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{{SI}}
{{CMG}}; {{AE}} {{Norina Usman}}
==Overview==
==Overview==
Hyperacusis is a condition characterized by an over-sensitivity to a specific frequency of sound or intolerance to reasonable environmental sounds. A person with hyperacusis has difficulty accepting everyday sounds; some sounds may seem offensively loud to that person but not to others. In hyperacusis, a person gives inappropriate or exaggerated responses to sounds that are neither uncomfortable nor threatening loud to an average person; even low-intensity sounds can elicit the reaction<ref name="pmid3631220">{{cite journal| author=Vernon JA| title=Pathophysiology of tinnitus: a special case--hyperacusis and a proposed treatment. | journal=Am J Otol | year= 1987 | volume= 8 | issue= 3 | pages= 201-2 | pmid=3631220 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3631220  }} </ref><ref name="pmid2329796">{{cite journal| author=Klein AJ, Armstrong BL, Greer MK, Brown FR| title=Hyperacusis and otitis media in individuals with Williams syndrome. | journal=J Speech Hear Disord | year= 1990 | volume= 55 | issue= 2 | pages= 339-44 | pmid=2329796 | doi=10.1044/jshd.5502.339 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2329796  }} </ref>.
Hyperacusis is a condition characterized by an over-sensitivity to a specific frequency of sound or intolerance to reasonable environmental sounds. A person with hyperacusis has difficulty accepting everyday sounds; some sounds may seem offensively loud to that person but not to others. In hyperacusis, a person gives inappropriate or exaggerated responses to sounds that are neither uncomfortable nor threatening loud to an average person; even low-intensity sounds can elicit the reaction.


==Classification==
==Classification==
Based on the symptoms, hyperacusis may be classified as Cochlear and vestibular hyperacusis<ref name="pmid14645606">{{cite journal| author=Baguley DM| title=Hyperacusis. | journal=J R Soc Med | year= 2003 | volume= 96 | issue= 12 | pages= 582-5 | pmid=14645606 | doi=10.1258/jrsm.96.12.582 | pmc=539655 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14645606  }} </ref><ref name="pmid12169120">{{cite journal| author=Baguley DM, Axon P, Winter IM, Moffat DA| title=The effect of vestibular nerve section upon tinnitus. | journal=Clin Otolaryngol Allied Sci | year= 2002 | volume= 27 | issue= 4 | pages= 219-26 | pmid=12169120 | doi=10.1046/j.1365-2273.2002.00566.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12169120  }} </ref><ref name="pmid9007578">{{cite journal| author=Scharf B, Magnan J, Chays A| title=On the role of the olivocochlear bundle in hearing: 16 case studies. | journal=Hear Res | year= 1997 | volume= 103 | issue= 1-2 | pages= 101-22 | pmid=9007578 | doi=10.1016/s0378-5955(96)00168-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9007578  }} </ref>.
Based on the symptoms, hyperacusis may be classified as cochlear and vestibular hyperacusis.<ref name="pmid14645606">{{cite journal |vauthors=Baguley DM |title=Hyperacusis |journal=J R Soc Med |volume=96 |issue=12 |pages=582–5 |date=December 2003 |pmid=14645606 |pmc=539655 |doi=10.1258/jrsm.96.12.582 |url=}}</ref>


1) [[Cochlear hyperacusis]]: (the most common form of hyperacusis) presents with ear pain, annoyance, and general intolerance to any sounds that most people don't notice or consider unpleasant. Crying spells or panic attacks may result from cochlear hyperacusis. As many as 86% of hyperacusis sufferers also have tinnitus.
====Cochlear hyperacusis:====


2) [[Vestibular hyperacusis]]: The sufferer may experience dizziness, nausea, or a loss of balance when certain pitched sounds are present.  
*The most common form of [[hyperacusis]]
*Presents with ear pain and general intolerance to any sounds that most people don't notice or consider unpleasant.
*Crying spells or [[panic attacks]] may result from cochlear hyperacusis.
*Most of the time cochlear hyperacusis is associated with [[tinnitus]].


Anxiety, stress, and phonophobia may be present in both types of hyperacusis. Someone with either form of hyperacusis may develop avoidant behavior to avoid a stressful sound situation or avoid embarrassing themself in a social case that might involve noise.
====Vestibular hyperacusis:====
 
*Associated with [[dizziness]], [[nausea]], or a loss of balance triggered by certain pitch sounds.
*[[Anxiety]], [[stress]], and [[phonophobia]] may be present in both types of hyperacusis.
*High risk for developing avoidant behavior to avoid a stressful sound situation.


==Pathophysiology==
==Pathophysiology==
Hyperacusis can be developed because of damage to the inner ear or hearing apparatus, affecting efferent part of the auditory nerve, and fibers that come out from the brain that control sounds. In this process, tissues of the auditory nerve are damaged, though the hair cells that permit us to hear pure tones remain integral. It can be as a result of injury to the neurological system of the brain. In some cases, hyperacusis may be triggered by a vestibular disorder. Stapes hypermobility can also be one of the causes of peripheral hyperacusis. Situations that comprise paralysis of the facial nerve (i.e., Ramsay-Hunt syndrome, Bell’s palsy, and Lyme disease) are involved in the causes of the condition.Hyperacusis increases in extent during anxiety, tiredness, or stress. The mechanism involved during stress, include endogenous dynorphins release into the synaptic region underneath inner hair cells. It potentiates the neurotransmitter glutamate, triggering sound to be seeming with excessive noise<ref name="pmid11347634">{{cite journal| author=Katzenell U, Segal S| title=Hyperacusis: review and clinical guidelines. | journal=Otol Neurotol | year= 2001 | volume= 22 | issue= 3 | pages= 321-6; discussion 326-7 | pmid=11347634 | doi=10.1097/00129492-200105000-00009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11347634  }} </ref><ref name="pmid11223280">{{cite journal| author=Sahley TL, Nodar RH| title=A biochemical model of peripheral tinnitus. | journal=Hear Res | year= 2001 | volume= 152 | issue= 1-2 | pages= 43-54 | pmid=11223280 | doi=10.1016/s0378-5955(00)00235-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11223280  }} </ref><ref name="pmid12169120">{{cite journal| author=Baguley DM, Axon P, Winter IM, Moffat DA| title=The effect of vestibular nerve section upon tinnitus. | journal=Clin Otolaryngol Allied Sci | year= 2002 | volume= 27 | issue= 4 | pages= 219-26 | pmid=12169120 | doi=10.1046/j.1365-2273.2002.00566.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12169120  }} </ref><ref name="pmid3631220">{{cite journal| author=Vernon JA| title=Pathophysiology of tinnitus: a special case--hyperacusis and a proposed treatment. | journal=Am J Otol | year= 1987 | volume= 8 | issue= 3 | pages= 201-2 | pmid=3631220 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3631220  }} </ref>.
 
*Hyperacusis can be developed because of damage to the inner ear or hearing apparatus, affecting efferent part of the [[auditory nerve]], and fibers that come out from the [[brain]] that control sounds.<ref name="pmid18404276">{{cite journal |vauthors=Jansen EJ, Helleman HW, Dreschler WA, de Laat JA |title=Noise induced hearing loss and other hearing complaints among musicians of symphony orchestras |journal=Int Arch Occup Environ Health |volume=82 |issue=2 |pages=153–64 |date=January 2009 |pmid=18404276 |doi=10.1007/s00420-008-0317-1 |url=}}</ref><ref name="pmid27569405">{{cite journal |vauthors=Paulin J, Andersson L, Nordin S |title=Characteristics of hyperacusis in the general population |journal=Noise Health |volume=18 |issue=83 |pages=178–84 |date=2016 |pmid=27569405 |pmc=5187659 |doi=10.4103/1463-1741.189244 |url=}}</ref>
*In this process, tissues of the [[auditory nerve]] are damaged, though the hair cells that permit us to hear pure tones remain integral.
*It can be as a result of injury to the neurological system of the brain. In some cases, hyperacusis may be triggered by a vestibular disorder.
*[[Stapes]] hypermobility can also be one of the causes of peripheral hyperacusis.
*Situations that comprise paralysis of the [[facial nerve]] (i.e., [[Ramsay-Hunt syndrome]], [[Bell's palsy|Bell’s palsy,]] and [[Lyme disease]]) are involved in the causes of the condition.
*Hyperacusis increases in extent during anxiety, tiredness, or stress.
*The mechanism involved during stress, include endogenous dynorphins release into the [[Synaptic cleft|synaptic region]] underneath inner hair cells.
*It potentiates the neurotransmitter [[glutamate]], triggering sound to be seeming with excessive noise.


==Causes==
==Causes==
The Common causes of hyperacusis include<ref name="pmid25104073">{{cite journal| author=Tyler RS, Pienkowski M, Roncancio ER, Jun HJ, Brozoski T, Dauman N | display-authors=etal| title=A review of hyperacusis and future directions: part I. Definitions and manifestations. | journal=Am J Audiol | year= 2014 | volume= 23 | issue= 4 | pages= 402-19 | pmid=25104073 | doi=10.1044/2014_AJA-14-0010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25104073 }} </ref><ref name="pmid30261653">{{cite journal| author=Di Stadio A, Dipietro L, Ricci G, Della Volpe A, Minni A, Greco A | display-authors=etal| title=Hearing Loss, Tinnitus, Hyperacusis, and Diplacusis in Professional Musicians: A Systematic Review. | journal=Int J Environ Res Public Health | year= 2018 | volume= 15 | issue= 10 | pages= | pmid=30261653 | doi=10.3390/ijerph15102120 | pmc=6209930 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30261653 }} </ref><ref name="pmid30261653">{{cite journal| author=Di Stadio A, Dipietro L, Ricci G, Della Volpe A, Minni A, Greco A | display-authors=etal| title=Hearing Loss, Tinnitus, Hyperacusis, and Diplacusis in Professional Musicians: A Systematic Review. | journal=Int J Environ Res Public Health | year= 2018 | volume= 15 | issue= 10 | pages= | pmid=30261653 | doi=10.3390/ijerph15102120 | pmc=6209930 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30261653 }} </ref><ref name="pmid25913555">{{cite journal| author=Halevi-Katz DN, Yaakobi E, Putter-Katz H| title=Exposure to music and noise-induced hearing loss (NIHL) among professional pop/rock/jazz musicians. | journal=Noise Health | year= 2015 | volume= 17 | issue= 76 | pages= 158-64 | pmid=25913555 | doi=10.4103/1463-1741.155848 | pmc=4918652 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25913555 }} </ref><ref name="pmid2329796">{{cite journal| author=Klein AJ, Armstrong BL, Greer MK, Brown FR| title=Hyperacusis and otitis media in individuals with Williams syndrome. | journal=J Speech Hear Disord | year= 1990 | volume= 55 | issue= 2 | pages= 339-44 | pmid=2329796 | doi=10.1044/jshd.5502.339 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2329796 }} </ref>:
Common causes of hyperacusis include:<ref name="pmid29488875">{{cite journal| author=Aazh H, Moore BCJ| title=Thoughts about Suicide and Self-Harm in Patients with Tinnitus and Hyperacusis. | journal=J Am Acad Audiol | year= 2018 | volume= 29 | issue= 3 | pages= 255-261 | pmid=29488875 | doi=10.3766/jaaa.16181 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29488875 }} </ref>
*Loud noise experience.
 
*Recreational and industrial noise acquaintance and noise-related hearing loss.
*Severe [[head trauma]]
*Expert musicians specifically those playing rock music and exposed to extended periods of amplified sound.
*[[Facial nerve]] dysfunction (to [[Stapedius]])
*Other causes that can lead to hyperacusis include migraine, Lyme disease, psychiatric illness such as post-traumatic stress disorder, and Williams syndrome.
*[[Surgery]]
*The most common cause of hyperacusis is overexposure to excessively high [[decibel]] levels (or [[sound pressure]] levels).
*Ear irrigation
*Some come down with hyperacusis suddenly by firing a gun, having an airbag deploy in their car, taking ear sensitive drugs.
*[[TMJ]] (Temporomandibular joint disorder)
Other causes can be due to the following:
*[[Adverse drug reaction]]
* Severe [[head trauma]]
*[[Williams Syndrome]]
* [[Facial nerve]] dysfunction (to [[Stapedius]])
*[[Autism]]
* [[Surgery]]
*[[Bell's palsy]]
* Ear irrigation
*[[Ménière's disease]]
* [[TMJ]] (Temporomandibular joint disorder)
*[[Asperger syndrome]]
* [[Adverse drug reaction]]
*Superior Canal Dehiscence Syndrome
* [[Williams Syndrome]]
*Chronic ear infections
* [[Autism]]
*Minor Head Injury
* [[Bell's palsy]]
*A vestibular disorder
* [[Ménière's disease]]
 
* [[Asperger syndrome]]
===Differentiating hyperacusis from other diseases===
* Superior Canal Dehiscence Syndrome
On the basis of signs and symptoms, hyperacusis must be differentiated from misophonia, phonophobia, tinnitus, William syndrome, Lyme disease, migraine.<ref name="pmid22413649">{{cite journal |vauthors=Schwartz P, Leyendecker J, Conlon M |title=Hyperacusis and misophonia: the lesser-known siblings of tinnitus |journal=Minn Med |volume=94 |issue=11 |pages=42–3 |date=November 2011 |pmid=22413649 |doi= |url=}}</ref><ref name="pmid23244506">{{cite journal |vauthors=Jüris L, Andersson G, Larsen HC, Ekselius L |title=Psychiatric comorbidity and personality traits in patients with hyperacusis |journal=Int J Audiol |volume=52 |issue=4 |pages=230–5 |date=April 2013 |pmid=23244506 |doi=10.3109/14992027.2012.743043 |url=}}</ref><ref name="pmid12169120">{{cite journal| author=Baguley DM, Axon P, Winter IM, Moffat DA| title=The effect of vestibular nerve section upon tinnitus. | journal=Clin Otolaryngol Allied Sci | year= 2002 | volume= 27 | issue= 4 | pages= 219-26 | pmid=12169120 | doi=10.1046/j.1365-2273.2002.00566.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12169120 }} </ref><ref name="pmid3631220">{{cite journal| author=Vernon JA| title=Pathophysiology of tinnitus: a special case--hyperacusis and a proposed treatment. | journal=Am J Otol | year= 1987 | volume= 8 | issue= 3 | pages= 201-2 | pmid=3631220 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3631220 }} </ref>.
* Chronic ear infections
{|
* Minor Head Injury
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
* A vestibular disorder:  see below.
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
| colspan="6" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
! rowspan="2" |Para-clinical findings
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Gold standard'''
|-
| colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
! colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination
|-
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Negative emotional reaction
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Ringing in the ears
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Psychiatric disorders
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Hearing loss
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Sound sensitivity
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Loudness discomfort level
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Misphonia
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |Limbic system involved
| style="background: #F5F5F5; padding: 5px;" |Clinical diagnosis
 
*Certain sounds trigger emotional or physiological responses
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Phonophobia
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |Limbic system involved
| style="background: #F5F5F5; padding: 5px;" |Clinical diagnosis
 
*Fear of loud sounds
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Tinnitus]]
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |8th cranial nerve palsy/ auditory system involved
| style="background: #F5F5F5; padding: 5px;" |Audiological exam
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |William Syndrome
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |Genetic disorder
| style="background: #F5F5F5; padding: 5px;" |Micro-array analysis/FISH and audiological exam
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Lyme disease|Lyme Disease]]
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |Auditory system involved
| style="background: #F5F5F5; padding: 5px;" |Audiological exam
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Migraine]]
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |Trigeminal ganglion stimulation
| style="background: #F5F5F5; padding: 5px;" |Clinical diagnosis
|}
 
==Epidemiology and Demographics==
 
'''Incidence'''
 
*The incidence of hyperacusis is approximately 1 in 50,000 people.
 
'''Prevalence'''
 
*The overall [[prevalence]] of [[hyperacusis]] among children and [[adolescents]] is between 3.2% to 1.7%. In adults, prevalence rate is between 8% to 15.2%.
 
'''Age'''
 
*Hyperacusis is commonly seen in individuals with any age group.
 
'''Gender'''
 
*Hyperacusis affects men and women equally.
 
'''Race'''
 
*There is no racial predilection to hyperacusis.
 
==Natural History, Complications, and Prognosis==
'''Natural History'''
 
*In the case of a [[hyperacusis]], patients can present with.<ref name="pmid25104073">{{cite journal| author=Tyler RS, Pienkowski M, Roncancio ER, Jun HJ, Brozoski T, Dauman N | display-authors=etal| title=A review of hyperacusis and future directions: part I. Definitions and manifestations. | journal=Am J Audiol | year= 2014 | volume= 23 | issue= 4 | pages= 402-19 | pmid=25104073 | doi=10.1044/2014_AJA-14-0010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25104073 }} </ref><ref name="pmid27569405">{{cite journal| author=Paulin J, Andersson L, Nordin S| title=Characteristics of hyperacusis in the general population. | journal=Noise Health | year= 2016 | volume= 18 | issue= 83 | pages= 178-84 | pmid=27569405 | doi=10.4103/1463-1741.189244 | pmc=5187659 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27569405 }} </ref>
 
 
*[[Ear pain]]
*[[Annoyance]]
*[[Fear]]
*General intolerance to many sounds
*[[Crying spells]]
*[[Panic attacks]]
*[[Tinnitus]]
*[[Anxiety]]
*[[Stress]]
*[[Phonophobia]]
*[[Avoidant behavior]]
*[[Dizziness]]
*[[Nausea]]
 
'''Complications'''
 
*Common complications of hyperacusis depend on the etiology.
 
'''Prognosis'''


==Symptoms==
*Depending on the extent of the disease progression at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good.
In cochlear hyperacusis (the most common form of hyperacusis), the symptoms are ear pain, annoyance, and general intolerance to any sounds that most people don't notice or consider unpleasant.  Crying spells or panic attacks may result from cochlear hyperacusis. As many as 86% of hyperacusis sufferers also have tinnitus.
*Patients with hyperacusis have profound psychological influence, patients presenting with self-harm or suicidal ideation.


In [[vestibular hyperacusis]], the sufferer may experience feelings of dizziness, nausea, or a loss of balance when certain pitched sounds are present.  For instance, someone with vestibular hyperacusis may feel like they are falling and as a result involuntarily grimace and clutch for something to brace themselves with.
==[[Hyperausis Diagnosis]]==


Anxiety, stress, and/or phonophobia may be present in both types of hyperacusis. Someone with either form of hyperacusis may develop avoidant behavior in order to try to avoid a stressful sound situation or to avoid embarrassing themself in a social situation that might involve noise.
===Diagnostic study of choice===
Pure tone audiometry is the gold standard test for the diagnosis of hyperacusis.
 
*Loudness discomfort level( LDL) measured in decibels (dB); LDL decreased by 16–18 dB than the general population is diagnostic of hyperacusis.
*95% of patients with hyperacusis have LDL ≤ 77 dB (average LDL in a normal person is 100 dB).
 
===History and Symptoms===
 
*The hallmark of hyperacusis is sensitivity to sounds.
*A positive history of over-sensitivity or distress to particular sounds is suggestive of hyperacusis.
*The most common symptoms of hyperacusis include annoyance, ear pain, loudness, and tinnitus.
 
===Physical Examination===
Physical examination of patients' hyperacusis is usually remarkable for fear, irritability, and avoidance behavior.
 
===Laboratory Findings===
There are no diagnostic laboratory findings associated with hyperacusis.
===CT scan===
There are no CT scan findings associated with hyperacusis.
 
===MRI===
There are no MRI findings associated with hyperacusis.
 
===Other Diagnostic Studies===
[[Health questionnaires]] may be helpful in the diagnosis of hyperacusis. Findings suggestive of hyperacusis include:
 
*HQ score of ≥ 22 is diagnostic of hyperacusis.
*[[Disability]]
*Functional impact
*[[Psychological]] factors
*Impacted [[quality of life]]


==Treatment==
==Treatment==
The most common treatment for hyperacusis is retraining therapy which uses broadband noise. Tinnitus Retraining Therapy (TRT), a treatment originally used to treat tinnitus, uses broadband noise to treat hyperacusis. [[Pink noise]] can also be used to treat hyperacusis.  By listening to broadband noise at soft levels for a disciplined period of time each day, patients can rebuild (i.e., re-establish) their tolerances to sound. When seeking treatment, it is important that the physician determine the patient's Loudness Discomfort Levels (LDL) so that hearing tests (brainstem auditory evoke response) or other diagnostic tests which involve loud noise ([[MRI]]) do not worsen the patient's tolerance to sound.
The most common treatment for hyperacusis is retraining therapy that includes:<ref name="pmid16329450">{{cite journal |vauthors=Andersson G, Jüris L, Kaldo V, Baguley DM, Larsen HC, Ekselius L |title=[Hyperacusis--an unexplored field. Cognitive behavior therapy can relieve problems in auditory intolerance, a condition with many questions] |language=Swedish |journal=Lakartidningen |volume=102 |issue=44 |pages=3210–2 |date=2005 |pmid=16329450 |doi= |url=}}</ref><ref name="pmid30261653">{{cite journal| author=Di Stadio A, Dipietro L, Ricci G, Della Volpe A, Minni A, Greco A | display-authors=etal| title=Hearing Loss, Tinnitus, Hyperacusis, and Diplacusis in Professional Musicians: A Systematic Review. | journal=Int J Environ Res Public Health | year= 2018 | volume= 15 | issue= 10 | pages=  | pmid=30261653 | doi=10.3390/ijerph15102120 | pmc=6209930 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30261653  }} </ref><ref name="pmid2329796">{{cite journal| author=Klein AJ, Armstrong BL, Greer MK, Brown FR| title=Hyperacusis and otitis media in individuals with Williams syndrome. | journal=J Speech Hear Disord | year= 1990 | volume= 55 | issue= 2 | pages= 339-44 | pmid=2329796 | doi=10.1044/jshd.5502.339 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2329796  }} </ref>
 
*Tinnitus Retraining Therapy (TRT), a treatment originally used to treat tinnitus, uses broadband noise to treat hyperacusis. By listening to broadband noise at soft levels for a disciplined period of time each day, patients can rebuild (i.e., re-establish) their tolerances to sound.
*[[Pink noise]] can also be used to treat hyperacusis.
 
Another possible treatment include:<ref name="pmid14645606">{{cite journal| author=Baguley DM| title=Hyperacusis. | journal=J R Soc Med | year= 2003 | volume= 96 | issue= 12 | pages= 582-5 | pmid=14645606 | doi=10.1258/jrsm.96.12.582 | pmc=539655 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14645606  }} </ref>
 
<br />
{| class="wikitable"
|+
!Non- pharmacological therapy
!Surgical Treatment
|'''Alternative treatments'''
|-
|Cognitive Behavioral Therapy
|Round window reinforcement
|Excercise, yoga, meditation
|-
|Tinnitus retraining therapy (TRT)
|Oval window reinforcement
|Massage, relaxing therapy, hypnosis
|-
|Directive Counselling
|
|Vitamin and supplements
|}


==People==
==People==
* [[Stephen Merritt]], of [[The Magnetic Fields]], suffers from this condition.
 
*[[Stephen Merritt]], of [[The Magnetic Fields]], suffers from this condition.


==See also==
==See also==
*[[Hearing impairment]]
*[[Hearing impairment]]
*[[Tinnitus]]
*[[Tinnitus]]


== External links ==
==External links==
* [http://www.aitinstiute.org AIT Institute for Auditory Integration Training. AIT helps remediate hyperacute hearing]
* [http://www.hyperacusis.net The Hyperacusis Network]
* [http://www.tinnitus-pjj.com/ Tinnitus & Hyperacusis Center by Pawel J. Jastreboff]
* [http://www.tinnitus.org/home/frame/THC1.htm The Tinnitus and Hyperacusis Centre, London UK]


{{Diseases of the ear and mastoid process }}
*[http://www.aitinstiute.org AIT Institute for Auditory Integration Training. AIT helps remediate hyperacute hearing]
[[fr:Hyperacousie]]
*[http://www.hyperacusis.net The Hyperacusis Network]
[[he:היפראקוזיס]]
*[http://www.tinnitus-pjj.com/ Tinnitus & Hyperacusis Center by Pawel J. Jastreboff]
[[nl:Hyperacusis]]
*[http://www.tinnitus.org/home/frame/THC1.htm The Tinnitus and Hyperacusis Centre, London UK]
[[fi:Hyperakusia]]


==References==
{{Reflist|2}}
[[Category:Otolaryngology]]
[[Category:Otolaryngology]]
{{WikiDoc Sources}}
[[Category: Up To Date]]

Latest revision as of 13:14, 14 April 2021

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List of terms related to Hyperacusis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Norina Usman, M.B.B.S[2]

Overview

Hyperacusis is a condition characterized by an over-sensitivity to a specific frequency of sound or intolerance to reasonable environmental sounds. A person with hyperacusis has difficulty accepting everyday sounds; some sounds may seem offensively loud to that person but not to others. In hyperacusis, a person gives inappropriate or exaggerated responses to sounds that are neither uncomfortable nor threatening loud to an average person; even low-intensity sounds can elicit the reaction.

Classification

Based on the symptoms, hyperacusis may be classified as cochlear and vestibular hyperacusis.[1]

Cochlear hyperacusis:

  • The most common form of hyperacusis
  • Presents with ear pain and general intolerance to any sounds that most people don't notice or consider unpleasant.
  • Crying spells or panic attacks may result from cochlear hyperacusis.
  • Most of the time cochlear hyperacusis is associated with tinnitus.

Vestibular hyperacusis:

  • Associated with dizziness, nausea, or a loss of balance triggered by certain pitch sounds.
  • Anxiety, stress, and phonophobia may be present in both types of hyperacusis.
  • High risk for developing avoidant behavior to avoid a stressful sound situation.

Pathophysiology

  • Hyperacusis can be developed because of damage to the inner ear or hearing apparatus, affecting efferent part of the auditory nerve, and fibers that come out from the brain that control sounds.[2][3]
  • In this process, tissues of the auditory nerve are damaged, though the hair cells that permit us to hear pure tones remain integral.
  • It can be as a result of injury to the neurological system of the brain. In some cases, hyperacusis may be triggered by a vestibular disorder.
  • Stapes hypermobility can also be one of the causes of peripheral hyperacusis.
  • Situations that comprise paralysis of the facial nerve (i.e., Ramsay-Hunt syndrome, Bell’s palsy, and Lyme disease) are involved in the causes of the condition.
  • Hyperacusis increases in extent during anxiety, tiredness, or stress.
  • The mechanism involved during stress, include endogenous dynorphins release into the synaptic region underneath inner hair cells.
  • It potentiates the neurotransmitter glutamate, triggering sound to be seeming with excessive noise.

Causes

Common causes of hyperacusis include:[4]

Differentiating hyperacusis from other diseases

On the basis of signs and symptoms, hyperacusis must be differentiated from misophonia, phonophobia, tinnitus, William syndrome, Lyme disease, migraine.[5][6][7][8].

Diseases Clinical manifestations Para-clinical findings Gold standard
Symptoms Physical examination
Negative emotional reaction Ringing in the ears Psychiatric disorders Hearing loss Sound sensitivity Loudness discomfort level
Misphonia + - + +/- - - Limbic system involved Clinical diagnosis
  • Certain sounds trigger emotional or physiological responses
Phonophobia + - + +/- - - Limbic system involved Clinical diagnosis
  • Fear of loud sounds
Tinnitus +/- + - - + + 8th cranial nerve palsy/ auditory system involved Audiological exam
William Syndrome +/- - +/- + + + Genetic disorder Micro-array analysis/FISH and audiological exam
Lyme Disease + + +/- + + + Auditory system involved Audiological exam
Migraine - + - - + +/- Trigeminal ganglion stimulation Clinical diagnosis

Epidemiology and Demographics

Incidence

  • The incidence of hyperacusis is approximately 1 in 50,000 people.

Prevalence

Age

  • Hyperacusis is commonly seen in individuals with any age group.

Gender

  • Hyperacusis affects men and women equally.

Race

  • There is no racial predilection to hyperacusis.

Natural History, Complications, and Prognosis

Natural History


Complications

  • Common complications of hyperacusis depend on the etiology.

Prognosis

  • Depending on the extent of the disease progression at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good.
  • Patients with hyperacusis have profound psychological influence, patients presenting with self-harm or suicidal ideation.

Hyperausis Diagnosis

Diagnostic study of choice

Pure tone audiometry is the gold standard test for the diagnosis of hyperacusis.

  • Loudness discomfort level( LDL) measured in decibels (dB); LDL decreased by 16–18 dB than the general population is diagnostic of hyperacusis.
  • 95% of patients with hyperacusis have LDL ≤ 77 dB (average LDL in a normal person is 100 dB).

History and Symptoms

  • The hallmark of hyperacusis is sensitivity to sounds.
  • A positive history of over-sensitivity or distress to particular sounds is suggestive of hyperacusis.
  • The most common symptoms of hyperacusis include annoyance, ear pain, loudness, and tinnitus.

Physical Examination

Physical examination of patients' hyperacusis is usually remarkable for fear, irritability, and avoidance behavior.

Laboratory Findings

There are no diagnostic laboratory findings associated with hyperacusis.

CT scan

There are no CT scan findings associated with hyperacusis.

MRI

There are no MRI findings associated with hyperacusis.

Other Diagnostic Studies

Health questionnaires may be helpful in the diagnosis of hyperacusis. Findings suggestive of hyperacusis include:

Treatment

The most common treatment for hyperacusis is retraining therapy that includes:[10][11][12]

  • Tinnitus Retraining Therapy (TRT), a treatment originally used to treat tinnitus, uses broadband noise to treat hyperacusis. By listening to broadband noise at soft levels for a disciplined period of time each day, patients can rebuild (i.e., re-establish) their tolerances to sound.
  • Pink noise can also be used to treat hyperacusis.

Another possible treatment include:[1]


Non- pharmacological therapy Surgical Treatment Alternative treatments
Cognitive Behavioral Therapy Round window reinforcement Excercise, yoga, meditation
Tinnitus retraining therapy (TRT) Oval window reinforcement Massage, relaxing therapy, hypnosis
Directive Counselling Vitamin and supplements

People

See also

External links

References

  1. ↑ 1.0 1.1 Baguley DM (December 2003). "Hyperacusis". J R Soc Med. 96 (12): 582–5. doi:10.1258/jrsm.96.12.582. PMC 539655. PMID 14645606.
  2. ↑ Jansen EJ, Helleman HW, Dreschler WA, de Laat JA (January 2009). "Noise induced hearing loss and other hearing complaints among musicians of symphony orchestras". Int Arch Occup Environ Health. 82 (2): 153–64. doi:10.1007/s00420-008-0317-1. PMID 18404276.
  3. ↑ 3.0 3.1 Paulin J, Andersson L, Nordin S (2016). "Characteristics of hyperacusis in the general population". Noise Health. 18 (83): 178–84. doi:10.4103/1463-1741.189244. PMC 5187659. PMID 27569405.
  4. ↑ Aazh H, Moore BCJ (2018). "Thoughts about Suicide and Self-Harm in Patients with Tinnitus and Hyperacusis". J Am Acad Audiol. 29 (3): 255–261. doi:10.3766/jaaa.16181. PMID 29488875.
  5. ↑ Schwartz P, Leyendecker J, Conlon M (November 2011). "Hyperacusis and misophonia: the lesser-known siblings of tinnitus". Minn Med. 94 (11): 42–3. PMID 22413649.
  6. ↑ Jüris L, Andersson G, Larsen HC, Ekselius L (April 2013). "Psychiatric comorbidity and personality traits in patients with hyperacusis". Int J Audiol. 52 (4): 230–5. doi:10.3109/14992027.2012.743043. PMID 23244506.
  7. ↑ Baguley DM, Axon P, Winter IM, Moffat DA (2002). "The effect of vestibular nerve section upon tinnitus". Clin Otolaryngol Allied Sci. 27 (4): 219–26. doi:10.1046/j.1365-2273.2002.00566.x. PMID 12169120.
  8. ↑ Vernon JA (1987). "Pathophysiology of tinnitus: a special case--hyperacusis and a proposed treatment". Am J Otol. 8 (3): 201–2. PMID 3631220.
  9. ↑ Tyler RS, Pienkowski M, Roncancio ER, Jun HJ, Brozoski T, Dauman N; et al. (2014). "A review of hyperacusis and future directions: part I. Definitions and manifestations". Am J Audiol. 23 (4): 402–19. doi:10.1044/2014_AJA-14-0010. PMID 25104073.
  10. ↑ Andersson G, Jüris L, Kaldo V, Baguley DM, Larsen HC, Ekselius L (2005). "[Hyperacusis--an unexplored field. Cognitive behavior therapy can relieve problems in auditory intolerance, a condition with many questions]". Lakartidningen (in Swedish). 102 (44): 3210–2. PMID 16329450.
  11. ↑ Di Stadio A, Dipietro L, Ricci G, Della Volpe A, Minni A, Greco A; et al. (2018). "Hearing Loss, Tinnitus, Hyperacusis, and Diplacusis in Professional Musicians: A Systematic Review". Int J Environ Res Public Health. 15 (10). doi:10.3390/ijerph15102120. PMC 6209930. PMID 30261653.
  12. ↑ Klein AJ, Armstrong BL, Greer MK, Brown FR (1990). "Hyperacusis and otitis media in individuals with Williams syndrome". J Speech Hear Disord. 55 (2): 339–44. doi:10.1044/jshd.5502.339. PMID 2329796.
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