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Fragile X (FX) syndrome is one of the leading inherited causes of autism spectrum disorder (ASD). A majority of FX and ASD patients exhibit sensory hypersensitivity, including auditory hypersensitivity or hyperacusis, a condition in which everyday sounds are perceived as much louder than normal. Auditory processing deficits in FX and ASD also afford the opportunity to develop objective and quantifiable outcome measures that are likely to translate between humans and animal models due to the well-conserved nature of the auditory system and well-developed behavioral read-outs of sound perception. Therefore, in this study we characterized auditory hypersensitivity in a Fmr1 knockout (KO) transgenic rat model of FX using an operant conditioning task to assess sound detection thresholds and suprathreshold auditory reaction time-intensity (RT-I) functions, a reliable psychoacoustic measure of loudness growth, at a variety of stimulus frequencies, bandwidths, and durations. Male Fmr1 KO and littermate WT rats both learned the task at the same rate and exhibited normal hearing thresholds. However, Fmr1 KO rats had faster auditory RTs over a broad range of intensities and steeper RT-I slopes than WT controls, perceptual evidence of excessive loudness growth in Fmr1 KO rats. Furthermore, we found that Fmr1 KO animals exhibited abnormal perceptual integration of sound duration and bandwidth, with diminished temporal but enhanced spectral integration of sound intensity. Because temporal and spectral integration of sound stimuli were altered in opposite directions in Fmr1 KO rats, this suggests that abnormal RTs in these animals are evidence of aberrant auditory processing rather than generalized hyperactivity or altered motor responses. Together, these results are indicative of fundamental changes to low-level auditory processing in Fmr1 KO animals. Finally, we demonstrated that antagonism of metabotropic glutamate receptor 5 (mGlu5) selectively and dose-dependently restored normal loudness growth in Fmr1 KO rats, suggesting a pharmacologic approach for alleviating sensory hypersensitivity associated with FX. This study leverages the tractable nature of the auditory system and the unique behavioral advantages of rats to provide important insights into the nature of a centrally important yet understudied aspect of FX and ASD.

Background: Autism spectrum conditions (ASC) and quantitative autistic traits (QATs) are associated with sensory symptoms, which may contribute to anxiety and adversely affect social and cognitive development. Although sensory symptoms can occur across all senses, the relative roles of specific sensory modalities as contributors to the autistic phenotype and to anxiety are not well understood. The objective of this study was to examine which sensory symptoms were most predictive of high anxiety., Methods: We recruited 257 female primary caregivers of children aged 6 to 11 years (49% girls) to a questionnaire study comprising parent-report measures for classical QATs (social, communicative, and rigid), autism-related sensorimotor symptoms (visual, auditory, tactile, olfactory, gustatory, vestibular, proprioceptive, and motor), and anxiety symptoms. First, Bayesian stochastic search variable selection (SSVS) was used to identify the most probable sensorimotor predictors of specific QATs as well as diagnosed ASC. Then, the selected predictors were used in another SSVS, using anxiety symptoms as a dependent variable, to identify which of the autism-relevant sensorimotor symptoms were most robustly predictive of anxiety. Finally, the effect sizes of anxiety-related sensory symptoms were estimated with linear regressions., Results: We found that auditory symptoms and motor difficulties were most predictive of ASC diagnosis. Developmental motor difficulties were also strongly related to all individual QATs, whereas auditory symptoms were more selectively predictive of rigid traits. Tactile symptoms robustly predicted social interaction QATs, and proprioceptive symptoms predicted communicative QATs. Anxiety outcomes were most strongly predicted by difficulties with auditory and olfactory processing., Conclusions: The results support the clinical importance of being alert to complaints about sounds and hearing in neurodevelopmental populations, and that auditory processing difficulties may be evaluated as an early marker of poor mental health in children with and without diagnosed autism. Olfactory processing differences appeared to be an anxiety marker less strongly associated with ASC or QATs, while motor difficulties were highly autism-relevant but not equally strongly associated with anxiety outcomes. We suggest that future studies may focus on the mechanisms and consequences of neurodevelopmental central auditory processing dysfunction and its potential relationship to anxiety disorders., (© 2024. The Author(s).)

Hyperacusis is a condition that is characterized by hypersensitivity to normal everyday sounds or reduced sound tolerance and can affect patients in distressing ways. Sound therapy is a treatment intervention that is used to desensitize patients. However, as yet, there is a lack of understanding on how it is used in clinical practice, the different types of devices, or how to use them. The aim of this scoping review was to establish the current use of sound therapy in adults with hyperacusis and identify any factors that may influence treatment. Methodology: An established methodological framework was used to formulate the research question and guide the search strategy and reporting. The inclusion criteria were studies reporting adult (>18 years) populations with hyperacusis and sound therapy treatments which were published in any language. Searches of electronic databases (CINAHL, Cochrane Library, Medline (EBSCO), Scopus, PsycINFO) identified 31 studies that met the inclusion criteria (completed in April 2024). Data from included records were collated and summarized descriptively.

Objective: Hyperacusis, characterized by an abnormal sensitivity to everyday sounds, is a condition associated with subjective distress and physiological changes in the auditory system. Prevalence rates have varied significantly worldwide, emphasizing the need for standardized criteria. This study aimed to determine the prevalence of hyperacusis among high school students in Mysore district and explore gender differences., Methods: A diverse cohort of 597 high school students participated in this cross-sectional study. The Modified Khalfa Hyperacusis Questionnaire assessed hyperacusis symptoms. Demographic data included age, gender, and educational status. A cut-off score of 28 on the questionnaire identified hyperacusis cases. Data were analyzed descriptively., Results: The study revealed a substantial hyperacusis prevalence of 17.25% among high school students in the Mysore district. Out of 103 identified cases, 43.69% were females, and 56.31% were males. These results suggest a potential gender disparity in hyperacusis prevalence. The study's findings highlight the high prevalence of hyperacusis among Indian high school students, emphasizing the need for attention to this issue in adolescent health. Gender differences observed in this study align with previous research, indicating a higher prevalence among males., Conclusions: Hyperacusis is a prevalent condition among Indian high school students, warranting further research on its causes and co-occurrence across different age groups. These findings underscore the significance of addressing hyperacusis as a critical health concern among adolescents and emphasize the need for tailored interventions and increased awareness., Competing Interests: Conflict of InterestThe authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper., (© Association of Otolaryngologists of India 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Recent evidence suggests a connection between hyperacusis and the motor system of the brain. For instance, our recent study reported that hyperacusis in participants with tinnitus and hearing loss is associated with smaller gray matter volumes in the supplementary motor area (SMA). Given that hearing loss can affect gray matter changes in tinnitus, this study aimed to determine if the changes reported in our previous findings of smaller SMA gray matter volumes in hyperacusis persist in the absence of hearing loss. Data for this study were gathered from four prior studies conducted between 2004 and 2019 at the University Medical Centre Groningen (UMCG). A total of 101 participants with tinnitus and either clinically normal hearing (normal hearing with tinnitus or NHT, n = 35) or bilateral sensorineural hearing loss (hearing loss with tinnitus or HLT, n = 66) were included across four studies. Hyperacusis was determined by a score of ≥22 on the Hyperacusis Questionnaire (HQ). In the NHT group, 22 (63%) participants scored ≥22 on the HQ (NHT with hyperacusis: mean age 44.1 years, 12 females), while in the HLT group, 25 (38%) participants scored ≥22 on the HQ (HLT with hyperacusis: mean age 59.5 years, 10 females). The 2 × 2 between-group ANOVAs revealed that hyperacusis is associated with smaller SMA gray matter volumes, regardless of hearing levels. Notably, the smaller SMA gray matter volumes in hyperacusis were primarily influenced by the attentional subscales of the HQ. The association between hyperacusis and the motor system may indicate a constant alertness to sounds and a readiness for motor action.

Purpose: Military-affiliated individuals (MIs) are at a higher risk of developing hearing loss and tinnitus. While these disorders are well-studied in MIs, their impact relative to non-military-affiliated individuals (non-MIs) remains understudied. Our study compared hearing, speech-in-noise (SIN) perception, and tinnitus characteristics between MIs and non-MIs., Method: MIs ( n = 84) and non-MIs ( n = 193) underwent hearing threshold assessment and Quick Speech-in-Noise Test. Participants with tinnitus completed psychoacoustic tinnitus matching, numeric rating scale (NRS) for loudness and annoyance, and Tinnitus Functional Index. Comorbid conditions such as anxiety, depression, and hyperacusis were assessed. We used a linear mixed-effects model to compare hearing thresholds and SIN scores between MIs and non-MIs. A multivariate analysis of variance compared tinnitus characteristics between MIs and non-MIs, and a stepwise regression was performed to identify predictors of tinnitus severity., Results: MIs exhibited better hearing sensitivity than non-MIs; however, their SIN scores were similar. MIs matched their tinnitus loudness to a lower intensity than non-MIs, but their loudness ratings (NRS) were comparable. MIs reported greater tinnitus annoyance and severity on the relaxation subscale, indicating increased difficulty engaging in restful activities. Tinnitus severity was influenced by hyperacusis and depression in both MIs and non-MIs; however, hearing loss uniquely contributed to severity in MIs., Conclusions: Our findings suggest that while MIs may exhibit better or comparable listening abilities, they were significantly more affected by tinnitus than non-MIs. Furthermore, our study highlights the importance of assessing tinnitus-related distress across multiple dimensions, facilitating customization of management strategies for both MIs and non-MIs.

Background: Despite substantial progress in investigating its psychophysical complexity, tinnitus remains a scientific and clinical enigma. The present study, through an ecological and multidisciplinary approach, aims to identify associations between electroencephalographic (EEG) and psycho-audiological variables., Methods: EEG beta activity, often related to stress and anxiety, was acquired from 12 tinnitus patients (TIN group) and 7 controls (CONT group) during an audio cognitive task and at rest. We also investigated psychological (SCL-90-R; STAI-Y; BFI-10) and audiological (THI; TQ12-I; Hyperacusis) variables using non-parametric statistics to assess differences and relationships between and within groups., Results: In the TIN group, frontal beta activity positively correlated with hyperacusis, parietal activity, and trait anxiety; the latter is also associated with depression in CONT. Significant differences in paranoid ideation and openness were found between groups., Conclusions: The connection between anxiety trait, beta activity in the fronto-parietal cortices and hyperacusis provides insights into brain functioning in tinnitus patients, offering quantitative descriptions for clinicians and new multidisciplinary treatment hypotheses.

Objective: Tonic tensor tympani syndrome is found in a subset of tinnitus patients who experience intra-aural and peri-aural symptoms, in addition to their tinnitus, in the absence of clinically detectable pathology. As the syndrome has not been widely reported, this study aims to determine its prevalence and evaluate the effectiveness of current management., Methods: The tinnitus management clinic records of patients over the past six years were assessed to identify tonic tensor tympani syndrome patients and track their progress based on patient-reported Tinnitus Handicap Index scores. Patients with reversible ear pathology and temporomandibular joint disorder were excluded., Results: It was found that 13 per cent of the tinnitus management patients fulfilled the criteria for tonic tensor tympani syndrome and 94 per cent of those who returned for follow up showed an improvement in their Tinnitus Handicap Index grades., Conclusion: This study suggests that tonic tensor tympani syndrome is a significant problem among tinnitus patients and current tinnitus management strategies contribute effectively to helping such patients habituate to their symptoms.

Objective: To validate the diagnoses and to investigate epidemiological data from an international, non‐clinic‐based, and large (n = 1604) survey of participants with cluster headache. Background: There are several limitations in current epidemiological data in cluster headache including a lack of large non‐clinic‐based studies. There is also limited information on several aspects of cluster headache, such as pediatric incidence. Methods: The International Cluster Headache Questionnaire was an internet‐based survey that included questions on cluster headache demographics, criteria from the International Classification of Headache Disorders (ICHD), and medications. Results: A total of 3251 subjects participated in the survey, and 1604 respondents met ICHD criteria for cluster headache. For validation, we interviewed a random sample of 5% (81/1604) of participants and confirmed the diagnosis of cluster headache in 97.5% (79/81). Pediatric onset was found in 27.5% (341/1583) of participants, and only 15.2% (52/341) of participants with pediatric onset were diagnosed before the age of 18. Men were more likely to have episodic cluster headache between ages 10 and 50, but the sex ratio was approximately equal for other ages. An overwhelming majority of respondents had at least one autonomic feature (99.0%, 1588/1604) and had restlessness (96.6%, 1550/1604), but many also had prototypical migrainous features including photophobia or phonophobia (50.1%, 804/1604), pain aggravated by physical activity (31.4%, 503/1604), or nausea and vomiting (27.5%, 441/1604). Interestingly, the first‐line medications for acute treatment (oxygen) and preventive treatment (calcium channel blockers) were perceived as significantly less effective in chronic cluster headache (3.2 ± 1.1 and 2.1 ± 1.0 respectively on a 5‐point ordinal scale) compared with episodic cluster headache (3.5 ± 1.0 and 2.4 ± 1.1, respectively, p < 0.001 for both comparisons). Conclusions: Cluster headache often occurs in the pediatric population, although they are typically not diagnosed until adulthood. The onset of cluster headache is the inverse of that in migraine; in migraine women are more likely to have migraine between ages 10 and 50 but the sex ratio is approximately equal otherwise. Prototypical migrainous features are not useful in differentiating cluster headache from migraine. Participant data from a large international study also suggest that chronic cluster headache is not only less responsive to newer treatments (like noninvasive vagus nerve stimulation and galcanezumab), but to traditional first‐line treatments as well. [ABSTRACT FROM AUTHOR]

Age-related hidden hearing loss is often described as a cochlear synaptopathy that results from a progressive degeneration of the inner hair cell (IHC) ribbon synapses. The functional changes occurring at these synapses during aging are not fully understood. Here, we characterized this aging process in IHCs of C57BL/6J mice, a strain which is known to carry a cadherin-23 mutation and experiences early hearing loss with age. These mice, while displaying a large increase in auditory brainstem thresholds due to 50% loss of IHC synaptic ribbons at middle age (postnatal day 365), paradoxically showed enhanced acoustic startle reflex suggesting a hyperacusis-like response. The auditory defect was associated with a large shrinkage of the IHCs' cell body and a drastic enlargement of their remaining presynaptic ribbons which were facing enlarged postsynaptic AMPAR clusters. Presynaptic Ca2+ microdomains and the capacity of IHCs to sustain high rates of exocytosis were largely increased, while on the contrary the expression of the fast-repolarizing BK channels, known to negatively control transmitter release, was decreased. This age-related synaptic plasticity in IHCs suggested a functional potentiation of synaptic transmission at the surviving synapses, a process that could partially compensate the decrease in synapse number and underlie hyperacusis. [ABSTRACT FROM AUTHOR]

Purpose: Tinnitus and hyperacusis are debilitating conditions often associated with age-, noise-, and drug-induced hearing loss. Because of their subjective nature, the neural mechanisms that give rise to tinnitus and hyperacusis are poorly understood. Over the past few decades, considerable progress has been made in deciphering the biological bases for these disorders using animal models. Method: Important advances in understanding the biological bases of tinnitus and hyperacusis have come from studies in which tinnitus and hyperacusis are consistently induced with a high dose of salicylate, the active ingredient in aspirin. Results: Salicylate induced a transient hearing loss characterized by a reduction in otoacoustic emissions, a moderate cochlear threshold shift, and a large reduction in the neural output of the cochlea. As the weak cochlear neural signals were relayed up the auditory pathway, they were progressively amplified so that the suprathreshold neural responses in the auditory cortex were much larger than normal. Excessive central gain (neural amplification), presumably resulting from diminished inhibition, is believed to contribute to hyperacusis and tinnitus. Salicylate also increased corticosterone stress hormone levels. Functional imaging studies indicated that salicylate increased spontaneous activity and enhanced functional connectivity between structures in the central auditory pathway and regions of the brain associated with arousal (reticular formation), emotion (amygdala), memory/spatial navigation (hippocampus), motor planning (cerebellum), and motor control (caudate/putamen). Conclusion: These results suggest that tinnitus and hyperacusis arise from aberrant neural signaling in a complex neural network that includes both auditory and nonauditory structures. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Acoustic shock can be a problem for people, such as call center operators, who use headsets to make or receive a large number of phone calls. Provided is a device which significantly reduces the likelihood of receiving an acoustic shock. Case 1: A 28-year-old women complaining of Tinnitus; Headache; Neck pain; Dizziness; Anxiety; Hyperacusis; Otalgia. Otoscopy and audiogram is normal. with anxiety and pain, symptomatic treatment is given with a 10-day rest because the patient is afraid of returning to work. Case 2: A 27-year-old women presenting with otalgia, hyperacusis, jaw pain, neck pain. Symptoms have lasted for three weeks An audiogram is requested which returns to normal various symptomatic treatments are prescribed without much improvement faced with the patient's stress, she was prescribed a rest period of 10 days, renewed once . [ABSTRACT FROM AUTHOR]

The article describes two cases of patients who had a prolonged history of headache which had characteristics of migraine but developed focal neurological deficit. Topics discussed include presumed cause of superficial siderosis, principal patterns of superficial siderosis, and diagnosis of idiopathic superficial siderosis in both patients.

Aminoglycosides (AG) antibiotics are a common treatment for recurrent infections in cystic fibrosis (CF) patients. AGs are highly ototoxic, resulting in a range of auditory dysfunctions. It was recently shown that the acoustic startle reflex (ASR) can assess behavioral evidence of hyperacusis and tinnitus in an amikacin cochleotoxicity mouse model. The goal of this study was to establish if tobramycin treatment led to similar changes in ASR behavior and to establish whether ebselen can prevent the development of these maladaptive neuroplastic symptoms. CBA/Ca mice were divided into three groups: Group 1 served as a control and did not receive tobramycin or ebselen, Group 2 received tobramycin (200 mg/kg/s.c.) and the vehicle (DMSO/saline/i.p.) daily for 14 continuous days, and Group 3 received the same dose/schedule of tobramycin as Group 2 and ebselen at (20 mg/kg/i.p.). Auditory brainstem response (ABR) and ASR hearing assessments were collected at baseline and 2, 6, 10, 14, and 18 weeks from the start of treatment. ASR tests included input/output (I/O) functions which assess general hearing and hyperacusis, and Gap-induced prepulse inhibition of the acoustic startle (GPIAS) to assess tinnitus. At 18 weeks, histologic analysis showed predominantly normal appearing hair cells and spiral ganglion neuron (SGN) synapses. Following 14 days of tobramycin injections, 16 kHz thresholds increased from baseline and fluctuated over the 18-week recovery period. I/O functions revealed exaggerated startle response magnitudes in 50% of mice over the same period. Gap detection deficits, representing behavioral evidence of tinnitus, were observed in a smaller subset (36%) of animals. Interestingly, increases in ABR wave III/wave I amplitude ratios were observed. These tobramycin data corroborate previous findings that AGs can result in hearing dysfunctions. We show that a 14-day course of tobramycin treatment can cause similar levels of hearing loss and tinnitus, when compared to a 14-day course of amikacin, but less hyperacusis. Evidence suggests that tinnitus and hyperacusis might be common side effects of AG antibiotics. [ABSTRACT FROM AUTHOR]

Age-related hidden hearing loss is often described as a cochlear synaptopathy that results from a progressive degeneration of the inner hair cell (IHC) ribbon synapses. The functional changes occurring at these synapses during aging are not fully understood. Here, we characterized this aging process in IHCs of C57BL/6J mice, a strain which is known to carry a cadherin-23 mutation and experiences early hearing loss with age. These mice, while displaying a large increase in auditory brainstem thresholds due to 50% loss of IHC synaptic ribbons at middle age (postnatal day 365), paradoxically showed enhanced acoustic startle reflex suggesting a hyperacusis-like response. The auditory defect was associated with a large shrinkage of the IHCs' cell body and a drastic enlargement of their remaining presynaptic ribbons which were facing enlarged postsynaptic AMPAR clusters. Presynaptic Ca2+ microdomains and the capacity of IHCs to sustain high rates of exocytosis were largely increased, while on the contrary the expression of the fast-repolarizing BK channels, known to negatively control transmitter release, was decreased. This age-related synaptic plasticity in IHCs suggested a functional potentiation of synaptic transmission at the surviving synapses, a process that could partially compensate the decrease in synapse number and underlie hyperacusis.

Background Misophonia is defined as significant distress (anger, distress or disgust) when exposed to certain sounds that would not affect most people, such as lip smacking or gum chewing. Although misophonia is common, the aetiology, prevalence and effective treatments are largely unknown. Aims Based on our proposed diagnostic criteria, we examined the prevalence of misophonia and its relationship with clinical and demographic variables in a large representative population sample. Method We used a household sample (N = 541) of all residents aged >15 years, living in 300 homes randomly selected in Ankara city centre, Turkey. All participants were assessed at their homes by trained interviewers, for sociodemographic variables, misophonic sounds and related factors, using a semi-structured interview (the Misophonia Interview Schedule) developed for the current research. Results The current misophonia diagnosis prevalence was 12.8% (n = 69 of 541), although 427 (78.9%) participants reported at least one sound that was distressing. The mean number of misophonic sounds was 8.6 (s.d. 8.9, range 0–44); the figure was 17.6 in those with misophonia compared with 7.3 in those without misophonia. Of those with misophonia, only 5.8% contacted services for their condition. Predictors of misophonia diagnosis included younger age, family history of misophonia and previous contact with mental health services. Conclusions Our study showed that misophonia is common in the general population, may cause significant disruption in daily life and is undertreated. Although more evidence is needed to classify misophonia as a psychiatric disorder, our findings support others who claim that the condition belongs to the group of mental disorders.

Aminoglycosides (AG) antibiotics are a common treatment for recurrent infections in cystic fibrosis (CF) patients. AGs are highly ototoxic, resulting in a range of auditory dysfunctions. It was recently shown that the acoustic startle reflex (ASR) can assess behavioral evidence of hyperacusis and tinnitus in an amikacin cochleotoxicity mouse model. The goal of this study was to establish if tobramycin treatment led to similar changes in ASR behavior and to establish whether ebselen can prevent the development of these maladaptive neuroplastic symptoms. CBA/Ca mice were divided into three groups: Group 1 served as a control and did not receive tobramycin or ebselen, Group 2 received tobramycin (200 mg/kg/s.c.) and the vehicle (DMSO/saline/i.p.) daily for 14 continuous days, and Group 3 received the same dose/schedule of tobramycin as Group 2 and ebselen at (20 mg/kg/i.p.). Auditory brainstem response (ABR) and ASR hearing assessments were collected at baseline and 2, 6, 10, 14, and 18 weeks from the start of treatment. ASR tests included input/output (I/O) functions which assess general hearing and hyperacusis, and Gap-induced prepulse inhibition of the acoustic startle (GPIAS) to assess tinnitus. At 18 weeks, histologic analysis showed predominantly normal appearing hair cells and spiral ganglion neuron (SGN) synapses. Following 14 days of tobramycin injections, 16 kHz thresholds increased from baseline and fluctuated over the 18-week recovery period. I/O functions revealed exaggerated startle response magnitudes in 50% of mice over the same period. Gap detection deficits, representing behavioral evidence of tinnitus, were observed in a smaller subset (36%) of animals. Interestingly, increases in ABR wave III/wave I amplitude ratios were observed. These tobramycin data corroborate previous findings that AGs can result in hearing dysfunctions. We show that a 14-day course of tobramycin treatment can cause similar levels of hearing loss and tinnitus, when compared to a 14-day course of amikacin, but less hyperacusis. Evidence suggests that tinnitus and hyperacusis might be common side effects of AG antibiotics.

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.

There is currently no singularly accepted definition of hyperacusis. The aim of this study was to determine a definition and description of hyperacusis by clinician consensus. A three-round Delphi survey involving hearing healthcare professionals built towards clinical consensus on a definition of hyperacusis. Round 1 involved three open-ended questions about hyperacusis. Seventy-nine statements were generated on descriptions, impact, sounds, and potential features of hyperacusis. Agreement on the relevance of each statement to defining or describing hyperacusis was then measured in Rounds 2 and 3. General consensus was defined a priori as ≥70% agreement, or ≥90 for clinical decision making. Forty-five hearing healthcare professionals were recruited to take part in this study. Forty-one completed Round 1, 36 completed Round 2, and 33 completed Round 3. Consensus was reached on 42/79 statements. From these a consensus definition includes "A reduced tolerance to sound(s) that are perceived as normal to the majority of the population or were perceived as normal to the person before their onset of hyperacusis". A consensus description of hyperacusis was also determined. This consensus definition of hyperacusis will help to determine the scope of clinical practice guidelines and influence needed research on hyperacusis. [ABSTRACT FROM AUTHOR]

This article details the rationale and creative process behind a collaborative – or more accurately in this case, dialogic – sound composition undertaken as part of research into the acoustic ecologies of people in the early stages of a dementia. Changes in abilities around hearing and listening are among the first symptoms of many types of dementia, making such auditory phenotypes an increasingly common part of lived experiences of sound. Following acoustic ecology practice in doing and presenting research in sound, and more specifically Steven Feld in doing so in dialogic or polyvocal ways, co-composition can be a way of exploring the particularities of others' hearing, listening and sound practices, which is less reliant on the discursive frames of interlocutors and researchers. The process of making sound art together draws attention to particular sounds and experiences, creating dialogic situations of companion listening, discussion and mutual learning. It also provides a framework for engaging interlocutors in soundscape and ethnographic fieldwork methods. The composition discussed here, Trevurr, documents my time working with Trevor, a keen amateur musician in Cornwall who has mild cognitive impairment, and gradually comes to simulate his experience of hyperacusis in a piece of dialogic, auraldiversity-oriented composition. [ABSTRACT FROM AUTHOR]

Objective: Sensory hypersensitivities such as photophobia, phonophobia, and osmophobia are common in patients with migraine. We investigated the burden of these multiple sensory hypersensitivities in migraine. Methods: In this cross-sectional study, 187 consecutive patients with migraine (26 men/161 women; age, 45.9 ± 13.2 years) were included. Sensory hypersensitivity symptoms such as photo−/phono−/osmophobia and accompanying symptoms were determined by neurologists in interviews. The Migraine Disability Assessment (MIDAS) was used to assess headache-related disability. The Kessler Psychological Distress Scale (K6) was also administered. Results: Photophobia, phonophobia and osmophobia were observed in 75.4%, 76.5% and 55.1% of the patients with migraine, respectively. A significant overlap in sensory hypersensitivities (photo−/phono−/osmophobia) was found; the proportions of patients with 2 and 3 coexisting sensory hypersensitivities were 33.2% and 41.7%, respectively. The MIDAS score was higher in those with 3 sensory hypersensitivity symptoms than in those with 0 to 2 sensory hypersensitivity symptoms. A generalized linear model with ordinal logistic regression analysis revealed that multiple sensory hypersensitivities, younger age, more migraine days per month, and a higher K6 score were significantly related to the higher MIDAS score. Conclusion: Our study showed that sensory hypersensitivities commonly occur and overlap in patients with migraine and that multiple sensory hypersensitivity symptoms have a significant impact on headache-related disability. [ABSTRACT FROM AUTHOR]

Background: Fremanezumab, a fully humanized monoclonal antibody (IgG2Δa) selectively targets the calcitonin gene-related peptide and has proven efficacy for the preventive treatment of migraine. In this study, we evaluated the long-term efficacy, safety, and tolerability of monthly and quarterly fremanezumab. Methods: Episodic migraine and chronic migraine patients completing the 12-week double-blind period of the FOCUS trial entered the 12-week open-label extension and received 3 monthly doses of fremanezumab (225 mg). Changes from baseline in monthly migraine days, monthly headache days of at least moderate severity, days of acute headache medication use, days with photophobia/phonophobia, days with nausea or vomiting, disability scores, and proportion of patients achieving a ≥50% or ≥75% reduction in monthly migraine days were evaluated. Results: Of the 807 patients who completed the 12-week double-blind treatment period and entered the open-label extension, 772 patients completed the study. In the placebo, quarterly fremanezumab, and monthly fremanezumab dosing regimens, respectively, patients had fewer average monthly migraine days (mean [standard deviation] change from baseline: − 4.7 [5.4]; − 5.1 [4.7]; − 5.5 [5.0]), monthly headache days of at least moderate severity (− 4.5 [5.0]; − 4.8 [4.5]; − 5.2 [4.9]), days per month of acute headache medication use (− 4.3 [5.2]; − 4.9 [4.6]; − 4.8 [4.9]), days with photophobia/phonophobia (− 3.1 [5.3]; − 3.4 [5.3]; − 4.0 [5.2]), and days with nausea or vomiting (− 2.3 [4.6]; − 3.1 [4.5]; − 3.0 [4.4]). During the 12-week open-label extension, 38%, 45%, and 46% of patients, respectively, achieved a ≥50% reduction and 16%, 15%, and 20%, respectively, achieved a ≥75% reduction in monthly migraine days. Disability scores were substantially improved in all 3 treatment groups. There were low rates of adverse events leading to discontinuation (<1%). Conclusion: Fremanezumab demonstrated sustained efficacy up to 6 months and was well tolerated in patients with episodic migraine or chronic migraine and documented inadequate response to multiple migraine preventive medication classes. Trial registration: ClinicalTrials.gov NCT03308968 (FOCUS). [ABSTRACT FROM AUTHOR]

Peduncular hallucinosis is a rare syndrome that develops with upper brainstem and diencephalon lesions. Patients complain of vivid hallucinations that usually occur in hypnagogic states but maintain critical appraisal. Pathological disruption of the reticular activating systems partially explains this clinical phenomenon. We report the case of a 47-year-old man presenting with peduncular hallucinosis associated with central auditory disturbance consisting of bilateral hypoacusis and left-sided paradoxical hyperacusis, secondary to a pontine haemorrhage. [ABSTRACT FROM AUTHOR]

There are currently no validated hyperacusis questionnaires available in Norway. This study evaluates a new (Norwegian) translation of Khalfa's Hyperacusis Questionnaire. Another aim was to examine levels of psychological distress in Norwegian hyperacusis patients. Seventy-six hyperacusis patients between 21 and 68 years of age completed an online survey on two occasions comprising the Norwegian Hyperacusis Questionnaire (HQ) and Hospital Anxiety and Depression Scale (HADS). The average scale score for HQ was 25.9 ± 9.0. The HQ scale and subscales had good to excellent internal consistency, except the emotional subscale, which had somewhat lower reliability. The test-retest reliability was moderate to good for the HQ scale and subscales. Fifty-five percent of patients had symptoms of anxiety, while 43% had symptoms of depression. This preliminary investigation found the Norwegian HQ to be a reliable and valid measure. Patients had more symptoms of anxiety than depression, which is in line with previous findings. Future studies may wish to evaluate the factor structure and measurement invariance of the Norwegian HQ. [ABSTRACT FROM AUTHOR]

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 painpredominant phenotype may be a meaningful prognostic marker in patients with hyperacusis. [ABSTRACT FROM AUTHOR]

Introduction: Øreblue® is a new treatment method for tinnitus and hyperacusis combining auditive rehabilitation and psychological therapy. The present monocentric observational retrospective study was to assess patient's judgements of its effectiveness. Material and methods: From January 2011 to June 2015, 74 consecutive healthy patients (male/female, 51/23; median age, 51.5 years) with chronic tinnitus (without hyperacusis, 32) or hyperacusis (with tinnitus, 42) were treated according to priority at the Hearing Institute of Resources (La Rochelle, France). They were asked to rank their discomfort, quality of life, and sleep quality before and 1 month after treatment using 10-point scales ranging from 0 (no discomfort, poor quality of life, or poor sleep quality) to 10 (unbearable discomfort, best quality of life, or best sleep quality) and their global impression of change on a 10-point scale ranging from 0% (not improved) to 100% (fully improved). These data which were reported in patient medical files were retrospectively analysed in 2018. Results: Discomfort, which was severe at inclusion (8.9±0.8), drastically decreased after treatment (change -8.5±1.3). Quality of life and sleep improved (from 2.2±1.4 and 2.0±1.9 to 8.3±1.4 and 4.9±2.1, respectively). 81.3% of the patients with tinnitus and 100% of patients with hyperacusis claimed to be fully improved. No adverse events were reported during the study. Conclusions: The Øreblue® method was effective on discomfort related to tinnitus or hyperacusis; it greatly improved quality of life and sleep. Further studies, especially multicentric or randomised, possibly with imaging and long-term follow-up, should be performed to strengthen these encouraging findings. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 20-year-old woman with recurrent headaches, eventually diagnosed with cytotoxic lesions of the corpus callosum (CLOCCs) through magnetic resonance imaging (MRI). Topics discussed include the diverse presentations of CLOCCs, potential differential diagnoses, and the reassuring self-resolving nature of CLOCCs evidenced by their disappearance on repeat MRI scans.

• First study to use NSS and HQ concurrently in large community and clinical samples. • Pioneering report of normative data using the newly translated 21-item French NSS. • Noise sensitivity and hyperacusis are multidimensional constructs. Noise sensitivity and hyperacusis are decreased sound tolerance conditions that are not well delineated or defined. This paper presents the correlations and distributions of the Noise Sensitivity Scale (NSS) and the Hyperacusis Questionnaire (HQ) scores in two distinct large samples. In Study 1, a community-based sample of young healthy adults (n = 103) exhibited a strong correlation (r = 0.74) between the two questionnaires. The mean NSS and HQ scores were 54.4 ± 16.9 and 12.5 ± 7.5, respectively. NSS scores displayed a normal distribution, whereas HQ scores showed a slight positive skew. In Study 2, a clinical sample of Veterans with or without clinical comorbidities (n = 95) showed a moderate correlation (r = 0.58) between the two questionnaires. The mean scores were 66.6 ± 15.6 and 15.3 ± 7.3 on the NSS and HQ, respectively. Both questionnaires' scores followed a normal distribution. In both samples, participants who self-identified as having decreased sound tolerance scored higher on both questionnaires. These findings provide reference data from two diverse sample groups. The moderate to strong correlations observed in both studies suggest a significant overlap between noise sensitivity and hyperacusis. The results underscore that NSS and HQ should not be used interchangeably, as they aim to measure distinct constructs, however to what extent they actually do remains to be determined. Further investigation should distinguish between these conditions through a comprehensive psychometric analysis of the questionnaires and a thorough exploration of psychoacoustic, neurological, and physiological differences that set them apart. [ABSTRACT FROM AUTHOR]

Keywords: auditory system; electrophysiology; neurotology; synaptic plasticity EN auditory system electrophysiology neurotology synaptic plasticity 3653 3655 3 08/17/22 20220815 NES 220815 Introduction The auditory system consists of a sophisticated balance of ascending and descending auditory signals that enable mammals to respond to stimuli of varying magnitude, adjust auditory gain and respond appropriately to saliency. To increase understanding of MOC neuron activity throughout the course of auditory stimuli, Torres Cadenas and colleagues investigated the short-term plasticity of the MNTB-MOC synapse. Taking a transgenic approach, the authors were able to isolate MOC neurons and adjust physiological parameters affecting neurotransmitter release and firing frequency of MNTB neurons. [Extracted from the article]

Hashir Aazh,1–3 Michael Landgrebe,4 Ali A Danesh,5 Brian CJ Moore6 1London Tinnitus and Hyperacusis Therapy Specialist Clinic, London, WC2N 5BW, UK; 2Guildford Tinnitus and Hyperacusis Therapy Specialist Clinic, Guildford, Surrey, GU2 4RG, UK; 3Audiology Department, Royal Surrey County Hospital, Guildford GU2 7XX, UK; 4Department of Psychiatry, Psychosomatics and Psychotherapy, Kbo Lech-Mangfall-Hospital Agatharied, Hausham 83734, Germany; 5Department of Communication Sciences and Disorders, Florida Atlantic University, Boca Raton, FL, 33431, USA; 6Department of Psychology, University of Cambridge, Cambridge CB2 3EB, UKCorrespondence: Hashir AazhGuildford Tinnitus and Hyperacusis Therapy Specialist Clinic, 1 Farnham Road, Guildford, Surrey GU2 4RG, UKEmail info@hashirtinnitusclinic.comAbstract: This article reviews the evidence related to the efficacy of Cognitive Behavioral Therapy (CBT) for alleviating the distress caused by tinnitus, hyperacusis and misophonia. Where available, the review was focused on meta-analyses of randomized controlled trials (RCTs) using either passive control groups (typically waiting list or education only) or active control groups (receiving some other form of treatment). Where data from RCTs were not available, case studies and retrospective studies were reviewed. Analyses were conducted separately for studies of patients with tinnitus, hyperacusis and misophonia. RCTs show that CBT is effective in alleviating the distress caused by tinnitus in comparison to passive control groups and sometimes active control groups. CBT for tinnitus can be effective both in individual and in group settings, whether delivered by psychiatrists, clinical psychologists, or specially trained audiologists. CBT for tinnitus can also be effective when delivered via the internet, when combined with help from audiologists. Usually, CBT does not reduce the loudness of tinnitus but it can improve quality of life. Case studies and some limited RCTs suggest that CBT can also be effective in alleviating the distress caused by hyperacusis and misophonia. However, RCTs with active control groups are currently lacking. There is strong evidence supporting the effectiveness of CBT in alleviating the distress caused by tinnitus. However, it is not yet clear whether CBT is more effective than some other forms of treatment. RCTs with active control groups are needed to establish more clearly the extent to which CBT is effective in alleviating the distress caused by hyperacusis and misophonia.Keywords: tinnitus, hyperacusis, misophonia, cognitive behavioral therapy, tinnitus specialist

Abstract Background Vestibular migraine (VM) has been recognized as a diagnostic entity over the past three decades. It affects up to 1% of the general population and 7% of patients seen in dizziness clinics. It is still underdiagnosed; consequently, it is important to conduct clinical studies that address diagnostic indicators of VM. The aim of this study was to assess auditory brainstem function in women with vestibular migraine using electrophysiological testing, contralateral acoustic reflex and loudness discomfort level. Methods The study group consisted of 29 women with vestibular migraine in the interictal period, and the control group comprised 25 healthy women. Auditory brainstem response, frequency following response, binaural interaction component and assessment of contralateral efferent suppression were performed. The threshold of loudness discomfort and the contralateral acoustic reflex were also investigated. The results were compared between the groups. Results There was a statistically significant difference between the groups in the frequency following response and the loudness discomfort level. Conclusions The current study suggested that temporal auditory processing and loudness discomfort levels are altered in VM patients during the interictal period, indicating that these measures may be useful as diagnostic criteria.

Context: Although studies of tinnitus exacerbated by sound exposure have indicated increased treatment challenges and intensified mental health and quality of life concerns, there is a lack of valid screening measures to differentiate or assess diagnostic factors and areas of impact unique to this specific symptom manifestation. Aims: The purpose of this study was to design a self-rated measurement tool that can accurately assess the subjective impact of tinnitus negatively modulated by external sound. Settings and Design: Based on review of established models of tinnitus and hyperacusis measurement and a two-part pilot study, the 20-item Sound-Sensitive Tinnitus Index (SSTI) was developed and administered in online survey format to 277 individuals worldwide. Methods and Material: Cronbach’s alpha was used to estimate reliability properties, and dimensional factor analysis was performed. To establish validity, statistical correlations of the SSTI were estimated with valid measures of related constructs including tinnitus, hyperacusis, depression, anxiety, and quality of life. Results: Statistical analysis yielded high levels of internal consistency reliability, and convergent validity was demonstrated through significant correlations with all established measures of related constructs. Initial factor analysis indicated two components split between overall functional impact and coping factors, while rotated factor analyses revealed four distinct scale dimensions, labeled: functional challenges, relational and communication challenges, coping factors, and prevention and hearing protection. Conclusions: As a valid and reliable measure, the SSTI fills an important gap as a clinical and research tool that can differentiate and assess severity and treatment progress in manifestations of combined tinnitus and auditory sensitivity symptoms.

Although tinnitus represents a major global burden, no causal therapy has yet been established. Ongoing controversies about the neuronal pathophysiology of tinnitus hamper efforts in developing advanced therapies. Hypothesizing that the unnoticed co-occurrence of hyperacusis and differences in the duration of tinnitus may possibly differentially influence the neural correlate of tinnitus, we analyzed 33 tinnitus patients without (T-group) and 20 tinnitus patients with hyperacusis (TH-group). We found crucial differences between the T-group and the TH-group in the increase of annoyance, complaints, tinnitus loudness, and central neural gain as a function of tinnitus duration. Hearing thresholds did not differ between T-group and TH-group. In the TH-group, the tinnitus complaints (total tinnitus score) were significantly greater from early on and the tinnitus intensity distinctly increased over time from ca. 12 to 17 dB when tinnitus persisted more than 5 years, while annoyance responses to normal sound remained nearly constant. In contrast, in the T-group tinnitus complaints remained constant, although the tinnitus intensity declined over time from ca. 27 down to 15 dB beyond 5 years of tinnitus persistence. This was explained through a gradually increased annoyance to normal sound over time, shown by a hyperacusis questionnaire. Parallel a shift from a mainly unilateral (only 17% bilateral) to a completely bilateral (100%) tinnitus percept occurred in the T-group, while bilateral tinnitus dominated in the TH-group from the start (75%). Over time in the T-group, ABR wave V amplitudes (and V/I ratios) remained reduced and delayed. By contrast, in the TH-group especially the ABR wave III and V (and III/I ratio) continued to be enhanced and shortened in response to high-level sound stimuli. Interestingly, in line with signs of an increased co-occurrence of hyperacusis in the T-group over time, ABR wave III also slightly increased in the T-group. The findings disclose an undiagnosed co-occurrence of hyperacusis in tinnitus patients as a main cause of distress and the cause of complaints about tinnitus over time. To achieve urgently needed and personalized therapies, possibly using the objective tools offered here, a systematic sub-classification of tinnitus and the co-occurrence of hyperacusis is recommended.