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Seminars in Pediatric Neurology Apr 2022Functional hearing disorder (FHD) is one of the conditions that fall under the umbrella of Functional Neurological Disorders. FHD is characterized by a decrease in... (Review)
Review
Functional hearing disorder (FHD) is one of the conditions that fall under the umbrella of Functional Neurological Disorders. FHD is characterized by a decrease in hearing sensitivity without correlation in standard audiological testing. In the general pediatric population, the prevalence of FHD is estimated at less than 2%. The prevalence is about 7% in children 6-17 years of age. Some authors, however, consider FHD the most common etiology of sudden onset bilateral hearing loss in children. The mean age at diagnosis of FHD is 11.3 years - the condition is rare below 7 years of age. FHD affects twice as many girls as boys. The loss tends to be of sudden onset, bilateral, impacts all frequencies to a similar degree, and can be associated with an identifiable psychosocial conflict. FHD can be diagnosed using two sets of tests: (1) behavioral tests: pure-tone and speech audiometry; and (2) physiological tests: immittance audiometry, otoacoustic emissions, auditory evoked potentials, and functional neuroimaging. The backbone of the treatment of FHD includes cognitive-behavioral therapy and as needed, psychiatric medications. In this paper, we review the diagnosis, treatment, differential diagnosis, and complications of FHD.
Topics: Audiometry, Pure-Tone; Child; Evoked Potentials, Auditory, Brain Stem; Female; Hearing; Hearing Disorders; Humans; Male; Otoacoustic Emissions, Spontaneous
PubMed: 35450670
DOI: 10.1016/j.spen.2022.100956 -
Journal of Clinical Pharmacy and... Nov 2022Evidence on whether the coronavirus disease 2019 (COVID-19) vaccination could cause hearing-related adverse events is still conflicting. This study aims to access the...
WHAT IS KNOWN AND OBJECTIVE
Evidence on whether the coronavirus disease 2019 (COVID-19) vaccination could cause hearing-related adverse events is still conflicting. This study aims to access the association between COVID-19 vaccine and hearing disorder.
METHODS
The Vaccine Adverse Event Reporting System (VAERS) was queried between January 2020 to November 2021. The disproportionality pattern for hearing impairment of COVID-19 vaccine was accessed by calculating the reporting odds ratio (ROR) and proportional reporting ratio (PRR). A further subgroup analysis based on the type of COVID-19 vaccine and the doses administered was performed. In addition, the disproportionalities for hearing dysfunction between COVID-19 and influenza vaccines were compared.
RESULTS AND DISCUSSION
A total of 14,956 reports of hearing-related adverse events were identified with COVID-19 vaccination and 151 with influenza vaccine during the analytic period in VAERS. The incidence of hearing disorder following COVID-19 vaccination was 6.66 per 100,000. The results of disproportionality analysis revealed that the adverse events of hearing impairment, after administration of COVID-19 vaccine, was significantly highly reported (ROR 2.38, 95% confidence interval [CI] 2.20-2.56; PRR: 2.35, χ 537.58), for both mRNA (ROR 2.37, 95% CI 2.20-2.55; PRR 2.34, χ 529.75) and virus vector vaccines (ROR 2.50, 95% CI 2.28-2.73; PRR 2.56, χ 418.57). While the disproportional level for hearing dysfunction was quite lower in influenza vaccine (ROR 0.36, 95% CI 0.30-0.42; PRR 0.36, χ 172.24).
WHAT IS NEW AND CONCLUSION
This study identified increased risk for hearing disorder following administration of both mRNA and virus vector COVID-19 vaccines compared to influenza vaccination in real-world settings.
Topics: Humans; Pharmacovigilance; COVID-19 Vaccines; Adverse Drug Reaction Reporting Systems; Influenza Vaccines; COVID-19; Vaccination; Hearing Disorders; RNA, Messenger
PubMed: 36089844
DOI: 10.1111/jcpt.13767 -
Acta Oto-laryngologica Jan 2018Music-induced acute acoustic trauma is not inevitably linked to hearing dysfunction as validated by conventional pure tone audiometry. Tinnitus is often in combination...
CONCLUSION
Music-induced acute acoustic trauma is not inevitably linked to hearing dysfunction as validated by conventional pure tone audiometry. Tinnitus is often in combination with hyperacusis. Our results point at 'silent hearing loss' as the underlying pathology, having afferent nerve terminal damage rather than hair cell loss as the structural correlate.
OBJECTIVES
Exposure to loud music is one of the most common causes of acute acoustic trauma, which adolescents and teenagers experience by voluntary exposure to loud music of sound levels up to 110 dB(A).
METHODS
The clinical and psychophysical data of 104 consecutive patients with music-induced hearing disorder (MIHD) were analyzed to construct individual hearing and tinnitus profiles. In all cases, tinnitus was the presenting symptom.
RESULTS
Hearing abilities were normal in about two-thirds of the tinnitus patients. Tinnitus was experienced most often as a high-frequency tone (83%). The Tinnitus Handicap Inventory (THI) scores ranged from 0 to 94 with an average score of 43.1. Visual analog scales (VAS) were used to assess tinnitus loudness (average 42.4) and annoyance (average 54.2), and tinnitus awareness was estimated (average 60.3). All VAS values correlated strongly with the THI. Hyperacusis was present in 65% and 71% of the patients reported sleeping disorders.
Topics: Adolescent; Adult; Audiometry, Pure-Tone; Auditory Threshold; Female; Hearing Loss, Noise-Induced; Humans; Hyperacusis; Male; Middle Aged; Music; Surveys and Questionnaires; Tinnitus; Young Adult
PubMed: 28859531
DOI: 10.1080/00016489.2017.1367100 -
Brain : a Journal of Neurology Apr 2022Susac syndrome is a disorder thought to be mediated by an autoimmune response towards endothelial cells, leading to a characteristic clinical triad of encephalopathy,... (Review)
Review
Susac syndrome is a disorder thought to be mediated by an autoimmune response towards endothelial cells, leading to a characteristic clinical triad of encephalopathy, visual disturbances due to branch arterial occlusions and sensorineural hearing impairment. Although it is a rare disease, three reasons make it important. First, given its variable presentation, Susac syndrome is underdiagnosed. Second, it is considered an important differential diagnosis in different neurological, psychiatric, ophthalmological and hearing disorders, and consequently is frequently misdiagnosed. Third, in many cases, Susac syndrome is diagnosed and treated late, with significant irreversible sequelae including dementia, blindness and hearing loss. Neuropathology findings derived from both Susac syndrome patient tissue and novel transgenic mouse models indicate cytotoxic CD8+ T cells adhere to microvessels, inducing endothelial cell swelling, vascular narrowing and occlusion, causing microinfarcts. Anti-endothelial cell antibodies are present in serum in 25% of Susac syndrome patients, but it is unclear whether they are aetiologically related to the disease, or an epiphenomenon. The clinical triad comprising encephalopathy, branch arterial occlusions, and sensorineural hearing impairment is considered pathognomonic, although great variability is found in presentation and natural course of disease. At first evaluation, only 13-30% of patients exhibit the full clinical triad, making diagnosis difficult. Retinal fluorescein angiography, optic coherence tomography, MRI and tonal audiometry are helpful methods for diagnosing and monitoring disease activity during treatment. By contrast, there are no reliable objective immune markers to monitor disease activity. Immunosuppression is the current treatment, with high-dose corticosteroid therapy as the mainstay, but additional therapies such as intravenous immunoglobulins, cyclophosphamide, rituximab and mycophenolate mofetil are often necessary, because the disease can be devastating, causing irreversible organ damage. Unfortunately, low rates of disease, variability in presentation and paucity of objective biomarkers make prospective controlled clinical trials for Susac syndrome treatment difficult. Current immunosuppressive treatments are therefore based on empirical evidence, mainly from retrospective case series and expert opinion. In this review, we draw attention to the need to take consider Susac syndrome in the differential diagnosis of different neurological, psychiatric, ophthalmological and hearing disorders. Furthermore, we summarize our current knowledge of this syndrome, in reference to its pathophysiology, diagnosis and management, emphasizing the need for prospective and controlled studies that allow a better therapeutic approach.
Topics: Animals; Brain Diseases; Diagnosis, Differential; Endothelial Cells; Hearing Disorders; Hearing Loss; Humans; Magnetic Resonance Imaging; Mice; Prospective Studies; Retrospective Studies; Susac Syndrome
PubMed: 35136969
DOI: 10.1093/brain/awab476 -
Anatomical Record (Hoboken, N.J. : 2007) Mar 2020This review presents many but not all the major historical events that have led to our current understanding of gene and stem cell therapies for the treatment of hearing... (Review)
Review
This review presents many but not all the major historical events that have led to our current understanding of gene and stem cell therapies for the treatment of hearing and balance disorders in animal models of these disorders. In order to better understand the application of these emerging therapies to the treatment of inner ear disorders in a clinical setting, it has been necessary to provide some genetic and pathobiology backgrounds from both animal models and clinical disorders. The current focus and goal of gene and stem cell therapies are directed toward understanding the effective treatment of animal models that mimic human disorders of hearing and balance. This approach not only addresses the most effective ways to deliver the gene or stem cell therapies to affected inner ears, it also provides an assessment of the efficacy of the applied therapy(s) in achieving either partial or full restoration of either hearing and/or balance within the animal models receiving these therapeutic interventions. This review also attempts to present a realistic assessment of how close the research fields of gene and stem cell therapies are to application for the treatment of human disorders in a clinical setting. Progress made in developing these novel therapies toward clinical applications would not have been possible without the many pioneering studies and discoveries achieved by the investigators cited in this review. There were also many other excellent studies performed by gifted investigators that were not able to be included within this review. Anat Rec, 303:390-407, 2020. © 2019 American Association for Anatomy.
Topics: Animals; Genetic Therapy; Hearing Disorders; History, 20th Century; History, 21st Century; Humans; Stem Cell Transplantation; Vestibular Diseases
PubMed: 31994336
DOI: 10.1002/ar.24332 -
Nature Reviews. Drug Discovery Nov 2016
Topics: Clinical Trials as Topic; Drug Discovery; Hearing Disorders; Humans; Tinnitus
PubMed: 27807361
DOI: 10.1038/nrd.2016.222 -
Continuum (Minneapolis, Minn.) Apr 2021This article reviews the causes of tinnitus, hyperacusis, and otalgia, as well as hearing loss relevant for clinicians in the field of neurology. (Review)
Review
PURPOSE OF REVIEW
This article reviews the causes of tinnitus, hyperacusis, and otalgia, as well as hearing loss relevant for clinicians in the field of neurology.
RECENT FINDINGS
Important causes of unilateral and bilateral tinnitus are discussed, including those that are treatable or caused by serious structural or vascular causes. Concepts of hyperacusis and misophonia are covered, along with various types of neurologic disorders that can lead to pain in the ear. Hearing loss is common but not always purely otologic.
SUMMARY
Tinnitus and hearing loss are common symptoms that are sometimes related to a primary neurologic disorder. This review, tailored to neurologists who care for patients who may be referred to or encountered in neurology practice, provides information on hearing disorders, how to recognize when a neurologic process may be involved, and when to refer to otolaryngology or other specialists.
Topics: Earache; Hearing Loss; Humans; Hyperacusis; Tinnitus
PubMed: 34351116
DOI: 10.1212/CON.0000000000000961 -
The New England Journal of Medicine Oct 1993
Review
Topics: Auditory Pathways; Child; Ear; Hearing Disorders; Humans; Wounds and Injuries
PubMed: 8371732
DOI: 10.1056/NEJM199310073291507 -
The Medical Clinics of North America Jan 1999Hearing loss may be caused by either a conductive problem, such that the sound waves do not reach the tympanic membrane, ossicles or inner ear, or a sensorineural...
Hearing loss may be caused by either a conductive problem, such that the sound waves do not reach the tympanic membrane, ossicles or inner ear, or a sensorineural problem, in which the cochlea and/or auditory nerve are somehow damaged. A combination of the above may also occur. The various causes of hearing loss and the options in treatment will be presented.
Topics: Cochlear Nerve; Ear Ossicles; Ear, Inner; Hearing; Hearing Disorders; Hearing Loss, Conductive; Hearing Loss, Sensorineural; Humans; Tympanic Membrane; Vestibulocochlear Nerve
PubMed: 9927965
DOI: 10.1016/s0025-7125(05)70092-4 -
Archives of Endocrinology and Metabolism 2017Endocochlear, retrocochlear and/or central origin hearing damage may be related to the absence of appropriate levels of thyroid hormone during morphogenesis and/or... (Review)
Review
Endocochlear, retrocochlear and/or central origin hearing damage may be related to the absence of appropriate levels of thyroid hormone during morphogenesis and/or auditory system development. Hearing disorders related to the thyroid are not well studied, despite speculation on the pathophysiological mechanisms. The objective of this review was to characterize the main pathophysiological mechanisms of congenital hypothyroidism and to evaluate the relationship with central and peripheral hearing disorders. We conducted a literature review using the databases MedLine, LILACS, Cochrane Library, SciELO, Institute for Scientific Information (ISI), Embase, and Science Direct between July and September on 2016. We identified the studies that address hearing disorder mechanisms on the congenital hypothyroidism. Congenital hypothyroidism may have clinical and subclinical manifestations that affect the auditory system and may be a potential risk factor for hearing impairment. Hearing impairment can severely impact quality-of-life, which emphasizes the importance of monitoring and evaluating hearing during the clinical routine of these patients.
Topics: Animals; Congenital Hypothyroidism; Disease Models, Animal; Hearing Loss; Humans
PubMed: 28977164
DOI: 10.1590/2359-3997000000292