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Hyperacusis: Understanding & Managing Auditory Hypersensitivity

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Hyperacusis is a hearing sensitivity that affects one’s quality of life. Your ears become extremely hypersensitive; even faint sounds can become highly audible and unbearable. Almost 80% of individuals with Hyperacusis also have accompanying tinnitus, where a person hears a constant ringing in the ears.1Cederroth, C.R., Lugo, A., Edvall, N.K., Lazar, A., Lopez-Escamez, J.A., Bulla, J., Uhlen, I., Hoare, D.J., Baguley, D.M., Canlon, B. and Gallus, S., 2020. Association between Hyperacusis and tinnitus. Journal of Clinical Medicine, 9(8), p.2412. Although Hyperacusis can develop through different mechanisms, loudness-induced hearing loss is the most common cause of Hyperacusis, where the inner ear’s cochlea, a shell-like structure, becomes damaged from noise trauma.
It is important to distinguish between Hyperacusis and ‘recruitment.’ Recruitment is an altered sound tolerance due to sensorineural hearing loss, where hair cells and nerves are damaged. Hence, the individuals with recruitment fluctuate between too-low and too-loud sound perceptions.
The first signs may be subtle, and people can confuse it with age. However, the healthcare provider will begin with a full medical assessment and questionaries. This is followed by more definitive tests like the Loudness Discomfort Level (LDL) test. It can be temporary or chronic; hence, early treatment intervention is required for a better prognosis.

What is Hyperacusis?

An alternating term for Hyperacusis is noise sensitivity. Even faint noises like the running of the water tap, the starting of a car engine, scrunching paper, the whistling of the wind, or even one’s voice can become too loud. Therefore, it can often be really challenging as the individuals feel irritable and anxious and sometimes can even be in physical pain.2Smit, A.L., Stegeman, I., Eikelboom, R.H., Baguley, D.M., Bennett, R.J., Tegg‐Quinn, S., Bucks, R.S., Stokroos, R.J., Hunter, M. and Atlas, M.D., 2021. Prevalence of Hyperacusis and its relation to health: the Busselton healthy aging study. The Laryngoscope, 131(12), pp.E2887-E2896. Hence, it can take a toll on one’s mental health and even result in self-isolation due to a fear of loud noises called phonophobia.
It targets all age groups, and almost 15% of adults suffer from Hyperacusis at a single point.3Smit, A.L., Stegeman, I., Eikelboom, R.H., Baguley, D.M., Bennett, R.J., Tegg‐Quinn, S., Bucks, R.S., Stokroos, R.J., Hunter, M. and Atlas, M.D., 2021. Prevalence of Hyperacusis and its relation to health: the Busselton healthy aging study. The Laryngoscope, 131(12), pp.E2887-E2896.

Causes of Hyperacusis

The exact cause of Hyperacusis is not fully understood and may involve various factors. Some common ones include the following:

Loud music & Leisure Sound:

A review done in 2021,4Pienkowski, M., 2021. Loud music and leisure noise is a common causes of chronic hearing loss, tinnitus, and Hyperacusis. International Journal of Environmental Research and public health, 18(8), p.4236. found that only loud noises from work, loud music in clubs, discos, and concerts, and leisure sounds like stadium matches, fireworks, and lawnmowers can lead to permanent hearing loss and Hyperacusis, especially in the young population.
It is important to highlight the growing risk of hearing loss in young adults due to risky listening habits, as the World Health Organisation has listed 1.1 billion young adults as high risk.5World Health Organization, 2015. Hearing loss due to recreational exposure to loud sounds: a review.

Biochemical Causes:

Hormonal changes play a big role in the development of Hyperacusis. Stress causes hormonal imbalance, various studies6GÖÇMENLER, H., 2021. Etiology of Hyperacusis: A Short Review of the Literature. Academic Researches in, p.592. reveal that patients with chronic stress, fatigue, and anxiety have an increased risk of this disease. If we look at the pathophysiology, stress damages the hair cells in the inner ear, which amplifies sound through increased neural sensitivity.

Sensitivity:

Anxiety, depression, and post-traumatic stress disorders are commonly accompanied by Hyperacusis. According to Research, this risk is linked with serotonin dysfunction (serotonin is a neurochemical responsible for happiness) as it causes dysregulation of central gain of the auditory system.7Jüris, L., Ekselius, L., Andersson, G. and Larsen, H.C., 2013. The Hyperacusis Questionnaire, loudness discomfort levels, and the Hospital Anxiety and Depression Scale: A cross-sectional study. Hearing, Balance and Communication, 11(2), pp.72-79.

Ototoxic Drugs:

Certain medications are damaging to the ears and can cause permanent hearing loss. Salicylate is highly ototoxic (oto means ear) and is commonly found in a very common medicine called aspirin.8Salvi, R., Radziwon, K., Manohar, S., Auerbach, B., Ding, D., Liu, X., Lau, C., Chen, Y.C. and Chen, G.D., 2021. Neural mechanisms of tinnitus and Hyperacusis in acute drug-induced ototoxicity. American Journal of Audiology, 30(3S), pp.901-915. One study found that salicylate caused temporary hearing loss by reducing the otoacoustic hearing signals. Since the signals were weak, they underwent progressive amplification when reaching the auditory cortex. Salicylate also increases corticosteroid, a stress hormone that is linked with it.

Trauma to the Head:

Direct injury involving the head can damage the neuronal pathway involved in hearing and perception, leading to Hyperacusis. Falling from a height, head-on collisions, or any blunt force can damage the brain structures involved in receiving and transmitting auditory signals. Sometimes, surgeries like mandibular reconstruction can also cause nerve damage, leading to noise sensitivity.

Hyperacusis & Autism

Hyperacusis is a common presentation with autism spectrum disorder (ASD). A pilot study9Danesh, A.A., Howery, S., Aazh, H., Kaf, W. and Eshraghi, A.A., 2021. Hyperacusis in autism spectrum disorders. Audiology Research, 11(4), pp.547-556. found a prevalence rate of 40% between Hyperacusis and autism. Patients with ASD mostly have hyper-serotonin conditions. Serotonin is a chemical that directly influences the area where nerve impulses are generated. Hence, it ends up acting as a regulator and influences neuronal sensitivity directly. Whenever serotonin is high, the sensitivity of the auditory neurons increases, and even the faint sounds are amplified. Since individuals with ASD have high levels of serotonin, their risk of Hyperacusis is significantly greater than non-ASD individuals.10Sato, K., 2022. What is the mechanism of loudness hyperacusis in autism? Medical Hypotheses, 159, p.110759.

Image of a kid sensitive to loud noise on TV, covering the ears in fear

Another mechanism for hyperacusis development in ASD is the delayed response of the stapedial acoustic reflex, a defense mechanism for reducing loud noises. Autism and Hyperacusis also share a genetic link since autistic individuals are positive for Contactin genes CNTN5 and CNTN6 that cause outgrowths in the neural hearing pathway.11Mercati, O., Huguet, G., Danckaert, A., André-Leroux, G., Maruani, A., Bellinzoni, M., Rolland, T., Gouder, L., Mathieu, A., Buratti, J. and Amsellem, F., 2017. CNTN6 mutations are risk factors for abnormal auditory sensory perception in autism spectrum disorders. Molecular psychiatry, 22(4), pp.625-633.

Hyperacusis and ADHD

When it comes to Hyperacusis, autism is the most studied condition, with a ton of literature. However, a recent study in 2020 found a link between hyperacusis and attention deficit hyperactivity disorder (ADHD).12Ralli, M., Greco, A., Altissimi, G., Natalia, T., Laura, C., Turchetta, R., Fusconi, M., Polimeni, A., Cianfrone, G. and de Vincentiis, M., 2018. Hyperacusis in children: a preliminary study on the effects of hypersensitivity to sound on speech and language. The International Tinnitus Journal, 22(1), pp.10-18. The study showed that almost 36.7% of children with ADHD suffered from it. Hence, apart from autism, ADHD is also linked with Hyperacusis.

Some rarer causes include:

  • Bell’s palsy
  • Down syndrome
  • Lyme’s disease
  • Ramsay Hunt syndrome
  • Temporomandibular joint syndrome

Symptoms of Hyperacusis

Symptoms of Hyperacusis exhibit themselves on a spectrum.
During early or for more milder cases, the symptoms include:

  • Ear discomfort
  • Heightened perception of soft sounds
  • Headaches
  • Problems with concentration

During severe Hyperacusis, symptoms are more constant and can include:

  • Constant popping sounds in ears
  • Physical pain when hearing sounds
  • Fear of public places
  • Anxiety
  • Fatigue
  • Phonophobia

However, it is essential to note that Hyperacusis is entirely different from phonophobia. It is a lower tolerance for loud sounds due to a physical disability. At the same time, phonophobia (phobia of sounds) is an emotionally driven response to loud sounds, but Hyperacusis can lead to phonophobia.
Rarely individuals can also experience seizures and suicide ideation.13Aazh, H. and Moore, B.C., 2018. Thoughts about suicide and self-harm in patients with tinnitus and Hyperacusis. Journal of the American Academy of Audiology, 29(03), pp.255-261.

Misophonia vs. Hyperacusis

Hyperacusis and misophonia can often be confused together. However, they share different characteristics. Misphonia is more of an emotional response to specific sounds, not always loud. The responses can range from anger, aggression, violence, and agitation to anxiety.14Schwartz, P., Leyendecker, J. and Conlon, M., 2011. Hyperacusis and misophonia: the lesser-known siblings of tinnitus. Minnesota Medicine, 94(11), pp.42-43. Simple sounds from other people, like eating, chewing, gasping, or even breathing, can trigger these individuals.
Hyperacusis is more of a physiological problem, while misophonia is psychological. It is important to distinguish between the two since the treatment options are different.
Once again, it is important to note that Hyperacusis, just like phonophobia, can lead to misophonia, as long-term Hyperacusis would make you easily irritable when hearing even the slightest of sounds.

Diagnosis of Hyperacusis

It can be hard for a general physician to pick up Hyperacusis. Hence, for a proper diagnosis, a referral to an ear, nose, and throat (ENT) specialist is a must. The specialist usually follows the following steps.

Medical History:

In any diagnostic scenario, the first step begins with history. A detailed explanation of the complaint is dotted, all accompanying symptoms are noted, and a list of possible diagnoses is compiled. Information about risk factors can help direct the doctor toward the right diagnosis.

Use of Questionnaires:

Questionnaires like the Hyperacusis Questionnaire (HQ), Psychometric Evaluation of the Hyperacusis Impact Questionnaire (HIQ), and Sound Sensitivity Symptoms Questionnaire (SSSQ) can play an important role in assessing the severity of the condition.15Aazh, H., Hayes, C., Moore, B.C., Danesh, A.A. and Vitoratou, S., 2022. Psychometric evaluation of the Hyperacusis Impact Questionnaire (HIQ) and Sound Sensitivity Symptoms Questionnaire (SSSQ) using a clinical population of adult patients with tinnitus alone or combined with Hyperacusis. Journal of the American Academy of Audiology, 33(05), pp.248-258. The ENT specialists can use this as a quick and effective baseline method to evaluate the degree of damage. However, their role is still questionable as a full-time diagnostic test. Questionnaires can help guide the doctor toward a more definitive one.

Physical Examination:

The specialist may check the inside of the ear using a camera instrument called an otoscope. Sometimes, the specialist might check the inside of the eardrum and evaluate your cranial nerves.

Hearing Tests:

The gold standard for the evaluation of hearing is pure tone audiometry. However, audiometry uses sound frequencies from 0.25 to 8 kHz (kilo-Hertz), but Hyperacusis manifests at loud noises, usually above 8 kHz. Hence, a more specific test for Hyperacusis is the loudness discomfort level (LDL) test.16Jahn, K.N., 2022. Clinical and investigational tools for monitoring noise-induced Hyperacusis. The Journal of the Acoustical Society of America, 152(1), pp.553-566. LDL uses headphones to deliver loud sounds to the patients. The patient then reports the sound as too loud the moment it becomes uncomfortable. This sound is labeled as LDL, and till now, LDL has been the most common test for diagnosing this disease.

Image of a medic performing hearing test on a patient
Pure tone audiometry

A New Psychoacoustic Test:

A trial in 2020,17Enzler, F., Fournier, P. and Norena, A.J., 2021. A psychoacoustic test for diagnosing Hyperacusis based on ratings of natural sounds. Hearing Research, 400, p.108124. came up with another psychoacoustic method for diagnosing Hyperacusis. The trial used four different audio and natural sounds as a stimulus (60,70,80, and 90 dB SPL). Afterward, the patients used a visual scale to rate their experience (from pleasant to unpleasant). Finally, the comparison of results with control leads to the development of a core discriminant sounds (CDS) scoring system. Although this method is in the beginning stages, the promising results of this trial can make it the official gold standard.

Treatment 

Most of the practiced treatments have more to do with mental therapy and relaxation unless your condition is so severe that you require surgery. Some treatment options are as follows:

Hyperacusis Sound Therapy:

Sound therapy is technical and requires the skills of a specialist. It has a standard 2-3 hour running time and is often held in a controlled environment or an acoustic room. Since Hyperacusis makes the patient avoidant of loud sounds, the first step is the introduction of light sounds back into the patient’s life. Mostly, the therapist starts with white noises like ‘shh’ or rushing sounds. The sounds are delivered either through a small hearing device or a sound generator. The hearing device is like a hearing aid delivering white sounds while the person works. Sometimes, a bedside sound generator is used instead. Sound therapy is time-consuming, long, and often requires a strong will. Hence, active counseling alongside hyperacusis sound therapy improves motivation and adherence.

Meditation &Yoga:

This is a lifestyle change advised by doctors to patients of Hyperacusis to help them deal with all the mental stress and strain of having it. While this does not directly treat your Hyperacusis, it does help deal with it.

Tinnitus Retraining Therapy (TRT):

In TRT, a device like a hearing aid is used to reduce hearing sensitivity by producing a soft, low-intensity sound constantly. While TRT is a treatment for tinnitus, as seen by its name, due to its decreasing hearing sensitivity, it also helps patients with Hyperacusis.

Cognitive Behavioral Therapy (CBT):

Stress, depression, and anxiety are common causes of Hyperacusis; patients with stress-induced Hyperacusis can often benefit greatly from a psychotherapy called CBT. It helps teach relaxation techniques to help manage stress and sometimes the accompanying misophonia in advanced Hyperacusis. The psychologist helps you desensitize from the sounds and helps restructure thoughts, especially negative emotions relating to loud sounds. CBT, coupled with sound therapy, works as an effective treatment plan for Hyperacusis.

Surgery:

If your Hyperacusis is severe and the above fails to work, your doctor would advise you to get a round and oval reinforcement surgery.18Silverstein, H., Wu, Y.H.E. and Hagan, S., 2015. Round and oval window reinforcement for the treatment of Hyperacusis. American Journal of Otolaryngology, 36(2), pp.158-162. Round and oval reinforcement surgery is a minimally invasive procedure in which support is provided to the bones to reduce hearing sensitivity by removing the tissue behind your ear and moving it around the window. For this, temporal fascia is used as the tissue and is placed trans-canal around the round window membrane and stapes footplate.

Things you can do at home to manage Hyperacusis

Using relaxation strategies like breathing exercises and maintaining regular sleep hygiene can help manage your stress levels. When exposed to loud noises like welding, lawn mowing, factories, or scaffolding, always use protective hearing equipment.
Living with Hyperacusis can be quite challenging, but there are a lot of support groups available. These help you provide a platform where you can share your experience with other hyperacusis patients. It is not a mental illness but rather a physical malfunction in the neural hearing pathways. Through awareness and early management, you can prevent this condition from becoming permanent.

Prevention

Although Hyperacusis has a lot of underlying causes, targeting the most common one can help protect your ears. So, limit using earphones, earplugs, or anything of the sort more than necessary, as they can cause serious damage to the ears.19Marshall, L., Lapsley Miller, J.A., Heller, L.M., Wolgemuth, K.S., Hughes, L.M., Smith, S.D. and Kopke, R.D., 2009. Detecting incipient inner-ear damage from impulse noise with otoacoustic emissions. The Journal of the Acoustical Society of America, 125(2), pp.995-1013. Also, avoiding places with frequent loud noises, such as construction zones, concerts, and industrial areas, can help avoid noise-induced hearing loss.

Conclusion

Hyperacusis is a medical condition where you become hypersensitive to noise, such that even the slightest of sounds can be piercing to you. It has a multitude of causes, while loud noise exposure is the most common; autism, stress, and even age play their role in damaging the hearing pathway. At the same time, there is no permanent cure for Hyperacusis other than surgery. There are several therapy options, such as sound therapy, TRT, and cognitive behavior therapy, which can help deal with Hyperacusis and make a patient’s life easier. Using protective equipment when exposed to loud noises and avoiding noise exposure can help protect your ears against damage leading to Hyperacusis.

Refrences
  • 1
    Cederroth, C.R., Lugo, A., Edvall, N.K., Lazar, A., Lopez-Escamez, J.A., Bulla, J., Uhlen, I., Hoare, D.J., Baguley, D.M., Canlon, B. and Gallus, S., 2020. Association between Hyperacusis and tinnitus. Journal of Clinical Medicine, 9(8), p.2412.
  • 2
    Smit, A.L., Stegeman, I., Eikelboom, R.H., Baguley, D.M., Bennett, R.J., Tegg‐Quinn, S., Bucks, R.S., Stokroos, R.J., Hunter, M. and Atlas, M.D., 2021. Prevalence of Hyperacusis and its relation to health: the Busselton healthy aging study. The Laryngoscope, 131(12), pp.E2887-E2896.
  • 3
    Smit, A.L., Stegeman, I., Eikelboom, R.H., Baguley, D.M., Bennett, R.J., Tegg‐Quinn, S., Bucks, R.S., Stokroos, R.J., Hunter, M. and Atlas, M.D., 2021. Prevalence of Hyperacusis and its relation to health: the Busselton healthy aging study. The Laryngoscope, 131(12), pp.E2887-E2896.
  • 4
    Pienkowski, M., 2021. Loud music and leisure noise is a common causes of chronic hearing loss, tinnitus, and Hyperacusis. International Journal of Environmental Research and public health, 18(8), p.4236.
  • 5
    World Health Organization, 2015. Hearing loss due to recreational exposure to loud sounds: a review.
  • 6
    GÖÇMENLER, H., 2021. Etiology of Hyperacusis: A Short Review of the Literature. Academic Researches in, p.592.
  • 7
    Jüris, L., Ekselius, L., Andersson, G. and Larsen, H.C., 2013. The Hyperacusis Questionnaire, loudness discomfort levels, and the Hospital Anxiety and Depression Scale: A cross-sectional study. Hearing, Balance and Communication, 11(2), pp.72-79.
  • 8
    Salvi, R., Radziwon, K., Manohar, S., Auerbach, B., Ding, D., Liu, X., Lau, C., Chen, Y.C. and Chen, G.D., 2021. Neural mechanisms of tinnitus and Hyperacusis in acute drug-induced ototoxicity. American Journal of Audiology, 30(3S), pp.901-915.
  • 9
    Danesh, A.A., Howery, S., Aazh, H., Kaf, W. and Eshraghi, A.A., 2021. Hyperacusis in autism spectrum disorders. Audiology Research, 11(4), pp.547-556.
  • 10
    Sato, K., 2022. What is the mechanism of loudness hyperacusis in autism? Medical Hypotheses, 159, p.110759.
  • 11
    Mercati, O., Huguet, G., Danckaert, A., André-Leroux, G., Maruani, A., Bellinzoni, M., Rolland, T., Gouder, L., Mathieu, A., Buratti, J. and Amsellem, F., 2017. CNTN6 mutations are risk factors for abnormal auditory sensory perception in autism spectrum disorders. Molecular psychiatry, 22(4), pp.625-633.
  • 12
    Ralli, M., Greco, A., Altissimi, G., Natalia, T., Laura, C., Turchetta, R., Fusconi, M., Polimeni, A., Cianfrone, G. and de Vincentiis, M., 2018. Hyperacusis in children: a preliminary study on the effects of hypersensitivity to sound on speech and language. The International Tinnitus Journal, 22(1), pp.10-18.
  • 13
    Aazh, H. and Moore, B.C., 2018. Thoughts about suicide and self-harm in patients with tinnitus and Hyperacusis. Journal of the American Academy of Audiology, 29(03), pp.255-261.
  • 14
    Schwartz, P., Leyendecker, J. and Conlon, M., 2011. Hyperacusis and misophonia: the lesser-known siblings of tinnitus. Minnesota Medicine, 94(11), pp.42-43.
  • 15
    Aazh, H., Hayes, C., Moore, B.C., Danesh, A.A. and Vitoratou, S., 2022. Psychometric evaluation of the Hyperacusis Impact Questionnaire (HIQ) and Sound Sensitivity Symptoms Questionnaire (SSSQ) using a clinical population of adult patients with tinnitus alone or combined with Hyperacusis. Journal of the American Academy of Audiology, 33(05), pp.248-258.
  • 16
    Jahn, K.N., 2022. Clinical and investigational tools for monitoring noise-induced Hyperacusis. The Journal of the Acoustical Society of America, 152(1), pp.553-566.
  • 17
    Enzler, F., Fournier, P. and Norena, A.J., 2021. A psychoacoustic test for diagnosing Hyperacusis based on ratings of natural sounds. Hearing Research, 400, p.108124.
  • 18
    Silverstein, H., Wu, Y.H.E. and Hagan, S., 2015. Round and oval window reinforcement for the treatment of Hyperacusis. American Journal of Otolaryngology, 36(2), pp.158-162.
  • 19
    Marshall, L., Lapsley Miller, J.A., Heller, L.M., Wolgemuth, K.S., Hughes, L.M., Smith, S.D. and Kopke, R.D., 2009. Detecting incipient inner-ear damage from impulse noise with otoacoustic emissions. The Journal of the Acoustical Society of America, 125(2), pp.995-1013.
Dr Faryal Tariq
Dr Faryal Tariq
Dr Faryal Tariq is an MBBS doctor, with a Masters degree in Public health from Brunel University London. She currently works as an MD and also has a strong passion for writing. Her exceptional writing and clinical experience coupled with a strong academic background has helped her in removing the stigma around taboo medical topics, raising awareness and helping numerous patients worldwide. Dr Faryal has written countless articles, blogs, and papers along with a publication in PubMed. Outside the clinical realm, she enjoys cooking, nutrition, shopping and exploring the world.

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