Hyperacusis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; 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[1][2].

Classification

Based on the symptoms, hyperacusis may be classified as Cochlear and vestibular hyperacusis[3][4][5].

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.

2) Vestibular hyperacusis: The sufferer may experience dizziness, nausea, or a loss of balance when certain pitched sounds are present.

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.

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[6][7][4][1].

Causes

The Common causes of hyperacusis include[8][9][9][10][2]:

  • Loud noise experience.
  • Recreational and industrial noise acquaintance and noise-related hearing loss.
  • Expert musicians specifically those playing rock music and exposed to extended periods of amplified sound.
  • Other causes that can lead to hyperacusis include migraine, Lyme disease, psychiatric illness such as post-traumatic stress disorder, and Williams syndrome.
  • 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, taking ear sensitive drugs.

Other causes can be due to the following:

Differentiating hyperacusis from other diseases

On the basis of sign and symptoms hyperacusis must be differentiated from misphonia, phonophobia, tinnitus, william syndrome,lyme disease,migraine[11][3][12][13][14][15].

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
Phonophobia + - + +/- - - Limbic system involved Clinical diagnosis
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[14].

Hyperausis Diagnosis

Diagnostic study of choice

Pure tone audiometry is the gold standard test for the diagnosis of hyperacusis[19][20][21].

  • 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[22][23]:

Treatment

The most common treatment for hyperacusis is retraining therapy that includes[24][25][26][27][28][29][30]:

  • 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:

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

Template:Diseases of the ear and mastoid process he:היפראקוזיס nl:Hyperacusis fi:Hyperakusia Template:WikiDoc Sources

  1. ↑ 1.0 1.1 Vernon JA (1987). "Pathophysiology of tinnitus: a special case--hyperacusis and a proposed treatment". Am J Otol. 8 (3): 201–2. PMID 3631220.
  2. ↑ 2.0 2.1 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.
  3. ↑ 3.0 3.1 Baguley DM (2003). "Hyperacusis". J R Soc Med. 96 (12): 582–5. doi:10.1258/jrsm.96.12.582. PMC 539655. PMID 14645606.
  4. ↑ 4.0 4.1 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.
  5. ↑ Scharf B, Magnan J, Chays A (1997). "On the role of the olivocochlear bundle in hearing: 16 case studies". Hear Res. 103 (1–2): 101–22. doi:10.1016/s0378-5955(96)00168-2. PMID 9007578.
  6. ↑ Katzenell U, Segal S (2001). "Hyperacusis: review and clinical guidelines". Otol Neurotol. 22 (3): 321–6, discussion 326-7. doi:10.1097/00129492-200105000-00009. PMID 11347634.
  7. ↑ Sahley TL, Nodar RH (2001). "A biochemical model of peripheral tinnitus". Hear Res. 152 (1–2): 43–54. doi:10.1016/s0378-5955(00)00235-5. PMID 11223280.
  8. ↑ 8.0 8.1 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.
  9. ↑ 9.0 9.1 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.
  10. ↑ Halevi-Katz DN, Yaakobi E, Putter-Katz H (2015). "Exposure to music and noise-induced hearing loss (NIHL) among professional pop/rock/jazz musicians". Noise Health. 17 (76): 158–64. doi:10.4103/1463-1741.155848. PMC 4918652. PMID 25913555.
  11. ↑ Palumbo DB, Alsalman O, De Ridder D, Song JJ, Vanneste S (2018). "Misophonia and Potential Underlying Mechanisms: A Perspective". Front Psychol. 9: 953. doi:10.3389/fpsyg.2018.00953. PMC 6034066. PMID 30008683.
  12. ↑ Nields JA, Fallon BA, Jastreboff PJ (1999). "Carbamazepine in the treatment of Lyme disease-induced hyperacusis". J Neuropsychiatry Clin Neurosci. 11 (1): 97–9. doi:10.1176/jnp.11.1.97. PMID 9990563.
  13. ↑ Levitin DJ, Menon V, Schmitt JE, Eliez S, White CD, Glover GH; et al. (2003). "Neural correlates of auditory perception in Williams syndrome: an fMRI study". Neuroimage. 18 (1): 74–82. doi:10.1006/nimg.2002.1297. PMID 12507445.
  14. ↑ 14.0 14.1 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.
  15. ↑ Schecklmann M, Lehner A, Schlee W, Vielsmeier V, Landgrebe M, Langguth B (2015). "Validation of Screening Questions for Hyperacusis in Chronic Tinnitus". Biomed Res Int. 2015: 191479. doi:10.1155/2015/191479. PMC 4628768. PMID 26557654.
  16. ↑ 16.0 16.1 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.
  17. ↑ Rosing SN, Schmidt JH, Wedderkopp N, Baguley DM (2016). "Prevalence of tinnitus and hyperacusis in children and adolescents: a systematic review". BMJ Open. 6 (6): e010596. doi:10.1136/bmjopen-2015-010596. PMC 4893873. PMID 27259524.
  18. ↑ Andersson G, Lindvall N, Hursti T, Carlbring P (2002). "Hypersensitivity to sound (hyperacusis): a prevalence study conducted via the Internet and post". Int J Audiol. 41 (8): 545–54. doi:10.3109/14992020209056075. PMID 12477175.
  19. ↑ Sheldrake J, Diehl PU, Schaette R (2015). "Audiometric characteristics of hyperacusis patients". Front Neurol. 6: 105. doi:10.3389/fneur.2015.00105. PMC 4432660. PMID 26029161.
  20. ↑ Anari M, Axelsson A, Eliasson A, Magnusson L (1999). "Hypersensitivity to sound--questionnaire data, audiometry and classification". Scand Audiol. 28 (4): 219–30. doi:10.1080/010503999424653. PMID 10572967.
  21. ↑ Aazh H, Knipper M, Danesh AA, Cavanna AE, Andersson L, Paulin J; et al. (2018). "Insights from the third international conference on hyperacusis: causes, evaluation, diagnosis, and treatment". Noise Health. 20 (95): 162–170. doi:10.4103/nah.NAH_2_18. PMC 6122267. PMID 30136676.
  22. ↑ Fackrell K, Fearnley C, Hoare DJ, Sereda M (2015). "Hyperacusis Questionnaire as a Tool for Measuring Hypersensitivity to Sound in a Tinnitus Research Population". Biomed Res Int. 2015: 290425. doi:10.1155/2015/290425. PMC 4628763. PMID 26557658.
  23. ↑ Aazh H, Moore BCJ (2017). "Factors related to uncomfortable loudness levels for patients seen in a tinnitus and hyperacusis clinic". Int J Audiol. 56 (10): 793–800. doi:10.1080/14992027.2017.1335888. PMID 28622055.
  24. ↑ Fackrell K, Potgieter I, Shekhawat GS, Baguley DM, Sereda M, Hoare DJ (2017). "Clinical Interventions for Hyperacusis in Adults: A Scoping Review to Assess the Current Position and Determine Priorities for Research". Biomed Res Int. 2017: 2723715. doi:10.1155/2017/2723715. PMC 5654244. PMID 29312994.
  25. ↑ Jüris L, Andersson G, Larsen HC, Ekselius L (2014). "Cognitive behaviour therapy for hyperacusis: a randomized controlled trial". Behav Res Ther. 54: 30–7. doi:10.1016/j.brat.2014.01.001. PMID 24508581.
  26. ↑ Sheppard A, Stocking C, Ralli M, Salvi R (2020). "A review of auditory gain, low-level noise and sound therapy for tinnitus and hyperacusis". Int J Audiol. 59 (1): 5–15. doi:10.1080/14992027.2019.1660812. PMID 31498009.
  27. ↑ Noreña AJ, Chery-Croze S (2007). "Enriched acoustic environment rescales auditory sensitivity". Neuroreport. 18 (12): 1251–5. doi:10.1097/WNR.0b013e3282202c35. PMID 17632277.
  28. ↑ Park JM, Kim WJ, Ha JB, Han JJ, Park SY, Park SN (2018). "Effect of sound generator on tinnitus and hyperacusis". Acta Otolaryngol. 138 (2): 135–139. doi:10.1080/00016489.2017.1386801. PMID 29043888.
  29. ↑ Silverstein H, Wu YH, Hagan S (2015). "Round and oval window reinforcement for the treatment of hyperacusis". Am J Otolaryngol. 36 (2): 158–62. doi:10.1016/j.amjoto.2014.10.014. PMID 25456168.
  30. ↑ Nikkar-Esfahani A, Whelan D, Banerjee A (2013). "Occlusion of the round window: a novel way to treat hyperacusis symptoms in superior semicircular canal dehiscence syndrome". J Laryngol Otol. 127 (7): 705–7. doi:10.1017/S0022215113001096. PMID 23732001.
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