A Phenotypic Comparison of Loudness & Pain Hyperacusis: Symptoms, Comorbidity, & Associated Features in a .. Patient Registry, 2021, Williams et al

[Andy]

Full title: A Phenotypic Comparison of Loudness and Pain Hyperacusis: Symptoms, Comorbidity, and Associated Features in a Multinational Patient Registry

Abstract

Purpose
Hyperacusis is a complex and poorly understood auditory disorder characterized by decreased tolerance to sound at levels that would not trouble most individuals. Recently, it has been suggested that individuals who experience otalgia in response to everyday sounds (termed pain hyperacusis) may differ clinically from those whose primary symptom is the perception of everyday sounds as excessively loud (termed loudness hyperacusis). Despite this theoretical distinction, there have been no empirical studies directly comparing these two populations of hyperacusis patients.


Method
Using data from a multinational patient registry (the Coordination of Rare Diseases at Sanford Registry), we examined self-reported demographics, symptoms, comorbidity, and response to treatment in a sample of 243 adults with hyperacusis, 152 of whom were classified as having pain hyperacusis based on reported symptoms. Bayesian statistical tests were used to investigate both the presence and absence of group differences between patients with loudness and pain hyperacusis.


Results
Individuals with pain hyperacusis presented with a more severe clinical phenotype, reporting a higher frequency of temporary symptom exacerbations (i.e., “setbacks”), less perceived symptom improvement over time, more severe comorbid headache disorders, and reduced benefit from sound therapy. However, the two hypothesized hyperacusis subtypes exhibited more similarities than differences, with the majority of symptoms and comorbidities being equally prevalent across groups. Multiple comorbidities were commonly observed, including tinnitus, primary headache disorders, psychiatric disorders, and functional somatic syndromes. Intolerance of sensory stimuli in other modalities was also frequently reported.


Conclusion
Although this study provides little evidence that loudness and pain hyperacusis are pathophysiologically distinct conditions, our findings indicate that a pain-predominant phenotype may be a meaningful prognostic marker in patients with hyperacusis.

Paywall, https://pubs.asha.org/doi/10.1044/2021_AJA-20-00209

 

[Hutan]

I was listening to an interview the other day with someone who was deaf and was given a cochlear implant. She found the sensation of sound overwhelming and, although she tried hard to get used to it, she couldn't. Her quality of life was greatly diminished, she got headaches, became irritable and had fatigue, and she had the device removed. What was particularly memorable was that she described the sensation of sound in terms of pain, like 'a knife through her brain'.

I don't really have sound sensitivity as part of my ME/CFS, not like many of you report. If in PEM, I experience noise as unwelcome but I wouldn't describe it is painful. When I heard this woman, I wondered if the mechanism for her feeling pain was similar to the sound sensitivity common in ME/CFS - perhaps a product of an overwhelmed processing system?

 

[Hutan]

When even soft noises feel like a knife to the eardrums
https://www.statnews.com/2016/02/18/noise-induced-ear-pain/

This article talks about pain receptors in the ear, and suggests that classifying hyperacusis as psychosomatic is out of date thinking:

Using new lab tools and techniques, pioneering scientists have identified what appear to be pain fibers in the inner ear, or cochlea. They are coining new terms, including “noxacusis” and “auditory nociception,” for this newly recognized sensation of noise-induced ear pain.

Few doctors or audiologists are even aware of the condition, though the new findings may encourage them to take patients who complain of ear pain more seriously. “It is time for pain hyperacusis to be recognized as a real symptom, not as a psychosomatic phenomenon,” García-Añoveros said. “This is, in a way, a new sensory modality — a hybrid of pain and hearing.”

It would be good to understand what is causing the sound sensitivity in ME/CFS - for someone to think beyond the vague 'HPA-axis, over-sensitive CNS' ideas.

 

[Sly Saint]

It's also the case for people who get migraines.

Why does sensitivity to sound happen with migraine?
Doctors do not know why a person becomes sensitive to sound during a migraine attack. It may be because migraine causes changes in how the brain processes light, sound, and smells. People with migraine may have lower thresholds for how stimuli affect them. Or, they process and combine stimuli differently.2

Imaging studies have shown unusual responses to stimuli throughout the brains of people living with migraine.2 One study of people with migraine with aura found more connections between the areas of the brain where light and sound stimuli are processed.3 This extra connectedness may help explain the increased sensitivity to sound, light and smells in people with migraine.

More research needs to be done to better understand what causes sound sensitivities in migraine.

https://migraine.com/migraine-symptoms/sensitivity-to-sound