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BACKGROUND: Superior Semicircular Canal Dehiscence (SSCD) is a dehiscence of the otic capsule which normally lies over the superior semicircular canal. This database constitutes the largest series of SSCD patients to date. OBJECTIVE: To determine what preoperative factors, if any, contribute to postoperative outcomes and evaluate symptom resolution in a large SSCD patient cohort. METHODS: A single-institution, retrospective chart review collected patient demographics, intraoperative findings, and pre-and postoperative symptoms. Fishers exact t-test was performed for unpaired categorical variables, with a significance level of p

BACKGROUND: Pulsatile tinnitus (PT) may be due to a spectrum of cerebrovascular etiologies, ranging from benign venous turbulence to life threatening dural arteriovenous fistulas. A focused clinical history and physical examination provide clues to the ultimate diagnosis; however, the predictive accuracy of these features in determining PT etiology remains uncertain. METHODS: Patients with clinical PT evaluation and DSA were included. The final etiology of PT after DSA was categorized as shunting, venous, arterial, or non-vascular. Clinical variables were compared between etiologies using multivariate logistic regression, and performance at predicting PT etiology was determined by area under the receiver operating curve (AUROC). RESULTS: 164 patients were included. On multivariate analysis, patient reported high pitch PT (relative risk (RR) 33.81; 95% CI 3.81 to 882.80) compared with exclusively low pitch PT and presence of a bruit on physical examination (9.95; 2.04 to 62.08; P=0.007) were associated with shunting PT. Hearing loss was associated with a lower risk of shunting PT (0.16; 0.03 to 0.79; P=0.029). Alleviation of PT with ipsilateral lateral neck pressure was associated with a higher risk of venous PT (5.24; 1.62 to 21.01; P=0.010). An AUROC of 0.882 was achieved for predicting the presence or absence of a shunt and 0.751 for venous PT. CONCLUSION: In patients with PT, clinical history and physical examination can achieve high performance at detecting a shunting lesion. Potentially treatable venous etiologies may also be suggested by relief with neck compression.

Tinnitus is a prevalent hearing-loss deficit manifested as a phantom (internally generated by the brain) sound that is heard as a high-frequency tone in the majority of afflicted persons. Chronic tinnitus is debilitating, leading to distress, sleep deprivation, anxiety, and even suicidal thoughts. It has been theorized that, in the majority of afflicted persons, tinnitus can be attributed to the loss of high-frequency input from the cochlea to the auditory cortex, known as deafferentation. Deafferentation due to hearing loss develops with aging, which progressively causes tonotopic regions coding for the lost high-frequency coding to synchronize, leading to a phantom high-frequency sound sensation. Approaches to tinnitus remediation that demonstrated promise include inhibitory drugs, the use of tinnitus-specific frequency notching to increase lateral inhibition to the deafferented neurons, and multisensory approaches (auditory-motor and audiovisual) that work by coupling multisensory stimulation to the deafferented neural populations. The goal of this review is to put forward a theoretical framework of a multisensory approach to remedy tinnitus. Our theoretical framework posits that due to visions modulatory (inhibitory, excitatory) influence on the auditory pathway, a prolonged engagement in audiovisual activity, especially during daily discourse, as opposed to auditory-only activity/discourse, can progressively reorganize deafferented neural populations, resulting in the reduced synchrony of the deafferented neurons and a reduction in tinnitus severity over time.

Attention plays an important role in not only the awareness and perception of tinnitus but also its interactions with external sounds. Recent evidence suggests that attention is heightened in the tinnitus brain, likely as a result of relatively local cortical changes specific to deafferentation sites or global changes that help maintain normal cognitive capabilities in individuals with hearing loss. However, most electrophysiological studies have used passive listening paradigms to probe the tinnitus brain and produced mixed results in terms of finding a distinctive biomarker for tinnitus. Here, we designed a selective attention task, in which human adults attended to one of two interleaved tonal (500 Hz and 5 kHz) sequences. In total, 16 tinnitus (5 females) and 13 age- and hearing-matched control (8 females) subjects participated in the study, with the tinnitus subjects matching the tinnitus pitch to 5.4 kHz (range = 1.9-10.8 kHz). Cortical responses were recorded in both passive and attentive listening conditions, producing no differences in P1, N1, and P2 between the tinnitus and control subjects under any conditions. However, a different pattern of results emerged when the difference was examined between the attended and unattended responses. This attention-modulated cortical response was significantly greater in the tinnitus than control subjects: 3.9-times greater for N1 at 5 kHz (95% CI: 2.9 to 5.0, p = 0.007, ηp2 = 0.24) and 3.0 for P2 at 500 Hz (95% CI: 1.9 to 4.5, p = 0.026, ηp2 = 0.17). We interpreted the greater N1 modulation as local neural changes specific to the tinnitus frequency and the greater P2 as global changes to hearing loss. These two cortical measures were used to differentiate between the tinnitus and control subjects, producing 83.3% sensitivity and 76.9% specificity (AUC = 0.81, p = 0.006). These results suggest that the tinnitus brain is more plastic than that of the matched non-tinnitus controls and that the attention-modulated cortical response can be developed as a clinically meaningful biomarker for tinnitus.

This report details a case of neurosyphilis manifesting as concurrent ocular and otosyphilis, an uncommon presentation of the disease. Here, we describe the diagnosis and treatment of a 27-year-old immunocompetent Caucasian male who presented with uveitis and tinnitus. Physical exam was consistent with uveitis and audiometric testing revealed bilateral sensorineural hearing loss. Serum rapid plasma reagin (RPR) was reactive at 1:512 with a follow-up cerebrospinal fluid (CSF) venereal disease research laboratory (VDRL) test likewise reactive at 1:2, confirming neurosyphilis. The patient was treated with intravenous penicillin G with improvement of symptoms and with subsequent improvement of serum and CSF RPR. However, he ultimately represented with recurrent symptoms and fluctuating serum RPR levels, necessitating repeat treatment and ongoing clinical monitoring. Neurosyphilis can occur at any point during the course of a syphilis infection and may present with a variety of nonspecific findings. This case documents a particularly uncommon instance of simultaneous ocular and otosyphilis, a presentation of neurosyphilis that has only been described a handful of times.

Tinnitus is a heritable, highly prevalent auditory disorder treated by multiple medical specialties. Previous GWAS indicated high genetic correlations between tinnitus and hearing loss, with little indication of differentiating signals. We present a GWAS meta-analysis, triple previous sample sizes, and expand to non-European ancestries. GWAS in 596,905 Million Veteran Program subjects identified 39 tinnitus loci, and identified genes related to neuronal synapses and cochlear structural support. Applying state-of-the-art analytic tools, we confirm a large number of shared variants, but also a distinct genetic architecture of tinnitus, with higher polygenicity and large proportion of variants not shared with hearing difficulty. Tissue-expression analysis for tinnitus infers broad enrichment across most brain tissues, in contrast to hearing difficulty. Finally, tinnitus is not only correlated with hearing loss, but also with a spectrum of psychiatric disorders, providing potential new avenues for treatment. This study establishes tinnitus as a distinct disorder separate from hearing difficulties.

Vestibular schwannoma (VS) is a benign peripheral nerve sheath tumor involving the vestibulocochlear nerve. Affected patients typically experience a gradual emergence of episodic imbalance and unilateral hearing loss, tinnitus, and headache. Less often, VS may be associated with facial pain; ocular, otic, and taste disturbance; paresthesia of the tongue and face; and temporomandibular disorder-like presentations. There is limited information in the dental literature relating the myriad of oral and maxillofacial manifestations of VS. The objective of this article is to underscore the importance for dental clinicians to seek clinicopathologic correlations with VS-related symptomatology, potentially resulting in a timelier diagnosis and improved patient outcomes. To convey this clinical challenge, a detailed narrative of a 45-year-old patient with an 11-year delay in diagnosis has been reported. In addition, the typical radiographic appearance of an implanted cranial device placed following VS resection has been provided. [ABSTRACT FROM AUTHOR]

Objective: Tinnitus may be associated with various brain changes. However, the degenerative changes in patients with tinnitus have not been extensively investigated. We aimed to evaluate degenerative, structural, and functional brain changes in patients with mild cognitive impairment (MCI) who also suffer from tinnitus. Materials and methods: This study included participants aged 60 to 80 years with MCI and a hearing level better than 40 dB. The participants were classified into two groups: MCI with tinnitus (MCI-T) and MCI without tinnitus (MCI-NT). All patients underwent Tinnitus Handicap Inventory (THI), 3 T brain MRI, F18-florapronol PET, and F18-FDG PET. Results: The MCI-T group exhibited higher ß-amyloid deposition in the superior temporal gyrus, temporal pole, and middle temporal gyrus compared to the MCI-NT group (p < 0.05 for all). Additionally, the MCI-T group showed increased metabolism in the inferior frontal gyrus, insula, and anterior cingulate cortex (ACC) (p < 0.005 for all). The THI score was strongly correlated with increased volume in the insula, ACC, superior frontal gyrus, supplementary motor area, white matter near the hippocampus, and precentral gyrus (p < 0.05 for all). Moreover, the MCI-T group demonstrated higher metabolic activity in the default mode network (DMN) and lower activity in the executive control network (ECN) (p < 0.05 for all). In the MCI-T group, the posterior DMN was positively correlated with the visual network and negatively with the ECN, whereas in the MCI-NT group, it correlated positively with the ECN. Conclusion: The MCI-T group exhibited greater ß-amyloid accumulation in the auditory cortex and more extensive changes across various brain networks compared with the MCI-NT group, potentially leading to diverse clinical symptoms such as dementia with semantic deficits or depression. Tinnitus in MCI patients may serve as a biomarker for degenerative changes in the temporal lobe and alterations in brain network dynamics. [ABSTRACT FROM AUTHOR]

AbstractBackgroundAims/ObjectivesMaterial and MethodsResultsConclusionSignificanceIdiopathic intracranial hypertension (IIH) can affect both hearing and balance due to increased inner ear pressure.This study aimed to evaluate the impact of increased inner ear pressure on hearing and balance in patients with IIH using auditory and vestibular tests.Twenty-four IIH patients and 28 healthy controls underwent oVEMP, pure tone audiometry, tympanometry, and acoustic reflex tests pre-lumbar punctures. IIH patients received acetazolamide. Pre- and post-treatment results, tinnitus, and vertigo scores were compared. Post-treatment oVEMP and audiometry results were compared between groups.Pre-treatment oVEMP showed a significant left N1 latency difference (p = 0.049). Post-treatment, left ear amplitude (p = 0.035) and both ear amplitude ratios (p = 0.044 and p = 0.047) increased significantly. Audiometry had no significant changes (p < 0.05). Tinnitus and vertigo scores decreased significantly (p ≤ 0.001).Prolonged oVEMP latency suggests IIH may impact the brain stem and vestibular nerve, while increased amplitude values indicate peripheral vestibular involvement. IIH affects hearing across all frequencies, especially at 4000 Hz, impacting both hearing and balance.Understanding the effects of IIH on auditory and vestibular functions can guide effective treatments, improving quality of life for patients by addressing both hearing and balance issues. [ABSTRACT FROM AUTHOR]

Purpose: Patients suffering from Ménière's disease (MD) experience vertigo, and impairments in hearing and quality of life (QoL). This study aims to investigate the impact of cochlear implantation (CI) on various aspects affecting patients with MD. Methods: A single tertiary centre's CI database for CI recipients with MD between 2014 and 2022 was screened retrospectively. Hearing, vertigo, tinnitus symptoms, and hearing-related QoL were assessed. Pre- and postoperative hearing tests in conjunction with subjective outcome measures by visual analogue scale (VAS) and validated tools such as the Dizziness Handicap Inventory (DHI), Tinnitus Handicap Inventory (THI) and Nijmegen Cochlear Implant Questionnaire (NCIQ), as well as the assessment of the pre- and postoperative Functional Level Scale (FLS) were examined. Results: Eleven ears were included (median age: 59 years at implantation). Following implantation, there was a significant enhancement in Word Recognition Scores at sound levels of 65 dB and 80 dB compared to before treatment (preop vs. 12 months postop: p = 0.012). However, no significant enhancement was observed for 50 dB. MD-related impairments improved significantly postoperatively, as measured by the VAS (vertigo: p = 0.017; tinnitus: p = 0.042), DHI (p = 0.043), THI (p = 0.043) and NCIQ (p < 0.001). The FLS improved significantly (p = 0.020). Conclusion: CI has positive effects on all areas examined in our cohort. However, discrimination of speech at low sound pressure levels remained problematic postoperatively. In patients suffering from MD, the prioritized treatment goals include not only improved hearing but also the rehabilitation of vertigo and tinnitus, as well as the enhancement of QoL. Validated instruments are useful screening tools. [ABSTRACT FROM AUTHOR]

Purpose: An up-to-date overview of epidemiology, etiology and pathophysiological mechanisms, diagnostic and evaluation methods, current treatment status and future directions of subjective tinnitus in adults. Methods: Review of current evidence-based literature on subjective tinnitus in adults. Results: The prevalence of subjective tinnitus in the adult population is estimated to be around 14%, and it tends to increase with age. Subjective tinnitus is a complex condition with multiple factors contributing to its origin. However, the exact causes and underlying mechanisms remain unknown. Potential causes may include hearing loss, dysfunction in the somatosensory system, and auditory cortical dysfunction, although severe underlying pathology is rare. Currently, diagnosis primarily relies on patient self-reported medical history and physician-based clinical assessment due to the lack of objective testing. Various treatment and management options have been proposed, but their effectiveness varies, and there is no universally agreed-upon treatment option. Conclusions: Tinnitus is a complex and heterogeneous disease with a high incidence rate and a tendency to increase with age. A holistic perspective is needed to understand the generation, perception, and emotional responses to tinnitus. Diagnosis requires a comprehensive assessment based on medical history and relevant examinations, identification of concurrent psychosomatic comorbidities, and active pursuit of objective diagnostic methods. At the same time, on the basis of existing treatment plans and combining emerging technologies, we will develop new personalized, precise, and combined treatment plans. [ABSTRACT FROM AUTHOR]

Objectives: Pendred syndrome, an autosomal recessive disorder, is often associated with pathogenic variants of the SLC26A4 gene that encodes the pendrin protein. Given its autosomal recessive inheritance, tracing the family history and screening siblings become crucial once a diagnosis of Pendred syndrome is confirmed. This case report aims to underscore the variability in inner ear morphology within a family diagnosed with Pendred syndrome, all carrying the same SLC26A4 gene mutation. Methods: A chart review and a review of the literature. Results: We present a family of 4, all of whom possess sensorineural hearing loss due to the same homozygous SLC26A4 variant c.919-2A>G. Intriguingly, clinical manifestations, especially inner ear deformities, displayed variability among family members. Notably, 1 family member exhibited a normal cochleovestibular structure morphology, which was rarely reported in the literature. Conclusions: This report highlights the significance of genetic testing and familial consultation when a proband exhibits typical Pendred syndrome symptoms. It also underscores that the inner ear morphology can exhibit variability among family members, even with the same homozygous SLC26A4 variant. [ABSTRACT FROM AUTHOR]

Tinnitus and misophonia are important "sound annoyance" disorders in pediatric otolaryngology and audiology practices. There is scant published literature to suggest increased anxiety and depression symptoms in these disorders. This study aimed at assessing the clinical characteristics of these 2 disorders and their prevalence in mental health–related symptoms in a 2-year retrospective chart review of a multi-disciplinary (otolaryngology, audiology, and psychology) clinic cohort. Analyses were based on 54 (tinnitus = 33 and misophonia = 21) children consisting of 19 males and 35 females with a mean age (standard deviation) of 14.3 (3.0) years. The entire cohort was negatively affected by diagnosis-based symptom severity instruments as assessed by Tinnitus Functional Index and Amsterdam Misophonia Scale. Both subgroups exhibited elevated anxiety and depression symptoms in psychometric instruments as assessed by Screen for Child Anxiety Related Emotional Disorders and Short Mood and Feelings Questionnaire. Evidence-based management of these disorders is lacking, and clinical trials are needed. [ABSTRACT FROM AUTHOR]

To establish the prevalence of tinnitus in adults who have sustained non-blast related traumatic brain injury (TBI), as well as the prevalence of tinnitus following TBI in the absence of hearing loss. A systematic search was carried out using MEDLINE, EMBASE, PsycINFO, CINAHL from January 1st 1990 to August 14th 2023. TBI, tinnitus and auditory findings were extracted from all eligible studies, and a descriptive synthesis performed. This systematic review was registered with PROSPERO (Registration number: CRD42022377637). Based on the Oxford Centre of Evidence-Based Medicine (OCEBM) (2011) criteria, the highest quality evidence identified was at Level 2b, with the bulk of the included studies predominantly populating the lower evidence tiers. While there was a substantial variability in the methods used to establish and report the presence of tinnitus, its occurrence following TBI was evident in adults with and without hearing loss. The need for prospective, longitudinal research into tinnitus following non-blast related TBI is evident. Such comprehensive studies hold the potential to inform and enhance the clinical diagnosis and management of this patient population. [ABSTRACT FROM AUTHOR]

Background: Tinnitus and uveitis have shared commonality in pathophysiology in terms of autoimmunity. However, no studies that have linked any association between the conditions of tinnitus and uveitis. Methods: This is a retrospective study conducted from the Taiwan National Health Insurance database in order to investigate whether tinnitus patients are at increased risk of uveitis. Patients newly diagnosed with tinnitus between 2001 and 2014 were recruited and followed up until 2018. The endpoint of interest was a diagnosis of uveitis. Results: A total of 31,034 tinnitus patients and 124,136 matched comparisons were analyzed. Tinnitus patients were found to have a significantly higher cumulative incidence for uveitis than those without the diagnosis of tinnitus with incidence rate of 1.68 (95% CI 1.55–1.82) per 10 000 person-months for tinnitus group and 1.48 (95% CI 1.42–1.54) per 10 000 person-months for non-tinnitus group. Conclusion: Tinnitus patients were found to have increased risk of developing uveitis. [ABSTRACT FROM AUTHOR]

• Combined static with dynamic analysis to explore the neural mechanisms of tinnitus. • The functional connectivity of inter-network decreased in tinnitus patients. • Tinnitus severity and duration were related to the transformation of brain networks. In order to comprehensively understand the changes of brain networks in patients with chronic tinnitus, this study combined static and dynamic analysis methods to explore the abnormalities of brain networks. Thirty-two patients with chronic tinnitus and 30 age-, sex- and education-matched healthy controls (HC) were recruited. Independent component analysis was used to identify resting-state networks (RSNs). Static and dynamic functional network connectivity (FNC) were performed. The temporal properties of brain network including mean dwell time (MDT), fraction time (FT) and numbers of transitions (NT) were calculated. Two-sample t test and Spearman's correlation were used for group compares and correlation analysis. Four RSNs showed abnormal FNC including auditory network (AUN), default mode network (DMN), attention network (AN) and sensorimotor network (SMN). For static analysis, tinnitus patients showed significantly decreased FNC in AUN-DMN, AUN-AN, DMN-AN, and DMN-SMN than HC [p < 0.05, false discovery rate (FDR) corrected]. For dynamic analysis, tinnitus patients showed significantly decreased FNC in DMN-AN in state 3 (p < 0.05, FDR corrected). MDT in state 3 was significantly decreased in tinnitus patients (t = 2.039, P = 0.046). In the tinnitus group, the score of tinnitus functional index (TFI) was negatively correlated with MDT and FT in state 4, and the duration of tinnitus was positively correlated with FT in state 1 and NT. Chronic tinnitus causes abnormal brain network connectivity. These abnormal brain networks help to clarify the mechanism of tinnitus generation and chronicity, and provide a potential basis for the treatment of tinnitus. [ABSTRACT FROM AUTHOR]

The influence of naps on tinnitus was systematically assessed by exploring the frequency, clinical and demographic characteristics of this phenomenon. 9,724 data from two different tinnitus databases (Tinnitus Hub: n = 6115; Tinnitus Research Initiative (TRI): n = 3627) were included. After separate analysis of the databases, these results were then compared with each other. In the Tinnitus Hub survey database, a total of 31.1% reported an influence on tinnitus by taking a nap (26.9% in the TRI database), with much more frequent worsening after a nap than improvement (23.0% a little or a lot worse; TRI: 17.7% worse; 8.1% a little or a lot better; TRI: 9.2% better). The influence of napping on tinnitus was associated in both databases with other clinical features, such as the dependence of tinnitus on night quality, stress and somatosensory maneuvers. The present study confirms the clinical observation that more tinnitus sufferers report worsening after a nap than tinnitus sufferers reporting an improvement. It was consistently shown that tinnitus sufferers reporting nap-induced modulation of tinnitus also report more frequently an influence of night sleep on their tinnitus. Further clinical and polysomnographic research is warranted to better understand the interaction between sleep and tinnitus. [ABSTRACT FROM AUTHOR]