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Frontiers in Aging Neuroscience 2016The brain activity of humans with tinnitus of various etiologies is typically studied with electro- and magneto-encephalography and functional magnetic resonance... (Review)
Review
The brain activity of humans with tinnitus of various etiologies is typically studied with electro- and magneto-encephalography and functional magnetic resonance imaging-based imaging techniques. Consequently, they measure population responses and mostly from the neocortex. The latter also underlies changes in neural networks that may be attributed to tinnitus. However, factors not strictly related to tinnitus such as hearing loss and hyperacusis, as well as other co-occurring disorders play a prominent role in these changes. Different types of tinnitus can often not be resolved with these brain-imaging techniques. In animal models of putative behavioral signs of tinnitus, neural activity ranging from auditory nerve to auditory cortex, is studied largely by single unit recordings, augmented by local field potentials (LFPs), and the neural correlates of tinnitus are mainly based on spontaneous neural activity, such as spontaneous firing rates and pair-wise spontaneous spike-firing correlations. Neural correlates of hyperacusis rely on measurement of stimulus-evoked activity and are measured as increased driven firing rates and LFP amplitudes. Connectivity studies would rely on correlated neural activity between pairs of neurons or LFP amplitudes, but are only recently explored. In animal models of tinnitus, only two etiologies are extensively studied; tinnitus evoked by salicylate application and by noise exposure. It appears that they have quite different neural biomarkers. The unanswered question then is: does this different etiology also result in different tinnitus?
PubMed: 27895575
DOI: 10.3389/fnagi.2016.00265 -
SAGE Open Medical Case Reports 2023Congenital hypoaldosteronism is a rare autosomal recessive or dominant endocrinopathy with variable penetrance, secondary to primary defects in aldosterone synthesis...
Congenital hypoaldosteronism is a rare autosomal recessive or dominant endocrinopathy with variable penetrance, secondary to primary defects in aldosterone synthesis that could lead to hypovolemia, hyponatremia, hyperkalemia, failure to thrive, microcephaly, seizures, neurodevelopmental delay, or hearing loss. We present the case of a Colombian patient with congenital hypoaldosteronism, who owns two variants in the gene, and a heterozygous pathogenic variant for nonclassical congenital adrenal hyperplasia. However, the patient missed follow-up and treatment for 6 years. At the age of 7 years, he resumed medical follow-up with laboratory findings of hyperreninemia and hypoaldosteronism, as well as clinical findings of strabismus, left mixed hyperacusis, and pathological short stature (-4.3 SD). Therefore, a trial of fludrocortisone therapy was started with subsequent improvement in renin levels, weight gain, and growth velocity. After 10 months of the start of the medication, he presented hypertension. There is no literature about the late treatment of this condition for pathological short stature.
PubMed: 37811351
DOI: 10.1177/2050313X231201724 -
Neuroscience and Biobehavioral Reviews Feb 2021Atypical behavioral responses to environmental sounds are common in autistic children and adults, with 50-70 % of this population exhibiting decreased sound tolerance... (Review)
Review
Atypical behavioral responses to environmental sounds are common in autistic children and adults, with 50-70 % of this population exhibiting decreased sound tolerance (DST) at some point in their lives. This symptom is a source of significant distress and impairment across the lifespan, contributing to anxiety, challenging behaviors, reduced community participation, and school/workplace difficulties. However, relatively little is known about its phenomenology or neurocognitive underpinnings. The present article synthesizes a large body of literature on the phenomenology and pathophysiology of DST-related conditions to generate a comprehensive theoretical account of DST in autism. Notably, we argue against conceptualizing DST as a unified construct, suggesting that it be separated into three phenomenologically distinct conditions: hyperacusis (the perception of everyday sounds as excessively loud or painful), misophonia (an acquired aversive reaction to specific sounds), and phonophobia (a specific phobia of sound), each responsible for a portion of observed DST behaviors. We further elaborate our framework by proposing preliminary neurocognitive models of hyperacusis, misophonia, and phonophobia that incorporate neurophysiologic findings from studies of autism.
Topics: Adult; Anxiety; Anxiety Disorders; Autistic Disorder; Child; Humans; Hyperacusis; Sound
PubMed: 33285160
DOI: 10.1016/j.neubiorev.2020.11.030 -
Ear, Nose, & Throat Journal Apr 2022Misophonia is a chronic condition in which patients experience a strong negative, emotional, or psychologic reaction to specific sounds. These sounds cause the...
Misophonia is a chronic condition in which patients experience a strong negative, emotional, or psychologic reaction to specific sounds. These sounds cause the individual to have a sudden, uncontrolled, and disproportionate negative reaction affecting their daily activities. The literature describes several cases of misophonia in the adult population; however, only 2 pediatric case studies are reported. Herein, we present 2 additional cases. An exaggerated response to an auditory stimulus is observed in other disorders such as tinnitus, hyperacusis, migraines, and many psychiatric disorders. Sound aversion has a broad differential diagnosis and may require visits to numerous specialists, placing strain on the patient and the healthcare system. Furthermore, misophonia is underdiagnosed in the pediatric population as it requires self-reporting of symptoms. The pathophysiology, prevalence, and treatment of misophonia continue to be relatively unknown. We attempt to highlight this rarely reported pediatric diagnosis and elaborate on its clinical presentation to increase awareness among otolaryngologists.
PubMed: 35414290
DOI: 10.1177/01455613221095606 -
The Primary Care Companion For CNS... Sep 2022
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European Journal of Medical Genetics Mar 2021Individuals with Williams Syndrome (WS) have specific auditory characteristics, including hypoacusis and hyperacusis, and music appreciation skills. Little is known...
Individuals with Williams Syndrome (WS) have specific auditory characteristics, including hypoacusis and hyperacusis, and music appreciation skills. Little is known about the functionality of the central auditory nervous system (CANS) for sound processing in WS. Thus, the objective of the present study was to evaluate the functionality of the CANS in individuals with WS, based on auditory event-related potentials, as far as cognitive and behavioral aspects are concerned. The study was carried out with 17 individuals, seven females and ten males, between seven and 17 years old, with WS, and 17 individuals with typical development matched by sex and chronological age to individuals with WS. None of these individuals had middle ear impairment or hearing loss. The subjects were evaluated for intelligence quotient, loudness discomfort level, and auditory event-related potentials with Tone Burst stimuli, on the oddball paradigm; the parents also answered the MTA-SNAP-IV questionnaire. Hyperacusis was found in six WS individuals and two individuals with typical development. In the present study, WS individuals present longer latency and reduced amplitude for P1, N1, N2 and P3 components. These results, suggesting a delay and hypoactive responses of the CANS in this syndrome, that cannot be related to the cognitive or behavioral aspects of these individuals, but it indicates a cortical immaturity to process acoustic stimuli.
Topics: Adolescent; Child; Evoked Potentials, Auditory; Female; Hearing; Humans; Male; Reaction Time; Williams Syndrome
PubMed: 33571693
DOI: 10.1016/j.ejmg.2021.104163 -
Neural Plasticity 2018Neurophysiological and neuroimaging data suggest that the brains of not only children but also adults are reorganized based on sensory inputs and behaviors. Plastic... (Review)
Review
Neurophysiological and neuroimaging data suggest that the brains of not only children but also adults are reorganized based on sensory inputs and behaviors. Plastic changes in the brain are generally beneficial; however, maladaptive cortical reorganization in the auditory cortex may lead to hearing disorders such as tinnitus and hyperacusis. Recent studies attempted to noninvasively visualize pathological neural activity in the living human brain and reverse maladaptive cortical reorganization by the suitable manipulation of auditory inputs in order to alleviate detrimental auditory symptoms. The effects of the manipulation of auditory inputs on maladaptively reorganized brain were reviewed herein. The findings obtained indicate that rehabilitation therapy based on the manipulation of auditory inputs is an effective and safe approach for hearing disorders. The appropriate manipulation of sensory inputs guided by the visualization of pathological brain activities using recent neuroimaging techniques may contribute to the establishment of new clinical applications for affected individuals.
Topics: Acoustic Stimulation; Auditory Cortex; Auditory Pathways; Brain Mapping; Correction of Hearing Impairment; Hearing Disorders; Humans; Hyperacusis; Neuronal Plasticity; Tinnitus
PubMed: 29887880
DOI: 10.1155/2018/2546250 -
Clinical Medicine Insights. Ear, Nose... 2017The frequency of tinnitus in children and adults is practically the same. However, although adults reveal their symptoms and seek for medical aid, the suffering often...
OBJECTIVES
The frequency of tinnitus in children and adults is practically the same. However, although adults reveal their symptoms and seek for medical aid, the suffering often remains unrecognized in the young. This is due to both the inability of children to properly describe their symptoms and the lack of recognition.
MATERIALS AND METHODS
Among 5768 patients entering our department with complaints of tinnitus between 2010 and 2015, there were only 112 children. A full clinical history and medical status had been determined at the time of presentation and were analyzed retrospectively.
RESULTS
The average duration from first complain to clinical presentation was approximately 12 months. A normal hearing capability of less than 25 dB was measured in 80% of the cases. Only 23 patients presented with a hearing impairment. The causes ranged from hearing loss, previous orthodontic treatment, noise trauma, middle ear aeration, muscular neck tension, and skull base fracture. Typical co-morbidities such as sleeping disorders, concentration disorders, and hyperacusis were observed.
CONCLUSIONS
This retrospective study shows that recognition of tinnitus in the childhood is generally delayed. A better characterization of complaints and triggers, however, is a prerequisite to sensitize medical personnel and caretakers for the suffering and to avoid developmental impairments.
PubMed: 29114160
DOI: 10.1177/1179550617736521 -
Medicina Oral, Patologia Oral Y Cirugia... Mar 2007This work seeks to alert medical and odontological staff to understanding and using interdisciplinary handling for detecting different pathologies common otic symptoms.... (Review)
Review
This work seeks to alert medical and odontological staff to understanding and using interdisciplinary handling for detecting different pathologies common otic symptoms. It offers better tools for this shared symptomatology during therapy s conservative phase. Tensor tympani muscle physiology and function in the middle ear have been veiled, even when their dysfunction and anatomical relationships may explain a group of confused otic symptoms during conventional clinical evaluation. Middle ear muscles share a common embryological and functional origin with chewing and facial muscles. This article emphasizes that these muscles share a functional neurological and anatomical dimension with the stomatognathic system; these muscles increased tonicity ceases to be a phenomenon having no logical connections. It offers functionality and importance in understanding referred otic symptoms in common with other extra-otical symptom pathologies. Tinnitus, vertigo, otic fullness sensation, hyperacusia, hypoacusia and otalgia are not only primary hearing organ symptoms. They should be redefined and related to the neighboring pathologies which can produce them. There is a need to understand temporomandibular disorders and craniofacial referred symptomatology from neurophysiologic and muscle-skeletal angles contained in the stomatognathic system. Common symptomatology is frequently observed in otic symptoms and temporomandibular disorders during daily practice; this should be understood by each discipline from a broad, anatomical and clinical perspective.
Topics: Ear Ossicles; Ear, Middle; Earache; Female; Hearing Disorders; Humans; Hyperacusis; Male; Masticatory Muscles; Muscle Contraction; Reflex; Temporomandibular Joint Disorders; Tensor Tympani; Tinnitus; Vertigo
PubMed: 17322813
DOI: No ID Found -
Photo-, osmo- and phonophobia in the premonitory phase of migraine: mistaking symptoms for triggers?The Journal of Headache and Pain Feb 2015Certain environmental stimuli are frequently reported as typical triggers of migraine pain. Whether these so-called triggers are independent precipitators of migraine...
BACKGROUND
Certain environmental stimuli are frequently reported as typical triggers of migraine pain. Whether these so-called triggers are independent precipitators of migraine pain or mere symptoms of the premonitory phase of migraine remains to be elucidated.
METHODS
In this retrospective cohort study of 1010 migraine patients of a tertiary headache center we assessed the frequency of common trigger factors, premonitory symptoms and accompanying symptoms as well as basic headache characteristics and demographic data.
RESULTS
Premonitory symptoms with an onset of 2 or more hours prior to the headache were present in 38.9% of migraine patients, the most frequent being a tense neck, phonophobia and difficulty concentrating. There was a clear overlap of certain trigger factors and the presence of corresponding premonitory symptoms: flickering or bright light as a trigger was associated with higher frequency of photophobia in the premonitory phase. The same applied to the presence of food craving and osmophobia in the premonitory phase and certain foods or odours as trigger factors.
CONCLUSIONS
Our data thus support the view that commonly reported trigger factors of migraine are not so much independent precipitators of migraine pain, but that they are most likely just misinterpreted results of enhanced attention to certain stimuli mediated by typical premonitory symptoms of migraine pain.
Topics: Adult; Cohort Studies; Female; Humans; Hyperacusis; Male; Migraine Disorders; Photophobia; Retrospective Studies
PubMed: 25904144
DOI: 10.1186/s10194-015-0495-7