Hyperacusis

Hyperacusis (or hyperacousis) is a debilitating hearing disorder[1] characterized by

Hyperacusis
an increased sensitivity to certain frequencies and volume ranges of sound (a
collapsed tolerance to usual environmental sound). A person with severe hyperacusis Specialty Otolaryngology
has difficulty tolerating everyday sounds, some of which may seem unpleasantly or
painfully loud to that person but not to others.[2][3]

Hyperacusis is often coincident with tinnitus. Both conditions have a prevalence of about 10–15% and hearing loss as a major risk
[1]
factor. However, there also appear to be important differences between the mechanisms involved in tinnitus and hyperacusis.

Contents
Signs and symptoms
Causes
Associated conditions
Neurophysiological mechanisms
Diagnosis
Treatment
Notable cases
See also
References
Further reading
External links

Signs and symptoms

In hyperacusis, the symptoms are ear pain, annoyance, and general intolerance to many sounds that most people are unaffected by.
fect either or both ears.[4] Hyperacusis can also
Crying spells or panic attacks may result from the experience of hyperacusis. It may af
be accompanied by tinnitus. Hyperacusis can result in anxiety, stress and phonophobia. Avoidant behaviour is often a response to
prevent the effects of hyperacusis and this can include avoiding social situations.

Causes
decibel (sound pressure) levels.[2]
The most common cause of hyperacusis is overexposure to excessively high

Some come down with hyperacusis suddenly as a result of taking ear sensitizing drugs, Lyme disease, Ménière's disease, head injury,
or surgery. Others are born with sound sensitivity, develop superior canal dehiscence syndrome, have had a history of ear infections,
or come from a family that has had hearing problems.

Some psychoactive drugs such as LSD, methaqualone, or phencyclidine (angel-dust) can cause hyperacusis.[5] An antibiotic,
ciprofloxacin has also been seen to be a cause, known asciprofloxacin-related hyperacusis.[6]

Associated conditions
 [7] include:
Some conditions that are associated with hyperacusis

Acoustic shock
Adverse drug reaction
Anxiety
Autism spectrum
Endolymphatic hydrops[8]
Lyme disease[7]
Migraine[7]
Ménière's disease
Multiple sclerosis[9]
Noise-induced hearing loss
Posttraumatic stress disorder[7]
Severe head trauma[7][10]
Superior canal dehiscence syndrome (SCDS)
Systemic lupus erythematosus (SLE)[11]
Tay–Sachs disease[12]
Williams syndrome[7][13]

Neurophysiological mechanisms
As one important mechanism, adaptation processes in the auditory brain that influence the dynamic range of neural responses are
[14]
assumed to be distorted by irregular input from the inner ear caused - mainly - by hearing loss related damages in the inner .ear

Diagnosis
The basic diagnostic test is similar to a normal audiogram. The difference is that
additionally to the hearing threshold at each test frequency also the lowest
uncomfortable sound level is measured. This level is called loudness discomfort
level (LDL) or uncomfortable loudness level(ULL). In patients with hyperacusis this
level is considerably lower than in normal subjects, and usually across most parts of
the auditory spectrum.[2][15]

Treatment
One possible treatment for hyperacusis is retraining therapy which uses broadband
Loudness discomfort levels
noise. Tinnitus retraining therapy, a treatment originally used to treat tinnitus, uses
(LDLs): group data of hyperacusis
broadband noise to treat hyperacusis. Pink noise can also be used to treat
patients without hearing loss. Upper
hyperacusis. By listening to broadband noise at soft levels for a disciplined period of line: average hearing thresholds.
time each day, patients can rebuild (i.e., re-establish) their tolerances to sound. Lower long line: LDLs of this group.
Although patients might not always make a complete recovery, the use of broadband Lower short line: LDLs of a reference
noise usually gives some of them a significant improvement in their symptoms, group with normal hearing.[15]
especially if this is combined with counseling.[16][17][3][18]

.[7] [19]
Another possible treatment iscognitive behavioral therapy(CBT), which may also be combined with retraining therapy

Notable cases
 Musician Jason DiEmilio ofAzusa Plane had hyperacusis. His story was told in BuzzFeed. [20]

Musician Stephin Merritt has monaural hyperacusis in his left ear, which influences the instrumentation of his band,
The Magnetic Fields, leads him to wear earplugs during performances and to cover his af fected ear during audience
applause.
Musician Laura Ballance of Superchunk has hyperacusis and no longer tours with the band.
American politician, activist, and film producerMichael Huffington has mild hyperacusis and underwent sound
therapy after finding that running tap water caused ear pain. [21]

Russian communist revolutionary, politician, and political theorist Vladimir Lenin was reported seriously ill by the
latter half of 1921, having hyperacusis and symptoms such as regular headache and insomnia. [22]

Musician Chris Singleton had hyperacusis, but made a full recovery.[23] His story was told in The Independent.[24]
Musician Peter Silberman of The Antlers had hyperacusis and tinnitus which put his musical career on hold, but was
quoted saying it died down to a 'manageable level'[25] He has now resumed his musical career .

See also
Misophonia
Tinnitus masker

References
1. Knipper M, Van Dijk P, Nunes I, Rüttiger L, Zimmermann U (December 2013)."Advances in the neurobiology of
hearing disorders: recent developments regarding the basis of tinnitus and hyperacusis"
(https://ac.els-cdn.com/S03
01008213000804/1-s2.0-S0301008213000804-main.pdf?_tid=ebe8c20a-acdc-11e7-b605-00000aab0f01&acdnat=1
507545232_c5f3bb0b5290a9f0529da948f5eae845)(PDF). Progress in Neurobiology. 111: 17–33.
doi:10.1016/j.pneurobio.2013.08.002(https://doi.org/10.1016%2Fj.pneurobio.2013.08.002) . PMID 24012803 (https://
www.ncbi.nlm.nih.gov/pubmed/24012803).
2. Tyler RS, Pienkowski M, Roncancio ER, JunHJ, Brozoski T, Dauman N, Dauman N, Andersson G, Keiner AJ,
Cacace AT, Martin N, Moore BC (December 2014)."A review of hyperacusis and future directions: part I. Definitions
and manifestations" (http://www.hyperacusis.net/media/1375/hyperacusis-part-1.pdf) (PDF). American Journal of
Audiology. 23 (4): 402–19. doi:10.1044/2014_AJA-14-0010(https://doi.org/10.1044%2F2014_AJA-14-0010) .
PMID 25104073 (https://www.ncbi.nlm.nih.gov/pubmed/25104073).
3. Pienkowski M, Tyler RS, Roncancio ER, JunHJ, Brozoski T, Dauman N, Coelho CB, Andersson G, Keiner AJ,
Cacace AT, Martin N, Moore BC (December 2014)."A review of hyperacusis and future directions: part II.
Measurement, mechanisms, and treatment"(https://pdfs.semanticscholar.org/a5ed/91aa23c1d830720013b3d2b8e4
1958345f01.pdf) (PDF). American Journal of Audiology. 23 (4): 420–36. doi:10.1044/2014_AJA-13-0037(https://doi.
org/10.1044%2F2014_AJA-13-0037). PMID 25478787 (https://www.ncbi.nlm.nih.gov/pubmed/25478787).
4. "Hyperacusis: An Increased Sensitivity to Everyday Sounds"(http://www.entnet.org/content/hyperacusis-increased-s
ensitivity-everyday-sounds). American Academy of Otolaryngology-Head and Neck Surgery . 21 April 2014.
5. Barceloux D (2012). Medical Toxicology of Drug Abuse : Synthesized Chemicals and Psychoactive Plants. Hoboken,
N.J: John Wiley & Sons. pp. 457, 507, and 616.ISBN 978-1-118-10605-1.
6. "Ciprofloxacin Related Hyperacusis, From FDA reports"(https://www.druginformer.com/search/side_effect_details/Ci
profloxacin/hyperacusis.html). 2017.
7. Baguley DM (December 2003)."Hyperacusis" (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC539655). Journal of
the Royal Society of Medicine. 96 (12): 582–5. doi:10.1177/014107680309601203(https://doi.org/10.1177%2F0141
07680309601203). PMC 539655 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC539655) . PMID 14645606 (http
s://www.ncbi.nlm.nih.gov/pubmed/14645606).
8. Møller A (2011). Textbook of tinnitus. Totowa, N.J. London: Humana Springer distributor. p. 457. ISBN 978-1-60761-
145-5.
9. Baguley D (2007). Hyperacusis : mechanisms, diagnosis, and therapies
. San Diego, CA: Plural Publishing Inc. p. 59.
ISBN 978-1-59756-808-1.
10. Granacher R (2008). Traumatic brain injury: methods for clinical and forensic neuropsychiatric assessment. Boca
Raton, Fla. London: CRC Taylor & Francis distributor. p. 181. ISBN 978-0-8493-8139-3.
11. Maciaszczyk K, Durko T, Waszczykowska E, Erkiert-Polguj A, Pajor A (February 2011)."Auditory function in patients
with systemic lupus erythematosus"(https://xa.yimg.com/kq/groups/18817297/928224434/name/g%C3%BCzide.pdf)
(PDF). Auris, Nasus, Larynx. 38 (1): 26–32. doi:10.1016/j.anl.2010.04.008(https://doi.org/10.1016%2Fj.anl.2010.04.
008). PMID 20576373 (https://www.ncbi.nlm.nih.gov/pubmed/20576373).
12. Desnick R (2001). Tay-Sachs disease. San Diego, Calif. London: Academic. p. 25.ISBN 978-0-08-049030-4.
13. Zarchi O, Attias J, Gothelf D (2010). "Auditory and visual processing in Williams syndrome".
The Israel Journal of
Psychiatry and Related Sciences. 47 (2): 125–31. PMID 20733255
(https://www.ncbi.nlm.nih.gov/pubmed/20733255).
14. Brotherton H, Plack CJ, Maslin M, Schaette R, Munro KJ (2015). "Pump up the volume: could excessive neural gain
explain tinnitus and hyperacusis?".Audiology & Neuro-Otology. 20 (4): 273–82. doi:10.1159/000430459 (https://doi.
org/10.1159%2F000430459). PMID 26139435 (https://www.ncbi.nlm.nih.gov/pubmed/26139435).
15. Sheldrake J, Diehl PU, Schaette R (2015)."Audiometric characteristics of hyperacusis patients"(https://www.ncbi.nl
m.nih.gov/pmc/articles/PMC4432660). Frontiers in Neurology. 6: 105. doi:10.3389/fneur.2015.00105 (https://doi.org/
10.3389%2Ffneur.2015.00105). PMC 4432660 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4432660) .
PMID 26029161 (https://www.ncbi.nlm.nih.gov/pubmed/26029161).
16. Lindsey, Heather (August 2014)."Help for Hyperacusis: Treatments Turn Down Discomfort" (https://journals.lww.co
m/thehearingjournal/Fulltext/2014/08000/Help_for_Hyperacusis___T reatments_Turn_Down.1.aspx). The Hearing
Journal. 67 (8): 22. doi:10.1097/01.HJ.0000453391.20357.f7(https://doi.org/10.1097%2F01.HJ.0000453391.20357.f
7). ISSN 0745-7472 (https://www.worldcat.org/issn/0745-7472).
17. Formby C, Hawley ML, Sherlock LP , Gold S, Payne J, Brooks R, Parton JM, Juneau R, Desporte EJ, Siegle GR
(May 2015). "A Sound Therapy-Based Intervention to Expand the Auditory Dynamic Range for Loudness among
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T (https://www.ncbi.nlm.
nih.gov/pmc/articles/PMC4906300). Seminars in Hearing. 36 (2): 77–110. doi:10.1055/s-0035-1546958(https://doi.o
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PMID 27516711 (https://www.ncbi.nlm.nih.gov/pubmed/27516711).
18. Marc Fagelson, David M. Baguley (2018).Hyperacusis and Disorders of Sound Intolerance Clinical and Research
Perspectives. Plural Publishing. pp. C15, C16.ISBN 978-1-94488-328-7.
19. Aazh H, Moore BC, Lammaing K, Cropley M (September 2016)."Tinnitus and hyperacusis therapy in a UK National
Health Service audiology department: Patients' evaluations of the ef
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47).
20. https://www.buzzfeed.com/joycecohen/noise-kills-when-everyday-sound-becomes-torture
21. http://www.huffingtonpost.com/michael-huffington/rejoining-society_b_7488310.html
22. Shub 1966, p. 426; Rice 1990, p. 187; Service 2000, p. 435.
23. "FAQs about Hyperacusis"(https://www.chrissingletonmusic.com/faqs). Chris Singleton. Retrieved 2018-03-29.
24. " 'I was allergic to sound' " (https://www.independent.co.uk/life-style/health-and-families/features/i-was-allergic-to-sou
nd-1987908.html). The Independent. 2010-06-01. Retrieved 2018-03-27.
25. "How Peter Silberman Lost His Hearing, Then Rediscovered Sound"(https://www.pastemagazine.com/articles/2017/
02/how-peter-silberman-lost-his-hearing-and-rediscove.html)
. pastemagazine.com. Retrieved 2018-03-27.

Further reading
Andersson, David M. Baguley, Gerhard (2007). Hyperacusis : mechanisms, diagnosis, and therapies . San Diego:
Plural Pub. ISBN 978-1597561044.
"Decreased Sound Tolerance", by Pawel J. Jastreboff and Margaret J Jastreboff, in: "Tinnitus: theory and
management", ed. James Byron Snow, 2004, ISBN 1-55009-243-X

External links
Classification ICD-10: H93.2 · D
ICD-9-CM: 388.42 ·
 MeSH: D01200178
· DiseasesDB:
29099

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