Association between Hyperacusis and Tinnitus

Cederroth, Christopher R.; Lugo, Alessandra; Edvall, Niklas K.; Lazar, Andra; Lopez-Escamez, José A.; Bulla, Jan; Uhlén, Inger; Hoare, Derek J.; Baguley, David; Canlon, Barbara; Gallus, Silvano

doi:10.3390/jcm9082412

Cited by 61 publications

(66 citation statements)

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“…The clinical information of patients with MD and tinnitus phenotypes is detailed in (Supplementary Tables 1 and 2). A second independent tinnitus cohort of 97 individuals from Sweden was selected as a replication cohort: the Tinnitus Swedish Tinnitus Outreach Project (STOP) [17] which originates from the LifeGene study [18] . A subgroup of 34 individuals with severe tinnitus was also selected according to the THI ≥ 56 (Supplementary Table 3).…”

Section: Methodsmentioning

confidence: 99%

Burden of rare variants in synaptic genes in patients with severe tinnitus: An exome based extreme phenotype study

et al. 2021

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Background tinnitus is a heterogeneous condition associated with audiological and/or mental disorders. Chronic, severe tinnitus is reported in 1% of the population and it shows a relevant heritability, according to twins, adoptees and familial aggregation studies. The genetic contribution to severe tinnitus is unknown since large genomic studies include individuals with self-reported tinnitus and large heterogeneity in the phenotype. The aim of this study was to identify genes for severe tinnitus in patients with extreme phenotype. Methods for this extreme phenotype study, we used three different cohorts with European ancestry (Spanish with Meniere disease (MD), Swedes tinnitus and European generalized epilepsy). In addition, four independent control datasets were also used for comparisons. Whole-exome sequencing was performed for the MD and epilepsy cohorts and whole-genome sequencing was carried out in Swedes with tinnitus. Findings we found an enrichment of rare missense variants in 24 synaptic genes in a Spanish cohort, the most significant being PRUNE2, AKAP9, SORBS1, ITGAX, ANK2, KIF20B and TSC2 ( p < 2E −04 ), when they were compared with reference datasets. This burden was replicated for ANK2 gene in a Swedish cohort with 97 tinnitus individuals, and in a subset of 34 Swedish patients with severe tinnitus for ANK2, AKAP9 and TSC2 genes ( p < 2E −02 ). However, these associations were not significant in a third cohort of 701 generalized epilepsy individuals without tinnitus. Gene ontology (GO) and gene-set enrichment analyses revealed several pathways and biological processes involved in severe tinnitus, including membrane trafficking and cytoskeletal protein binding in neurons. Interpretation a burden of rare variants in ANK2, AKAP9 and TSC2 is associated with severe tinnitus . ANK2, encodes a cytoskeleton scaffolding protein that coordinates the assembly of several proteins, drives axonal branching and influences connectivity in neurons.

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Section: Methodsmentioning

confidence: 99%

Burden of rare variants in synaptic genes in patients with severe tinnitus: An exome based extreme phenotype study

et al. 2021

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“…Some studies focus on patients with cognitive impairment (n = 7), such as patients with WS (7,15,25,26), autism (9,22), and Asperger's syndrome (AS) (36). In addition, some studies (n = 12) are specific to patients with other comorbidities [tinnitus (2,20,32,33,35,40,46), hearing disorders (HD) (10, 44), Bell 's palsy (24), attention deficit hyperactivity disorder (ADHD) (45) and complex regional pain syndrome related dystonia (CRPS related dystonia) ( 28 2). Additionally, all use the word hyperacusis.…”

Section: Study Characteristicsmentioning

confidence: 99%

“…But only 2 presented the same discomfort loudness (80 dB HL) (9,22). Different definitions were also used to diagnose hyperacusis (5,21,24,26,29,33,35,36,(38)(39)(40)(41)(43)(44)(45)(46)(47)(48). Coelho (27) used both the hyperacusis definition and the discomfort loudness to diagnose.…”

Section: Diagnosis and Evaluationmentioning

confidence: 99%

“…As for methods to evaluate hearing status, there are many types and combinations. Except for 2 studies that do not provide listening evaluation methods (6, 7), the rest of the studies describe their own listening assessment methods, with 10 studies using different questionnaires (18,19,23,26,34,38,39,46) or surveys (10,36), while 21 studies (2, 9, 15, 17, 20-22, 24, 27, 28, 31-33, 35, 37, 40, 42-45, 48) carrying out by a method of audiology, such as pure tone audiometry (17,21), hearing tests (27,32,42), auditory brainstem response examination (9), and other methods. Also, some studies used both (4,5,16,25,41,47).…”

Section: Diagnosis and Evaluationmentioning

confidence: 99%

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Prevalence of Hyperacusis in the General and Special Populations: A Scoping Review

Ren

Xiang

et al. 2021

Front. Neurol.

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Objectives: To study the prevalence of hyperacusis in the general population and the special population, and to determine the effect of population differences on hyperacusis.Methods: The two authors followed a scoping review methodology and screened nearly 30 years of English literature in Pubmed, Web of Science, OVID, and EBSCO. Then, the extracted results of each study were discussed in groups and subgroups.Results: The authors selected 42 pieces of scientific literature that met the requirements, studying a total of 34,796 subjects, including the general population (28,425 subjects), the special occupation population (2,746 subjects), and the patients with concomitant diseases (5,093 subjects). The prevalence was 0.2–17.2% in the general population, 3.8–67% in the special occupation population, and 4.7–95% in the patients with special diseases. It was found that in the general population, the high prevalence occurs in adolescents and older adults. The prevalence of hyperacusis in women is significantly higher than in men. In people with hearing disorders, the prevalence of hyperacusis is significantly higher than in people with normal hearing. Various diseases (such as Williams syndrome, tinnitus, and autism), as well as various occupations (musicians, music students, teachers, and others), have been found to be high risk factors for hyperacusis.Conclusion: The high prevalence of hyperacusis and the large differences between reported prevalence in different studies deserves our great attention. Additionally, in order to increase the comparability of the studies, a standardized set of criteria are needed to study the prevalence of hyperacusis.

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“…The authors concluded that the presence of hyperacusis may intensify the severity of perceived tinnitus. Cederroth et al [ 34 ] also showed that hyperacusis is strongly associated with tinnitus, and the relationship increased with tinnitus severity. We have provided evidence that subjects with weak/mild, strong, or very strong tinnitus differ in terms of how they perceive loud sounds—the more severe the tinnitus, the more severe is the hyperacusis.…”

Section: Discussionmentioning

confidence: 99%

Decreased Sound Tolerance in Tinnitus Patients

Raj-Koziak

Gos

Kutyba

et al. 2021

Life

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(1) Background: Decreased sound tolerance is a significant problem in tinnitus sufferers. The aim of the study was to evaluate the relationship between tinnitus and decreased sound tolerance (hyperacusis and misophonia). (2) Methods: The study sample consisted of 74 patients with tinnitus and decreased sound tolerance. The procedure comprised patient interviews, pure tone audiometry, impedance audiometry, measurement of uncomfortable loudness levels, and administration of the Hyperacusis Questionnaire, Tinnitus Handicap Inventory, and Visual Analogue Scales. (3) Results: The majority (69%) of the patients reported that noise aggravated their tinnitus. The correlation between tinnitus and hyperacusis was found to be statistically significant and positive: r = 0.44; p < 0.01. The higher the tinnitus severity, the greater the hyperacusis. There was no correlation between misophonia and hyperacusis (r = 0.18; p > 0.05), or between misophonia and tinnitus (r = 0.06; p > 0.05). (4) Conclusions: For tinnitus patients the more significant problem was hyperacusis rather than misophonia. The diagnosis and treatment of decreased sound tolerance should take into account not only audiological, but also psychological problems of the patients.

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Association between Hyperacusis and Tinnitus

Cited by 61 publications

References 60 publications

Burden of rare variants in synaptic genes in patients with severe tinnitus: An exome based extreme phenotype study

Burden of rare variants in synaptic genes in patients with severe tinnitus: An exome based extreme phenotype study

Prevalence of Hyperacusis in the General and Special Populations: A Scoping Review

Decreased Sound Tolerance in Tinnitus Patients

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