Hyperacusis questionnaire and event-related potential correlation in migraine patients.

This study aims to investigate auditory hypersensitivity and cortical function in migraine patients using the Hyperacusis Questionnaire and the Event-Related Potential (ERP) technique. The study analyzes alterations in the latency and amplitude of the event-related potentials MMN and P300 components. The findings contribute to a better understanding of the physiological relationship between migraine and auditory hypersensitivity. Seventeen migraine patients were admitted to the outpatient clinic of the Department of Otorhinolaryngology-Head and Neck Surgery at Peking University People's Hospital from June 2023 to September 2023. Nineteen matched healthy subjects were also selected. All participants underwent the pure tone audiometry and the auditory brainstem response test to determine hearing thresholds, the Hyperacusis Questionnaire, the Tinnitus Handicap Inventory, and an ERP examination. The Oddball classical paradigm was used as the stimulation task, and electroencephalography signals were recorded synchronously. The scores of the Hyperacusis Questionnaire, latency and amplitude of MMN and P300 component were compared between the migraine group and the control group, and their correlation was analyzed. The latency of MMN at the Fz and Cz sites in migraine patients was significantly shorter than that in the control group (P < 0.05), and the amplitudes were significantly higher than those in the control group (P < 0.05). The variances in latency and amplitude of P300 at Cz and Pz sites in migraine patients were not statistically significant when compared with the control group. (P > 0.05). The Hyperacusis Questionnaire was negatively correlated with MMN latency, with a correlation coefficient of - 0.374 (P = 0.025), and positively correlated with MMN amplitude, with a correlation coefficient of 0.378 (P = 0.023). There was no significant similarity between the Hyperacusis Questionnaire and P300 latency and amplitude (P > 0.05). Overall, auditory hypersensitivity was enhanced in individuals with migraines compared to healthy individuals, leading to faster information processing, while there may be less impairment in cognitive function.

Confirmatory factor analysis of the Tinnitus Impact Questionnaire using data from patients seeking help for tinnitus alone or tinnitus combined with hyperacusis.

A confirmatory factor analysis (CFA) of the Tinnitus Impact Questionnaire (TIQ) was performed. In contrast to commonly used tinnitus questionnaires, the TIQ is intended solely to assess the impact of tinnitus by not including items related to hearing loss or tinnitus loudness. This was a psychometric study based on a retrospective cross-sectional analysis of clinical data. Data were available for 155 new patients who had attended a tinnitus and hyperacusis clinic in the UK within a five-month period and had completed the TIQ. The mean age was 54 years (standard deviation = 14 years). The TIQ demonstrated good internal consistency, with Cronbach's α = 0.84 and McDonald's ω = 0.89. CFA showed that two items of the TIQ had low factor loadings for both one-factor and two-factor models and their scores showed low correlations with scores for other items. Bi-factor analysis gave a better fit, indicated by a relative chi-square (χ2) of 18.5, a Root-Mean Square Error of Approximation (RMSEA) of 0.103, a Comparative Fit Index (CFI) of 0.97, a Tucker Lewis Index (TLI) of 0.92, and a Standardized Root-Mean Residual (SPMR) of 0.038. Total TIQ scores were moderately correlated with scores for the Visual Analogue Scale of effect of tinnitus on life and the Screening for Anxiety and Depression-Tinnitus questionnaire, supporting the convergent validity of the TIQ. The TIQ score was not correlated with the pure-tone average hearing threshold, indicating discriminant validity. A multiple-causes multiple-indicator (MIMIC) model showed no influences of age, gender or hearing status on TIQ item scores. The TIQ is an internally consistent tool. CFA suggests a bi-factor model with sufficient unidimensionality to support the use of the overall TIQ score for assessing the impact of tinnitus. TIQ scores are distinct from the impact of hearing impairment among patients who have tinnitus combined with hearing loss.

Perceptual Consequences of Cochlear Deafferentation in Humans.

Cochlear synaptopathy, a form of cochlear deafferentation, has been demonstrated in a number of animal species, including non-human primates. Both age and noise exposure contribute to synaptopathy in animal models, indicating that it may be a common type of auditory dysfunction in humans. Temporal bone and auditory physiological data suggest that age and occupational/military noise exposure also lead to synaptopathy in humans. The predicted perceptual consequences of synaptopathy include tinnitus, hyperacusis, and difficulty with speech-in-noise perception. However, confirming the perceptual impacts of this form of cochlear deafferentation presents a particular challenge because synaptopathy can only be confirmed through post-mortem temporal bone analysis and auditory perception is difficult to evaluate in animals. Animal data suggest that deafferentation leads to increased central gain, signs of tinnitus and abnormal loudness perception, and deficits in temporal processing and signal-in-noise detection. If equivalent changes occur in humans following deafferentation, this would be expected to increase the likelihood of developing tinnitus, hyperacusis, and difficulty with speech-in-noise perception. Physiological data from humans is consistent with the hypothesis that deafferentation is associated with increased central gain and a greater likelihood of tinnitus perception, while human data on the relationship between deafferentation and hyperacusis is extremely limited. Many human studies have investigated the relationship between physiological correlates of deafferentation and difficulty with speech-in-noise perception, with mixed findings. A non-linear relationship between deafferentation and speech perception may have contributed to the mixed results. When differences in sample characteristics and study measurements are considered, the findings may be more consistent.

Device and Fitting Protocol for a Transitional Intervention for Debilitating Hyperacusis.

PURPOSE: This report describes a hearing device and corresponding fitting protocol designed for use in a transitional intervention for debilitating loudness-based hyperacusis. METHOD: The intervention goal is to transition patients with hyperacusis from their typical counterproductive sound avoidance behaviors (i.e., sound attenuation and limited exposure to healthy low-level sounds) into beneficial sound therapy treatment that can expand their dynamic range to the point where they can tolerate everyday sounds and experience an improved quality of life. This requires a combination of counseling and sound therapy, the latter of which is provided via the hearing device technology, signal processing, and precision fitting approach described in this report. The device combines a miniature behind-the-ear sound processor and a custom earpiece designed to maximize the attenuation of external sounds. Output-limiting loudness suppression is used to restrict exposure to offending high-level sounds while unity gain amplification maximizes exposure to healthy and tolerable lower level sounds. The fitting process includes measurement of the real-ear unaided response, the real-ear measurement (REM) system noise floor, the real-ear occluded response, real-ear insertion gain, and the output limit. With these measurements, the device can achieve the prescribed unity gain needed to provide transparent access to comfortable sound levels. It also supports individualized configuration of the therapeutic noise from an on-board sound generator and adaptive output limiting based on treatment-induced increases in dynamic range. RESULTS AND CONCLUSION: The utility of this device and fitting protocol, in combination with structured counseling, is highlighted in the outcomes of a successful 6-month trial of the transitional intervention described in a companion report in this issue.

Results of a 6-Month Field Trial of a Transitional Intervention for Debilitating Hyperacusis.

PURPOSE: We present results from a 6-month field trial of a transitional intervention for debilitating primary hyperacusis, including a combination of structured counseling; promotion of safe, comfortable, and healthy sound exposure; and therapeutic broadband sound from sound generators. This intervention is designed to overcome barriers to successful delivery of therapeutic sound as a tool to downregulate neural hyperactivity in the central auditory pathways (i.e., the maladaptive mechanism believed to account for primary hyperacusis) and, together with the counseling, reduce the associated negative emotional and physiological reactions to debilitating hyperacusis. METHOD: Twelve adults with normal or near-normal audiometric thresholds, complaints consistent with their pretreatment loudness discomfort levels ≤ 75 dB HL at multiple frequencies, and hearing questionnaire scores ≥ 24 completed the sound therapy-based intervention. The low-level broadband therapeutic sound was delivered by ear-level devices fitted bilaterally with either occluding earpieces and output-limiting loudness suppression (LS; to limit exposure to offensive sound levels) or open domes to maximize comfort and exposure to sound therapy. Thresholds for LS (primary outcome) were incrementally adjusted across six monthly visits based on treatment-driven change in loudness judgments for running speech in sound field. Secondary outcomes included categorical loudness judgments, speech understanding, and questionnaires to assess the hyperacusis problem, quality of life, and depression. An exit survey assessed satisfaction with and benefit from the intervention and the counseling, therapeutic sound, and LS components. RESULTS: The mean change in LS (34.8 dB) was highly significant (effect size = 2.045). Eleven of 12 participants achieved ≥ 16-dB change in LS, consistent with highly significant change in sound-based questionnaire scores. Exit surveys indicated satisfaction with and benefit from the intervention. CONCLUSION: The transitional intervention was successful in improving the hyperacusis conditions of 11 of 12 study participants while reducing their sound avoidance behaviors and reliance on sound protection.

Counseling Protocol for a Transitional Intervention for Debilitating Hyperacusis.

INTRODUCTION: This clinical focus article describes a structured counseling protocol for use with protected sound management and therapeutic sound in a transitional intervention for debilitating hyperacusis. The counseling protocol and its associated visual aids are crafted as a teaching tool to educate affected individuals about hyperacusis and encourage their acceptance of a transitional intervention. DESCRIPTION OF COUNSELING COMPONENTS: The counseling protocol includes five components. First, the patient's audiometric results are reviewed with the patient, and the transitional intervention is introduced. An overview of peripheral auditory structures and central neural pathways and the concept of central gain are covered in the second and third components. Maladaptive hyper-gain processes within the auditory neural pathways, which underlie the hyperacusis condition, and associated connections with nonauditory processes responsible for negative reactions to hyperacusis are covered in the fourth component. Detrimental effects from misused hearing protection devices (HPDs) and the necessity to wean the patient from overuse of HPDs are also discussed. In the fifth component, the importance of therapeutic sound is introduced as a tool to downregulate hyper-gain activity within the auditory pathways; its implementation in uncontrolled and controlled sound environments is described. It is explained that, over the course of the transitional intervention, recalibration of the hyper-gain processes will be ongoing, leading to restoration of normal homeostasis within the auditory pathways. In turn, associated activation of reactive nonauditory processes, which contribute to hyperacusis-related distress, will be reduced or eliminated. As recalibration progresses, there will be less need for protected sound management and sound therapy. Sound tolerance will improve, hyperacusis will subside, and daily activities in typical healthy sound environments will again become routine. RESULTS AND CONCLUSION: The combination of counseling with protected sound management and therapeutic sound is highlighted in companion reports, including a summary of the outcomes of a successful trial of the transitional intervention.

Background and Rationale for a Transitional Intervention for Debilitating Hyperacusis.

PURPOSE: This report provides the experimental, clinical, theoretical, and historical background that motivated a patented transitional intervention and its implementation and evaluation in a field trial for mitigation of debilitating loudness-based hyperacusis (LH). BACKGROUND AND RATIONALE: Barriers for ameliorating LH, which is differentiated here from other forms of hyperacusis, are delineated, including counterproductive management and treatment strategies that may exacerbate the condition. Evidence for hyper-gain central auditory processes as the bases for LH and the associated LH-induced distress and stress responses are presented. This presentation is followed by an overview of prior efforts to use counseling and therapeutic sound as interventional tools for recalibrating the hyper-gain LH response. We also consider previous efforts to use output-limiting sound-protection devices in the management of LH. This historical background lays the foundation for our transitional intervention protocol and its implementation and evaluation in a field trial. CONCLUSIONS: The successful implementation and evaluation of a transitional intervention, which we document in the outcomes of a companion proof-of-concept field trial in this issue, build on our prior efforts and those of others to understand, manage, and treat hyperacusis. These efforts to overcome significant barriers and vexing long-standing challenges in the management and treatment of LH, as reviewed here, are the pillars of the transitional intervention and its primary components, namely, counseling combined with protective sound management and therapeutic sound, which we detail in separate reports in this issue.

A Preliminary Analysis of the Clinical Effectiveness of Audiologist-Delivered Cognitive Behavioral Therapy Delivered via Video Calls for Rehabilitation of Misophonia, Hyperacusis, and Tinnitus.

PURPOSE: Cognitive behavioral therapy (CBT) is a key intervention for management of misophonia, hyperacusis, and tinnitus. The aim of this study was to perform a preliminary analysis comparing the scores for self-report questionnaires before and after audiologist-delivered CBT via video calls for adults with misophonia, hyperacusis, or tinnitus or a combination of these. METHOD: This was a retrospective cross-sectional study. The data for 37 consecutive patients who received CBT for misophonia, hyperacusis, or tinnitus from a private institute in the United Kingdom were analyzed. Self-report questionnaires taken as part of routine care were as follows: 4C Questionnaires for tinnitus, hyperacusis, and misophonia (4C-T, 4C-H, and 4C-M, respectively), Tinnitus Impact Questionnaire (TIQ), Hyperacusis Impact Questionnaire (HIQ), Misophonia Impact Questionnaire (MIQ), Sound Sensitivity Symptoms Questionnaire (SSSQ), and Screening for Anxiety and Depression in Tinnitus (SAD-T). Responses were also obtained to other questionnaires related to tinnitus, hyperacusis, insomnia, and anxiety and mood disorders. A linear mixed-model method was used to assess the changes in response to the questionnaires pretreatment and posttreatment. RESULTS: Pretreatment-posttreatment comparisons showed that scores for the 4C-T, 4C-H, 4C-M, TIQ, HIQ, MIQ, SSSQ, and SAD-T improved, with effect sizes of 1.4, 1.2, 1.3, 2.6, 0.9, 0.7, 0.9, and 1.4, respectively (all p < .05). CONCLUSIONS: This preliminary analysis suggests that CBT via video calls may be effective in reducing the impact of misophonia, hyperacusis, and tinnitus. However, this study did not have a control group, so its results need to be interpreted with caution.

Comprehensive Characterization of Hearing Loss and Tinnitus in Military-Affiliated and Non-Military-Affiliated Individuals.

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.

Noise sensitivity or hyperacusis? Comparing the Weinstein and Khalfa questionnaires in a community and a clinical samples.

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.

Oval and round window reinforcement surgery leads to improvements in sound tolerance and quality of life for hyperacusis patients.

PURPOSE: Hyperacusis is an audiological disorder in which patients become persistently sensitive and intolerant to everyday environmental sounds. For those patients that fail conservative options, a minimally invasive surgical procedure has been developed. MATERIALS & METHODS: Retrospective case series of 73 adult patients with hyperacusis who underwent oval and round window reinforcement surgery between 1/2017-6/2023. Small pieces of temporalis fascia were used to reinforce the round and oval windows. Patients were separated into two groups based on their preoperative speech Loudness Discomfort Level (LDL). Patients with a preoperative speech LDL ≤ 70 dB were placed in the "low LDL group" whereas patients with a preoperative speech LDL >70 dB were placed in the "high LDL group." Preoperative and one-week postoperative audiogram and speech LDLs were compared. Quality of life was assessed using the Glasgow Benefit Inventory (GBI) survey. RESULTS: 73 patients met inclusion criteria - 21 patients in the low LDL group and 52 in the high LDL group. Patients in the high LDL group significantly improved their LDLs by an average of 3.5 dB (P < 0.0001). 42 patients (80.8 %) in the high LDL group had improvement and would recommend the surgery for hyperacusis. Patients in the low LDL group significantly improved their LDL by an average of 12.9 dB (P = 0.032). Ten patients (47.6 %) from the low LDL group experienced improvement and would recommend hyperacusis surgery. CONCLUSION: Many patients with hyperacusis who undergo oval and round window reinforcement can receive significant improvement in sound tolerance and quality of life. Patients with a pre-op speech LDL > 70 dB have the greatest potential for improvement with surgery (80.8 %), probably because their hyperacusis was less severe. In the high LDL group(>70dB) the improvement in 1-10 scale went from 8.6 pre-op to 2.4 post op. In the low LDL group(<70dB) went from 9.2 pre-op to 6.8 post-op. These findings were consistent with the GBI results.

Tensor Tympani and Stapedial Tendon Reconstruction for Postoperative Hyperacusis after Tendon Lysis for Middle Ear Myoclonus.

OBJECTIVE: To present a method for repair of the stapedial and tensor tympani tendons in a patient with hyperacusis after a tendon lysis procedure. PATIENTS: A 71-year-old professional musician who presented to clinic with debilitating hyperacusis following a tensor tympani and stapedial tendon lysis procedure to treat middle ear myoclonus. INTERVENTIONS: A novel procedure for reapproximation of the tensor tympani and stapedial tendons into their native insertion points using periosteal grafts and nitinol wire. MAIN OUTCOMES MEASURES: Stapedial reflex measurements, uncomfortable loudness level, and subjective patient experience. RESULTS: Postoperatively, the patient had objective improvement in hyperacusis with return of acoustic reflexes in the affected ear and durable improvements in their frequency-specific uncomfortable loudness levels. CONCLUSIONS: This case describes the debilitating complication of hyperacusis following tendon lysis and highlights the importance of maximizing behavioral and medical measures prior to undergoing surgical intervention for middle ear myoclonus.

COVID-19 Vaccination Effects on Tinnitus and Hyperacusis: Longitudinal Case study.

The case study explores COVID-19 vaccination connection to tinnitus and hyperacusis, considering its onset and exacerbation post vaccination. The subject is a 47-year-old woman with a history of bilateral tinnitus, and her hearing history was tracked from 2014 to 2023. An intense episode of tinnitus occurred in 2021, distinct from previous experiences post COVID-19 vaccination, second dose. Symptoms manifested as sudden onset of hyperacusis, pronounced "roar" type tinnitus, and a sudden decline in hearing. Audiometric results showed reduce thresholds in low frequencies and lower speech scores in the left ear. This escalation significantly affects speech understanding in group conditions and noisy environments. There was a gradual improvement in tinnitus and hyperacusis severity, but the subject has a greater problem with speech understanding. The subject's journey involved visits to specialists, multiple testing including neuroimaging, naturopath consultations, and anxiety medication. It emphasizes the importance of healthcare practitioners recognizing and documenting these issues and need for timely multidisciplinary intervention and support. Further research is necessary to better understand the relationship between COVID-19, vaccination, and auditory symptoms.

A Systematic Review on the Genetic Contribution to Tinnitus.

PURPOSE: To assess the available evidence to support a genetic contribution and define the role of common and rare variants in tinnitus. METHODS: After a systematic search and quality assessment, 31 records including 383,063 patients were selected (14 epidemiological studies and 17 genetic association studies). General information on the sample size, age, sex, tinnitus prevalence, severe tinnitus distribution, and sensorineural hearing loss was retrieved. Studies that did not include data on hearing assessment were excluded. Relative frequencies were used for qualitative variables to compare different studies and to obtain average values. Genetic variants and genes were listed and clustered according to their potential role in tinnitus development. RESULTS: The average prevalence of tinnitus estimated from population-based studies was 26.3% for any tinnitus, and 20% of patients with tinnitus reported it as an annoying symptom. One study has reported population-specific differences in the prevalence of tinnitus, the white ancestry being the population with a higher prevalence. Genome-wide association studies have identified and replicated two common variants in the Chinese population (rs2846071; rs4149577) in the intron of TNFRSF1A, associated with noise-induced tinnitus. Moreover, gene burden analyses in sequencing data from Spanish and Swede patients with severe tinnitus have identified and replicated ANK2, AKAP9, and TSC2 genes. CONCLUSIONS: The genetic contribution to tinnitus is starting to be revealed and it shows population-specific effects in European and Asian populations. The common allelic variants associated with tinnitus that showed replication are associated with noise-induced tinnitus. Although severe tinnitus has been associated with rare variants with large effect, their role on hearing or hyperacusis has not been established.

The effects of acute and chronic noise trauma on stimulus-evoked activity across primary auditory cortex layers.

Exposure to intense noise environments is a major cause of sensorineural hearing loss and auditory perception disorders, such as tinnitus and hyperacusis, which may have a central origin. The effects of noise-induced hearing loss on the auditory cortex have been documented in many studies. One limitation of these studies, however, is that the effects of noise trauma have been mostly studied at the granular layer (i.e, the main cortical recipient of thalamic input), while the cortex is a very complex structure, with six different layers each having its own pattern of connectivity and role in sensory processing. The present study aims to investigate the effects of acute and chronic noise trauma on the laminar pattern of stimulus-evoked activity in the primary auditory cortex of the anesthetized guinea pig. We show that acute and chronic noise trauma are both followed by an increase in stimulus-evoked cortical responses, mostly in the granular and supragranular layers. The cortical responses are more monotonic as a function of the intensity level after noise trauma. There was minimal change, if any, in local field potential (LFP) amplitude after acute noise trauma, while LFP amplitude was enhanced after chronic noise trauma. Finally, LFP and the current source density analysis suggest that acute but more specifically chronic noise trauma is associated with the emergence of a new sink in the supragranular layer. This result suggests that supragranular layers become a major input recipient. We discuss the possible mechanisms and functional implications of these changes.NEW & NOTEWORTHY Our study shows that cortical activity is enhanced after trauma and that the sequence of cortical column activation during stimulus-evoked response is altered, i.e. the supragranular layer becomes a major input recipient. We speculate that these large cortical changes may play a key role in the auditory hypersensitivity (hyperacusis) that can be triggered after noise trauma in human subjects.

Cochlear Nucleus Transcriptome of a Fragile X Mouse Model Reveals Candidate Genes for Hyperacusis.

OBJECTIVE: Fragile X Syndrome (FXS) is a hereditary form of autism spectrum disorder. It is caused by a trinucleotide repeat expansion in the Fmr1 gene, leading to a loss of Fragile X Protein (FMRP) expression. The loss of FMRP causes auditory hypersensitivity: FXS patients display hyperacusis and the Fmr1- knock-out (KO) mouse model for FXS exhibits auditory seizures. FMRP is strongly expressed in the cochlear nucleus and other auditory brainstem nuclei. We hypothesize that the Fmr1-KO mouse has altered gene expression in the cochlear nucleus that may contribute to auditory hypersensitivity. METHODS: RNA was isolated from cochlear nuclei of Fmr1-KO and WT mice. Using next-generation sequencing (RNA-seq), the transcriptomes of Fmr1-KO mice and WT mice (n = 3 each) were compared and analyzed using gene ontology programs. RESULTS: We identified 270 unique, differentially expressed genes between Fmr1-KO and WT cochlear nuclei. Upregulated genes (67%) are enriched in those encoding secreted molecules. Downregulated genes (33%) are enriched in neuronal function, including synaptic pathways, some of which are ideal candidate genes that may contribute to hyperacusis. CONCLUSION: The loss of FMRP can affect the expression of genes in the cochlear nucleus that are important for neuronal signaling. One of these, Kcnab2, which encodes a subunit of the Shaker voltage-gated potassium channel, is expressed at an abnormally low level in the Fmr1-KO cochlear nucleus. Kcnab2 and other differentially expressed genes may represent pathways for the development of hyperacusis. Future studies will be aimed at investigating the effects of these altered genes on hyperacusis. LEVEL OF EVIDENCE: N/A Laryngoscope, 134:1363-1371, 2024.

Investigation of the relationship between hyperacusis and auditory processing difficulties in individuals with normal hearing.

PURPOSE: This study aimed to investigate the prevalence of hyperacusis and auditory processing (listening) difficulties (APDiff) in normal-hearing young adults and to explore the relationship between these two conditions. METHODS: A total of 333 participants aged 18-24 underwent pure-tone audiometry tests and completed the Khalfa Hyperacusis Questionnaire (KHQ) and the University of Cincinnati Auditory Processing Inventory (UCAPI) online. RESULTS: Hyperacusis was identified in 26.43% of participants, and APDiff in 36.04%. A strong, positive correlation was found between KHQ and UCAPI scores (r = 0.603, p < 0.001). Logistic regression models indicated that hyperacusis significantly increased the likelihood of having APDiff (OR 7.688, p < 0.001). CONCLUSION: Despite the high prevalence of hyperacusis and APDiff in young adults, few seek help from audiology clinics. Our findings highlight a significant link between hyperacusis and APDiff, emphasizing the need for screening protocols for auditory processing skills in young adults due to these conditions' potential social and academic impacts.

Recognising and managing migraine.

Migraine is a common neurological disorder characterised by a severe, pulsating headache, sometimes accompanied with photophobia or phonophobia and nausea and/or vomiting. The symptoms of migraine can have a significant adverse effect on a person's ability to undertake normal activities. Nurses have an important role in assisting patients in identifying migraine triggers and in supporting them to manage the symptoms of migraines through lifestyle changes and pharmacological treatments. This article describes different types of migraines and some differential diagnoses and 'red flag' symptoms that could indicate a more serious condition. The author also discusses non-pharmacological and pharmacological management strategies and treatments.

Phenotypes and clinical subgroups in vestibular migraine: a cross-sectional study with cluster analysis.

OBJECTIVE: The aim of this multicentric cross-sectional study was to collect phenotypes and clinical variability on a large sample of 244 patients enrolled in different university centers in Italy, trying to differentiate subtypes of VM. BACKGROUND: VM is one of the most frequent episodic vertigo characterized by a great clinical variability for duration of attacks and accompanying symptoms. Diagnosis is based only on clinical history of episodic vertigo in 50% of cases associated with migrainous headache or photo/phonophobia. METHODS: We enrolled in different university centers 244 patients affected by definite VM according to the criteria of the Barany Society between January 2022 and December 2022. An audiometric examination and a CNS MRI were performed before inclusion. Patients with low-frequency sensorineural hearing loss were not included, as well as patients with an MRI positive otherwise that for microischemic lesions. Patients were asked to characterize vestibular symptoms choosing among (multiple answers were allowed): internal vertigo, dizziness, visuo-vestibular symptoms/external vertigo; onset of vertigo and duration, neurovegetative, and cochlear accompanying symptoms (hearing loss, tinnitus, and fullness during attacks) were collected as well as migrainous headache and/or photo/phonophobia during vertigo; autoimmune disorders were also analyzed. A bedside examination was performed including study of spontaneous-positional nystagmus with infrared video goggles, post head shaking ny, skull vibration test, and video head impulse test. RESULTS: We included 244 subjects, 181 were females (74.2%). The age of onset of the first vertigo was 36.6 ± 14.5 while of the first headache was 23.2 ± 10.1. A positive correlation has been found between the first headache and the first vertigo. The mean duration of vertigo attacks was 11 ± 16 h. We carried on a cluster analysis to identify subgroups of patients with common clinical features. Four variables allowed to aggregate clusters: age of onset of vertigo, duration of vertigo attacks, presence of migrainous headache during vertigo, and presence of cochlear symptoms during vertigo. We identified 5 clusters: cluster 1/group 1 (23 subjects, 9.4%) characterized by longer duration of vertigo attacks; cluster 2/group 2 (52 subjects, 21.3%) characterized by absence of migrainous headache and cochlear symptoms during vertigo; cluster 3/group 3 (44 subjects, 18%) characterized by presence of cochlear symptoms during vertigo but not headache; cluster 4/group 4 (57 subjects, 23.4%) by the presence of both cochlear symptoms and migrainous headache during vertigo; cluster 5/group 5 (68 subjects, 27.9%) characterized by migrainous headache but no cochlear symptoms during vertigo. CONCLUSION: VM is with any evidence a heterogeneous disorder and clinical presentations exhibit a great variability. In VM, both symptoms orienting toward a peripheral mechanism (cochlear symptoms) and central ones (long lasting positional non-paroxysmal vertigo) may coexist. Our study is the first published trying to characterize subgroups of VM subjects, thus orienting toward different pathophysiological mechanisms.

Misophonia and Hearing Comorbidities in a Collegiate Population.

OBJECTIVES: Misophonia is a little-understood disorder in which certain sounds cause a strong emotional response in those who experience it. People who are affected by misophonia may find that noises like loud chewing, pen clicking, and/or sniffing trigger intense frustration, anger, or discomfort. The relationship of misophonia with other auditory disorders including loudness hyperacusis, tinnitus, and hearing loss is largely underexplored. This project aimed to investigate the prevalence and hearing-health comorbidities of misophonia in a college-aged population by using an online survey. DESIGN: A total of 12,131 undergraduate and graduate students between the ages of 18 and 25 were given the opportunity to answer an in-depth online survey. These students were sampled in a roughly 50 of 50 sex distribution. The survey was created using Qualtrics and included the following components: electronic consent, demographics questionnaire, Misophonia Questionnaire (MQ), Khalfa's Hyperacusis Questionnaire (HQ), Tinnitus and Hearing Survey, and Tinnitus Functional Index (TFI). To be eligible for compensation, answers for each of the above components were required, with the exception of the TFI, which was only presented to students who indicated that they experienced tinnitus. Respondents were determined to have high or possible likelihood of having misophonia if they gave specific answers to the MQ's Emotion and Behavior Scale or the MQ Severity Scale. RESULTS: After excluding duplicate responses and age-related outliers, 1,084 responses were included in the analysis. Just over 20% (n = 217) of the sample was determined to have a high or probable likelihood of having misophonia. The sample was primarily White, female, and of mid-to-high socioeconomic status. There was a strong positive correlation between MQ total scores and HQ total scores. High likelihood misophonia status showed a significant relationship with self-reported hearing loss and tinnitus. No statistically significant relationship was found between misophonia and age, ethnicity, or socioeconomic status. MQ total scores differed significantly when separating respondents by sex, self-reported tinnitus, and loudness hyperacusis. White respondents had significantly higher MQ total scores than Asian/Asian American respondents. CONCLUSIONS: The estimated prevalence of misophonia was about 8% to 20% of the sample, which agrees with most of the currently published research examining misophonia symptoms in collegiate populations. Results of data analysis suggest that misophonia severity may be related to loudness hyperacusis, sex, and possibly tinnitus. Future studies are needed to further examine the characteristics of these relationships, possibly in populations more optimized to reflect the general population or those with hearing-health disorders.