Search

BACKGROUND: Chronic rhinosinusitis frequently occurs in people with cystic fibrosis. Several medical interventions are available for treating chronic rhinosinusitis in people with cystic fibrosis; for example, different concentrations of nasal saline irrigations, topical or oral corticosteroids, antibiotics ‐ including nebulized antibiotics ‐ dornase alfa and modulators of the cystic fibrosis transmembrane conductance regulator (CFTR) (such as lumacaftor, ivacaftor or tezacaftor). However, the efficacy of these interventions is unclear. This is an update of a previously published review. OBJECTIVES: The objective of this review is to compare the effects of different medical interventions in people diagnosed with cystic fibrosis and chronic rhinosinusitis. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and hand searching of journals and conference abstract books. Date of last search of trials register: 09 September 2021. We also searched ongoing trials databases, other medical databases and the reference lists of relevant articles and reviews. Date of latest additional searches: 22 November 2021. SELECTION CRITERIA: Randomized and quasi‐randomized trials of different medical interventions compared to each other or to no intervention or to placebo. DATA COLLECTION AND ANALYSIS: Two review authors independently assessed trials identified for potential inclusion in the review. We planned to conduct data collection and analysis in accordance with Cochrane methods and to independently rate the quality of the evidence for each outcome using the GRADE guidelines. MAIN RESULTS: We identified no trials that met the pre‐defined inclusion criteria. The most recent searches identified 44 new references, none of which were eligible for inclusion in the current version of this review; 12 studies are listed as excluded and one as ongoing. AUTHORS' CONCLUSIONS: We identified no eligible trials assessing the medical interventions in people with cystic fibrosis and chronic rhinosinusitis. High‐quality trials are needed which should assess the efficacy of different treatment options detailed above for managing chronic rhinosinusitis, preventing pulmonary exacerbations and improving quality of life in people with cystic fibrosis.

Despite progress in vestibular research in the last twenty years, much remains poorly understood about vestibular pathophysiology and its management. A shared language is a critical first step in understanding vestibular disorders and is under development. Telehealth will continue for patients with dizziness, and ambulatory monitoring of nystagmus will become a diagnostic tool. In the next two decades, we anticipate that vestibular perceptual threshold testing will become common in tertiary centers, that imaging with improved spatial resolution will yield better understanding of vestibular pathophysiology, and that vestibular implants will become a part of clinical practice.

Objective To perform a systematic review to determine if a total score of ≥14.5 (mean score ≥ 2.1) on the ETDQ‐7 accurately identifies patients with obstructive Eustachian tube dysfunction (OETD) on impedance tympanometry (peak compliance

Tympanic membrane (TM) perforations can occur at any age, but limited population-level data are available. Using data from the National Health and Nutrition Examination Survey, we performed a cross-sectional analysis of the prevalence and population estimates for TM perforations among individuals ≥12 years old in the United States. Overall, TM perforations have a prevalence of 2.1% (95% CI, 1.7%-2.6%), corresponding to 5.8 million Americans. Across the life course, older adults have the highest prevalence of TM perforations at 6.1% (95% CI, 4.7%-7.6%), corresponding to nearly 3 million Americans, as opposed to a prevalence of 0.6% (95% CI, 0.3%-0.9%) in adolescents, which equates to 0.2 million Americans. Males and females have a similar prevalence at 2.3% (95% CI, 1.6%-3.0%) among males and 2.0% (95% CI, 1.4%-2.6%) among females. These prevalence and population estimates provide the first US-based population estimates of the burden of TM perforations over the life course.

Objective To describe the procedure and results of an adapted closure and reconstruction technique for translabyrinthine surgery that focuses on identifying and managing potential pathways for CSF egress to the middle ear and Eustachian tube. Methods Retrospective review of a cohort of translabyrinthine acoustic neuroma cases that were reconstructed using this technique. Results In addition to meticulous packing of potential conduits using soft tissue, hydroxyapatite cement is used to seal opened air cell tracts prior to obliteration of the mastoid defect using adipose tissue. Early results of a small patient cohort using this technique are encouraging and there were no wound infections. There was a single case of CSF rhinorrhea associated with incomplete sealing of opened petrous apex cells, with no recurrence after appropriate implementation of the described protocol during revision surgery. Conclusion Proactive management of potential conduits of CSF egress including opened air cell tracts has a high likelihood of reducing rates of rhinorrhea and need for revision surgery after the translabyrinthine approach to the posterior fossa.

A 28-year-old man with congenital hypogonadotropic hypogonadism (CHH) was found to be heterozygous for the GNRH1 p.R31C mutation, reported in the literature as pathogenic and dominant. The same mutation was found in his son at birth, but the testing of the infant at 64 days confirmed the hormonal changes associated with minipuberty. This led to further genetic sequencing of the patient and his son, which found a second variant, AMHR2 p.G445_L453del, in the heterozygous form, reported as pathogenic in the patient but not in his son. This suggests a digenic cause of the patient’s CHH. Together, these mutations are postulated to contribute to CHH by the lack of anti-Müllerian hormone (AMH) signalling, leading to the impaired migration of gonadotrophin releasing hormone (GnRH) neurons, the lack of the AMH effect on GnRH secretion, and altered GnRH decapeptide with reduced binding to GnRH receptors. This led us to the conclusion that the observed GNRH1 mutation in the heterozygous state is not certain to be dominant or, at least, exhibits incomplete penetrance and variable expressivity. This report also emphasises the opportunity afforded by the time window of minipuberty in assessing the inherited genetic disorders of hypothalamic function.

Background Bilateral vestibular hypofunction is associated with chronic disequilibrium, postural instability, and unsteady gait owing to failure of vestibular reflexes that stabilize the eyes, head, and body. A vestibular implant may be effective in alleviating symptoms. Methods Persons who had had ototoxic (7 participants) or idiopathic (1 participant) bilateral vestibular hypofunction for 2 to 23 years underwent unilateral implantation of a prosthesis that electrically stimulates the three semicircular canal branches of the vestibular nerve. Clinical outcomes included the score on the Bruininks-Oseretsky Test of Motor Proficiency balance subtest (range, 0 to 36, with higher scores indicating better balance), time to failure on the modified Romberg test (range, 0 to 30 seconds), score on the Dynamic Gait Index (range, 0 to 24, with higher scores indicating better gait performance), time needed to complete the Timed Up and Go test, gait speed, pure-tone auditory detection thresholds, speech discrimination scores, and quality of life. We compared participants' results at baseline (before implantation) with those at 6 months (8 participants) and at 1 year (6 participants) with the device set in its usual treatment mode (varying stimulus pulse rate and amplitude to represent rotational head motion) and in a placebo mode (holding pulse rate and amplitude constant). Results The median scores at baseline and at 6 months on the Bruininks-Oseretsky test were 17.5 and 21.0, respectively (median within-participant difference, 5.5 points; 95% confidence interval [CI], 0 to 10.0); the median times on the modified Romberg test were 3.6 seconds and 8.3 seconds (difference, 5.1; 95% CI, 1.5 to 27.6); the median scores on the Dynamic Gait Index were 12.5 and 22.5 (difference, 10.5 points; 95% CI, 1.5 to 12.0); the median times on the Timed Up and Go test were 11.0 seconds and 8.7 seconds (difference, 2.3; 95% CI, -1.7 to 5.0); and the median speeds on the gait-speed test were 1.03 m per second and 1.10 m per second (difference, 0.13; 95% CI, -0.25 to 0.30). Placebo-mode testing confirmed that improvements were due to treatment-mode stimulation. Among the 6 participants who were also assessed at 1 year, the median within-participant changes from baseline to 1 year were generally consistent with results at 6 months. Implantation caused ipsilateral hearing loss, with the air-conducted pure-tone average detection threshold at 6 months increasing by 3 to 16 dB in 5 participants and by 74 to 104 dB in 3 participants. Changes in participant-reported disability and quality of life paralleled changes in posture and gait. Conclusions Six months and 1 year after unilateral implantation of a vestibular prosthesis for bilateral vestibular hypofunction, measures of posture, gait, and quality of life were generally in the direction of improvement from baseline, but hearing was reduced in the ear with the implant in all but 1 participant. (Funded by the National Institutes of Health and others; ClinicalTrials.gov number, NCT02725463.).

This paper describes the diagnostic criteria for superior semicircular canal dehiscence syndrome (SCDS) as put forth by the classification committee of the Bárány Society. In addition to the presence of a dehiscence of the superior semicircular canal on high resolution imaging, patients diagnosed with SCDS must also have symptoms and physiological tests that are both consistent with the pathophysiology of a ‘third mobile window’ syndrome and not better accounted for by another vestibular disease or disorder. The diagnosis of SCDS therefore requires a combination of A) at least one symptom consistent with SCDS and attributable to ‘third mobile window’ pathophysiology including 1) hyperacusis to bone conducted sound, 2) sound-induced vertigo and/or oscillopsia time-locked to the stimulus, 3) pressure-induced vertigo and/or oscillopsia time-locked to the stimulus, or 4) pulsatile tinnitus; B) at least 1 physiologic test or sign indicating that a ‘third mobile window’ is transmitting pressure including 1) eye movements in the plane of the affected superior semicircular canal when sound or pressure is applied to the affected ear, 2) low-frequency negative bone conduction thresholds on pure tone audiometry, or 3) enhanced vestibular-evoked myogenic potential (VEMP) responses (low cervical VEMP thresholds or elevated ocular VEMP amplitudes); and C) high resolution computed tomography (CT) scan with multiplanar reconstruction in the plane of the superior semicircular canal consistent with a dehiscence. Thus, patients who meet at least one criterion in each of the three major diagnostic categories (symptoms, physiologic tests, and imaging) are considered to have SCDS.

SUMMARY: Analogous to hearing restoration via cochlear implants, vestibular function could be restored via vestibular implants that electrically stimulate vestibular nerve branches to encode head motion. This study presents the technical feasibility and first imaging results of CT for vestibular implants in 8 participants of the first-in-human Multichannel Vestibular Implant Early Feasibility Study. Imaging characteristics of 8 participants (3 men, 5 women; median age, 59.5 years; range, 51–66 years) implanted with a Multichannel Vestibular Implant System who underwent a postimplantation multislice CT (n = 2) or flat panel CT (n = 6) are reported. The device comprises 9 platinum electrodes inserted into the ampullae of the 3 semicircular canals and 1 reference electrode inserted in the common crus. Electrode insertion site, positions, length and angle of insertion, and number of artifacts were assessed. Individual electrode contacts were barely discernible in the 2 participants imaged using multislice CT. Electrode and osseous structures were detectable but blurred so that only 12 of the 18 stimulating electrode contacts could be individually identified. Flat panel CT could identify all 10 electrode contacts in all 6 participants. The median reference electrode insertion depth angle was 9° (range, −57.5° to 45°), and the median reference electrode insertion length was 42 mm (range, −21−66 mm). Flat panel CT of vestibular implants produces higher-resolution images with fewer artifacts than multidetector row CT, allowing visualization of individual electrode contacts and quantification of their locations relative to vestibular semicircular canals and ampullae. As multichannel vestibular implant imaging improves, so will our understanding of the relationship between electrode placement and vestibular performance.

Peripheral vestibular disorders are caused by pathology of the inner ear. The majority of these disorders are diagnosed with a detailed history and vestibular physical exam. Imaging is rarely a part of diagnostic work up as the pathologies of these disorders are "invisible," or undetectable on imaging. However, these "invisible" diagnoses are becoming increasingly visible with advancements in imaging technology. Developments in magnetic resonance imaging are allowing for increased spatial resolution and better image contrast, improving our ability to see soft tissue structures including the membranous labyrinth, sensory epithelia and nerves. With these improvements in imaging, clinicians will be able to understand better atypical presentations of peripheral vestibular disorders, disease intractable to traditional therapy, disorders with unclear pathoetiology and disease only seen on histopathological studies. This review assesses the current state of imaging in the neurotology clinic with a special focus on magnetic resonance imaging and then gathers diseases identified by vestibular testing and histopathological studies that could be better understood with developments in imaging. In doing so, we hope to guide advancement in neurotologic imaging and apprise clinicians of the utility of imaging in peripheral vestibular disease diagnosis.

The Johns Hopkins Otologic Research Laboratory was founded in 1924 as the first human temporal bone laboratory within the United States. To better understand the contributions of the Johns Hopkins Otologic Research Laboratory to our understanding of presbycusis, we consulted with a medical librarian and archivist to search the Alan Mason Chesney Medical Archives, PubMed, JSTOR, and Johns Hopkins Bulletin for published and unpublished works from the lab. Between 1924 and 1938, Samuel J. Crowe, the Chairman of Otolaryngology, and anatomist Stacy R. Guild amassed a collection of ∼1,800 temporal bones. This collection allowed for an unprecedented period of discovery related to otologic disease. They combined hearing thresholds measured by the recently invented audiometer with new techniques for temporal bone decalcification, sectioning, and staining, and a method for the graphic reconstruction of the cochlea. Crowe and Guild used this unique opportunity to correlate otopathology with hearing and to make the first detailed descriptions of the otopathology of presbycusis. In 1931 and 1934, they observed spiral ganglion neuron and outer hair cell loss in the basal turn of the cochlea in individuals with high-frequency hearing loss. These were the first studies to reveal that stria vascularis degeneration and middle ear pathology were not the most common causes for high-frequency hearing loss. Aside from revealing the primary driving factors of presbycusis, this work provided insight into the tonotopic organization of the cochlea. After initially being recruited to help raise money for the laboratory, medical illustrator Max Brödel used the vertical histologic cross-sections of the cochlea to produce illustrations of the ear. The decision to produce histologic sections in the plane of the superior semicircular canal likely influenced Brödel's illustrations that share a similar orientation and would later become widely circulated. Significant contributions from the Otologic Research Laboratory were also made by Mary Hardy, D.Sc., a woman who has previously received little recognition for her work. The sectioning of temporal bones was stopped in 1938 due to World War II, but much of Crowe's and Guild's work continued into the 1940s until a rift between the two resulted in the temporary closure of the laboratory in 1949. Nearly 100 years after its founding, discoveries from the Johns Hopkins Otologic Research Laboratory remain relevant and emphasize the importance of continued human temporal bone research to improve our understanding and treatment of otologic disease.

Identification of variants in the calcium-sensing receptor (CASR) gene is an important means of distinguishing between familial hypocalciuric hypercalcaemia (FHH) and primary hyperparathyroidism. However, identification and bioinformatics analysis of genetic variants alone is now considered insufficient as definitive proof; additional functional assessment is required to diagnose FHH with certainty. We identified two novel variants, D433Y and C739Y, and one previously reported variant G509R in the CASR of four kindreds provisionally diagnosed with FHH and aimed to functionally characterise these variants to confirm the diagnosis. Variant receptors were cloned as FLAG-tagged constructs into the mammalian expression vector, pcDNA3.1. Wild type and variant receptor constructs were expressed in HEK293 cells and their expression assessed by Western blot analysis and their functionality analysed using an IP-One assay which measures myo-inositol 1-phosphate accumulation following CaSR activation. Western blot analysis showed that the D433Y receptor had diminished mature glycosylated receptor compared with wild type CaSR whereas the G509R receptor had a complete lack of mature receptor. The C739Y receptor was consistently overexpressed. Functional assessment showed the D433Y receptor to be mildly inactivating at physiological calcium concentrations whereas the G509R receptor was inactive at all calcium concentrations. By contrast, the C739Y variant was activating compared to wild type receptor which is inconsistent with it causing FHH. We conclude that functional assessment of CaSR variants using the IP-One assay was useful in the investigation of suspected FHH probands, confirming the D433Y and G509R variants as likely pathogenic/pathogenic, but dismissing the C739Y variant as causing FHH.

Facial nerve tumors within the temporal bone present several surgical challenges due to the tortuous course of the facial nerve and the nerve’s close relationship to other important structures. Surgical approaches often have either sacrificed hearing/vestibular function or involved brain retraction. We present a case of a patient who was diagnosed with a facial nerve schwannoma (House-Brackmann IV/VI). Magnetic resonance imaging (MRI) showed the tumor was limited to the middle ear. The patient had already undergone facial reanimation procedures and elected to have the tumor removed. A transmastoid trans-facial canal surgical approach was used to remove the tumor without disturbing the ossicular chain. The patient’s hearing and vestibular function remained intact. Operating from within the facial canal provides the surgeon additional room to dissect facial nerve tumors from the middle ear to the geniculate ganglion when using a transmastoid approach. This surgical approach is similar to the previously described transmastoid/supralabyrinthine approach to excise facial nerve tumors within the temporal bone but modified to keep the ossicles intact. While the described approach has a limited application, in certain cases of facial nerve tumors within the temporal bone when surgery is warranted, a transmastoid trans-facial canal approach may have advantages over previously-described approaches.

Context In the clinic it is important to differentiate primary hyperparathyroidism (PHPT) from the more benign, inherited disorder, familial hypocalciuric hypercalcemia (FHH). Since the conditions may sometimes overlap biochemically, identification of calcium-sensing receptor (CASR) gene variants causative of FHH (but not PHPT) is the most decisive diagnostic aid. When novel variants are identified, bioinformatics and functional assessment are required to establish pathogenicity. Objective We identified 3 novel CASR transmembrane domain missense variants, Thr699Asn, Arg701Gly, and Thr808Pro, in 3 probands provisionally diagnosed with FHH and examined the variants using bioinformatics and functional analysis. Methods Bioinformatics assessment utilized wANNOVAR software. For functional characterization, each variant was cloned into a mammalian expression vector; wild-type and variant receptors were transfected into HEK293 cells, and their expression and cellular localization were assessed by Western blotting and confocal immunofluorescence, respectively. Receptor activation in HEK293 cells was determined using an IP-One ELISA assay following stimulation with Ca++ ions. Results Bioinformatics analysis of the variants was unable to definitively assign pathogenicity. Compared with wild-type receptor, all variants demonstrated impaired expression of mature receptor reaching the cell surface and diminished activation at physiologically relevant Ca++ concentrations. Conclusion Three CASR missense variants identified in probands provisionally diagnosed with FHH result in receptor inactivation and are therefore likely causative of FHH. Inactivation may be due to inadequate processing/trafficking of mature receptor and/or conformational changes induced by the variants affecting receptor signaling. This study demonstrates the value of functional studies in assessing genetic variants identified in hypercalcemic patients.

Objectives: Hypothesized causes of vestibular neuritis/labyrinthitis include neuroinflammatory or vascular disorders, yet vascular disorders of the inner ear are poorly understood. Guided by known microvascular diseases of the retina, we developed 2 hypotheses: (1) there exist vascular vulnerabilities of artery channels in cases of hypothetical nerve swelling for the superior, inferior, and vestibulocochlear artery and (2) there are arteriovenous crossings that could compromise vascular flow in disease states. Methods: Two fully mounted and stained temporal bones were used to render three-dimensional reconstructions of the labyrinth blood supply. Using these maps, areas of potential vascular compression were quantified in 50 human temporal bones. Results: Although inner ear arteries and veins mostly travel within their own bony channels, they may be exposed (1) at the entrance into the otic capsule, and (2) where the superior vestibular vein crosses the inferior vestibular artery. At the entry into the otic capsule, the ratio of the soft tissue to total space for the superior vestibular artery was significantly greater than the inferior vestibular artery/cochleovestibular artery (median 44, interquartile range 34–55 vs. 14 [9–17], p < 0.0001). Conclusions: Three-dimensional reconstruction of human temporal bone histopathology can guide vascular studies of the human inner ear. Studies of retinal microvascular disease helped identify areas of vascular vulnerability in cases of hypothetical nerve swelling at the entrance into the otic capsule and at an arteriovenous crossing near the saccular macula. These data may help explain patterns of clinical findings in peripheral vestibular lesions.

Background: A clinical pattern of damage to the auditory, visual, and vestibular sensorimotor systems, known as multi-sensory impairment, affects a significant percent of the US population. Within the population of US military service members exposed to mild traumatic brain injury (mTBI), 15 – 44% will develop multi-sensory impairment following a mild traumatic brain injury. In the US civilian population, multi-sensory impairment related symptoms are also a common sequela of damage to the vestibular system and affect ~ 300-500/100,000 population. Vestibular rehabilitation is recognized as a critical component of the management of multi-sensory impairment. Unfortunately, the current clinical practice guidelines for the delivery of vestibular rehabilitation are not evidence-based and primarily rely on expert opinion. The focus of the INVENT VPT trial is gaze stability training, which represents the unique component of vestibular rehabilitation. The aim of the INVENT VPT trial is to assess the efficacy of a non-invasive, incremental vestibular adaptation training device for normalizing the response of the vestibulo-ocular reflex. Methods: The INVENT VPT trial is a multicenter randomized controlled crossover trial in which military service members with mTBI and civilian patients with vestibular hypofunction are randomized to begin traditional vestibular rehabilitation or incremental vestibular adaptation and then cross over to the alternate intervention after a prescribed washout period. Vestibulo-ocular reflex function and other functional outcomes are measured to identify the best means to improve delivery of vestibular rehabilitation. We incorporate ecologically valid outcome measures that address the common symptoms experienced in those with vestibular pathology and multi-sensory impairment. Discussion: The INVENT VPT Trial will directly impact the health care delivery of vestibular rehabilitation in patients suffering from multi-sensory impairment in three critical ways: 1) Compare optimized traditional methods of vestibular rehabilitation to a novel device that is hypothesized to improve vestibulo-ocular reflex performance; 2) Isolate the ideal dosing of vestibular rehabilitation considering patient burden and compliance rates; 3) Examine whether recovery of the vestibulo-ocular reflex can be predicted in participants with vestibular symptoms. Trial registration: ClinicalTrials.gov, NCT03846830. Registered 20 February 2019,https://clinicaltrials.gov/ct2/show/NCT03846830

Objectives To compare hearing outcomes between traditional microsurgical transmastoid repairs and underwater, endoscopic transmastoid repairs of superior semicircular canal dehiscence syndrome (SCDS). Methods A retrospective review of 14 patients undergoing transmastoid repair of SCDS between 2013 and 2017. Preoperative, immediate (7-10 d) postoperative, and long-term (>6 wk) postoperative speech discrimination scores, pure-tone averages, as well as pure-tone air conduction and bone conduction thresholds were obtained at 250 Hz, 500 Hz, 1000 Hz, 2000 Hz, 4000 Hz, and 8000 Hz. Hearing threshold changes were all calculated as preoperative thresholds subtracted from postoperative threshold at each frequency. Wilcoxon rank-sum tests were conducted to assess changes in postoperative hearing thresholds at both the immediate and long-term postoperative period between those undergoing the traditional transmastoid technique and the underwater, endoscopic transmastoid technique. Results Our results demonstrate patients who underwent underwater transmastoid repairs had significantly less increase in their high-frequency bone conduction thresholds at both time periods, indicating less sensorineural hearing loss, compared to the traditional microscopic repair (p = 0.02 [effect size, r = 0.4], p = 0.006 [r = 0.6]). At long-term follow-up, there was also significantly improved pure-tone averages in the patients in the underwater repair cohort compared to the traditional group (p = 0.004 [r = 0.6]). There were no differences in air-bone gaps at any frequency or any time period between the two cohorts. Conclusions Preliminary findings show that the underwater endoscopic technique may prevent sensorineural hearing loss during transmastoid repair of SCDS.

OBJECTIVES: The aim of the present study was to investigate current treatment practices and self-reported effectiveness in Meniere’s disease. MATERIALS and METHODS: Members of two Italian Meniere’s disease support (n=170) with ≥6-month history of Meniere’s disease were administered an online survey about recent treatments. Vertigo episode count, work absenteeism, and limitations in family life, social life, work, or travel as included in the Social Life and Work Impact of Dizziness Questionnaire before and after recent treatments were queried. RESULTS: Twenty-four different treatments were reported for Meniere’s disease, with dietary modifications (55%), diuretics (47%), and betahistine (41%) being the most common. The majority (71%) received multiple simultaneous treatments. Prior to the most recent treatments, 78%-89% of respondents indicated limitations in family or social life, work, or traveling. After their most recent treatment, respondents reported improvements in mean vertigo episode counts (5.7±7.6 vs. 2.6±4.6, p

Background:According to population-based studies that estimate disease prevalence, the majority of patients evaluated at dizziness clinics receive a single vestibular diagnosis. However, accumulating literature supports the notion that different vestibular disorders are interrelated and often underdiagnosed.Objective:Given the complexity and richness of these interrelations, we propose that a more inclusive conceptual framework to vestibular diagnostics that explicitly acknowledges this web of association will better inform vestibular differential diagnosis.Methods:A narrative review was performed using PubMed database. Articles were included if they defined a cohort of patients, who were given specific vestibular diagnosis. The interrelations among vestibular disorders were analyzed and placed within a conceptual framework.Results:The frequency of patients currently receiving multiple vestibular diagnoses in dizziness clinic is approximately 3.7% (1263/33 968 patients). The most common vestibular diagnoses encountered in the dizziness clinic include benign paroxysmal positional vertigo (BPPV), vestibular migraine, vestibular neuritis, and Ménière’s disease.Conclusions:A review of the literature demonstrates an intricate web of interconnections among different vestibular disorders such as BPPV, vestibular migraine, Ménière’s disease, vestibular neuritis, bilateral vestibulopathy, superior canal dehiscence syndrome, persistent postural perceptual dizziness, anxiety, head trauma, and aging, among others.

Hypothesis Magnetic vestibular stimulation (MVS) elicits nystagmus in C57BL/6J mice but not head tilt mice lacking Nox3, which is required for normal otoconial development. Background Humans have vertigo and nystagmus in strong magnetic fields within magnetic resonance imaging machines. The hypothesized mechanism is a Lorentz force driven by electrical current entering the utricular neuroepithelium, acting indirectly on crista hair cells via endolymph movement deflecting cupulae. We tested an alternate hypothesized mechanism: Lorentz action directly on crista hair cell stereocilia, driven by their currents independent of the utricle. Methods Before MVS, vestibulo-ocular reflex responses of eight C57BL/6J mice and six head tilt mice were measured during whole-body sinusoidal rotations and tilts using video-oculography. Mice were then placed within a 4.7 Tesla magnetic field with the horizontal semicircular canals approximately Earth-horizontal for ≥1 minute in several head orientations, while eye movements were recorded via infrared video in darkness. Results Outside the magnet, both C57BL/6J and head tilt mice had intact horizontal vestibulo-ocular reflex, but only C57BL/6J mice exhibited static counter-roll responses to tilt (normal utiruclo-ocular reflex). When placed in the magnet nose-first, C57BL/6J mice had left-beating nystagmus, lasting a median of 32.8 seconds. When tail-first, nystagmus was right-beating and similar duration (median 28.0 s, p > 0.05). In contrast, head tilt mice lacked magnetic field-induced nystagmus (p Conclusions C57BL/6J mice generate nystagmus in response to MVS, while mice deficient in Nox3 do not. This suggests 1) a normal utricle is necessary, and 2) functioning semicircular canals are insufficient, to generate MVS-induced nystagmus in mice.

PURPOSE OF THE REVIEW: The goal of this review is to highlight current approaches to diagnosis and treatment for adult-onset hearing loss in patients likely to present to a neurologist’s office. The review will discuss primary and secondary causes of acute and chronic hearing loss, and will discuss common situations that can be managed by a neurologist as well as situations that require immediate care and referral for further management by an otolaryngologist-head and neck surgeon. RECENT FINDINGS: Hearing screening assessments using mobile applications and tablet devices are now available and can be integrated into many clinical practice settings, including in the evaluation of hearing concerns related to various neurological pathologies. For patients presenting with a sudden worsening in hearing, bedside evaluation, including with objective measures of hearing, can inform neurologists about diagnosis and subsequent management. For patients who present with gradual worsening in hearing, particularly those related to neurologic disorders, hearing care can be an important adjunct to ongoing neurologic care. More commonly encountered, age-related hearing loss is highly prevalent among older adults and may affect overall neurological assessment, including neurocognitive testing, as well as patient-provider communication, patient satisfaction, and care outcomes. Hearing loss is increasingly recognized as a potentially modifiable risk factor for dementia. Neurologists can support the hearing health of their patients through the routine use of communication strategies and by integrating simple, low-cost technology with their current clinical practices. SUMMARY: Both acute and chronic hearing loss can be a symptom of many conditions managed by neurologists. Few conditions are emergent, requiring immediate referral to and treatment by an otolaryngologist-head and neck surgeon. Despite the range of hearing interventions available, including hearing aids, over-the-counter devices, and aural rehabilitation, hearing loss is a common and under-treated chronic health condition. By promptly addressing a patient’s hearing concerns, neurologists can improve patients’ awareness of the deficit and support the overall importance of maintaining sensory health across the life course.

OBJECTIVE To measure the vestibular nerve bony channels, applying a 3D measurement to account for the oblique trajectory of the singular nerve. BACKGROUND The clinical syndrome vestibular neuritis affects structures innervated by the superior vestibular nerve more commonly than the inferior vestibular nerve. Anatomical differences such as a longer, narrower bony channel of the superior vestibular nerve may increase its susceptibility to entrapment. MAIN OUTCOME MEASURES Length of the narrow segment of each vestibular nerve in which the nerve occupies more than 80% of the bony channel was measured. RESULTS Forty six normal ears sectioned in the axial plane were measured. The narrow channel for the lateral semicircular canal (SCC, mean [SD] 2.94 ± 0.54) mm was longer than that of the singular nerve innervating the posterior SCC (1.95 ± 0.58 mm [p

Objectives: To report a case of profound bilateral sensorineural hearing and vestibular loss from relapsing polychondritis and hearing outcomes after cochlear implantation. Methods: Case report and literature review. Results: A 43 year-old woman developed sudden loss of hearing and balance that progressed over several weeks to bilateral, profound hearing and vestibular loss. Steroid treatments were ineffective. She underwent vestibular physical therapy and left cochlear implantation. About 10 months after her initial presentation, she developed erythema, warmth, swelling, and pain of the left auricle sparing the lobule, flattening of the bridge of her nose, and right ankle swelling, warmth, and skin erythema. A biopsy of the left auricle revealed histopathologic findings consistent with relapsing polychondritis. She was treated with high dose prednisolone. The ear inflammation resolved, however, despite excellent auditory response to pure tone thresholds, the patient reported no improvement in speech perception after cochlear implantation. Conclusions: Relapsing polychondritis can present with rapidly progressive, profound loss of hearing and vestibular function. Hearing outcomes after cochlear implantation can include poor speech discrimination despite good pure tone detection thresholds.

Introduction Discordant thyroid function tests are routinely encountered in clinical practice. Differential diagnoses include acute thyroxine (T4) ingestion, laboratory interference from heterophilic antibodies, thyroid hormone resistance, thyroid-stimulating hormone (TSH)-secreting pituitary adenomas, and T4 protein binding abnormalities. The impact of abnormal binding proteins may be less recognized since widespread use of free T4 (FT4) assays compared to older total T4 assays. Case report A 69-year-old female was referred for assessment of discordant thyroid function tests. Biochemistry since July 2015 showed persistently elevated FT4 levels by immunoassay ranging between 25 to 34 pmol/L with normal or slightly decreased TSH ranging between 0.05 to 2.74 mU/L. The patient was clinically euthyroid on 100 mcg daily of levothyroxine for Hashimoto’s thyroiditis. FT4 measured using liquid chromatography-tandem mass spectrometry (LC-MS/MS) was 19.5 pmol/L. Exome sequencing (confirmed by Sanger sequencing) detected a guanine to adenine substitution at residue 725 of the ALB gene previously associated with dysalbuminemic hyperthyroxinemia. The patient’s daughter had similar thyroid function tests and the same genetic variant. FT4 results from 3 different automated immunoassays showed the Roche Cobas and Siemens Centaur platforms to be most affected by the variant, and Abbott Architect had the best agreement with LC-MS/MS. Conclusion Familial dysalbuminemic hyperthyroxinemia is a potential cause of discordant thyroid function tests. Clinicians suspecting protein-binding abnormalities may further investigate using reference methods such as LC-MS/MS and equilibrium dialysis if available. The increasing accessibility of exome sequencing offers a cost-effective method of diagnosing genetic variants that cause discordant thyroid function tests.

INTRODUCTION Superior canal dehiscence syndrome (SCDS) is characterized by a defect in the bone overlying the superior semicircular canal. This third mobile window generates an abnormal low-impedance pathway for sound/pressure transmission into the inner ear resulting in the characteristic symptoms. OBJECTIVE To perform a prospective analysis of symptoms in patients with SCDS. METHODS An aggregated symptom set was studied prospectively and compared with a healthy control group. The 31 items inquired about the presence and severity of symptoms. Initial comparison between both groups was performed using the Kolmogorov-Smirnov test and nonparametric Mann-Whitney U test. For analysis of the internal consistency, the Cronbach's alpha coefficient was determined. Finally, the prevalence of symptoms obtained in the present study was compared to the prevalence of symptoms obtained as part of a recent retrospective systematic review. RESULTS Responses from the 29 patients with SCDS in the case group and the 58 healthy controls were not normally distributed. Those with SCDS had a higher prevalence and more severe symptoms for almost all items. The Cronbach's alpha coefficient of 0.969 indicates an excellent internal consistency. The prospective prevalence of most symptoms was higher than the retrospective prevalence reported earlier. CONCLUSIONS SCDS causes a variety of vestibular and auditory symptoms. This prospective study demonstrates that their prevalence and severity are higher than reported previously. This symptom set demonstrates excellent internal consistency and will serve as a foundation toward developing a disease-specific patient-reported outcome measure for SCDS. LEVEL OF EVIDENCE NA.