Hyperacusis
Encyclopedia
Hyperacusis is a health condition characterized by an over-sensitivity to certain frequency ranges of sound (a collapsed tolerance to normal 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.
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 (Temporomandibular joint disorder
), head injury, or surgery. Others are born with sound sensitivity (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:
.
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.
, 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.
It can be acquired as a result of damage sustained to the hearing apparatus, or inner ear
Inner ear
The inner ear is the innermost part of the vertebrate ear. In mammals, it consists of the bony labyrinth, a hollow cavity in the temporal bone of the skull with a system of passages comprising two main functional parts:...
. There is speculation that the efferent
Efferent nerve
In the nervous system, efferent nerves, otherwise known as motor or effector neurons, carry nerve impulses away from the central nervous system to effectors such as muscles or glands...
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
Vestibular hyperacusis
The word hyperacusis generally refers to cochlear hyperacusis, which is associated with tinnitus and an intolerance for normal, every day sounds, such as the refrigerator running. However, vestibular hyperacusis is equally disabling for the lives it affects, albeit in different ways. Dr...
, 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
Tensor tympani
The tensor tympani, the larger of the two muscles of the tympanic cavity, is contained in the bony canal above the osseous portion of the auditory tube...
and stapedius
Stapedius
The stapedius is the smallest skeletal muscle in the human body. At just over one millimeter in length, its purpose is to stabilize the smallest bone in the body, the stapes....
muscles which function in the normal acoustic reflex
Acoustic reflex
The acoustic reflex is an involuntary muscle contraction that occurs in the middle ear of mammals in response to high-intensity sound stimuli....
response that protects the inner ear from loud sounds.
40% of tinnitus
Tinnitus
Tinnitus |ringing]]") is the perception of sound within the human ear in the absence of corresponding external sound.Tinnitus is not a disease, but a symptom that can result from a wide range of underlying causes: abnormally loud sounds in the ear canal for even the briefest period , ear...
patients complain of mild hyperacusis.
Causes
The most common cause of hyperacusis is overexposure to excessively high decibelDecibel
The decibel is a logarithmic unit that indicates the ratio of a physical quantity relative to a specified or implied reference level. A ratio in decibels is ten times the logarithm to base 10 of the ratio of two power quantities...
levels (or sound pressure
Sound pressure
Sound pressure or acoustic pressure is the local pressure deviation from the ambient atmospheric pressure caused by a sound wave. Sound pressure can be measured using a microphone in air and a hydrophone in water...
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
Lyme disease
Lyme disease, or Lyme borreliosis, is an emerging infectious disease caused by at least three species of bacteria belonging to the genus Borrelia. Borrelia burgdorferi sensu stricto is the main cause of Lyme disease in the United States, whereas Borrelia afzelii and Borrelia garinii cause most...
, Ménière's disease
Ménière's disease
Ménière's disease is a disorder of the inner ear that can affect hearing and balance to a varying degree. It is characterized by episodes of vertigo and tinnitus and progressive hearing loss, usually in one ear. It is named after the French physician Prosper Ménière, who, in an article published...
, TMD (Temporomandibular joint disorder
Temporomandibular joint disorder
Temporomandibular joint disorder , or TMJ syndrome, is an umbrella term covering acute or chronic inflammation of the temporomandibular joint, which connects the mandible to the skull. The disorder and resultant dysfunction can result in significant pain and impairment...
), head injury, or surgery. Others are born with sound sensitivity (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
DSM
-Business:* DSM , an international life science and performance materials company from the Netherlands* Delhi school of music, a music school in India...
) that is published by the American Psychiatric Association
American Psychiatric Association
The American Psychiatric Association is the main professional organization of psychiatrists and trainee psychiatrists in the United States, and the most influential worldwide. Its some 38,000 members are mainly American but some are international...
lists hyperacusis as one of the possible signs indicating phencyclidine
Phencyclidine
Phencyclidine , commonly initialized as PCP and known colloquially as angel dust, is a recreational dissociative drug...
(PCP or Angel-dust) intoxication.
Causes include, but are not limited to:
- MigraineMigraineMigraine is a chronic neurological disorder characterized by moderate to severe headaches, and nausea...
- Severe head trauma
- Facial nerveFacial nerveThe facial nerve is the seventh of twelve paired cranial nerves. It emerges from the brainstem between the pons and the medulla, and controls the muscles of facial expression, and functions in the conveyance of taste sensations from the anterior two-thirds of the tongue and oral cavity...
dysfunction (to stapediusStapediusThe stapedius is the smallest skeletal muscle in the human body. At just over one millimeter in length, its purpose is to stabilize the smallest bone in the body, the stapes....
) - SurgerySurgerySurgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
- Ear irrigation
- Tension myositis syndromeTension myositis syndromeTension myositis syndrome , also known as tension myoneural syndrome, is a name given by John E. Sarno to a condition he describes as characterized by psychosomatic musculoskeletal and nerve symptoms, most notably back pain...
- Temporomandibular joint disorderTemporomandibular joint disorderTemporomandibular joint disorder , or TMJ syndrome, is an umbrella term covering acute or chronic inflammation of the temporomandibular joint, which connects the mandible to the skull. The disorder and resultant dysfunction can result in significant pain and impairment...
(TMJ) - Adverse drug reactionAdverse drug reactionAn adverse drug reaction is an expression that describes harm associated with the use of given medications at a normal dosage. ADRs may occur following a single dose or prolonged administration of a drug or result from the combination of two or more drugs...
- MAO inhibitor discontinuation syndrome
- Williams syndromeWilliams syndromeWilliams syndrome is a rare neurodevelopmental disorder characterized by a distinctive, "elfin" facial appearance, along with a low nasal bridge; an unusually cheerful demeanor and ease with strangers; developmental delay coupled with strong language skills; and cardiovascular problems, such as...
- AutismAutismAutism is a disorder of neural development characterized by impaired social interaction and communication, and by restricted and repetitive behavior. These signs all begin before a child is three years old. Autism affects information processing in the brain by altering how nerve cells and their...
- Bell's palsyBell's palsyBell's palsy is a form of facial paralysis resulting from a dysfunction of the cranial nerve VII that results in the inability to control facial muscles on the affected side. Several conditions can cause facial paralysis, e.g., brain tumor, stroke, and Lyme disease. However, if no specific cause...
- Tay–Sachs disease
- Ménière's diseaseMénière's diseaseMénière's disease is a disorder of the inner ear that can affect hearing and balance to a varying degree. It is characterized by episodes of vertigo and tinnitus and progressive hearing loss, usually in one ear. It is named after the French physician Prosper Ménière, who, in an article published...
- Asperger syndromeAsperger syndromeAsperger's syndrome that is characterized by significant difficulties in social interaction, alongside restricted and repetitive patterns of behavior and interests. It differs from other autism spectrum disorders by its relative preservation of linguistic and cognitive development...
- Superior canal dehiscence syndromeSuperior canal dehiscence syndromeSuperior canal dehiscence syndrome is a rare medical condition of the inner ear, first described in 1998 by Dr. Lloyd B. Minor of Johns Hopkins University, Baltimore, USA, leading to hearing and balance disorders in those affected....
(SCDS) - Chronic ear infections
- Minor Head Injury
- A vestibular disorder: see below.
Symptoms
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 tinnitusTinnitus
Tinnitus |ringing]]") is the perception of sound within the human ear in the absence of corresponding external sound.Tinnitus is not a disease, but a symptom that can result from a wide range of underlying causes: abnormally loud sounds in the ear canal for even the briefest period , ear...
.
In vestibular hyperacusis
Vestibular hyperacusis
The word hyperacusis generally refers to cochlear hyperacusis, which is associated with tinnitus and an intolerance for normal, every day sounds, such as the refrigerator running. However, vestibular hyperacusis is equally disabling for the lives it affects, albeit in different ways. Dr...
, 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
Minimum audibility curve
Minimum audibility curve is a standardised graph of the threshold of hearing versus frequency for an average human, and is used as the reference level when measuring hearing loss with an audiometer as shown on an audiogram....
sounds in that frequency range at the volume in question, as well as on the person's preexisting muscle tone
Muscle tone
In physiology, medicine, and anatomy, muscle tone is the continuous and passive partial contraction of the muscles, or the muscle’s resistance to passive stretch during resting state. It helps maintain posture, and it declines during REM sleep.-Purpose:Unconscious nerve impulses maintain the...
and severity of startle response.
Anxiety
Anxiety
Anxiety is a psychological and physiological state characterized by somatic, emotional, cognitive, and behavioral components. The root meaning of the word anxiety is 'to vex or trouble'; in either presence or absence of psychological stress, anxiety can create feelings of fear, worry, uneasiness,...
, 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.
Treatment
The most common treatment for hyperacusis is retraining therapy which uses broadband noise. Tinnitus Retraining Therapy (TRT)Tinnitus retraining therapy
Tinnitus retraining therapy is a form of habituation therapy designed to help people who suffer from tinnitus . TRT uses counselling to explain to the patient how a combination of tinnitus retraining and sound enrichment can end their negative reaction to the tinnitus sound, and then reduce and...
, a treatment originally used to treat tinnitus, uses broadband noise to treat hyperacusis. Pink noise
Pink noise
Pink noise or 1/ƒ noise is a signal or process with a frequency spectrum such that the power spectral density is inversely proportional to the frequency. In pink noise, each octave carries an equal amount of noise power...
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.
People
- Stephin MerrittStephin MerrittStephin Merritt is an American singer-songwriter based in Los Angeles , best known as the principal singer and songwriter in the band The Magnetic Fields...
, of The Magnetic FieldsThe Magnetic FieldsThe Magnetic Fields is the principal creative outlet of singer-songwriter Stephin Merritt...
, suffers from this condition. - Dublin singer-songwriter Chris Singleton was diagnosed with hyperacusis.
- The late Belgian composer Henri PousseurHenri PousseurHenri Pousseur was a Belgian composer.-Biography:Pousseur studied at the Academies of Music in Liège and in Brussels from 1947 to 1953. He was closely associated with Pierre Froidebise and André Souris...
suffered from hyperacusis. - Jazz musician Melody GardotMelody GardotMelody Gardot is a Grammy-nominated American singer, writer and musician in Philadelphia, Pennsylvania, though she considers herself a "citizen of the world". She has been influenced by such blues and jazz artists as Judy Garland, Janis Joplin, Miles Davis, Duke Ellington, Stan Getz and George...
has hyperacusis as a result of head trauma. - Ludwig van BeethovenLudwig van BeethovenLudwig van Beethoven was a German composer and pianist. A crucial figure in the transition between the Classical and Romantic eras in Western art music, he remains one of the most famous and influential composers of all time.Born in Bonn, then the capital of the Electorate of Cologne and part of...
suffered from hyperacusis, alongside tinnitus and hearing loss.
Further reading
- "Decreased Sound Tolerance", by Pawel J. Jastreboff and Margaret J Jastreboff, in: "Tinnitus: theory and management", ed. James Byron Snow, 2004, ISBN 155009243X