hyperacusis

Hyperacusis is an increased sensitivity to sound (including pain) and a low tolerance for environmental noise. Definitions of hyperacusis can vary significantly; it often revolves around damage to or dysfunction of the stapes bone, stapedius muscle or tensor tympani. It is often categorized into four subtypes: loudness, pain (also called noxacusis), annoyance, and fear. It can be a highly debilitating hearing disorder. There are a variety of causes and risk factors, with the most common being exposure to loud noise. It is often coincident with tinnitus. Proposed mechanisms in the literature involve dysfunction in the brain, inner ear, or middle ear. Little is known about the prevalence of hyperacusis, in part due to the degree of variation in the term's definition. Reported prevalence estimates vary widely, and further research is needed to obtain strong epidemiological data.

Hyperacusis is an increased sensitivity to sound (including pain) and a low tolerance for environmental noise. Definitions of hyperacusis can vary significantly; it often revolves around damage to or dysfunction of the stapes bone, stapedius muscle or tensor tympani. It is often categorized into four subtypes: loudness, pain (also called noxacusis), annoyance, and fear. It can be a highly debilitating hearing disorder. There are a variety of causes and risk factors, with the most common being exposure to loud noise. It is often coincident with tinnitus. Proposed mechanisms in the literature involve dysfunction in the brain, inner ear, or middle ear. Little is known about the prevalence of hyperacusis, in part due to the degree of variation in the term's definition. Reported prevalence estimates vary widely, and further research is needed to obtain strong epidemiological data. ==Signs and symptoms== Hyperacusis symptoms can include an increased perception of the loudness of sounds (loudness hyperacusis), pain (noxacusis/pain hyperacusis/sound-induced otalgia), annoyance, and/or fear in response to sounds by which most people are unaffected. It may affect one or both ears. The majority of patients experience bilateral symptoms but often have one ear that is more affected than the other. Annoyance hyperacusis is often considered synonymous with misophonia. Fear hyperacusis is often considered synonymous with phonophobia. Many researchers more narrowly define hyperacusis to only include loudness hyperacusis and pain hyperacusis. Hyperacusis can also be accompanied by tinnitus. The latter is more common and there are important differences between their involved mechanisms. Hyperacusis can result in anxiety and stress. Avoidant behavior is often a response to prevent the effects of hyperacusis and this can include avoiding social situations. ==Causes and risk factors== The most common cause of hyperacusis is overexposure to excessively high decibel (sound pressure) levels, which can cause acoustic trauma. An acoustic shock, which can lead to symptoms such as hyperacusis and ear pain, can also occur after exposure to an unexpected moderately loud to loud noise, even if this does not necessarily result in permanent cochlear damage. Some affected people acquire hyperacusis suddenly as a result of taking ototoxic drugs (which can damage the cells responsible for hearing), Lyme disease, Ménière's disease, head injury, or surgery. Others are born with sound sensitivity or develop superior canal dehiscence syndrome. Bell's palsy can trigger hyperacusis if the associated flaccid paralysis affects the tensor tympani, and stapedius, two small muscles of the middle ear. Paralysis of the stapedius muscle prevents its function in dampening the oscillations of the ossicles, causing sound to be abnormally loud on the affected side. Age may also be a significant factor, with younger patients exhibiting more severe hyperacusis. Recently, it has been discovered that individuals with one copy of the GJB2 (Cx26) genetic mutation exhibit hearing that is more sensitive than average, akin to hyperacusis. These individuals appear to be at greater risk for damage from noise. Some psychoactive drugs such as LSD, methaqualone, benzodiazepines, or phencyclidine can cause hyperacusis. An antibiotic, ciprofloxacin, has also been seen to be a cause, known as ciprofloxacin-related hyperacusis. Benzodiazepine withdrawal syndrome is also a possible cause. ==Management and treatment== There are currently no evidence-based guidelines regarding the treatment of patients with hyperacusis. The majority of audiologists report insufficient formal education in this area, likely due in part to the current lack of consensus in the literature regarding definitions and treatment of hyperacusis. Dr. Kelly Jahn surveyed 32 patients with severe pain hyperacusis and found that 95% of them had experienced a lack of empathy or support from doctors (20% somewhat agree and 75% strongly agree, with a 5% non response rate). ==Stories of hyperacusis patients== The Hyperacusis Memorial Wall of Remembrance is dedicated to hyperacusis patients and their stories. Musician Jason DiEmilio, who recorded under the name Azusa Plane, had hyperacusis and ultimately went on to die by suicide due in part to his sensitivity to noise. Dietrich Hectors, a hyperacusis sufferer in Belgium, took his life in 2009, after years of struggling with catastrophic hyperacusis and tinnitus. A local government in Belgium made a short film based on his life to warn others of the dangers of hyperacusis and tinnitus. 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 affected ear during audience applause. Vladimir Lenin, the Russian communist revolutionary, politician, and political theorist, was reported seriously ill by the latter half of 1921, having symptoms possibly consistent with hyperacusis, such as regular headache and insomnia. Musician Peter Silberman of The Antlers had hyperacusis and tinnitus resulting from Cochlear hydrops which put his musical career on hold until the conditions reduced down to a "manageable level". Racing driver Wolfgang Reip developed severe hyperacusis after several noise traumas during his racing career. Ludwig van Beethoven possibly suffered from hyperacusis alongside his hearing loss. In a letter, he wrote: "As soon as anybody shouts, I can't bear it. Heaven alone knows what is to become of me." British writer Linda Stratmann suffers from hyperacusis and reports noises "cut through her like a scalpel." Japanese man Ohama Matsuzo suffered from hyperacusis, leading to him murdering three neighbours over the sound of a piano. Musician Laura Ballance, bassist of rock band Superchunk and co-founder of Merge Records, suffers from hyperacusis and has quit touring as a result.

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