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Air-bone gaps (ABGs) are commonly found in patients with conductive or mixed hearing loss generally due to outer- and/or middle-ear diseases such as otitis externa, tympanic membrane perforation, interruption or fixation of the ossicular chain, and chronic suppurative otitis media. ABGs can also be found in correlation with inner-ear disorders, such as endolymphatic hydrops, enlarged vestibular aqueduct syndrome, semicircular canal dehiscence, gusher syndrome, cochlear dehiscence, and Paget disease's as well cerebral vascular anomalies including dural arteriovenous fistula. The typical clinical presentation of inner-ear conditions or cerebral vascular anomalies causing ABGs includes audiological and vestibular symptoms like vertigo, oscillopsia, dizziness, imbalance, spinning sensation, pulsatile or continuous tinnitus, hyperacusis, autophony, auricular fullness, Tullio's phenomenon, and Hennebert's sign. Establishing a definitive diagnosis of the underlying condition in patients presenting with an ABG is often challenging to do and, in many patients, the condition may remain undefined. Results from an accurate clinical, audiological, and vestibular evaluation can be suggestive for the underlying condition; however, radiological assessment by computed tomography and/or magnetic resonance imaging is mandatory to confirm any diagnostic suspicion. In this review, we describe and discuss the most recent updates available regarding the clinical presentation and diagnostic workup of inner-ear conditions that may present together with ABGs. [ABSTRACT FROM AUTHOR]

Introduction: Vertigo and disequilibrium are common symptoms in idiopathic Parkinson's disease (PD) and in Multiple System Atrophy (MSA). Hearing loss has been recently recognized as an additional non-motor feature in PD. The aim of this study is to evaluate audio-vestibular function in patients affected by PD and MSA.Methods: Fifteen patients with PD, 16 patients with MSA and 20 age-matched healthy controls (HC) were enrolled. Audio-vestibular examination included pure-tone audiometry (PTA), vestibular bed-side examination, video Head Impulse Test (vHIT), and cervical Vestibular-Evoked Myogenic Potentials (cVEMPs).Results: PD and MSA patients showed worse PTA thresholds compared to HC at high frequencies. MSA patients showed worse PTA thresholds at 125 Hz compared to HC. In patients with PD, a direct correlation between disease duration and PTA thresholds was found at 2000 Hz and 4000 Hz. In patients with MSA, disease duration was directly related to PTA thresholds at 125 Hz and 250 Hz. Among PD patients, cVEMPs were absent bilaterally in 46.7% and unilaterally in 13.3% of the subjects. Among MSA patients, cVEMPs were absent bilaterally in 26.7% and unilaterally in 40% of the subjects; p13 latency was significantly increased in PD patients as compared to HC. A significant inverse relationship was found between disease duration and cVEMP amplitude in MSA patients.Conclusion: We found that high-frequency hearing loss and cVEMP abnormalities are frequent features of both MSA and PD, suggesting that an audio-vestibular dysfunction may be present in these patients even in the absence of self-reported auditory or vestibular symptoms. [ABSTRACT FROM AUTHOR]

In recent years, there has been increasing research interest in the correlation between hearing impairment and cognitive decline, two conditions that have demonstrated a strong association. Hearing loss appears as a risk factor for cognitive impairment, especially among certain populations, notably nursing home residents. Furthermore, hearing loss has been identified as a modifiable age-related condition linked to dementia, and it has been estimated that midlife hearing loss, if eliminated, might decrease the risk of dementia in the general population. Several mechanisms have been suggested to explain the pathologic connections between hearing loss and dementia; however, clear evidence is missing, and the common pathophysiological basis is still unclear. In this review, we discussed current knowledge about the relationship between hearing loss and dementia, and future perspectives in terms of the effects of hearing rehabilitation for early prevention of cognitive decline. [ABSTRACT FROM AUTHOR]

Purpose: Most ELST data in the literature are case studies or limited to small cohorts (< 16 patients). We evaluated the main clinical signs observed at endolymphatic sac tumor (ELST) diagnosis in patients with or without Von Hippel-Lindau disease. Methods: We conducted a comprehensive literature search in PubMed, Scopus, and Web of Science. We included studies with at least 1 patient, of any age, affected by sporadic or VHL-related ELSTs reporting levels of hearing loss and facial nerve function and a comprehensive description of presenting symptoms at ELST diagnosis. We combined data for proportional meta-analysis. p values of 0.05 were considered statistically significant. Methodological quality was evaluated. Analyses were performed with MedCalc 14.8.1 software. Results: A total of 26 studies, including 113 patients and 118 cases of ELSTs were included. Pooled proportion rates (95% CI) of overall hearing loss was 88.7%, (82.4–93.4), severe hearing loss was 21.6% (12.8–32.1) profound hearing loss was 39.8% (28.7–51.5), vertigo/imbalance was 42.0% (33.8–50.5), tinnitus was 61.8% (53.4–69.8) and facial nerve palsy was 30.6% (23.2–38.9). Generally, symptoms were homogeneous or moderately heterogeneous among included studies. Conclusion: This is the first systematic review of clinical presentations at ELST diagnosis. The most serious clinical events include profound hearing loss and facial impairment. Fluctuating hearing loss, tinnitus and vertigo are frequently reported and may confound correct and prompt ELST diagnosis. [ABSTRACT FROM AUTHOR]

Age-related sensorineural hearing loss (HL) leads to localized brain changes in the primary auditory cortex, long-range functional alterations, and is considered a risk factor for dementia. Nonhuman studies have repeatedly highlighted cross-modal brain plasticity in sensorial brain networks other than those primarily involved in the peripheral damage, thus in this study, the possible cortical alterations associated with HL have been analyzed using a whole-brain multimodal connectomic approach. Fifty-two HL and 30 normal hearing participants were examined in a 3T MRI study along with audiological and neurological assessments. Between-regions functional connectivity and whole-brain probabilistic tractography were calculated in a connectome-based manner and graph theory was used to obtain low-dimensional features for the analysis of brain connectivity at global and local levels. The HL condition was associated with a different functional organization of the visual subnetwork as revealed by a significant increase in global efficiency, density, and clustering coefficient. These functional effects were mirrored by similar (but more subtle) structural effects suggesting that a functional repurposing of visual cortical centers occurs to compensate for age-related loss of hearing abilities. [ABSTRACT FROM AUTHOR]

Dementia and hearing loss share radiologic and biologic findings that might explain their coexistence, especially in the elderly population. Brain atrophy has been observed in both conditions, as well as the presence of areas of gliosis. The brain atrophy is usually focal; it is located in the temporal lobe in patients with hearing loss, while it involves different part of brain in patients with dementia. Radiological studies have shown white matter hyperintensities (WMHs) in both conditions. WMHs have been correlated with the inability to correctly understand words in elderly persons with normal auditory thresholds and, the identification of these lesion in brain magnetic resonance imaging studies has been linked with an increased risk of developing cognitive loss. In addition to WMHs, some anatomopathological studies identified the presence of brain gliosis in the elderly's brain. The cause-effect link between hearing loss and dementia is still unknown, despite they might share some common findings. The aim of this systematic review is to analyze radiologic and biomolecular findings that these two conditions might share, identify a common pathological basis, and discuss the effects of hearing aids on prevention and treatment of cognitive decline in elderly patients with hearing loss. [ABSTRACT FROM AUTHOR]