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Hyperacusis (also spelled hyperacousis) is a health condition characterized by an over-sensitivity to certain frequency ranges of sound (a collapsed tolerance to usual environmental sound). A person with severe hyperacusis has difficulty tolerating everyday sounds, some of which may seem unpleasantly loud to that person but not to others.
It can be acquired as a result of damage sustained to the hearing apparatus, or inner ear. There is speculation that the efferent portion of the auditory nerve (olivocochlear bundle) has been affected (efferent meaning fibers that originate in the brain which serve to regulate sounds). This theory suggests that the efferent fibers of the auditory nerve are selectively damaged, while the hair cells that allow the hearing of pure tones in an audiometric evaluation remain intact. In cases not involving aural trauma to the inner ear, hyperacusis can also be acquired as a result of damage to the brain or the neurological system. In these cases, hyperacusis can be defined as a cerebral processing problem specific to how the brain perceives sound. In rare cases, hyperacusis may be caused by a vestibular disorder. This type of hyperacusis, called vestibular hyperacusis, is caused by the brain perceiving certain sounds as motion input as well as auditory input.
Although severe hyperacusis is rare, a lesser form of hyperacusis affects musicians, making it difficult for them to play in the very loud environment of a rock band or orchestra which previously gave them no problems. It also makes attendance at loud discos or live events difficult for a portion of the population. Given that sound levels at such events usually exceed recommended safe levels of exposure, this is a problem which is probably showing up variations between people, which may be genetic, or the result of stress or ill-health, or it may be caused by abnormal response in the tensor tympani and stapedius muscles which function in the normal acoustic reflex response that protects the inner ear from loud sounds.
40% of tinnitus patients complain of mild hyperacusis.
The most common cause of hyperacusis is overexposure to excessively high decibel levels (or sound pressure levels). Some come down with hyperacusis suddenly by firing a gun, having an airbag deploy in their car, experiencing any extremely loud sound, taking ear sensitizing drugs, Lyme disease, Ménière's disease, TMD/TMJ (Temporomandibular joint disorder), 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. The Diagnostic and Statistical Manual of Mental Disorders (DSM) that is published by the American Psychiatric Association lists hyperacusis as one of the possible signs indicating phencyclidine (PCP or Angel-dust) intoxication.
Causes include, but are not limited to:
- Severe head trauma
- Facial nerve dysfunction (to stapedius)
- Post traumatic stress disorder
- Ear irrigation
- Tension myositis syndrome
- Temporomandibular joint disorder (TMJ)
- Adverse drug reaction
- MAO inhibitor discontinuation syndrome
- Williams syndrome
- Bell's palsy
- Tay–Sachs disease
- Ménière's disease
- Lyme disease
- Superior canal dehiscence syndrome (SCDS)
- Chronic ear infections
- Minor Head Injury
- Noise-induced hearing loss
- Developmental Coordination Disorder/Dyspraxia
- A vestibular disorder: see below.
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.
In vestibular hyperacusis, the sufferer may experience feelings of dizziness, nausea, or a loss of balance when sounds of certain pitches are present. For instance, people with vestibular hyperacusis may feel like they are falling and as a result involuntarily grimace and clutch for something to brace themselves with. The degree to which a sufferer is affected depends not only on the overall severity of that person's symptoms but also on whether the person can detect sounds in that frequency range at the volume in question, as well as on the person's preexisting muscle tone and severity of startle response.
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 themselves in a social situation that might involve noise.
A person suffering from hyperacusis might be startled by very low sound levels. Everyday sounds like shutting doors, ringing phones, television, running water, ticking clocks, chewing gum, cooking, normal conversation, eating, dishes, and other sounds will hurt his/her ears. In extreme situations even the use of earplugs fails to bring relief and the patient may spend their life trying to avoid all sounds and just stay at home.
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. Another treatment method is the Berard Auditory Integration Training. 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. Steroids are used to treat hyperacusis within 72 hours of the onset of the condition.
- The late Belgian composer Henri Pousseur suffered from hyperacusis.
- Jazz musician Melody Gardot has hyperacusis as a result of head trauma.
- Ludwig van Beethoven suffered from hyperacusis, alongside tinnitus and hearing loss.
- Stephin Merritt, best known as the principal singer and songwriter in the band The Magnetic Fields, suffers from hyperacusis.
- Crime-writer Linda Stratmann suffers from hyperacusis.
- In the documentary Bobby Fischer Against the World, it was suggested by Dr. Anthony Saidy that former World Chess champion Bobby Fischer suffered from hyperacusis.
See also 
- Baguley DM (December 2003). "Hyperacusis". J R Soc Med 96 (12): 582–5. PMC 539655. PMID 14645606.
- Zarchi O, Attias J, Gothelf D (2010). "Auditory and visual processing in Williams syndrome". Isr J Psychiatry Relat Sci 47 (2): 125–31. PMID 20733255.
- Nelson JJ, Chen K (July 2004). "The relationship of tinnitus, hyperacusis, and hearing loss". Ear Nose Throat J 83 (7): 472–6. PMID 15372918.
- "Molecular Interventions - CLOCKSS". Molinterv.aspetjournals.org. Retrieved 2012-10-29.
- "Stephin Merritt - Wikipedia, the free encyclopedia". En.wikipedia.org. Retrieved 2012-10-29.
Further reading 
- "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