Hyperacusis

By Marcello Cherchi, MD PhD

For patients

Hyperacusis broadly refers to the state in which one person experiences a sound as louder or more uncomfortable than how most other people would perceive that sound. Hyperacusis can be the result of range of ear problems and neurological problems. After taking a history and performing a physical examination, your clinician may suggest checking several tests of auditory function. Less commonly, imaging of the ear or brain may be appropriate. Management is often supervised by an audiologist experienced with hyperacusis, sometimes working in tandem with a psychologist or psychiatrist. In some circumstances an otolaryngologist may also become involved in care.

For clinicians

Overview

There is no universally agreed-upon set of criteria for hyperacusis, though most literature relies on definitions including terminology such as “increased sensitivity to sounds,” “intolerance to sounds that other individuals would not ordinarily find bothersome,” “disorder of loudness perception,” etc.  Part of the difficulty lies in the fact that hyperacusis involves an objective component (measurable amplitude of a sound stimulus), a subjective percept, and a behavioral response.  The epidemiology of hyperacusis has been poorly studied; literature reports prevalence among children of 3.2%, and prevalence among adults as ranging from 8.6% to 15.2%, with differences likely due to different definitions and study methodologies.  Etiologies of hyperacusis include peripheral (otologic), central (neurologic) and psychiatric.  Testing usually entails audiometry with “loudness discomfort levels (LDL)” or “uncomfortable loudness (UCL).”  Ideally management of hyperacusis should be overseen by an audiologist specializing in this topic.

Introduction

There is no universally agreed-upon set of criteria for hyperacusis, though most literature relies on definitions including terminology such as “increased sensitivity to sounds,” “intolerance to sounds that other individuals would not ordinarily find bothersome,” “disorder of loudness perception,” etc.

After conducting a thorough history, at the clinician’s discretion a workup for hyperacusis may include:

Physical examination:

• Assess for facial paralysis (as can be found in Bell’s palsy, Ramsay-Hunt syndrome, Lyme disease).

Audio-vestibular workup:

• Audiometry with loudness discomfort levels. This can also assess for conductive hyperacusis (which can be found with semicircular canal dehiscence), low frequency loss (often associated with Ménière’s disease), high frequency hearing loss (often associated with labyrinthitis) or a “noise notch” (often associated with noise exposure).
• Otoacoustic emissions.
• Possibly brainstem auditory evoked responses.
• Cervical and/or ocular vestibular evoked myogenic potentials with thresholds (to assess for semicircular canal dehiscence)

Imaging:

• Depending on the circumstances (e.g., status post head trauma; suspicion for multiple sclerosis; audio-vestibular workup suggestive of semicircular canal dehiscence), imaging with a temporal bone CT or MRI of the brain and internal auditory canals without and with contrast may be appropriate.

Management may involve referral to:

• Audiologist experienced with hyperacusis.
• Otolaryngologist, for example if there is suspicion for perilymphatic fistula.
• Psychologist/psychiatrist if there is evidence for depression or post-traumatic stress disorder.

Definitions of hyperacusis:

Frequently cited definitions of hyperacusis include:

“Increased sensitivity to sound in levels that would not trouble a normal individual. This auditory sensitivity is characterized by a painful or troublesome sensation when the patient is exposed to sound” (Katzenell and Segal 2001).

“Unusual tolerance to ordinary environmental sounds and… consistently exaggerated or inappropriate responses to sounds that are neither threatening nor uncomfortably loud to a typical person” (Baguley 2003).

“Disorder of loudness perception, in which sound intensities that are considered comfortable by most people are perceived unbearably loud” (Knipper, Van Dijk et al. 2013).

However, it turns out that there is no universally accepted definition of hyperacusis. Tyler and colleagues (Tyler, Pienkowski et al. 2014) review a wide variety of definitions that have been proposed for hyperacusis based on different aspects of the phenomenon, which might broadly be grouped into perception (awareness), sensitivity, discomfort, tolerance, response, emotional valence and auditory function:

• Perception
  • “Heightened awareness of sounds”
• Sensitivity
  • “Pathological auditory hypersensitivity”
  • “Increased auditory sensitivity”
  • “Noise sensitivity”
  • “Audio-sensitivity”
  • “Soft sound sensitivity”
  • “Select sound sensitivity”
• Discomfort
  • “Discomfort for sounds that would be acceptable to most normally hearing people”
• Tolerance
  • “Sound intolerance”
  • “Loudness tolerance problem”
  • “Abnormally low tolerance for sound levels”
• Abnormal response
  • “Abnormally strong response to moderate sound”
  • “Hyperresponsiveness to sound stimuli”
  • “Hyperresponsiveness to noise”
  • “Aversive response”
• Emotional valence (Tyler, Pienkowski et al. 2014)
  • “Annoyance”
  • “Irritability”
  • “Fear”
• Auditory function
  • “Disturbed loudness function”

The variety of definitions in turn raises the question of whether each represents a different perspective on one and the same underlying problem, or whether there are actually distinct pathologies.

Loudness

At a very basic level, hyperacusis involves sounds being experienced/perceived/responded to as “too loud.” But what is loudness?

“Loudness is determined mainly by physical sound intensity (i.e., pressure), although it is also affected by other stimulus attributes such as duration, frequency, band-width, and monaural versus binaural presentation, as well as psychological factors such as mood and emotional exhaustion. Considerable evidence… suggests that the relationship between sound intensity and loudness is not static in time, but can be modified by sound deprivation or stimulation. Deprivation increases sound sensitivity, whereas stimulation decreases it. The neurological bases of those loudness shifts remain largely unknown. A hypothetical central gain process, which regulates supra-threshold sensitivity somewhere in the auditory system, is one of the most commonly proposed mechanisms” (Fournier, Schonwiesner et al. 2014).

In other words, amplitude of sound waves (and thus the energy they contain) is the main, though not the only determinant of loudness.

Epidemiology

There are few studies of the epidemiology of hyperacusis, and their results are difficult to compare because they employed different definitions of hyperacusis and different data collection methodologies, as reviewed by Tyler and colleagues (Tyler, Pienkowski et al. 2014).

Andersson and colleagues (Andersson, Lindvall et al. 2002) analyzed the results of a survey of 1147 people (median age 47 years, SD 16.9 years) conducted in Sweden by mail and internet and reported the prevalence of hyperacusis to be 8.6%.

Fabijanska and colleagues (Fabijanska, Rogowski et al. 1999) analyzed the results of a questionnaire survey from 10,349 adult respondents in Poland and reported the prevalence of hyperacusis to be 15.2% overall (12.5% of women and 17.6% of men).

Coelho and colleagues (Coelho, Sanchez et al. 2007) conducted a questionnaire survey of 506 children aged 5 – 12 years and reported the prevalence of hyperacusis to be 3.2%.

Etiologies

The differential diagnosis of hyperacusis includes (Katzenell and Segal 2001, Baguley 2003, Tyler, Pienkowski et al. 2014):

• Peripheral etiologies:
  • Acute acoustic trauma (Axelsson and Hamernik 1987).
  • Noise-induced hearing loss. Hyperacusis can occur in individuals with chronic exposure to industrial sounds (Niemeyer 1971, Tzaneva 1996).
  • Loudness recruitment. “Converging physiological evidence indicates that intensifying central responsiveness to an existing sound causes loudness recruitment in subjects with hyperacusis” (Knipper, Van Dijk et al. 2013).
  • Ménière’s disease (Brandy William and Lynn James 1995, Gordon 2000, Vattoth, Shah et al. 2010).
  • Bell’s palsy (mediated by failure of the stapedial reflex from damage of the facial nerve). Hyperacusis is reported to occur in 29% of patients with Bell’s palsy (Adour and Wingerd 1974).
  • Ramsay-Hunt syndrome. Up to 46% of cases of Ramsay-Hunt syndrome are reported to be associated with hyperacusis (Bartnik, Fabijanska et al. 2001).
  • Stapedectomy (Katzenell and Segal 2001).
  • Perilymphatic fistula (Fukaya and Nomura 1988).
  • Semicircular canal dehiscence (Tyler, Pienkowski et al. 2014). One series reported that 85% (17 out of 20 patients) with superior semicircular canal dehiscence had hyperacusis (Yuen, Eikelboom et al. 2009).
• Central etiologies:
  • Migraine (Woodhouse and Drummond 1993, Vingen, Pareja et al. 1998).
  • Williams syndrome. Up to 95% of patients with Williams syndrome are reported to suffer from hyperacusis (Nigam and Samuel 1994).
  • Learning disabilities (Gordon 1986).
  • Dysfunction of auditory efferent system and its effects on auditory gain (Baguley 2003).
  • Multiple sclerosis (Cohen, Rudge et al. 1988, Weber, Pfadenhauer et al. 2002).
  • Fibromyalgia (Geisser, Glass et al. 2008, Staud, Godfrey et al. 2021).
• Potentially a combination of central and peripheral etiologies:
  • Head trauma (Waddell and Gronwall 1984). Although this is usually categorized under “central etiologies” of hyperacusis, head injury can easily cause labyrinthine concussion with cochlear damage, and thus include a peripheral component of hyperacusis.
  • Lyme disease. In some cases this may be due to the disease’s predilection for causing facial nerve paralysis and thus interference with the stapedial reflex (Peltomaa, Pyykko et al. 2002), but some cases of hyperacusis in Lyme disease appear to occur independently of facial paralysis (Nields, Fallon et al. 1999).
• Hormonal etiologies:
  • Addison’s disease (Henkin and Daly 1968).
• Psychiatric etiologies:
  • Depression (Carman 1973).
  • Post-traumatic stress disorder (Westcott 2002).

Loudness recruitment

Loudness recruitment describes, “an experience commonly associated with cochlear hearing loss and specifically with dysfunction of the outer hair cells of the organ of Corti: with a rising sound level, the perceived loudness increases faster than normal” (Baguley 2003).

Patients who have sustained cochlear damage, “conceivably possess the ability of recruitment because weak sounds cannot stimulate the few remaining hair cells and only a minimum number of impulses will be conducted through the acoustic nerve. Strong stimulation, on the other hand, is able to stimulate the remaining intact hair cells (presumably inner hair cells) and the impulses are then conducted through the normal fibers of the acoustic nerve and its connections to the cerebral cortex, which then becomes relatively well saturated” (Alexander 1959).

Testing

Contemporary audiologic assessment of hyperacusis often involves characterization of “loudness discomfort levels” (LDL) or “uncomfortable loudness” (UCL), defined as “the lowest sound level judged by the listener to be uncomfortably loud” (Tyler, Pienkowski et al. 2014).

Imaging

Few imaging studies have been conducted to investigate presumptive centrally-mediated hyperacusis (Tyler, Pienkowski et al. 2014).

Management

When initially experienced, hyperacusis can be uncomfortable and frightening, so a natural strategy is one of avoidance, or at least diminution of input. While this may appear sensible and even appropriate in some circumstances, there is some evidence that it may be maladaptive in others.

“For many patients, the first reaction to hyperacusis is to protect themselves with ear plugs, muffs or other devices. There is, however, reason to believe that such strategies to decrease the intensity of sound entering the auditory system may further increase the central gain, exacerbating rather than improving the hyperacusis” (Baguley 2003).

After other treatable causes have been excluded, consultation with an audiologist specializing in hyperacusis is medically reasonable.

References

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