Hyperacusis

What is Hyperacusis?

Hyperacusis is an uncommon condition which makes regular sounds intolerable and often perceived as painful, scary, unwelcome, or too loud. Patients frequently experience hyperacusis along with tinnitus, a ringing or buzzing in the ears, which can lead to significant distress. This distress often affects a person’s ability to participate in social, work, and leisure activities. Because of this, it’s common for people with this condition to avoid places or situations with certain noises, and seek medical help.

Two conditions that can be associated with sound intolerance are phonophobia, which can be described as a fear of sound, usually linked with migraines, and misophonia, which is a dislike of certain sounds. Both conditions might have an emotional aspect.

As of now, there isn’t a definitive cure for hyperacusis, but efforts are being made to understand and treat this condition more effectively. In fact, in the last forty years, the amount of research studies dedicated to understanding this condition has increased tenfold.

What Causes Hyperacusis?

Hyperacusis, a condition that makes normal sounds seem incredibly loud, can be triggered by various factors, though a direct cause is often hard to determine. The most frequent cause of hyperacusis is exposure to high levels of noise. People often think that hyperacusis and hearing loss are closely connected; it’s true that factors like exposure to constant loud noise at work or during leisure activities can lead to both conditions. In fact, studies have found that professional musicians, especially those playing pop/rock music, are more prone to hyperacusis due to their prolonged exposure to amplified sound.

However, just because someone experiences hearing loss doesn’t necessarily mean they’ll also have hyperacusis. While the two conditions can occur together, hearing loss doesn’t directly cause hyperacusis.

In addition, hyperacusis is often seen in people with other health problems. These can range from mental health conditions to physical ailments like fibromyalgia and chronic fatigue syndrome, or problems with joints and back. For example, over half of the patients who sought specialized care for hyperacusis also met the criteria for at least one mental health condition, with almost half experiencing an anxiety disorder. It’s also rather common for people with hyperacusis to have tinnitus, a condition marked by constant ringing in the ears. Other conditions, such as migraines, post-traumatic stress disorder, Lyme disease, and Williams syndrome, a rare genetic disorder, can also be connected to hyperacusis.

Risk Factors and Frequency for Hyperacusis

Hyperacusis, or increased sensitivity to certain levels and frequencies of sound, is a condition that affects a range of age groups. In children and teens, it is estimated to affect about 3.2% to 17.1% of individuals. The wide range in estimates is due to differences in the individuals’ ages and hearing capabilities. As for adults, current research shows that anywhere from 8% to 15.2% may have hyperacusis.

Some studies suggest that hyperacusis may be more common in older people and in women who have a higher level of education. However, getting a clear picture of how many people actually have this condition is quite difficult. This is because the symptoms of hyperacusis can be highly personal and subjective. Because of this, doctors and researchers rely heavily on a set of specific screening questions.

Just like research on tinnitus, or ringing in the ears, studies on hyperacusis often have notable differences in terms of design and methods used. This makes comparing the results of different studies a challenge.

• The prevalence of hyperacusis in children and adolescents is between 3.2% and 17.1%, but varies based on age and hearing status.
• In adults, the prevalence rates are between 8% and 15.2%.
• Hyperacusis may be more common in older individuals and in women with higher educational attainment.
• Identifying the prevalence of hyperacusis is a complex process due to its subjective presentation and the reliance on screening questions.
• The inconsistencies in design and methodology among studies make it difficult to compare research results on hyperacusis.

Signs and Symptoms of Hyperacusis

Hyperacusis is a condition where sounds become unusually sensitive or loud to the point of discomfort. This can be categorized into four types:

• Loudness hyperacusis: ordinary sounds are perceived as uncomfortably loud
• Annoyance hyperacusis: sounds cause anxiety and irritability
• Fear hyperacusis: certain sounds trigger avoidance behaviors
• Pain hyperacusis: certain sounds cause physical pain, typically in the ear

Hyperacusis can affect both ears. If it appears on one side, it could be due to other causes, like an acoustic shock.

Hyperacusis is often confused with two other conditions: phonophobia and misophonia. However, these are different. Hyperacusis is related to sound sensitivity in the auditory system. Any quiet sound can trigger it. On the other hand, phonophobia and misophonia are connected to the limbic system. Phonophobia is fear associated with a specific sound, while misophonia is a psychological reaction to specific sounds leading to emotional and physical responses.

Conducting a complete neurotological examination is crucial to identify any causes associated with hyperacusis, some of which are reversible. Checking the mobility of the eardrum and function of the facial nerves, as well as taking a detailed patient’s history can highlight potential risk factors, like noise exposure and acoustic shock.

Hyperacusis is different from Tinnitus. While hyperacusis usually affects both ears, is rarely intermittent, and is more pronounced and noticeable, requiring more investigation for self-help, tinnitus is often one-sided, intermittent, and linked to somatic modulation with unclear or basic perception and can be alleviated by self-help.

Testing for Hyperacusis

Diagnosing hyperacusis, a condition linked to heightened sensitivity to normal sound levels, usually involves a specific hearing test and filling out a questionnaire.

In the hearing test, known as pure-tone audiometry, a measure called Loudness Discomfort Level (LDL) is determined. Average people without hyperacusis usually find sounds unbearable when they hit about 100 decibels (the volume of a motorcycle). But for those with hyperacusis, this point, called LDL, is usually 77 decibels or even lower.

Also, patients typically answer questions about how much their condition affects their daily routine. There are various questionnaires used for this like the Geräuschüberempfindlichkeit (GÜF) and the Multiple Activity Scale for Hyperacusis (MASH), but the most common is the Hyperacusis Questionnaire (HQ).

There isn’t a widely agreed score that would immediately indicate a diagnosis of hyperacusis. But if a patient’s LDL and HQ scores indicate sensitivity, that gives a pretty reliable sign. For context, 95% of people with hyperacusis have an LDL of 77 decibels or lower and an HQ score of 22 or more.

Unfortunately, these scores only give information about whether hyperacusis exists or not and don’t tell much about how it affects a person’s life. It isn’t also easy finding a balance in evaluating the patient without making them uncomfortable and risking the doctor-patient relationship.

After the initial tests and questionnaires, further tests might be needed if the doctor suspects another cause. For instance, Lyme disease could be a culprit, and your doctor may want to run a blood test for that. They may also order high-resolution scans of the brain if the hyperacusis comes with something unusual like facial paralysis.

For patients with hyperacusis who also have psychological issues, doctors may use a variety of other questionnaires to understand the extent of these problems. These could include the Generalized Anxiety Disorder 7 item scale (GAD-7), Short Health Anxiety Inventory (SHAI), Mini-Social Phobia Inventory (Mini-SPIN), Obsessive-Compulsive Inventory-Revised (OCI-R), Penn State Worry Questionnaire-Abbreviated (PSWQ-A), and Patient Health Questionnaire (PHQ-9).

Treatment Options for Hyperacusis

Treatment for hyperacusis, which is increased sensitivity to everyday sounds, has two main goals. The first is to address the physical symptoms, and the second is to help reduce the emotional impact of the condition.

Cognitive-behavioral therapy (CBT) is often used alongside counseling and patient education. CBT is a type of therapy that helps you manage your problems by changing how you think and behave. It has been found to be very effective in helping manage the emotional reaction to sound. It can increase your ability to tolerate loud sounds (known as LDL) and reduce the severity of hyperacusis.

Directive counseling is another approach that helps identify and address certain behaviors. It’s often used as a treatment for conditions like tinnitus, which is a ringing or buzzing in your ears.

Tinnitus retraining therapy (TRT) is another method that is becoming increasingly popular. TRT educates you about your condition and gradually exposes you to sound to help desensitize your reaction to it. This kind of therapy can help reverse the increased sensitivity to sound, which is thought to be the main cause of hyperacusis. The use of sound generator therapy for six months has been shown to improve your tolerance to loud sounds. Another possible treatment approach is to increase your overall level of sound exposure – basically, creating a noisy environment – which has been shown to help manage symptoms.

Surgery might be necessary in some cases. This is particularly true if other treatments haven’t worked or if the hyperacusis is connected to another condition, such as superior semi-circular canal dehiscence syndrome. This syndrome is a small hole in the bone that separates the middle and inner ear. In such situations, a simple, reversible surgery called round and oval window reinforcement can be performed. This procedure has been shown to be highly successful and can sustainably reduce symptoms.

Alternative treatments are also often considered, especially for chronic pain. These might include supplements and vitamins, acupuncture, exercise, yoga, meditation, massage, relaxation therapy, and hypnosis.

What else can Hyperacusis be?

When a person is being examined for a loss of sound tolerance, doctors usually contemplate two main conditions: misophonia (hatred of sound) and phonophobia (fear of or aversion to certain sounds).

However, there are still other health issues that can cause these symptoms and need to be ruled out:

• Bell’s Palsy – a condition that causes a temporary weakness or paralysis of the muscles in the face
• Ramsay-Hunt syndrome – a rare form of shingles that can cause facial paralysis and hearing loss
• Migraines – severe headaches that can cause sensitivity to sounds
• Lyme disease – an infectious disease caused by ticks
• Neurosyphilis – an infection of the brain or spinal cord caused by syphilis
• Williams syndrome – a genetic disorder that affects many parts of the body
• Post-traumatic stress disorder (PTSD) – a mental health condition triggered by a traumatic event
• Depression – a serious mood disorder that affects how you feel, think, and handle daily activities
• Superior Canal Dehiscence Syndrome – a balance disorder that can cause dizziness, hearing loss, and sound sensitivity
• Autism – a developmental disorder that impairs the ability to communicate and interact
• Cri-du-Chat syndrome – a rare genetic condition present at birth that results in a variety of physical and mental issues
• Tay–Sach disease – a very rare, inherited neurodegenerative disease
• Temporomandibular disorders – disorders related to the jaw joint
• Fibromyalgia – a disorder characterized by widespread musculoskeletal pain

The doctor will conduct tests to exclude these possibilities and pinpoint the exact cause of the patient’s sound intolerance.

What to expect with Hyperacusis

The outlook for a person’s condition depends largely on the cause. People who struggle with hyperacusis, which is a heightened sensitivity to normal levels of sound, can experience different levels of distress. Often, these individuals may be more affected than those with tinnitus, a condition characterized by hearing a constant ringing or noise when none is present.

According to discussions in patient forums, hyperacusis is a long-term condition for some. However, it’s crucial to remember that those who have recovered might not continue sharing their experiences in these online communities. Hyperacusis can have severe psychological effects. Around 13% of patients have expressed thoughts of self-harm or suicide, emphasizing the need for doctors to check for signs of anxiety and depression in these individuals.

Furthermore, they noted that emerging adults (those between 18 and 29 years old) are especially vulnerable. They are more likely to develop hyperacusis due to their tendency to engage in risk-taking behaviors. On top of that, they may also face these psychological challenges during what is already a particularly stressful stage of life.

Possible Complications When Diagnosed with Hyperacusis

In certain cases, if the reason for the illness is identified, the complications might differ based on that cause.

Hyperacusis, which refers to heightened sensitivity to everyday sounds, doesn’t lead to further physical problems on its own. However, it can carry a significant psychological toll, which can seriously impact a person’s quality of life.

Also, it’s worth noting that some patients may express thoughts of suicide or self-harm.

Considerations:

• Complications vary based on the cause of the condition
• Hyperacusis doesn’t cause physical complications but can affect mental health significantly
• Some patients might express thoughts of self-harm or suicide

Preventing Hyperacusis

Learning about hyperacusis, a condition characterized by increased sensitivity to normal environmental sounds, can play a big role in treatment. This education can happen one-on-one, or it might be part of Cognitive Behavioral Therapy (CBT) – a type of therapy that helps you manage problems by changing the way you think and behave, or Tinnitus Retraining Therapy (TRT) – a treatment that helps to reduce the perceived intensity of tinnitus, a ringing in the ears.

During these sessions, patients will learn about their condition, understand their test results, get information about how the body functions, learn strategies to cope with their condition, and relaxation techniques. Group education sessions can also be beneficial. In these sessions, patients get to meet and share experiences with others going through similar struggles.

Educational approaches have been found to be as effective as CBT and counseling in treating hyperacusis. In fact, in the United Kingdom, nearly all services for tinnitus, a common symptom of hyperacusis, include education as an important part of the treatment plan.