Clinical Practice Guideline: Age-Related Hearing Loss Executive Summary.

OBJECTIVE: Age-related hearing loss (ARHL) is a prevalent but often underdiagnosed and undertreated condition among individuals aged 50 and above. It is associated with various sociodemographic factors and health risks including dementia, depression, cardiovascular disease, and falls. While the causes of ARHL and its downstream effects are well defined, there is a lack of priority placed by clinicians as well as guidance regarding the identification, education, and management of this condition. PURPOSE: The purpose of this clinical practice guideline is to identify quality improvement opportunities and provide clinicians trustworthy, evidence-based recommendations regarding the identification and management of ARHL. These opportunities are communicated through clear actionable statements with an explanation of the support in the literature, the evaluation of the quality of the evidence, and recommendations on implementation. The target patients for the guideline are any individuals aged 50 years and older. The target audience is all clinicians in all care settings. This guideline is intended to focus on evidence-based quality improvement opportunities judged most important by the Guideline Development Group (GDG). It is not intended to be a comprehensive, general guide regarding the management of ARHL. The statements in this guideline are not intended to limit or restrict care provided by clinicians based on their experience and assessment of individual patients. ACTION STATEMENTS: The GDG made strong recommendations for the following key action statements (KASs): (KAS 4) If screening suggests hearing loss, clinicians should obtain or refer to a clinician who can obtain an audiogram. (KAS 8) Clinicians should offer, or refer to a clinician who can offer, appropriately fit amplification to patients with ARHL. (KAS 9) Clinicians should refer patients for an evaluation of cochlear implantation candidacy when patients have appropriately fit amplification and persistent hearing difficulty with poor speech understanding. The GDG made recommendations for the following KASs: (KAS 1) Clinicians should screen patients aged 50 years and older for hearing loss at the time of a health care encounter. (KAS 2) If screening suggests hearing loss, clinicians should examine the ear canal and tympanic membrane with otoscopy or refer to a clinician who can examine the ears for cerumen impaction, infection, or other abnormalities. (KAS 3) If screening suggests hearing loss, clinicians should identify sociodemographic factors and patient preferences that influence access to and utilization of hearing health care. (KAS 5) Clinicians should evaluate and treat or refer to a clinician who can evaluate and treat patients with significant asymmetric hearing loss, conductive or mixed hearing loss, or poor word recognition on diagnostic testing. (KAS 6) Clinicians should educate and counsel patients with hearing loss and their family/care partner(s) about the impact of hearing loss on their communication, safety, function, cognition, and quality of life. (KAS 7) Clinicians should counsel patients with hearing loss on communication strategies and assistive listening devices. (KAS 10) For patients with hearing loss, clinicians should assess if communication goals have been met and if there has been improvement in hearing-related quality of life at a subsequent health care encounter or within 1 year. The GDG offered the following KAS as an option: (KAS 11) Clinicians should assess hearing at least every 3 years in patients with known hearing loss or with reported concern for changes in hearing.

Clinical Practice Guideline: Age-Related Hearing Loss.

OBJECTIVE: Age-related hearing loss (ARHL) is a prevalent but often underdiagnosed and undertreated condition among individuals aged 50 and above. It is associated with various sociodemographic factors and health risks including dementia, depression, cardiovascular disease, and falls. While the causes of ARHL and its downstream effects are well defined, there is a lack of priority placed by clinicians as well as guidance regarding the identification, education, and management of this condition. PURPOSE: The purpose of this clinical practice guideline is to identify quality improvement opportunities and provide clinicians trustworthy, evidence-based recommendations regarding the identification and management of ARHL. These opportunities are communicated through clear actionable statements with explanation of the support in the literature, evaluation of the quality of the evidence, and recommendations on implementation. The target patients for the guideline are any individuals aged 50 years and older. The target audience is all clinicians in all care settings. This guideline is intended to focus on evidence-based quality improvement opportunities judged most important by the guideline development group (GDG). It is not intended to be a comprehensive, general guide regarding the management of ARHL. The statements in this guideline are not intended to limit or restrict care provided by clinicians based on their experience and assessment of individual patients. ACTION STATEMENTS: The GDG made strong recommendations for the following key action statements (KASs): (KAS 4) If screening suggests hearing loss, clinicians should obtain or refer to a clinician who can obtain an audiogram. (KAS 8) Clinicians should offer, or refer to a clinician who can offer, appropriately fit amplification to patients with ARHL. (KAS 9) Clinicians should refer patients for an evaluation of cochlear implantation candidacy when patients have appropriately fit amplification and persistent hearing difficulty with poor speech understanding. The GDG made recommendations for the following KASs: (KAS 1) Clinicians should screen patients aged 50 years and older for hearing loss at the time of a health care encounter. (KAS 2) If screening suggests hearing loss, clinicians should examine the ear canal and tympanic membrane with otoscopy or refer to a clinician who can examine the ears for cerumen impaction, infection, or other abnormalities. (KAS 3) If screening suggests hearing loss, clinicians should identify sociodemographic factors and patient preferences that influence access to and utilization of hearing health care. (KAS 5) Clinicians should evaluate and treat or refer to a clinician who can evaluate and treat patients with significant asymmetric hearing loss, conductive or mixed hearing loss, or poor word recognition on diagnostic testing. (KAS 6) Clinicians should educate and counsel patients with hearing loss and their family/care partner(s) about the impact of hearing loss on their communication, safety, function, cognition, and quality of life (QOL). (KAS 7) Clinicians should counsel patients with hearing loss on communication strategies and assistive listening devices. (KAS 10) For patients with hearing loss, clinicians should assess if communication goals have been met and if there has been improvement in hearing-related QOL at a subsequent health care encounter or within 1 year. The GDG offered the following KAS as an option: (KAS 11) Clinicians should assess hearing at least every 3 years in patients with known hearing loss or with reported concern for changes in hearing.

VM-PATHI Correlates With Cognitive Function Improvement After Successful Treatment in Patients With Vestibular Migraine.

OBJECTIVE: To assess changes in cognitive function in vestibular migraine patients undergoing treatment. STUDY DESIGN: Prospective cohort. SETTING: Single-institution tertiary-care center. PATIENTS: Thirty-four patients with vestibular migraine were included in the study. Average age at diagnosis was 47.9 years. A majority of patients (91.2%) were female. INTERVENTIONS: Vestibular therapies included pharmacologic treatment (67.6%), mindfulness-based stress reduction (58.8%), vestibular physical therapy (20.6%), and lifestyle changes only (2.9%). MAIN OUTCOME MEASURES: Pretreatment and posttreatment questionnaires were collected including the Cognitive Failures Questionnaire (CFQ), Vestibular Migraine Patient Assessment Tool and Handicap Inventory (VM-PATHI), and Dizziness Handicap Inventory. RESULTS: Median time between pretreatment and posttreatment questionnaire was 4.4 months (range, 2.8-15.6. mo). CFQ scores decreased in subjects who responded to treatment, as defined by those with a positive change in VM-PATHI score (average decrease, 6.5; p = 0.03). CFQ scores did not improve in subjects who had no improvement in their vestibular condition, as defined by no change or an increase in VM-PATHI score (average increase, 2.0; p = 0.53). Univariate linear regression showed that VM-PATHI score change was highly predictive of CFQ change ( p < 0.01, r2 = 0.36). Multivariate regression demonstrated that the VM-PATHI ( p = 0.03) and not the Dizziness Handicap Inventory ( p = 0.10) predicted changes in CFQ score. CONCLUSIONS: Self-reported cognitive dysfunction improves with successful treatment of vestibular migraine.

Abnormal Subjective and Audiometric Auditory Function in Migraine.

OBJECTIVE: To identify if migraine is associated with auditory deficits and if the auditory profile of migraine is distinct from other pain syndromes, such as chronic pain. STUDY DESIGN: Cross-sectional, retrospective. SETTING: A total of 5273 respondents of the 1999 to 2004 National Health and Nutrition Examination Survey. METHODS: Regression analyses assessed the association between migraine (n = 1245) and chronic pain (n = 430) status with subjectively endorsed hearing loss, tinnitus, pure-tone average (PTA) at 500, 1000, 2000, and 4000 Hz, and subjective-audiometric hearing mismatch (endorsed hearing loss but with a PTA ≤ 25 dB), correcting for confounding factors. RESULTS: Migraine was associated with increased tinnitus (adjusted odds ratio [aOR] = 1.77, 95% confidence interval [CI]: 1.47-2.13, p < .001) and subjective hearing loss (aOR = 1.58, 95% CI: 1.29-1.94, p < .001). Migraine was associated with higher PTA (ß = .89, p = .023). Migraine decreased the PTA threshold at which individuals endorsed subjective hearing loss (ß = -1.94, p = .013) and was associated with a more subjective-audiometric hearing mismatch (aOR = 1.50, 95% CI: 1.18-1.89, p < .001). Chronic pain was not associated with tinnitus (aOR = 1.26, 95% CI: 0.97-1.63, p = 0.079), subjective hearing loss (aOR = 0.94, 95% CI: 0.71-1.23, p = .64), changes in PTA (ß = -.22, p = .69), altered PTA threshold for endorsing hearing loss (ß = 1.40, p = .19), or subjective-audiometric hearing mismatch (aOR = 0.98, 95% CI: 0.70-1.34, p = .88). CONCLUSION: Migraine is associated with both worse pure-tone audiometry and higher sensitivity to changes in hearing ability, suggesting both peripheral and central auditory function abnormalities. In contrast, patients with chronic pain did not demonstrate these abnormalities. The etiology of abnormal auditory processing in migraine may be different from that of other pain syndromes.

Generalized vestibular hyporeflexia and chronic upbeat nystagmus due to thiamine deficiency.

BACKGROUND: Ocular motor and vestibular manifestations of Wernicke's thiamine deficiency (WTD) are frequent and heterogeneous. Previous neuropathological and neuroimaging findings identified brainstem and cerebellar lesions responsible for these findings, however, peripheral vestibular lesions are probably uncommon in human WTD, though noted on an avian thiamine deficient study. MATERIAL: Single case study of a WTD patient post-gastric bypass who developed ataxia, oscillopsia and nystagmus, with low serum thiamine, and increased MRI T2 signal in the thalami, but normal brainstem and cerebellum. Vestibular evaluation showed significant vestibular hyporreflexia affecting all six canals, and a chronic upbeat nystagmus, now for 14 months after WTD onset. METHODS: Serial clinical, video head impulse, nystagmus analysis, cervical and ocular vestibular evoked responses. She is undergoing treatment with Memantine, Clonazepam and vestibular rehabilitation, and feels improvement. CONCLUSION: This report shows a novel combination of central and peripheral vestibular findings, of relevance for diagnosis and treatment, in addition to the development of a coherent hypothesis on the ocular motor and vestibular findings in WTD.

Vestibular Test Results in Patients With Horizontal Canal Benign Paroxysmal Positional Vertigo.

Introduction While the mechanism of posterior canal benign paroxysmal positional vertigo (BPPV) is widely accepted as canalolithiasis, the pathophysiology of horizontal canal BPPV remains controversial. We seek to analyze vestibular test results of patients with horizontal canal BPPV with ageotropic nystagmus (AHC) and geotropic nystagmus (GHC) in comparison to patients with posterior canal BPPV (PC) to better understand its pathophysiology. Methods In a retrospective chart review of adults with BPPV at a tertiary referral balance center, we reviewed the clinical characteristics and compared videonystagmography, caloric, rotary chair, subjective visual vertical (SVV)/ subjective visual horizontal (SVH), and vestibular evoked myogenic potential (VEMP) results between groups. Results We included 11 AHC and seven GHC patients and randomly selected 20 PC patients as the comparison group. All groups had a high rate of migraine and low rates of diabetes and head trauma, but no difference between groups. Ipsilateral caloric weakness was more prevalent in the GHC group compared to the PC group (p=0.02). One of two AHC patients and both GHC patients who had SVV/SVH testing had abnormal findings. The only AHC patient who had ocular VEMP testing had abnormal results. Additionally, we observed a significant downbeating component to nystagmus (4 deg/sec or greater) exclusively in the AHC group (5/10 patients, p=0.001). Conclusions Patients with AHC and GHC have unique vestibular testing results. In particular, only AHC patients showed a downbeating component to their nystagmus, which may suggest utricular dysfunction in the pathophysiology of AHC.

Visually induced dizziness.

PURPOSE OF REVIEW: Visually induced dizziness (VID) is a common phenomenon in vestibular disorders of both peripheral and central causes. This article provides a review of the most updated understandings of definition, pathophysiology, and treatment options. RECENT FINDINGS: The pathophysiology is complex and its severity or persistence may be related both to the underlying cause and heritable factors. Environmental and psychological factors may influence the degree of impact of VID on daily life function. Treatment is mostly empiric at this point but includes pharmacologic, desensitization, cognitive behavioral therapies, visual rehabilitation, and treatment of the underlying cause whenever present. Additional research is needed to clarify the best management of this vestibular symptom as well as some of the other conditions with which it is commonly associated. SUMMARY: VID is a fairly common vestibular syndrome constitutng spatial disorientation without illusory motion. As it is seen in both peripheral and central vestibular disorders, it should be considered a syndrome or constellation of symptoms rather than a discrete disorder. In some cases, it may be the presenting symptom with no other clear disorder linked to it.

Nonvestibular Dizziness.

Dizziness is a common chief complaint with an extensive differential diagnosis that ranges from peripheral, central, to nonvestibular conditions. An understanding of nonvestibular conditions will aid accurate diagnosis and initiation of appropriate management. Thus, the objective of this article is to present an overview of nonvestibular etiologies that may plague a dizzy patient and the recommended treatment options.

Acute Vestibular Syndrome.

PURPOSE OF REVIEW: This article provides a practical approach to acute vestibular syndrome while highlighting recent research advances. RECENT FINDINGS: Acute vestibular syndrome is defined as sudden-onset, continuous vertigo lasting longer than 24 hours with associated nausea and vomiting, all of which are worsened with head movement. Acute vestibular syndrome is provoked by a variety of central and peripheral causes, the most common of which are vestibular neuritis and acute stroke (posterior circulation). A clinical approach focusing on timing, associated history, and ocular motor findings can improve diagnostic accuracy and is more sensitive and specific than early neuroimaging. Because of the shared neurovascular supply, both peripheral and central vestibular disorders can manifest overlapping signs previously considered solely peripheral or central, including vertical skew, nystagmus, abnormal vestibular ocular reflex, hearing loss, and gait instability. Although acute vestibular syndrome is typically benign, stroke should be considered in every person with acute vestibular syndrome because it can act as a harbinger of stroke or impending cerebellar herniation. Treatment is focused on physical therapy because the evidence is minimal for the long-term use of medication. SUMMARY: The diagnosis of acute vestibular syndrome first requires the elimination of common medical causes for dizziness. Next, underlying pathology must be determined by distinguishing between the most common causes of acute vestibular syndrome: central and peripheral vestibular disorders. Central vestibular disorders are most often the result of ischemic stroke affecting the cerebellar arteries. Peripheral vestibular disorders are assumed to be caused mostly by inflammatory sources, but ischemia of the peripheral vestibular apparatus may be underappreciated. By using the HINTS Plus (Head Impulse test, Nystagmus, Test of Skew with Plus referring to hearing loss assessment) examination in addition to a comprehensive neurologic examination, strokes are unlikely to be missed. For nearly all acute vestibular disorders, vestibular physical therapy contributes to recovery.

Acute Vestibular Syndrome and ER Presentations of Dizziness.

Acute vestibular syndrome (AVS) describes sudden onset, severe, continuous dizziness that persists for more than 24 hours. Its wide differential presents a diagnostic challenge. Vestibular neuritis is the most common cause, but stroke, trauma, medication effects, infectious, and inflammatory causes all present similarly. The TiTrATE model (Timing, Triggers, And Targeted Exam) is systematic way to evaluate these patients, and the HINTS Plus exam (Head Impulse, Nystagmus, Test of Skew, plus hearing loss) is critical in differentiating central and peripheral causes. The importance of recognizing risk factors for stroke and the role of imaging is also discussed.

Ocular Vestibular-Evoked Myogenic Potential Amplitudes Elicited at 4 kHz Optimize Detection of Superior Semicircular Canal Dehiscence.

Introduction: High-resolution temporal bone computed tomography (CT) is considered the gold standard for diagnosing superior semicircular canal dehiscence (SCD). However, CT has been shown over-detect SCD and provide results that may not align with patient-reported symptoms. Ocular vestibular-evoked myogenic potentials (oVEMPs)-most commonly conducted at 500 Hz stimulation-are increasingly used to support the diagnosis and management of SCD. Previous research reported that stimulation at higher frequencies such as 4 kHz can have near-perfect sensitivity and specificity in detecting radiographic SCD. With a larger cohort, we seek to understand the sensitivity and specificity of 4 kHz oVEMPs for detecting clinically significant SCD, as well as subgroups of radiographic, symptomatic, and surgical SCD. We also investigate whether assessing the 4 kHz oVEMP n10-p15 amplitude rather than the binary n10 response alone would optimize the detection of SCD. Methods: We conducted a cross-sectional study of patients who have undergone oVEMP testing at 4 kHz. Using the diagnostic criteria proposed by Ward et al., patients were determined to have SCD if dehiscence was confirmed on temporal bone CT by two reviewers, patient-reported characteristic symptoms, and if they had at least one positive vestibular or audiometric test suggestive of SCD. Receiver operating characteristic (ROC) analysis was conducted to identify the optimal 4 kHz oVEMP amplitude cut-off. Comparison of 4 kHz oVEMP amplitude across radiographic, symptomatic, and surgical SCD subgroups was conducted using the Mann-Whitney U test. Results: Nine hundred two patients (n, ears = 1,804) underwent 4 kHz oVEMP testing. After evaluating 150 temporal bone CTs, we identified 49 patients (n, ears = 61) who had radiographic SCD. Of those, 33 patients (n, ears = 37) were determined to have clinically significant SCD. For this study cohort, 4 kHz oVEMP responses had a sensitivity of 86.5% and a specificity of 87.8%. ROC analysis demonstrated that accounting for the inter-amplitude of 4 kHz oVEMP was more accurate in detecting SCD than the presence of n10 response alone (AUC 91 vs. 87%). Additionally, using an amplitude cut-off of 15uV reduces false positive results and improves specificity to 96.8%. Assessing 4 kHz oVEMP response across SCD subgroups demonstrated that surgical and symptomatic SCD cases had significantly higher amplitudes, while radiographic SCD cases without characteristic symptoms had similar amplitudes compared to cases without evidence of SCD. Conclusion: Our results suggest that accounting for 4 kHz oVEMP amplitude can improve detection of SCD compared to the binary presence of n10 response. The 4 kHz oVEMP amplitude cut-off that maximizes sensitivity and specificity for our cohort is 15 uV. Our results also suggest that 4 kHz oVEMP amplitudes align better with symptomatic SCD cases compared to cases in which there is radiographic SCD but no characteristic symptoms.

Vestibular Lab Testing: Interpreting the Results in the Headache Patient with Dizziness.

PURPOSE OF REVIEW: To provide an overview of vestibular lab testing and to familiarize the reader with common results observed in headache patients with dizziness. RECENT FINDINGS: The latest research indicates variable levels of both peripheral and central vestibular dysfunction in headache populations with dizziness. Mechanisms may include vestibulocerebellar loss of inhibition, central vestibular network misfiring, and peripheral pathology aggravating central hypersensitization. Headache patients are commonly affected by dizziness stemming from various etiologies. Although history is still the gold standard in diagnosis, vestibular lab testing can identify the integrity of vestibular function. Research is emerging and future directions are encouraging.

Superior Semicircular Canal Dehiscence Syndrome.

Superior canal dehiscence syndrome (SCDS) is a vestibular disorder caused by a pathologic third window into the labyrinth that can present with autophony, sound- or pressure-induced vertigo, and chronic disequilibrium among other vestibulocochlear symptoms. Careful history taking and examination in conjunction with appropriate diagnostic testing can accurately diagnose the syndrome. Key examination techniques include fixation-suppressed ocular motor examination investigating for sound- or pressure-induced eye movements in the plane of the semicircular canal. Audiometry, vestibular evoked myogenic potentials, and computed tomography confirm the diagnosis. Corrective surgical techniques can be curative, but many patients find their symptoms are not severe enough to undergo surgery. Although a primarily peripheral vestibular disorder, as first-line consultants for most dizziness complaints, neurologists will serve their patients well by understanding SCDS and its role in the differential diagnosis of vestibular disorders.

Post-transplant primary central nervous system lymphoma after Epstein-Barr virus cerebellitis.

Post-transplantation lymphoproliferative disorder (PTLD) is a complication of solid organ and hematopoietic stem cell transplantation. Cases with isolated central nervous system (CNS) disease are rare. Epstein-Barr virus (EBV) plays a causative role. We present a patient with EBV cerebellitis documented 5 months prior to development of primary CNS PTLD (PCNS-PTLD). This case report demonstrates progression from EBV CNS infection to lymphoproliferative disorder, highlighting the importance of serial clinical and imaging monitoring in transplant patients post-EBV CNS infection. PCNS-PTLD should always be considered in the differential diagnosis for transplant patients presenting with CNS symptoms, even in cases with no evidence of EBV viremia. Earlier diagnosis and appropriate treatment could result in improved outcomes.

Bilateral Vestibular Dysfunction Associated With Chronic Exposure to Military Jet Propellant Type-Eight Jet Fuel.

We describe three patients diagnosed with bilateral vestibular dysfunction associated with the jet propellant type-eight (JP-8) fuel exposure. Chronic exposure to aromatic and aliphatic hydrocarbons, which are the main constituents of JP-8 military aircraft jet fuel, occurred over 3-5 years' duration while working on or near the flight line. Exposure to toxic hydrocarbons was substantiated by the presence of JP-8 metabolite n-hexane in the blood of one of the cases. The presenting symptoms were dizziness, headache, fatigue, and imbalance. Rotational chair testing confirmed bilateral vestibular dysfunction in all the three patients. Vestibular function improved over time once the exposure was removed. Bilateral vestibular dysfunction has been associated with hydrocarbon exposure in humans, but only recently has emphasis been placed specifically on the detrimental effects of JP-8 jet fuel and its numerous hydrocarbon constituents. Data are limited on the mechanism of JP-8-induced vestibular dysfunction or ototoxicity. Early recognition of JP-8 toxicity risk, cessation of exposure, and customized vestibular therapy offer the best chance for improved balance. Bilateral vestibular impairment is under-recognized in those chronically exposed to all forms of jet fuel.