadband noise is delivered through noise
(sound) generators custom fit to the
patient’s ears or headphones using a tape
or CD player. Broadband noise comes in
many colours with white noise offering
equal energy in all frequencies. This can be
a problem for the hyperacusis patient
because high frequency sound is especially
difficult to tolerate and is not dominant in
the mainstream of life. The frequency band
that most mimics day to day living is
captured in pink noise. Pink noise features
less energy in the higher frequencies
(above 8,000Hz) and helps the patient
recover more rapidly. The patient is
instructed to listen to broadband noise at
soft tolerable levels for up to eight hours a
day. The patient must make a leap of faith
and understand that even though sound
may have caused their hyperacusis, sound,
if administered correctly, will help them
recover. In turn, if they stick with the
programme, most improve dramatically
and return to the mainstream of life.
The Hyperacusis Network lists clinicians
worldwide who are specially trained to
treat hyperacusis. In most cases the patient
will have to travel to see a qualified trained
clinician because many countries do not
have anyone qualified. Even in the United
States fewer than 20 states have qualified
clinicians. Travel and all the noise associated
with it are often very difficult for hyperacusis patients. Now, with the available technology of noise cancellation headphones
the hyperacusis patient can travel in
comfort. Once they arrive the clinician will
establish a patient’s sound tolerances,
perform hearing tests within those sound
tolerances, rule out any pathological problems, explain the dynamics of hyperacusis,
phonophobia and misophonia, fit the
patient with sound generators, and
customise a sound retraining protocol
specific to the patient’s needs. Ongoing
counselling is also a necessary component
to treatment because many patients do not
experience improvements in their sound
tolerances for weeks or even months. As
with any treatment programme, there are
rough spots where the patient must be
convinced to stay the course or they will
not improve. To help the patient understand that they are making progress typically their LDLs are retested after three
months. During these months patients
yearn to meet others face to face so they
can share their experience and support one
another. Although it has often been stated
that 40% of individuals who have tinnitus
have some degree of hyperacusis, few have
it to the degree where it affects their livelihood and ability to be productive. More
specifically, until the patient improves they
often cannot return to work. For those who
are deeply impacted with severe hyperacusis they find themselves in a position
where even their family and friends cannot
comprehend what decreased sound tolerances are or why it would be such a barrier
to normal living. Hearing loss is easily
understood but not hearing sensitivity. So
where can they find help?
The Hyperacusis Network maintains a
website (www.hyperacusis.net), a message
board (www.chat-hyperacusis), and
publishes a biannual newsletter. Because
severe hyperacusis is rare, there are no local
support networks. This makes hyperacusis
even more isolating. The Hyperacusis
Network offers a support network where
people can share their experiences, frustrations, and treatment with one another. For
the past 21 years the network has heard
from individuals from virtually every
country. There is no membership fee and
no advertising is accepted. Any and all
information is kept confidential and
everyone works as a volunteer. We
welcome the contributions of all clinicians,
patients, and caregivers. We are in particular awe of clinicians who, for some reason,
share their expertise and valuable resources
to explore and help unlock the mystery of
hyperacusis. The only real dilemma
remaining for individuals in the United
States who come down with severe hyperacusis is cost.
Have you thought of working together with
ENT & audiology news to produce a
supplement featuring a selection of our
published articles tailoured to your topic of
interest and specialty?
You choose the relevant articles and we will add a front cover branded
with your company identity, an editorial welcome and a declaration
from ENT & audiology news that you are the supplements sponsor.
For more information contact Nova McMillan,
[email protected]
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
example of the supplement
sponsored by Mertz
73
Sound Tolerance in Autism
Spectrum Disorder
Teresa Tavassoli,
PhD,
Postdoctoral Fellow,
Seaver Autism Center,
Mount Sinai School of
Medicine,
Department of
Psychiatry, Box 1668,
1 Gustave L. Levy Place,
New York,
NY 10029, USA.
Correspondence
E: teresa.tavassoli@
gmail.com
Declaration of
Competing Interests
None declared.
“My hearing is
like having a
sound amplifier
set on maximum
loudness”
Temple Grandin, 1996
74
ost of us can organise sensory stimuli
such as sounds from the environment
appropriately, and often even have a
pleasurable experience, allowing us to enjoy
listening to the wind in the trees. However, for
some individuals even mild sensory stimuli can be
overwhelming and potentially unpleasant.
Anecdotal reports of individuals with Autism
Spectrum Disorder (ASD), such as the quote by
Temple Grandin, show that hearing and sound
tolerance might be affected. Once someone with
Autism Spectrum Disorder told me that their day
was not enjoyable because the ‘wind is too loud’.
Autism Spectrum Disorders are conditions
affecting up to 1% of the population [1], and are
characterised by difficulties in the development of
social relationships and communication alongside
unusually narrow interests and repetitive behaviour
[2]. However, sensory reactivity issues are associated with core features of ASD and may even form
the basis for some of the deficits [3] but also some
of the strengths noted with the condition [4].
Changes have been proposed to the new
Diagnostic and Statistical Manual for Mental
Disorders (DSM) V criteria, which include “hyperor hypo-reactivity to sensory input or unusual
interest in sensory aspects of environment (such as
apparent indifference to pain / heat / cold, adverse
response to specific sounds or texture, etc.)”.
Well-known anecdotal reports about sensory
perception, such as sound intolerance, in ASD
come from Temple Grandin, a professor in the USA
with high functioning autism, who describes some
of her early sensory experiences as the following:
“My hearing is like having a sound amplifier set on
maximum loudness” [5]. Grandin also reported
“some of the sounds that are most disturbing to
autistic children are the high-pitched, shrill noises
made by electrical drills, blenders, saws, and
vacuum cleaners”. Indeed, there is a wealth of
personal accounts, for example Darren White
reports that he “could rarely hear a sentence
because his hearing distorted them”. Furthermore,
Darren White writes “Another trick which my ears
played was to change the volume of the sounds
around me. Sometimes when the other kids spoke
to me I could scarcely hear them and sometimes
they sounded like bullets… Life was terrifying in
those days” [6]. Anecdotal reports are important
windows into the way in which individuals with
ASD might perceive the world around them. They
teach us about high variability seen in ASD, potential links to autistic traits and the importance of a
functional sensory perception for everyday life.
M
Anecdotal reports can also help develop ideas
about sound tolerance in ASD. However one limitation of anecdotal reports is that they are not
quantitative. To address the issue of quantification,
sensory questionnaires and laboratory studies have
been used to measure hearing issues in ASD
showing that individuals with ASD have unusual
auditory processing. Regarding low-level auditory
processing, individuals with ASD show superior
pitch processing [7]. However looking at more
complex auditory processing such as speech, individuals with ASD sometimes show difficulties [8, 9].
Alcantara et al. [9] measured speech reception
thresholds (level of correctly identifying speech)
and showed that individuals with ASD perform
worse across auditory conditions and show lower
speech-to-noise perception. This study used
complex auditory stimuli, which requires the ability
to integrate and filter information. In contrast, on a
lower perceptual level of speech processing children with ASD show superior processing
compared to typically developing children [10].
Further evidence for enhanced low-level auditory processing comes from Mottron et al. [10]
reporting exceptional absolute judgment and
production of pitch in a case study. In 2006,
Mottron et al. [11] summarised this line of sensory
research by postulating the ‘enhanced perceptual
functioning’ (EPF) model of ASD, characterised by
superior low-level perceptual processing [11,12] .
O’Riordan & Passetti [13] also report superior
auditory discrimination ability in children with
ASD, and as noted above Järvinen-Pasley et al.
show superior perceptual processing of speech in
children with ASD [14]. Bonnel et al. reported
superior pitch sensitivity in individuals with ASD
using psychoacoustic tasks (judging the pitch of
pure tones in a ‘same-different’ discrimination task
and in a ‘high-low’ categorisation task) [7]. They
also performed audiometric tests beforehand
using various frequencies: the usual 250Hz, 500Hz,
1kHz, 2kHz, 4kHz, and 8kHz, plus five additional
frequencies: 750Hz, 1062Hz, 1.5kHz, 3kHz, and
6kHz. They report no difference between groups
on audiometric tests. Nevertheless individuals
with ASD seem to be affected by high pitched
auditory stimuli, such as electrical drills [5] and
tests using higher frequencies might be needed.
Furthermore, individuals with ASD show hyperacusis, which can be described as increased perception of loudness [15].
Several hypotheses have been put forward to
explain sensory atypicalities seen in individuals
with ASD. Early theories such as a chronic state of
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
feature
physiological overarousal or sensory deprivation in children with ASD have been disregarded as possible explanations. A more
recent review also suggests that over-arousal
might not be true for everyone on the
autistic spectrum [16]. Ornitz (1974) also put
forward the idea of sensory modulation
dysfunction, which can be due to differences
in filtering information or an imbalance in
excitation and inhibition [17]. The idea of
sensory modulation dysfunction: the inability
to regulate sensory input in an adaptive way;
is also supported by current research [18].
Another recent explanation as mentioned
above is Mottron et al.’s ‘enhanced sensory
functioning’ model, which suggests
‘enhanced low-level discrimination and
perception, diminished perception of
complex movement and autonomy of lowlevel information processing toward higherorder operations’ [11].
Even though there is a wide range of
studies to date showing sensory issues in
ASD, such as sound intolerance, there is no
consensus on how to measure hearing in
individuals with ASD. The proposed changes
to the DSM V include ‘hyper-or hypo-reactivity to sensory input or unusual interest in
sensory aspects of environment’, but we still
need to decide how to measure sensory reactivity. As seen in the literature sensory questionnaires, laboratory tests such as auditory
pitch or discrimination thresholds and even
brain imaging studies to measure neural activation towards sounds are used [19]. So far,
sensory questionnaires such as the widely
used Sensory Profile show robust differences
in hearing in ASD across different ages [20,21].
When conducting my PhD at the Autism
Research Centre at the University of
Cambridge under the tutelage of Prof BaronCohen we used the Sensory Profile in children
and adults with ASD as well. The caregiver
Sensory Profile for example is a 125 item
sensory questionnaire investigating children's
reactivity towards everyday stimuli and it is
commercially available (www.pearsonassessments.com). Two out of the eight items
regarding hearing are: ‘Holds hands over ears
to protect ears from sound’ or ‘Responds
negatively to unexpected or loud noises (for
example, cries or hides at noise from vacuum
cleaner, dog barking or hair dryer)’. The lower
the score on the Sensory Profile the more
sensory difficulties the child has (classifications are based on the performance of over
1,000 children without disabilities). We found
that children with ASD showed more auditory processing difficulties and scored lower
on the Sensory Profile auditory processing
subscale (average score of 21 out of 40)
compared to typically developing children
(average score of 36 out of 40). In addition we
used another sensory questionnaires, the
Sensory Over-Responsivity Scale (SenSOR)
[22]. The SenSOR was developed to specifically investigate exaggerated responses to
one or more type of sensory stimuli in children and adults. Some questions would for
example ask by which items in the environment someone is bothered, such as a clock
ticking. The higher the score on the SenSOR,
the more sensory difficulties someone has.
We asked adults with and without ASD
about their sound tolerance using this
measure. Adults with ASD were bothered by
more auditory items in the environment
(average score of 9) compared to typical
controls (average score of 4). To date sensory
questionnaires seem to be the most widely
used tools to screen for sound intolerance in
individuals with ASD. In addition more objective hearing tests should be used to establish
if hearing thresholds or discrimination are
affected in children and adults with ASD.
Further research on sound tolerance in
children and adults with ASD is needed to
help individuals with ASD to enjoy listening
to the wind in the trees. Besides the present
scientific inquiries into the nature of sensory
differences in ASD, it is important to use
sensory-related research findings to improve
the lives of individuals with ASD. For two
years (from 2009-2011) I have been part of
the Royal College of Art and the Helen
Hamlyn Center for Design’s Expert Panel for
housing for adults with autism. A design
guideline booklet was published in 2010 with
design themes such as growth, development
and triggers [23]. Understanding the differences in sensory perception was critical to
development of the ‘triggers’ section,
including guidelines to designing environments with comfortable acoustics. The environment we live in has a great impact on our
wellbeing, and rather than trying to change
individuals themselves, we can just change
the environment around them.
9.
17. Ornitz, E. M. (1974). The modulation of sensory input
and motor output in autistic children. J Autism Dev
Disord 1974;4(3):197-215.
References
1.
Baird, G, Simonoff E, Pickles A, Chandler S, Loucas T,
Meldrum D, Charman T. Prevalence of disorders of the
autism spectrum in a population cohort of children in
South Thames: the Special Needs and Autism Project
(SNAP). Lancet 2006;368(9531):210-5.
2.
DSM-IV: Diagnostic and Statistical Manual of Mental
Disorders (4th Edition). Washington DC, USA: American
Psychiatric Association; 1994.
3.
Boyd BA, Baranek GT, Sideris J, Poe MD, Watson LR,
Patten E, Miller H. Sensory features and repetitive behaviors in children with autism and developmental delays.
Autism Res 2010;3(2):78-87.
4.
Baron-Cohen S, Ashwin E, Ashwin C, Tavassoli T,
Chakrabarti B. Talent in autism: hyper-systemizing,
hyper-attention to detail and sensory hypersensitivity.
Philos Trans R Soc Lond B Biol Sci 2009;364(1522):137783.
Alcántara JI, Weisblatt EJ, Moore BC, Bolton PF. Speechin-noise perception in high-functioning individuals with
autism or Asperger's syndrome. J Child Psychol Psychiatry
2004;45(6):1107-14.
10. Mottron L, Burack JA, Stauder JE, Robaey P. Perceptual
processing among high-functioning persons with
autism. J Child Psychol Psychiatry 1999;40(2):203-11.
11. Mottron L, Dawson M, Soulières I, Hubert B, Burack J.
Enhanced perceptual functioning in autism: an update,
and eight principles of autistic perception. J Autism Dev
Disord 2006;36(1):27-43.
12. Samson F, Mottron L, Soulières I, Zeffiro TA. Enhanced
visual functioning in autism: An ALE meta-analysis. Hum
Brain Mapp 2011;doi: 10.1002/hbm.21307.
13. O'Riordan, M., & Passetti, F. (2006). Discrimination in
autism within different sensory modalities. J Autism Dev
Disord 2006;36(5):665-75.
5.
Grandin T. Thinking in pictures. Vancouver, WA, USA:
Vintage Books; 1996.
6.
White BB, White MS. Autism from the inside. Medical
Hypotheses 1987;24(3):223-9.
14. Järvinen-Pasley A, Wallace GL, Ramus F, Happé F,
Heaton P. Enhanced perceptual processing of speech in
autism. Dev Sci 2008;11(1):109-21.
7.
Bonnel A, Mottron L, Peretz I, Trudel M, Gallun E,
Bonnel AM. Enhanced pitch sensitivity in individuals
with autism: a signal detection analysis. J Cogn Neurosci
2003;15(2):226-35.
15. Khalfa S, Bruneau N, Rogé B, Georgieff N, Veuillet E,
Adrien JL, Barthélémy C, Collet L. Increased perception
of loudness in autism. Hear Res 2004;198(1-2):87-92.
8.
Teder-Sälejärvi WA, Pierce KL, Courchesne E, Hillyard SA.
Auditory spatial localization and attention deficits in
autistic adults. Brain Res Cogn Brain Res 2005;23(23):221-34.
16. Rogers SJ, Ozonoff S. Annotation: What do we know
about sensory dysfunction in autism? A critical review of
the empirical evidence. J Child Psychol Psychiatry
2005;46(12):1255-68.
18. Ben-Sasson A, Hen L, Fluss R, Cermak SA, Engel-Yeger B,
Gal E. A Meta-analysis of Sensory Modulation symptoms in Individuals with Autism Spectrum Disorders. J
Autism Dev Disord 2008;39(1):1-11.
19. Gomot M, Belmonte MK, Bullmore ET, Bernard FA,
Baron-Cohen S. Brain hyper-reactivity to auditory novel
targets in children with high-functioning autism. Brain
2008;131(Pt 9):2479-88.
20. Tomchek SD, Dunn W. Sensory processing in children
with and without autism: a comparative study using the
short sensory profile. American Journal of Occupational
Therapy, 2007;61(2):190-200.
21. Crane L, Goddard L, Pring L. Sensory processing in
adults with autism spectrum disorders. Autism
2009;13(3):215-28.
22. Schoen SA, Miller LJ, Green KE. Pilot study of the
Sensory Over-Responsivity Scales: assessment and inventory. Am J Occup Ther 2008;62(4):393-406.
23. Brand A. Living in the Communitiy: Housing Design for
Adults with Autism. London: Helen Hamlyn Centre;
2010. [www.hhc.rca.ac.uk/CMS/files/
1.Living_in_the_Community.pdf]
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
75
New Findings on Hyperacusis
in Williams Syndrome
Prof Joseph Attias,
Department of
Audiology & Clinical
Neurophysiology,
Schneider Children's
Medical Center of Israel,
Petach Tikva, affiliated
with Sackler Faculty of
Medicine, Tel Aviv
University, Tel Aviv, and
Department of
Communication Sciences
& Disorders, University
of Haifa, Haifa, Israel.
Correspondence
E: [email protected]
Declaration of
Competing Interests
None declared.
illiams syndrome is a genetic
neurodevelopmental disorder
caused by a hemizygous
microdeletion of about 1.55-1.83Mb on the
long arm of chromosome 7 which is located
at position 11.23 (7q11.23). Its estimated
prevalence ranges from 1:7,500 to 1:20,000
live births [1]. Williams syndrome is manifested by a wide range of medical conditions and a unique behavioural and cognitive profile. The main physical features are
typical facies, supravalvular aortic stenosis,
failure to thrive, short stature, transient
neonatal hypercalcaemia, and delayed
language and motor development.
Behaviourally, patients have a strong social
appetite and a low level of social fear [2].
The mean cognitive level is within the range
of mild to moderate retardation, with some
peaks and troughs in mental domains,
particularly severe visuospatial construction
deficits accompanied by a relative strength
in expressive language [3] and relatively
spared face and object recognition.
One of the chief and obvious symptoms
of Williams syndrome is an oversensitivity to
sound which is reflected in three auditory
behaviours: auditory fascination, defined as
an above-normal attraction to, or fascination
with, certain sounds [4]; phonophobia,
defined as an aversion to, or morbid fear of,
normal sounds; and hyperacusis, defined as
an oversensitivity or collapsed tolerance to
usual environmental sounds. An additional
salient feature is a strong attraction to music.
W
Hyperacusis in Williams Syndrome
Hyperacusis should be distinguished from
another phenomenon called auditory
recruitment, which is always a by-product
of sensorineural hearing loss and is better
understood. It causes the perception of
sound to be exaggerated (sounds getting
too loud too fast), resulting in sound distortion and patient discomfort. It has been
attributed to the ‘recruitment’ of intact hair
cells by damaged hair cells, mainly outer
ones, in neighbouring critical bands. The
recruited (working) cells ‘hear’ both their
own frequency and the frequency of the
damaged cells, thereby increasing their
signal. The net effect is a short loudness
dynamic range between the softest sound
the subject can hear (owing to the hearing
76
loss) and the loudest sound they can
comfortably tolerate (owing to the recruitment).
Hyperacusis in Williams syndrome apparently begins before one year of age and
tends to decrease somewhat during
adolescence. Although it is debilitating,
research into its aetiology and pathophysiology has been limited. The behavioural
reactions of affected subjects may be
extreme, including covering the ears, crying,
or avoiding noise-related situations. For
example, children with Williams syndrome
may refrain from attending birthday parties
due to their fear of the noise of bursting
balloons [5,6]. In our previous study with a
relatively large sample of 49 patients with
Williams syndrome aged 1-35 years [7],
83.7% reported being frightened or bothered by normal environmental sounds;
most were sensitive to more than one
sound. Hyperacusis was most severe at age
5.7 years. The most common behavioural
responses were covering the ears (67.4%),
leaving the area (62.8%), complaining
(51.4%), crying (44.2%), hugging (25.6%),
asking to stop the noise (16.3%), panic
behaviour (14.0%), and getting into bed
(14.0%). Most instances of hyperacusis were
associated with high-intensity noise of lowmedium frequency and variable degrees of
continuity, such as that emitted by electrical
devices, thunder, bursting balloons, sirens /
alarms, shouting, loud music, and motor
vehicles. Other disturbing noises reported
by a minority of patients were people
crying, television, ringing telephones, and
applause. On a scale of 1 to 5, the mean
rating of the degree to which noise sensitivity interfered with the children’s lives was
3.5 (grade 3: ‘markedly distracts the child or
makes the child stop his / her activity’; grade
4: ‘runs away from the site of the sound or
needs to be prepared before going to a
place where he / she will be exposed to the
sound’).
Genetics of hyperacusis
The prevalance and early emergence of
hyperacusis in children with Williams
syndrome suggests that one or more of the
25-28 genes from the deleted 7q11 region
play a role in auditory processing and
perception. Indeed, Williams syndrome
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
feature
offers an attractive and unique model for
investigating the genetics and biology of
congenital hyperacusis, with potential therapeutic implications. Studies have shown
direct and indirect associations among
several genes that may be involved in the
Williams syndrome phenotype, especially in
the hearing loss and oversensitivity to
sounds. The main candidate genes are ELN,
LIMK1, and GTF2.
Elastin gene (ELN)
In mammals, two types of sensory cells, the
inner and outer hair cells, are essential to the
mechano-electrical transduction process
leading to sound detection. It is generally
accepted that the inner hair cells function as
true sensors whereas the outer hair cells
participate in the feedback mechanism of
the ‘cochlear amplifier’, an active tuning
process responsible for the extreme sensitivity and frequency selectivity of the hearing
organ. At the heart of the feedback mechanism are the rapid length changes in isolated
outer hair cells brought on by conformational changes in voltage-sensitive motor
units situated in their plasma transmembrane. The motor molecule has been putatively identified as the membrane protein
prestin [8]. The hair cell changes are driven
by the shearing motion of their stereocilia in
response to sound-induced vibrations of the
cochlear fluid, which opens the ion channels
and leads to depolarisation of the hair cells.
This action depends largely on the actin
cytoskeleton and elastic extracellular filaments of the stereocilia. Although the exact
contents of the extracellular link remain
unclear, studies have reported that the
protease enzyme elastase disintegrates tip
links [9].
ELN encodes elastin, the ‘elastic’ protein in
connective tissue that allows tissues to
resume their shape after stretching or
contracting. It has been associated with the
Williams syndrome phenotype of
supravalvular aortic stenosis. Elastin is
expressed in the blood vessels of the brain
and probably also the cochlear vessels.
Studies suggest that a haploinsufficiency
(one functional copy) of ELN may be
involved in the cochlear dysfunction in
hyperacusis via several potential mechanisms: (1) reduced perfusion of the cochlea
due to vascular stenosis, resulting in hypoxia
and cell damage, primarily to the outer hair
cells, and ultimately, outer and inner hair cell
death; (2) increasing rigidity of tissue in the
organ of Corti, especially the basilar
membrane; and (3) desynchronisation of
the movement of the stereocilia with
acoustic neural firing, leading to dysfunc-
Figure 1: Mean air-conduction thresholds and standard error (both right and left ears). Hearing
thresholds were poorer bilaterally in the Williams syndrome (WS) group than in the typical developing
(TD) controls, especially in the high frequencies (repeated measure ANOVA: P<001 for both ears).
tions in the sensory (cochlear amplifier;
hearing loss) neural, and neural-motor
(acoustic reflexes) activities of the auditory
pathways.
General transcription factor IIi gene
(GTF2I)
The GTF2I gene encodes a multifunctional
phosphoprotein involved in transcription
and signal transduction. It is highly
expressed during development and in
normal neuronal tissues. There is strong
evidence that two members of the GTF2I
gene family, GTF2I and GTF2IRD1, play a role
in the craniofacial features of Williams
syndrome and some of its neurobehavioural features [10]. In 2010, Lucena et al.
[11] generated a mutant mouse model with
a frame deletion of exon 2 of the GTF2I
gene. The animals heterozygous for the
mutation showed a remarkable neurobehavioural phenotype consisting of
decreased exploratory activity despite
normal motor co-ordination, enhancer
anxiety, and a low threshold for sound
tolerance; oversensitivity to sounds was
evident at 65dB.
Lim Domain Kinase 1 Gene (LIMK1)
LIMK1 encodes a serine / threonine kinase
that regulates actin reorganisation. It is probably a component of an intracellular
signalling pathway that mediates proteinprotein interactions and may be involved in
brain development. On a fear-conditioning
test, LIMK1 knockout mice showed significantly longer and more constant freezing
than wild-type mice after exposure to certain
sounds [12]. Recently, Matsumoto et al. [13]
linked LIMK to outer cell hair motility and
cochlear amplification, suggesting that ‘any
disruption in the signalling pathways
involving these molecules could result in
extreme physiological responses such as
hyperacusis or deafness’. They found that
activation or inhibition of LIMK-mediated
pathways increased or decreased, respectively, both electromotile amplitude and
total length of the cochlear outer hair cells,
without affecting the performance of the
motor proteins (prestin) embedded in the
plasma membrane. Thus, genetic or physiological aberrations that lead to disruption or
malfunction of the mechanism(s) regulating
outer hair cell motility, such as the LIMK1
deficiency in subjects with Williams
syndrome, could be the underlying cause of
hyperacusis and of the increasing risk of
mechanical damage of the inner hair cell
bundle which necessarily leads to progressive
hearing loss.
Other Auditory Phenotypes in
Williams Syndrome
Hearing Loss
Figure 1 depicts the average audiogram of
patients with Williams syndrome at our
tertiary medical centre. Most of the patients
had bilateral, asymmetrical, high-frequency
cochlear hearing loss across 3-8kHz. Severity
ranged from 25-55dB on the right and 25110dB on the left. The hearing loss began
early in life and was progressive. Conductive
hearing loss associated with otitis media
with effusion has been well documented in
children with Williams syndrome [14]. In our
patients, about 10% had pure conductive
hearing loss at a frequency range of 0.252kHz, and the rest had either mixed hearing
loss or normal hearing thresholds. The
asymmetric configuration (left > right) of
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
77
feature
the cochlear hearing loss resembles the
hearing loss induced by repeated exposure
to high-level noise in typically developing
subjects. However, none of the children in
our sample was exposed to noise at a highrisk level, and all lived in a normal environment. The origin of the cochlear hearing
loss in Williams syndrome is still under
investigation. It may be associated with a
deficiency in one or more of the ELN or
GTF2I genes through a dysfunction
primarily of the outer hair cells in the
cochlea.
Low otoacoustic emissions
Patients with Williams syndrome who have
normal or abnormal audiograms but healthy
middle-ear functions show significantly
lower otoacoustic emissions (OAE) amplitudes either for transient-evoked [15] or
distortion products [7], than age-matched
controls. These findings may reflect a hereditary cochlear amplification disorder. The
lower OAEs probably increase the predisposition of the patients to noise-induced
damage even in everyday environments.
Absent acoustic stapedial reflexes
Hyperacusis is a chief symptom in clinical
conditions involving the absence or
abnormal function of the stapedial muscles,
either due to facial malfunction, such as in
Bell’s palsy or Ramsay Hunt syndrome, or
following resection of the stapedial muscle
in stapedectomy. Unexpectedly, ipsilateral
acoustic stapedial reflexes (ASRs), even to
wide-band intensive stimulation, are absent
in more than 70% of individuals with
Williams syndrome, irrespective of the
severity of the cochlear hearing loss
[7,14,15]. The reason remains unclear. The
absence of ASRs may represent an overt
reaction of the genetic disorders in Williams
syndrome or may be a consequence of an
auditory or facial nerve dysfunction along
the acoustic stapedial arc. In one study, individuals with Williams syndrome exhibited a
delayed wave I on auditory brainstem
response test [7], suggesting desynchronisation of the activity of the acoustic nerve
which initiates stapedial muscle contraction.
Dysfunctional auditory efferent
system
Another auditory system that may play a
significant role in the hypersensitivity to
sounds in Williams syndrome as well as to
the high tone losses is the olivocochlear
system of the auditory efferent pathway. A
previous study showed that unexpectedly,
in response to low-level contralateral noise,
patients with Williams syndrome showed
78
higher OAE amplitudes compared to
controls, both globally and in discrete
frequency bands. This may reflect neural
hyperexcitability of the auditory efferent
system. Since auditory efferent activity is
initiated and largely affected by the afferent
auditory system, it is possible that the
medial olivocochlear hyperexcitability in
Williams syndrome is caused by the provision of abnormal loudness information by
the afferent auditory pathways to the
efferent neural system or by hyperactivity of
the auditory medial olivocochlear system.
The pattern of efferent auditory malfunction in Williams syndrome is different from
that in patients with other auditory
complaints, such as tinnitus, and in patients
with difficulty listening to background
noise, who usually exhibit hypoaction of
the efferent olivocochlear system [18].
Treatment of hyperacusis
Therapeutic approaches to the hyperacusis
in Williams syndrome vary from ear protectors (ear plugs, muffs) to acoustic-visualbehavioural training or gradual exposure to
sound sources with a progressive increase in
intensity. The exact type of treatment
should be matched to the age, disease
severity, and the individual patient’s ability
to mentally co-operate. Behavioural training
can also be specific to certain sounds, with
recognition of the source of familiar noises
or noises that are directly related to the
patient’s hyperacusis. Alternatively, binaural
or controlled free-field exposure in soundproof rooms to non-specific sounds and
noises of increasing intensity could directly
affect the plasticity (adaptation) of the
central and peripheral structures responsible for the hyperacusis. This should be
accompanied by behavioural relaxation
techniques and, in some cases, medication.
In the event of significant cochlear hearing
loss, hearing aids are recommended, with
low gain at the first fitting and a progressive
increase in intensity.
Conclusion
Hyperacusis in Williams syndrome is characterised by an early onset and steady
depreciation during life. It is accompanied
by a progressive high-tone cochlear hearing
loss and associated with a lack of acoustic
reflexes, hyperaction of the efferent auditory system, and reduced level of otoacoustic emissions. Hyperacusis is probably
a phenotype of the genetic disorders in
Williams syndrome. Its early diagnosis and
treatment, primarily by acoustic training, is
highly recommended, along with fitting
hearing aids when necessary.
References
1. Meyer-Lindenberg A, Mervis CB, Berman KF.
Neural mechanisms in Williams syndrome: a
unique window to genetic influences on cognition
and behaviour. Nat Rev Neurosci 2006;7(5):380-93.
2. Mobbs D, Garrett AS, Menon V, Rose FE, Bellugi U,
Reiss AL. Anomalous brain activation during face
and gaze processing in Williams syndrome.
Neurology 2004;62(11):2070-6.
3. Mervis CB, Robinson BF, Bertrand J, Morris CA,
Klein-Tasman BP, Armstrong SC. The Williams syndrome cognitive profile. Brain Cogn
2000;44(3):604-28.
4. Levitin DJ, Cole K, Lincoln A, Bellugi U. Aversion,
awareness, and attraction: investigating claims of
hyperacusis in the Williams syndrome phenotype.
J Child Psychol Psychiatry 2005;46(5):514-23.
5. Van Borsel J, Curfs LM, Fryns JP. Hyperacusis in
Williams syndrome: a sample survey study. Genet
Couns 1997;8(2):121-6.
6. Klein AJ, Armstrong BL, Greer MK, Brown FR.
Hyperacusis and otitis media in individuals with
Williams syndrome. J Speech Hear Disord
1990;55(2):339-44.
7. Gothelf D, Farber N, Raveh E, Apter A, Attias J.
Hyperacusis in Williams syndrome Characteristics
and associated neuroaudiologic abnormalities.
Neurology 2006;66(3):390-5.
8. Homma K, Dallos P. Evidence that prestin has at
least two voltage-dependent steps. J Biol Chem
2011;286(3):2297-307.
9. Meyer J, Furness DN, Zenner HP, Hackney CM,
Gummer AW. Evidence for opening of hair-cell
transducer channels after tip-link loss. J Neurosci
1998;18(17):6748-56.
10. Hirota H, Matsuoka R, Chen XN, Salandanan LS,
Lincoln A, Rose FE, Sunahara M, Osawa M, Bellugi
U, Korenberg JR. Williams syndrome deficits in
visual spatial processing linked to GTF2IRD1 and
GTF2I on chromosome 7q11.23. Genet Med
2003;5(4):311-21.
11. Lucena J, Pezzi S, Aso E, Valero MC, Carreiro C,
Dubus P, Sampaio A, Segura M, Barthelemy I,
Zindel MY, Sousa N, Barbero JL, Maldonado R,
Pérez-Jurado LA, Campuzano V. Essential role of
the N-terminal region of TFII-I in viability and
behavior. BMC Med Genet 2010;11:61.
12. Meng Y, Zhang Y, Tregoubov V, Falls DL, Jia Z.
Regulation of spine morphology and synaptic
function by LIMK and the actin cytoskeleton. Rev
Neurosci 2003;14(3):233-40.
13. Matsumoto N, Kitani R, Kalinec F. Linking LIMK1
deficiency to hyperacusis and progressive hearing
loss in individuals with Williams syndrome.
Commun Integr Biol 2011;4(2): 208-10.
14. Klein AJ, Armstrong BL, Greer MK, Brown FR.
Hyperacusis and otitis media in individuals with
Williams syndrome. J Speech Hear Disord
1990;55(2):339-44.
15. Attias J, Raveh E, Ben-Naftali NF, Zarchi O, Gothelf
D. Hyperactive auditory efferent system and lack
of acoustic reflexes in Williams syndrome. J Basic
Clin Physiol Pharmacol 2008;19(3-4):193-207.
16. Zarchi O, Attias J, Gothelf D. Auditory and visual
processing in Williams syndrome. Isr J Psychiatry
Relat Sci 2010;47(2):125-31.
17. Marler JA, Elfenbein JL, Ryals BM, Urban Z,
Netzloff ML. Sensorineural hearing loss in children
and adults with Williams syndrome. Am J Med
Genet A 2005;138(4):318-27.
18. Attias J, Bresloff I, Furman V. The influence of the
efferent auditory system on otoacoustic emissions
in noise induced tinnitus: clinical relevance. Acta
Otolaryngol 1996;116(4):534-9.
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
Military Trauma and its Influence
on Loudness Perception
Prof Marc Fagelson,
PhD, CCC-A,
Director of Audiology,
Department of
Audiology & Speech
Language Pathology
East Tennessee State
University Johnson City,
TN 37614, USA.
he sensory mislabelling of environmental events is one of the more
pronounced disruptions associated with posttraumatic stress disorder
(PTSD) [1]. Mislabelling, in this context,
results from an erroneous or exaggerated
neural representation of a sound, sight,
tactile sensation, or smell producing a
perception that does not correspond accurately to the magnitude of the related stimulus. The exaggerated perception may
trigger in the patient with PTSD a state of
hyperarousal that produces profound
negative reactions and ultimately
contributes to avoidance behaviours. The
resulting sensations of pain and anguish
experienced by the affected individual may
upset their most valued relationships, drive
family members apart, and produce in the
sufferer a sense of despair or self-loathing.
In order to facilitate a patient’s ability to
manage hyperacusic responses, and to
understand the physiologic mechanisms of
sensory mislabelling, it is essential for clinicians to address the contributions of
psychological distress to the auditory experience. A management plan that incorporates interdisciplinary teams of professionals stands the best chance of
improving the coping skills of individuals
thus affected.
It is often the case that veterans who
experience hyperacusis have hearing loss
and tinnitus; what sets the patient with
PTSD apart is an increased likelihood that
they will rate sound tolerance problems as
more severe than tinnitus and hearing loss.
Patients report that they avoid certain
social and occupational situations due to
T
Correspondence
E: FAGELSON@
mail.etsu.edu
Declaration of
Competing Interests
None declared.
Figure 1: Patient ratings of tinnitus loudness
and degree of sound tolerance problems
experienced during routine activities.
Ratings are based on verbal response and a
0-10 scale.
sound-triggered pain and discomfort when
in the presence of everyday sounds that do
not bother their colleagues, friends, or
family. Table 1 summarises findings from
the last 500 patients observed at a
Veterans’ Affairs clinic in the US with
respect to the rating of sound tolerance
problems compared to pure-tone sensitivity and ratings of hearing loss and
tinnitus. It is clear that the veterans with
PTSD experience the loudness of environmental sounds in a substantially different
way from patients with similar absolute
threshold, and similar ratings of tinnitus
loudness. Patients with PTSD are also three
times more likely to state that exposure to
loud sounds exacerbates tinnitus loudness
than members of the other groups [2]. The
difference extends to comparisons of
patients with tinnitus and psychological
disorder (that is, anxiety, depression, or
panic attacks) other than PTSD (Figure 1).
To what do we attribute the apparent
mislabelling of sound that affects patients
with PTSD to a greater degree than
patients without PTSD?
The DSM-IV [3] specifies the symptoms
that must be present to endorse a PTSD
diagnosis as including, 1) exposure to traumatic stressor, 2) re-experiencing symptoms (flashbacks), 3) avoidance and
numbing symptoms, 4) hyperarousal, 5)
duration of symptoms > one month, and
6) significant distress or impairment of
functioning. The symptoms associated
with hyperarousal are consistent with
patients’ complaints regarding the experience of excessive loudness associated with
moderate sound levels. When confronted
Table 1. Patient group information regarding tinnitus, hyperacusis, and hearing loss. Ratings are based on verbal
response and a 1-10 scale.
Patients with:
80
Tinnitus Only
Tinnitus + Psych Dx
Tinnitus +PTSD
Mean
SD
Mean
SD
Mean
SD
PTA (better ear)
33.8
7.6
33.2
8.1
34.4
7.7
PTA (worse ear)
38.1
8.8
38.8
9.2
38.3
8.9
Hyperacusis Rating (1-10)
4.40
3.4
4.46
2.8
7.51
2.7
Tinnitus Loudness Rating (1-10)
6.37
2.5
6.51
2.1
6.78
2.2
Hearing Loss Rating (1-10)
5.13
2.6
5.28
2.7
6.10
2.7
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
feature
by the perception of excessive loudness,
patients respond as though they are in
danger, they maintain an alert state that is
disproportionate to the stimulus magnitude.
Hyperarousal is recognised as a condition in which patients experience reduced
pain tolerance, anxiety, exaggerated startle
responses, insomnia, and heightened
sympathetic nervous system activation. If
we consider an exaggerated neural
response as an element of PTSD-related
hyperarousal, then it is reasonable to think
of hyperacusis as a sound-based analogue
of reduced pain tolerance. Patients who
experience startle responses that compel
withdrawing from social situations illustrate the power of the sensory mislabelling.
Clearly, the individual with PTSD who
experiences an episode of hyperacusis
perceives something that is not present in
the environment, and their reaction,
although perhaps appropriate at some
time in the past when a similar sound signified danger, would not be appropriate in
their present circumstance.
The concept of hyperarousal and its relation to the fight-or-flight response is attributed, as early as 1915, to Walter Bradford
Cannon [4]. The Cannon-Bard theory of
emotion centres on the thalamus and its
output to cortical regions associated with
responses to powerful emotions. Thalamic
activity could trigger aversive conditioned
responses when, for example, its output is
associated consistently with specific events
that evoke powerful negative emotions.
For the hyperacusic veteran, the response
to unexpected impulse sounds is learned;
the soldier is trained to recognise the
sounds’ value as a survival threat. Combat
veterans consistently lash out, sometimes
violently, at friends and family when startled or when woken suddenly from sleep.
For those veterans with experience in
combat zones, the responses are difficult
to extinguish, they are not easily unlearned.
Therefore, while such patients recognise
they behave inappropriately, or antisocially, they are unable to adopt new
strategies to cope with arousing environmental conditions.
Bremner and colleagues [5] attributed
poor coping strategies among veterans
with PTSD to trauma-related changes in
neural circuitry, specifically involving the
hippocampus, that impaired recalling or
learning effective coping strategies.
Bremner measured reductions in
hippocampal volume among returning
combat veterans and related the changes
to specific deficits in memory and learning.
Such impairment affected both declarative
memory (that is, remembering facts) and
non-declarative memory (that is, how to
brush one’s teeth, or, for our purposes,
remembering how to react to an environmental event) [6]. The inability to develop
or retain appropriate emotional (and ultimately physical) responses to environmental stimuli was viewed as a direct
consequence of hippocampus impairment.
PTSD-related hyperarousal is attributed
to a variety of neural and biochemical
sources. In addition to hippocampus
involvement, chronic excessive cortisol
levels associated with prolonged stress
such as those experienced by combat
veterans or trauma victims contribute to
central nervous system irritability and
hyperarousal. In this way, the combat experience produces similar effects to those
suffered by victims of sexual abuse or
captivity.
Judith Herman [7] described victimised
women who were known to Freud and
other psychoanalysts as experiencing an
‘elevated baseline of arousal’ in combination with ‘an extreme startle response to
unexpected stimuli, as well as an intense
reaction to specific stimuli associated with
the traumatic event’ (p. 36). She stated
further that trauma victims had difficulty
tuning out repetitive stimuli, even if
innocuous, as each presentation appeared
to be processed as ‘a new, dangerous
surprise’. It is worth noting here the relatively high prevalence of tinnitus among
this population, as tinnitus could be
considered a repetitive stimulus generating
a similar response. The physiologic consequences for such women mirrored those of
veterans who reported feeling ‘on patrol’
when they have misinterpreted environmental events to a degree that they could
not function in routine social situations.
Victims of sexual abuse or captivity were
long known to exhibit a tendency to
startle, withdraw, suffer nightmares, and
experience psychosomatic symptoms that
were similar to the effects seen in veterans
who had survived violent episodes, had
perpetrated violence on others, or who
had chanced upon horrific scenes of battle
recently ended (for example, those soldiers
who came upon the village of My Lai after
the massacre).
Hyperacusis may be difficult to quantify,
however when such patients are asked
directly to rate the annoyance and impact
on routine life of their sound tolerance
problems, the ratings typically exceed
those applied by patients to their hearing
loss and / or tinnitus. Although the lack of
a well-established hyperacusis assessment
clouds the interpretation of patient
comments, their reports consistently reveal
that they experience enduring discomfort
and anxiety. Repeated exposures do not
change their experience; as Herman [7]
described, it is as if the sensation is new
each time it is experienced. Adaptation to
such stimuli appears beyond the reach of
these veterans.
Mislabelling of stimuli is the result of
several factors: training, experience, traumatic memories, and wholesale changes to
the baseline activity in the CNS. The aberrant behaviours that result, such as avoidance, irritability, and violent reactions to
neutral events, have the potential to rend
relationships and upend aspects of daily life
that most people take for granted. It is
likely that some veterans and military
personnel learn to be hyperacusic over
time. Their strong physical and emotional
responses to sounds that would not
bother other people have had substantial
ecological value at one time and are reinforced in life-threatening situations. It is the
misfortune of such individuals that a
plethora of environmental sounds
resemble the sounds of war enough so that
they trigger extremely disturbing thoughts,
memories and actions. The scope of these
disruptions warrants the collaboration of
professionals devoted to ameliorating their
severity. As Shay [8] points out, it may be
that to serve one’s nation renders the
veteran unfit to be its citizen. It is our privilege to help individuals enjoy the benefits
that should accrue to their time in the military service.
References
1. Schnurr PP, Jankowski MK. Physical health and
posttraumatic stress disorder: Review and synthesis.
Sem Clin Neuropsychiatry 1999;4(4):295-304.
2. Fagelson MA. The association between tinnitus
and posttraumatic stress disorder. Am J Audiol
2007;16(2):107-17.
3. American Psychiatric Association. Diagnostic and
statistical manual of mental disorders (4th edition).
Washington, DC: American Psychiatric Association;
1994.
4. Cannon WB. The James-Lange Theory of Emotions:
A Critical Examination and an Alternative Theory.
Am J Psychol 1927;39(1/4):106-24.
5. Bremner JD. Brain Imaging Handbook. New York,
NY: WW Norton; 2005.
6. Bremner JD. Does stress damage the brain? Biol
Psychiatry 1999;45(7):797-805.
7. Herman JL. Trauma and Recovery. New York, NY:
Basic Books; 1997.
8. Shay J. Achilles in Vietnam: the undoing of character. New York, NY: Scribner; 1994.
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
81
Complaints about Low
Frequency Environmental Noise
Andy Moorhouse,
Professor of Engineering
Acoustics and Vibration,
University of Salford,
Newton Building,
Salford, M5 4WT, UK.
Tim Husband,
Principal Audiologist,
The Tinnitus Clinic,
Manchester, UK.
Correspondence
E: A.T.Moorhouse@
salford.ac.uk
E: [email protected]
Declaration of
Competing Interests
TH was awarded an
honorarium by the
British Society of
Audiology for the
presentation of the
preliminary results at
The European Tinnitus
Course 2010. TH acted
as a subcontractor to
DEFRA to deliver aspects
of this paper.
AM's participation was
supported by Defra, who
funded the study.
here are a number of individuals
who present both to Environmental
Health Officers (EHOs) and to audiology clinics complaining about a low
frequency noise which causes them
considerable distress. Sometimes the
perception of noise, usually in the home
and worse at night, is accompanied by
physical sensations such as vibrations
through the furniture. Sleep disturbance in
these cases is common and sufferers have
even moved house, slept in their car or in a
draughty hallway in an attempt to escape
the noise.
EHOs may follow a standard assessment
procedure [1] and in some cases will identify a noise source which can then potentially be subject to noise control. However,
in a proportion of cases (perhaps as high as
70%) no noise source can be identified that
could be responsible for the complainant’s
reaction and, in the absence of a clearly
identified source, no action by the EHO is
possible. Lacking reprieve, the complainant
may then continue to contact the EHO
simply through lack of alternatives,
resulting in a cycle of frustration which
consumes resources while providing no
real prospect of a resolution. A number of
such cases consequently end up being
referred to the Ombudsman.
In March of 2011 a study was completed
which set out to assess whether, irrespective of the (unknown) cause of the LFN
perception, the perception may be lessened through modification or application
of the conventional audiological treatment
for tinnitus [2]. A concurrent study also
investigated whether computer based
Cognitive Behavioural Therapy would be
helpful in these cases [3]. The essential
premise for this investigation is that the
mechanisms underlying these individuals’
perception appear to be highly analogous
T
Figure 1: The LFN network.
to those proposed for both tinnitus and
hyperacusis, that is, heightened vigilance,
increased psychological arousal and raised
central auditory gain.
The study
The study entitled UK-wide Support
Network for Low Frequency Noise
Sufferers was conducted across nine
centres located around England and
Scotland (Figure 1). Contact was made in
these areas with the local EHO offices. A
treatment protocol was developed for LFN
complainants whose main elements were:
● The exclusion of treatable disease by
clinical history, otoscopy, audiometry
and ENT opinion as local protocols dictated
● Discussion of the distress and agitation
evoked by the perceived LFN
● Environmental sound therapy to reduce
the starkness of the signal
● Relaxation therapy to reduce the arousal
and agitation associated with the signal
● Identification of those individuals with
clinically significant anxiety and / or
depression and referral to Psychological
Services (using the Hospital Anxiety and
Depression Scale) [4].
Whilst not large in number, those individuals
with LFN complaint have a significant
clinical need
82
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
feature
Table 1: Number of participants by centre.
Centre
A
B
C
D
E
F
G
H
I
Referrals
2
5
2
2
1
0
1
1
0
Table 2: Summary of audiometric data for participants.
Summary of audiometric data for
participants hearing loss
Number
% of sample
Nil
2.00
14.29%
Mild
7.00
50.00%
Moderate
1.00
7.14%
Severe
0.00
0.00%
Not available for this article
4.00
28.57%
d. EQ–5D questionnaire [7].
e. Visual analogue scales for: LFN loudness, pitch and distress.
In addition an innovative assessment for
these individuals was developed known
as the Kenyon Quiet Room Protocol,
designed to determine if the client
became aware of their LFN within a quiet
room which could indicate low
frequency tinnitus as a cause.
A total of 14 individuals took part in the
study, 11 of which were referred from
EHOs to the LFN network and three of
which were self-referred (Table 1).
The mean age of participants was 62
years (range 35-87 years, standard deviation 13.4 years). Eight (58%) of the participants were female and 6 (42%) male.
These gender and age distribution figures
are broadly consistent with previously
reported figures for LFN sufferers, for
example an average age of 55 with two
thirds female is reported by Leventhall
[8]. The average length of the complaint
prior to seeing the audiologist was 17
months (range 9 to 31 months, standard
deviation 8 months). The maximum
number of appointments was 5 with a
mean of 3 per case. Audiometric data is
summarised in Table 2.
Half of the complainants had a clear
idea about the origin of the perceived
LFN, the remainder being unsure
although all had thought about various
possibilities. The likely sources
mentioned were digital TV, factories or
works, neighbours using machinery, fish
tanks or hot tubs, water pipes or heavy
duty pumps, telecommunication masts
and refrigerators.
Evaluation of benefit
Figure 2: Hospital Anxiety and Depression Scale (HADS) scores: upper – anxiety, lower – depression.
The referral pathway was bi-directional
such that each suitable candidate was
given a letter to take to their GP requesting
referral on to ENT services and notification
was sent directly to the clinician at each
site involved in the program. Each host site
had previously sought and obtained the
support of an ENT Consultant who agreed
to medically assess each candidate.
Outcome measures used included:
a. Hospital Anxiety and Depression Scale
(HADS) questionnaire [4].
b. Tinnitus Handicap Inventory (THI)
questionnaire [5], but substituting LFN
experience for the concept of tinnitus.
c. Hyperacusis was measured using a
validated 14 item self report questionnaire [6].
Example before-after comparisons are
shown in Figure 2 for the HADS anxiety
and depression scales. The results are
mixed with some subjects indicating a
marked improvement (D2, G2) and
others showing little effect or even a
slight worsening.
A single-sided, paired t-test of the
seven indicators revealed that the means
of six out of seven had moved in a
favourable direction but that the
improvement was only significant at the
p<0.05 level for one of the indicators.
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
83
feature
Thus, more subjects would be required to
confirm the benefit.
Metrics of distress and handicap all indicated a clinical population that was
agitated and distressed by their situation,
and self report of length of complaint
evidenced a situation that was chronic and
long-standing. Whilst not large in number,
those individuals with LFN complaint have
a significant clinical need.
The present study indicates that audiology-based therapy provides a context in
which people with LFN complaint can be
assessed, treatable audiological conditions
can be excluded, and where some
(modest) improvement can be made in
some individuals. The factors likely to influence success are the quality of the referral
by the EHO, the quality of the audiology
input and the attitude of the complainant.
A model proposed in the study of stress
and increased auditory gain is a plausible
explanation for the symptoms noted in
LFN cases [2]. In particular, the involvement of the sympathetic autonomic
nervous system, and of the emotional
brain, is likely to be a faithful representation
of the clinical situation.
References
Whilst not large in
1. Moorhouse AT, Waddington DC, Adams MD. A
procedure for the assessment of low frequency
noise complaints. (NANR45). Technical Report.
Defra; London: 2011.
number, those
2. Moorhouse AT, Baguley DM, Husband T. (2012)
UK-wide Support Infrastructure for Low Frequency
Noise Sufferers ('LFN Network'). Technical Report.
Defra; London: 2012 (in press).
3. Leventhall G. Development of a course in computerised Cognitive Behavioural Therapy aimed at
relieving the problems of those suffering from noise
exposure, in particular, exposure to low frequency
noise (NANR 237). Interim Report. Defra; London:
2009.
individuals with
LFN complaint
have a significant
clinical need
4. Zigmond AS, Snaith RP. The hospital anxiety and
depression scale. Acta psychiatrica scandinavica
1983;67(6):361-70.
5. Newman CW, Jacobson GP, Spitzer JB.
Development of the tinnitus handicap inventory.
Archives of Otolaryngology - Head and Neck Surgery,
Am Med Assoc 1996;122(2):143.
6. Khalfa S, Dubal S, Veuillet E, Perez-Diaz F, Jouvent R,
Collet L. Psychometric normalization of a hyperacusis questionnaire. ORL 2000;64(6):436-42.
7. EuroQol – a new facility for the measurement of
health-related quality of life. The EuroQol Group;
http://www.euroqol.org/1990.
8. Leventhall G. A Review of Published Research on
Low Frequency Noise and its Effects. Technical
Report. Defra; London: 2003.
Helping the
world to hear
with portable, easy to use audiological equipment
Impedance
measurements
Otowave
Fast, accurate middle
ear measurements
Intuitive
Handheld
Ideal for primary
care use
Reduced test times
Clinical
audiometry
Amplivox 240 and 260
Amplivox 270
AC, BC
and speech
Range of clinical tests
E-mail: [email protected]
World class solutions for audiological and occupational health requirements
84
Amplivox 116
Diagnostic
audiometry
AC and BC audiometry (240)
AC, BC and speech
audiometry (260)
Totally portable
Tel: +44 (0)1865 842411
Screening
audiometry
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
www.amplivox.ltd.uk
The Treatment of Hyperacusis
with Cognitive Behaviour Therapy
Gerhard Andersson,
PhD,
Professor in Clinical
Psychology,
Linköping University and
Karolinska Institutet
Department of
Behavioural Sciences
and Learning,
Linköping University,
SE-581 83 Linköping,
Sweden.
Correspondence
E: Gerhard.Andersson@
liu.se
Declaration of
Competing Interests
None declared.
C
ognitive behaviour therapy (CBT) is the psychological treatment approach
that has received most empirical support for a range of conditions such as
depression, anxiety, chronic pain [1], but also for tinnitus [2]. However for
the problem of hyperacusis, often comorbid with tinnitus, there is much less evidence
for any treatment approach [3]. The aim of this article is to describe why psychological
treatment could be called for in the management of hyperacusis.
Hyperacusis and avoidance
There are several theories regarding hyperacusis [3], but a majority focus on the
neural underpinnings and for example a
possible link between serotonin (5-hydroxytryptamine) system malfunction and
hyperacusis [4]. The possibility that hyperacusis could be at least partly viewed as a
psychological problem has also been
suggested, which does not preclude the
role of auditory dysfunctions or central
factors relating to brain functioning (for
example, central auditory gain). Indeed,
hyperacusis and the term phonophobia
are often referring to similar problems even
if there are distinctions (phonophobia
being fear of specific sounds). From a
psychological and clinical point of view,
patients with severe hyperacusis often
present with a distinct pattern of avoidance behaviours, which can be sorted into
three different categories (Figure 1). The
first relates to avoidance of sound due to
actual pain in the ears and severe discomfort. This avoidance can be very specific
and associated with certain environments.
A form of avoidance relating to this is to
use ear protection to ‘avoid’ the risk of
feeling discomfort when being exposed to
unexpected sounds. I would argue that this
discomfort / pain-related avoidance is the
Since hyperacusis is characterised by marked
avoidance which the patient finds difficult
to confront, it is important not to push the
patient too hard without consent
86
main problem for many people with
hyperacusis. The second form of avoidance
is less driven by pain and discomfort, but
rather avoidance of sounds and environments that can lead to irritation and
annoyance. This is similar to the construct
of misophonia, which is a term used by
advocates of Tinnitus Retraining Therapy
[5]. The third form of avoidance, perhaps
being more common among persons with
tinnitus and hearing loss, is fear of sound
that might damage hearing and lead to
worse hyperacusis (and tinnitus). While
this avoidance can be motivated by environments that might damage hearing it is
often exaggerated (even to the extent that
patients have catastrophic beliefs about
what sound can do to their hearing). I need
to stress that beliefs regarding the consequences of being exposed to sounds is
always a part of the symptom profile of
adults with hyperacusis. With children and
persons with neurological dysfunctions, it
is less clear that they have any thoughts
and beliefs about sound.
Finally, at least in the clinic, many
persons with severe hyperacusis have
comorbid psychiatric problems such as
depression and anxiety disorders [6]. These
psychiatric problems often share the characteristic with hyperacusis of being closely
tied to behavioural avoidance and
maladaptive thoughts / beliefs. Having said
that, I do not regard hyperacusis as a
psychiatric disorder. It needs to be
managed as a problem on its own that can
occur in the context of other problems
that can aggravate the problem.
Cognitive behaviour therapy for
hyperacusis
This brief text does not allow a full description of how CBT for hyperacusis is
conducted. (For a brief overview, see Table
1.) However, our form of CBT always starts
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
feature
with a medical examination to rule out
any treatable condition and / or problems
that call for referral before we can start
treating hyperacusis (for example, neck
problems associated with whiplash
disorder, other pain conditions such as
headaches). Once this is done, a cognitive-behavioural analysis is performed
based on a patient interview. This is a
collaborative venture which might require
that the patient keeps a symptom diary
for a week. The goal is to reach a shared
case formulation which can inform the
subsequent CBT techniques.
Since hyperacusis is characterised by
marked avoidance which the patient finds
difficult to confront, it is important not to
push the patient too hard without
consent. In cases where there is a need to
boost motivation, I have found that motivational interviewing (MI) can be a
feasible approach [7]. MI can be viewed as
a way to support the patient in making
informed decisions. It acknowledges the
short-term benefits of avoidance and the
long-term benefits of gradually
confronting sounds and leaves it up to
the patient to decide which way to go.
An important part of CBT is education
which is part of the first session and
repeated for all subsequent treatment
steps (so called rationale). Basically, the
patient needs to understand why it is
important to cease avoiding sounds but
should also be met with empathy and
understanding.
In our CBT for hyperacusis we have
included three main techniques that are
presented in six sessions. The first is to
practise applied relaxation which can
relieve tension and be a tool to be used
in everyday life [8]. Briefly, this consists of
four steps beginning with tension-relax
and ending with rapid relaxation. As with
most forms of CBT, exercises are
prescribed as homework assignments.
The second technique is to construct an
exposure hierarchy for sound environments. This is in the form of gradual
exposure. Exposure should be gradual
and built on success. There is no point in
having the patient fail. Hence we include
exposure to background sounds that are
perceived as ‘safe’, such as music in the
home. The rationale is grounded in how
physical strength is built up after injury
(that is, gradual activity). However,
gradual exposure to avoided and feared
sounds is also included during the
treatment. Since CBT focusses on function it can sometimes be better to ‘move
in the right direction’ with the tempo-
Noise sensitivity / pain
Fear of injury or
becoming worse
Annoyance / irritation
Figure 1: Multidimensional view of hyperacusis.
Table 1. Key elements in CBT for hyperacusis.
Cognitive-behavioural analysis of avoidance behaviours and strengths
Rationale for the treatment
Applied relaxation in four steps
Graded exposure to sounds
Behavioural activation
Cognitive techniques
In addition techniques derived from motivational interviewing can be included
rary aid of hearing protection as long as
the patient knows and understands that
protecting the ears from everyday
sounds is a cause rather than a solution
to the problem. Indeed, we also include
what is called behavioural activation [9],
which is more than exposure to sound
and an evidence-based treatment for
depression. The third technique, which is
characteristic of CBT, is to include
throughout the process techniques
aimed at changing how the patient views
the problems. For example, catastrophic
beliefs are targeted and also what the
patient values and regards as important
(so called values work) that can serve as
a motive for activation and gradual
exposure.
Final comments
To date there are no randomised
controlled trials on CBT for hyperacusis.
We have however recently completed a
study with 58 patients who were
randomised to either treatment or
waiting for treatment (Jüris et al., in preparation). The preliminary results show a
clear effect of the treatment described
above. Given the lack of evidence-based
treatments for hyperacusis CBT should be
considered as a treatment option.
References
1. Butler AC, Chapman JE, Forman EM, Beck AT. The
empirical status of cognitive-behavioral therapy: A
review of meta-analyses. Clin Psychol Rev
2006;26:17-31.
2. Hesser H, Weise C, Zetterqvist Westin V,
Andersson G. A systematic review and metaanalysis of randomized controlled trials of cognitive-behavioral therapy for tinnitus distress. Clin
Psych Rev 2011;31:545-53.
3. Baguley DM, Andersson G. Hyperacusis:
Mechanisms, diagnosis, and therapies. San Diego:
Plural Publishing Inc; 2007.
4. Marriage J, Barnes NM. Is central hyperacusis a
symptom of 5-hydroxytryptamine (5-HT) dysfunction? J Laryngol Otol 1995;109:915-21.
5. Jastreboff PJ, Hazell J. Tinnitus retraining therapy:
Implementing the neurophysiological model.
Cambridge: Cambridge University Press; 2004.
6. Jüris L, Larsen H-C, Andersson G, Ekselius L.
Psychiatric comorbidity and personality factors in
patients with hyperacusis. Int J Audiol (in press).
7. Miller WR, Rollnick S. Motivational interviewing. 2
ed. New York: Guilford Press; 2002.
8. Andersson G, Kaldo V. Cognitive-behavioral therapy with applied relaxation. In: Tinnitus treatment
Clinical protocols. Edited by Tyler RS. New York:
Thieme; 2006: 96-115.
9. Martell CR, Dimidjian S, Herman-Dunn R.
Behavioral activation for depression. A clinician's
guide. New York: Guilford Press; 2010.
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
87
Using TRT to Treat Hyperacusis,
Misophonia and Phonophobia
1
Pawel J Jastreboff,
PhD, ScD, MBA,
Professor,
Department of
Otolaryngology,
Emory University School
of Medicine,
Atlanta, GA, USA.
2
Margaret M
Jastreboff, PhD,
President,
Jastreboff Hearing
Disorders Foundation,
Inc., Ellicott City,
MD, USA.
Correspondence
1
E: [email protected]
2
E: [email protected]
Declaration of
Competing Interests
None declared.
88
Decreased sound tolerance
While acknowledging the presence in the
literature of a variety of definitions, we
promote the use of the term ‘decreased
sound tolerance’ (DST) defined as being
present when a subject exhibits negative
reactions when exposed to sound that
would not evoke the same response in an
average listener [1]. Reported reactions
include a variety of negative emotional
responses, discomfort, dislike, distress,
annoyance, anxiety, pain and fear. DST can
present as the patient's sole complaint,
but is also reported in many medical
conditions including head injury,
migraine, Lyme disease, Williams
Syndrome, autism, Bell's palsy, benzodiazepine withdrawal and post stapedectomy. DST is particularly frequent in
patients with tinnitus.
DST patients are composed of two
distinct groups. In the first group, patients'
reactions depend primarily on the physical characterisation of a sound; these
patients have pure hyperacusis. In the
second group negative reactions to sound
occur to specific patterns of sound (for
example, a neighbour's music; chewing
sounds; sound of swallowing encountered
at home or at school; voices of specific
people; a clicking sound such as a copy
machine; running water; crackling sounds
such as paper or a fireplace; high-flying
airplanes; and so on). At the same time
patients in this group can tolerate a high
level of other sounds such as loud music
or the noise on a busy street. Moreover,
their reactions are frequently (but not
always) context-specific. Thus, the eating
sounds made at the dinner table at home
or in a school cafeteria evoke a negative
reaction but the same sounds at a friend's
house evoke no such reaction. To describe
these patients, we coined the term ‘misophonia’ [1,2]. Many patients with misophonia react to bothersome sounds with
discomfort, dislike, annoyance or pain. A
small subset of patients in this group
express a fearful reaction to sound and are
diagnosed with phonophobia. DST results
from the combined effects of hyperacusis
and misophonia.
Definitions and mechanisms for
hyperacusis and misophonia
In hyperacusis (present in about 30% of
tinnitus patients), negative reactions to a
sound depend only on its physical characteristics (that is, its spectrum and intensity). The sound's meaning and the
context in which it occurs are irrelevant.
Misophonia (present in about 60% of
our tinnitus patients), is characterised by
negative reaction to a sound with a
specific pattern and meaning to a given
patient. The physical characteristics of a
sound are secondary. Reactions to the
sound depend on the patients' past
history and on non-auditory factors like
the patient's previous evaluation of the
sound, her / his psychological profile, and
the context in which the sound is
presented. Patients with misophonia have
an increased awareness of external sounds
and of somatosounds (for example, one's
own eating).
Notably, patients with hyperacusis or
misophonia report the same negative
reactions. This leads us to conclude that
all cases of bothersome DST involve the
emotional (that is, limbic) system and
other systems in the brain in addition to
the auditory system as well as connections
between the auditory and other systems
[3,4]. Because a sound evokes negative
reactions in DST patients, some enhancement of activity in the brain's non-auditory systems must occur. This enhancement results from modifications in the
processing of sound-evoked neuronal
activity within these systems. Taking this
information into account we propose the
following mechanisms for hyperacusis and
misophonia. It is possible to envision three
scenarios.
In the first scenario, the increased
amplification of sound-evoked activity
occurs within the auditory pathways and
all other systems and their connections
work within the norm. The hypothesis of
increased gain within the auditory system
has received experimental support in
humans [5]. Furthermore, laboratory
results indicate an increase in gain occurs
at the subconscious level of the auditory
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
feature
pathways [6]. Since the other systems in
the brain have only a limited capacity to
modulate gain within the auditory pathways, the sound's meaning, past history,
and the environment where the patient is
exposed to the sound have little impact
on the patient's reaction to it. This
scenario corresponds to pure hyperacusis.
In the second scenario, functional
connections between the auditory
system and other systems in the brain are
enhanced while the auditory system functions normally. As higher levels of the
brain are involved, the functional properties of these connections depend on the
sound's meaning and past association,
involve learning and occur to specific
sound patterns. It can be argued that
these conceptions are governed by principles of conditioned reflexes [3,7]. At a
behavioural level, this scenario corresponds to pure misophonia.
A third scenario combines the first
and second scenarios, wherein an
enhancement of sound-evoked neural
activity within the auditory pathways
and the enhancement of connections
occur between the auditory system and
other systems in the brain. (Both hyperacusis and misophonia are present).
Actually, misophonia is automatically
created in patients with significant
hyperacusis, as some sounds evoke
discomfort and therefore create a negative association of these sounds with
suffering. In turn, this situation will create
conditioned reflexes which link the auditory system with other systems in the
brain, thus yielding misophonia.
Furthermore, the properties of the
emotional and other non-auditory
systems in the brain as reflected in the
patient's personality profile and, in some
cases, accompanying psychological
disorders (for example, Obsessive
Compulsive Disorder), affect the extent
of negative reactions the patient has to a
given sound and the patient's susceptibility to develop DST.
Differential diagnosis
Because hyperacusis and misophonia
evoke the same emotional and autonomic reactions, it is impossible to
discriminate between them on the basis
of observed reactions. Patients with DST
may have normal hearing or hearing loss
and may complain about tinnitus. The
audiological evaluation of DST usually
involves making a determination of sound
levels that evoke discomfort (Loudness
Discomfort Levels – LDLs) using voice and
/ or pure tones. We use label LDLs to indicate measurements performed with pure
tone stimuli.
During this test, the patient is asked to
report when he / she experiences strong
discomfort while exposed to gradually
increasing sound levels. An individual
without DST has an average LDL for all
tested frequencies of about 100dB HL [8].
However, LDLs alone are insufficient to
diagnose hyperacusis or misophonia.
When a patient has hyperacusis, his / her
LDLs show lower values - typically in 6085dB HL range, but low values alone are
not sufficient for proving the presence of
hyperacusis as these values may be due
to misophonia. Indeed, in misophonia
both low and normal LDLs are possible
within a range of 20 to 120dB HL.
Therefore, in addition to a properly
administered LDL test, a specific, detailed
interview is crucial for diagnosis. In the
interview, it is important for clinicians to
identify both sounds which evoke negative reactions as well as sounds which are
well-tolerated by the patient in order to
detect any discrepancies between reactions and the intensity of the sound.
Moreover, medical evaluation is important as DST can be a symptom of many
clinical conditions and some treatable
diseases as noted above.
Use of TRT to treat DST
Tinnitus Retraining Therapy is a method
aimed at the habituation of negative reactions evoked by an internal signal (that is,
tinnitus-related neuronal activity) or
external sounds which evoke negative
reactions in the patient when DST is
present. TRT is based on the neurophysiological model of tinnitus and consists of
counselling and sound therapy, both
based on the model and tailored to
problem(s) affecting a given patient.
Counselling for patients with DST
provides information regarding potential
mechanisms which cause the problem, as
well as the mechanisms used in treatment (for example, brain plasticity, mechanisms of habituation, extinction of
conditioned reflexes, the role of the
conscious and subconscious systems in
the brain, the role of the limbic and autonomic nervous systems, and other relevant systems in the brain). Counselling
also addresses patients' concerns and
discusses goals and expectations. Sound
therapy in TRT always follows the rule
‘never use a sound that is annoying or
bothersome or creates problems because
of any reason’.
Basis of approach for hyperacusis
and misophonia
Because the presumed mechanisms for
hyperacusis and misophonia are substantially different, the sound therapies used
in TRT to treat these phenomena are
distinctively different as well. Notably, the
protocols for sound therapy that are
effective for hyperacusis are not helpful in
treating misophonia; conversely, the
protocols that are effective for misophonia are not helpful for treating hyperacusis.
The proposed mechanism of hyperacusis involves an increased gain within the
auditory pathways and therefore a desensitisation protocol with a variety of
sounds is used for treatment. Background
sound enrichment is recommended using
table-top sound machines, CD, radio and
TV. For patients with normal hearing, earlevel sound generators are also recommended to assure the patient is exposed
to a stable, well-controlled, consistent
sound level that is under the patient's full
control. The patient adjusts the volume
to a non-annoying level, which he / she
may temporarily increase upon entering a
louder environment. An evaluation of the
sound level inside of the ear canal
performed by the Real Ear Measurements
system revealed the average sound level
used by our patients was about 10dB SL.
Consequently, the use of sound generators does not interfere with speech understanding.
Sound generators are not recommended for patients with hearing loss as
the sound emitted by the generators typically includes frequencies in the speech
range and may hinder speech understanding. To overcome this problem we
recommend using devices called combination instruments, so-called because
they combine hearing aid and a sound
generator in one shell. Amplification
provided by the hearing aid component,
programmed by an audiologist, counteracts the impact of introducing low level
sound. The patient sets the sound level in
the same manner as when using separate
sound generators.
TRT is very effective (~80% success
rate) for treating hyperacusis [9,10].
Typically, improvement is seen within
months and in many cases it is possible to
resolve the hyperacusis.
The treatment of misophonia is much
more complex and takes a similar time as
the treatment of tinnitus (for example,
nine to 18 months). As noted above, the
potential mechanisms for misophonia
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
89
feature
involve enhanced functional connections
between the auditory system and other
systems in the brain, particularly the limbic
and autonomic nervous systems. We have
proposed that these connections are
governed by the principles of conditioned
reflexes, with the subconscious part of the
brain playing a significant role.
Consequently, misophonia treatment is
aimed at attenuating these functional
connections by using the principle of the
active extinction of conditioned reflexes,
where sound is paired with something that
evokes a positive reaction. Therefore, the
essential feature of the misophonia protocols involves an attempt to create an association between a variety of sounds with a
positive emotional status, such as listening
to one's favourite music [1,3].
The specific protocol used by a given
patient to treat misophonia differs with
respect to the extent of the patient’s
control (for example, listening to music at
home, watching a movie in a theatre,
watching a movie at home with the sound
level set by a spouse), type of sound (for
example, music, books on tape, TV show,
movies), the sound level used, and the
environment where the patient is exposed
(for example, home, movie theatre,
school). In one protocol, the offensive
sound (for example, the sound of eating
produced by other people) is mixed with a
sound which evokes a positive reaction (for
example, music) which is initially set at a
level that partially masks the offensive
sound. Over time the volume level of the
positive sound is gradually decreased.
Protocols are always modified to fit the
individual patient's needs. Typically, several
protocols are used. While misophonic
patients frequently benefit from using earlevel sound generators or combination
instruments, these devices are unnecessary
for the successful outcome of the treatment. Sound generators alone without
specific protocols for misophonia are inef-
fective. However, when patients are treated
properly the success rate is high and a cure
is achieved in many cases [1].
In clinical practice hyperacusis and misophonia typically occur together and must
be treated concurrently. In cases of
moderate or severe hyperacusis, misophonia is inevitable and is created automatically, as negative reactions initially
evoked by sound due to hyperacusis
provide negative reinforcement and create
a conditioned reflex arc.
to a majority of patients. In many cases it is
possible to achieve cure for both hyperacusis and misophonia. Specific evaluation is
necessary, and different protocols for
hyperacusis and misophonia, applied
concurrently, are essential for a positive
treatment outcome.
References
Psychological factors in
misophonia
Many misophonic patients exhibit some
psychological problems, and may also try
to control their environment using misophonia as a tool (for example, control of
parents). Misophonia frequently emerges
in teenagers or children and may persevere
for many years. Collaboration with a
psychologist may be advisable in some of
these cases.
Phonophobia in DST and tinnitus
patients
A subset of tinnitus patients develop misophonia due to a phobic reaction to
sounds, particularly when patients believe
specific sounds enhance their tinnitus.
These patients do not have classical
phonophobic reactions to sound in
general, but are afraid that exposure to
certain sounds will make their tinnitus or
hearing worse. In such cases, appropriate
counselling in combination with treatment
for hyperacusis and misophonia is sufficient. However, when a more generalised
phonophobic reaction to sound is present,
the patient needs to be treated by a
psychologist.
Conclusion
Properly applied Tinnitus Retraining
Therapy is an effective method to treat
DST that provides significant improvement
coming up in the March April 2013 issue
1. Jastreboff MM, Jastreboff PJ. Decreased sound tolerance and Tinnitus Retraining Therapy (TRT).
Aust N Z J Audiol 2002;(2):74-81.
2. Jastreboff PJ, Jastreboff MM. Decreased sound tolerance. In: Snow JB, Jr, editor. Tinnitus: Theory and
Management. BC Decker, Hamilton; London:
2004:8-15.
3. Jastreboff PJ, Hazell JWP. Tinnitus Retraining
Therapy: Implementing the Neurophysiological
Model. Cambridge University Press; Cambridge;
2004:276.
4. Kumar S, von Kriegstein K, Friston K, Griffiths TD.
Features versus Feelings: Dissociable
Representations of the Acoustic Features and
Valence of Aversive Sounds. The Journal of
Neuroscience 2012;32(41):14184 –92.
5. Formby C, Sherlock LP, Gold SL. Adaptive plasticity
of loudness induced by chronic attenuation and
enhancement of the acoustic background. J Acoust
Soc Am 2003;114(1):55-8.
6. Salvi RJ, Wang J, Powers N. Rapid functional reorganization in the inferior colliculus and cochlear
nucleus after acute cochlear damage. In: Salvi RJ,
Henderson D, Fiorino F, Colletti V, editors. Auditory
system plasticity and regeneration. Thieme Medical
Publishers ; New York: 1996: 275–96.
7. Wilson PH. Classical conditioning as the basis for
the effective treatment of tinnitus-related distress.
ORL J Otorhinolaryngol Relat 2006;68(1):6-11.
8. Sherlock LP, Formby C. Estimates of loudness, loudness discomfort, and the auditory dynamic range:
normative estimates, comparison of procedures,
and test-retest reliability. J Am Acad Audiol
2005;16(2):85-100.
9. Baguley DM, Andersson G. Hyperacusis:
Mechanisms, Diagnosis, and Therapies. Plural
Publishing Inc.; Plymouth: 2007:144.
10. Formby C, Hawley M, Sherlock L, Gold S, Segar A,
Gmitter C, Cannavo J. Intervention for restricted
dynamic range and reduced sound tolerance. J
Acoust Soc Am 2008;123:3717.
AUDIOLOGY MATTERS
Humanitarian Audiology guest edited by Jackie L Clark
Articles Overview of Humanitarian Audiology
Cultural awareness (Browne & Clark)
TeleAudiology Network
Pathologies encountered on Humanitarian Mission
Hearing Aid Candidacy and Strategies in Developing Countries
Hearing Aid Provision in Developing Countries
90
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
An Interview with
James W Hall III
Prof James W Hall
III, PhD,
Clinical Professor,
Department of Speech,
Language, and Hearing
Sciences,
P.O. Box 3432,
St Augustine,
FL 32085, USA.
Correspondence
E: [email protected]
P
rof James W Hall III, received his PhD in audiology from Baylor College of
Medicine in 1979, under the direction of Dr James Jerger. Thirty years on, he
holds adjunct appointments as Professor of Audiology at Nova Southeastern
University in Fort Lauderdale, Florida, and Extraordinary Professor in the Department of
Communication Pathology at the University of Pretoria in South Africa.
Declaration of
Competing Interests
None declared.
Prof Hall’s major clinical and research
interests are clinical electrophysiology,
auditory processing disorders, tinnitus /
hyperacusis, and audiology applications of
tele-health. A prolific author, Prof Hall has
written seven textbooks and over 125
journal articles and book chapters, and
despite his busy schedule, took time out
to speak with our Audiology Editor, Dr
David Baguley.
preparing for my ABR presentation at the
conference. Suddenly I realised there was a
nun sitting next to me. In the ensuing
conversation I rather evangelically
described the importance of newborn
screening and early identification for
hearing loss. When I finally finished my
spontaneous sermon the nun simply
stated, “God led you to the profession of
audiology.” I know she is correct.
How do you find the time to write
so many books?
Our mutual friend Dr Roger Ruth
passed away two years ago. What
do you think will be his enduring
legacy?
I have a bad habit of working almost
constantly. Perhaps I should rephrase that
response. I have a good habit of looking
upon my writing as a hobby. For most of
my career I’ve taken more satisfaction
from writing than any other professional
activity. To paraphrase the US Naval hero
John Paul Jones, “I have not yet begun to
write”. I trust it’s not too impolite to cite a
person who fought the British. Did you
know he was a Scot by birth?
You are known as a very
thoughtful person, and I know
that your spiritual life is very
important to you. How has that
influenced your audiology work?
If this were a face-to-face oral interview,
my answer to this question would go on
for at least 15 minutes. When I was 14
years old I read the Albert Schweitzer
autobiography ‘Out of My Life and
Thought’. From that time onward I had a
vague notion that someday I wanted to
do good through my profession. One day
in 1993 I had an interesting experience
while waiting in an airport for a flight to
the NIH Consensus Conference on Early
Identification of Hearing Loss in Infants. I
was concentrating on my slides while
92
A week never passes without some
reminder of Roger. The remembrances
may be related to audiology or to one of
the sports teams Roger liked. Most of
them didn’t win very often but Roger was
a loyal fan. Without question Roger’s
enduring legacy is his former students.
Roger was a kind, dedicated and rigorous
teacher at the University of Virginia and
later at nearby James Madison University. I
regularly encounter his former students
who invariably comment about how
much they learned from Roger in the
classroom and also in the clinic. Roger also
made a lasting contribution to the profession of audiology as a Founder of the
American Academy of Audiology.
You are often to be found
teaching audiology abroad, and in
some far-flung places. I would be
interested to hear your reflections
upon those experiences.
I love to travel. You and I are blessed to
have audiology friends around the world
who share our love of and commitment
to the profession and the patients we
serve. Travel certainly involves frustrations
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
feature
and fatigue. However, I can honestly
confirm that each and every one of my
professional journeys has been incredibly
rewarding and enriching. In the next 20 or
30 years of my career, I hope to focus even
more on international audiology. I now
have a motto on my business cards and
website (www.audiologyworld.net) that
reads: “The world is my clinic and classroom.” I’ll readily acknowledge that John
Wesley inspired the line.
Your knowledge and clinical
expertise on auditory objective
testing is huge. What test do we
not yet have that you yearn for?
That’s another good question that should
be answered in a 50 minute PowerPoint
presentation. I really would like to see a
portable combination device for
recording three existing objective procedures: acoustic immittance measures like
tympanometry (with 220 and 1,000Hz
probe tones) and acoustic reflexes, plus
OAEs and ABR. The technological
capacity exists for such a wonderful
device. With it and video-otoscopy I
could travel the world identifying hearing
loss in young children and everyone else.
The device would necessarily be on a
computer platform so with broadband
Internet connection I could even transmit
all of the data to you to verify my analyses
and interpretation!
We have talked a lot about
tinnitus and hyperacusis over the
years. What is your assessment of
the current state of knowledge
and clinical practice for these
conditions?
We have talked frequently about tinnitus
and hyperacusis, often over a session beer
or two. Almost 20 years ago you actually
provided the inspiration and challenge for
me to develop tinnitus and hyperacusis
clinics at two different university medical
centres.
I’m quite impressed with the relatively
rapid transition from totally inadequate
services for patients with tinnitus to
rather widespread high quality services, at
least on the western side of the Atlantic
Ocean. Actually, in my travels I’ve
observed a worldwide expansion of
tinnitus services. At the risk of insulting
some readers, I must say that audiologists
are generally more aware than physicians
(at least in the USA) of the seriousness of
tinnitus as a health problem and of the
availability of effective management
options.
Prof Hall and colleague Dr De Wet Swanepoel (University of Pretoria, South Africa) performing hearing
screenings simultaneously on triplets.
Tell us about your background
I grew up in a small town in northeastern
Connecticut. All but a few of my ancestors emigrated from England to what is
now New England in 1620 and the early
1630s (five ancestors arrived on the
Mayflower). I had a typical 1950s childhood in the USA. I even once was the
proud owner of a Davy Crockett racoonskin hat. Don’t worry… it was a fake.
My favourite childhood and adolescent
activities included reading, music, sailing,
running and working in a tiny regional
hospital. In college I majored in biology
and was part of a resurrection of the
rowing team. My dad and grandfather
(James W Hall, Senior and Junior) were
optometrists and I was supposed to
follow in their footsteps.
Who has inspired you in your
career and why? Who have been
your heroes and mentors?
My first mentor was my dad. As a young
child I spent time at his optometry office
in my small hometown. He was very
confident and competent, with a good
‘bedside manner’. I subconsciously learned
from my dad about patient care and the
importance of sensory health care on
quality of life. The learning experience
must have been subconscious because I
didn’t appreciate the powerful impact of
those early experiences until I was 25 years
into my audiology career.
I graduated from college at the age of
21 totally unaware of the profession of
audiology. I applied to several universities
to pursue a master’s degree in speech
pathology. Why you might ask? My soonto-be wife expressed an interest in the
field and the pathology part of the phrase
reminded me of exciting days working
with the pathologist at our local hospital.
Fortunately, Northwestern University
offered me a full scholarship with tuition.
My new wife and I set off for the Midwest
part of the USA to what was even then a
leading programme in audiology.
To make a long story a little bit shorter,
I was introduced at Northwestern to
some of the biggest names in audiology…
Raymond Carhart, Earl Harford, Tom
Tillman and Noel Matkin. I soon began
the conversion from speech pathology to
audiology. My first gainful employment
following graduation was at Baylor
College of Medicine and Methodist
Hospital in Houston, Texas. There in the
early 1970s I came under the influence of
my most important mentor… James
Jerger. The remarkable educational, clinical
and research experiences I enjoyed during
those years permanently shaped my
career.
What has been the best piece of
advice that you have received in
your career? What advice would
you offer to those following in
your footsteps?
I can’t recall specific advice received in a
lecture or in a meeting with a teacher or
colleague that I’ve taken to heart and
followed closely in my career. I tended to
follow what people practised rather than
what they preached. For example, in the
1970s Jim Jerger published an article every
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6
93
feature
I’m quite impressed with the relatively rapid transition from totally
inadequate services for patients with tinnitus to rather widespread high
quality services, at least on the western side of the Atlantic Ocean
month or two. He once said: “You are
what you publish.” I’ve tried to follow his
example and advice ever since. Also, Jim
had a map of the world on a wall in the
clinic in Houston. It was filled with
colourful little pins identifying all of the
countries and cities where he had given
presentations. I love maps anyway so I was
naturally attracted to the display. The
subliminal message was successful audiologists spread the audiology gospel
throughout the world.
My advice to audiologists of all ages is
simple: work and study hard, care for your
patients and take advantage of every
opportunity to encourage and inspire
young students or junior audiologists. The
latter suggestion is not easy to implement
when you’re busy, facing deadlines and
generally under duress. But sometimes
years later you’ll discover when you meet
an audiology student in a graduate
programme or an audiologist in a chance
encounter at a convention that the
minute or two you spent years earlier with
that person actually planted the seed that
led to their career in audiology.
What keeps you motivated? What
drives you?
I guess I’m internally motivated and driven.
Maybe it’s a product of my essentially
introverted personality. I wake up most
days knowing what I want to accomplish.
Give me some time to work alone and I’ll
accomplish the goal. Nowadays opportunities for new and exciting professional
projects seem to arise on a regular basis. I
can honestly say that I don’t have a grand
scheme or game plan. One good professional experience seems to lead to another.
What’s your earliest memory of a
patient? Why has it stayed with
you?
My earliest memory of a patient is the
least memorable. I was a master’s student
in speech pathology at Northwestern
University just entering a therapy room to
94
evaluate a five year old boy with a severe
articulation problem. The room had what
looked like a mirror with my supervisor
and other students in a little room on the
other side. Within the past few weeks I’d
grown a beard because adult patients in
the clinic thought I appeared too young to
be a graduate student. My patient entered
the room, took one look at me, and ran
out of the room screaming hysterically. So
much for the cardinal rule of speech
pathology… ‘establish rapport with your
patient.’ I later discovered that the child’s
parents were recently divorced. The child’s
bearded father had been charged with
child abuse.
Since then I’ve completely enjoyed the
process of getting to know a patient, diagnosing their hearing loss or related
problem, and then developing an effective
management plan. I’ve come to appreciate
deeply the enormous effect we audiologists can have on the lives of people,
young and old.
If you could make every
audiologist read one book what
would it be and why?
Why only one book? Why not begin with
the Handbook of Auditory Evoked
Responses and continue on to
Otoacoustic
Emissions:
Principles,
Protocols, and Procedures? Seriously, I’m
reluctant to recommend a single book. I
tend to read biographies (mostly on
airplanes) but I would recommend a
recent non-textbook that’s relevant to
audiology entitled ‘The Idea Factory: Bell
Labs and the Great Age of American
Innovation’ by Jon Gertner.
What are you working on at the
moment?
In recent years I’ve been working almost
non-stop on an undergraduate textbook
called Introduction to Audiology Today. It’s
by far the most difficult writing task I’ve ever
undertaken. I find it challenging to write for
students who have no background in audiology or hearing science. The book will be
published in time for the 2013 American
Academy of Audiology Convention in
Anaheim, California. I truly hope it encourages many bright and motivated young
students to select audiology as a career.
What are the challenges ahead?
What do you predict is coming
next on the horizon?
There are many naysayers and cynics
among us, but I’ve always viewed my cup
as half full if not overflowing. Every profession faces challenges. Creatively and effectively addressing the challenges will
produce a stronger and more vibrant
profession of audiology.
In the USA the Doctor of Audiology
movement has resulted in a new generation of audiologists who are generally well
prepared, motivated, and committed to
their new profession. Unfortunately,
worldwide 90% of the people with hearing
impairment have no access to a proper
hearing care. We need more high quality
educational programs for audiology in
developing countries.
Finally, are there any other topics
that you would like to address?
Please check back with me in 2032 for an
update!
Interviewed by
Dr David Baguley, PhD, MBA,
Consultant Clinical Scientist (Audiology),
Cambridge University Hospitals NHS Foundation Trust,
Hills Road,
Cambridge, CB2 2QQ, UK.
Email: [email protected]
ENT & audiology news | JANUARY/FEBRUARY 2013 | VOL 21 NO 6