Search results for " Hearing"
showing 10 items of 301 documents
Cardiac phenotype in ATP1A3-related syndromes: A multicentre cohort study
2020
ObjectiveTo define the risks and consequences of cardiac abnormalities in ATP1A3-related syndromes.MethodsPatients meeting clinical diagnostic criteria for rapid-onset dystonia-parkinsonism (RDP), alternating hemiplegia of childhood (AHC), and cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS) with ATP1A3 genetic analysis and at least 1 cardiac assessment were included. We evaluated the cardiac phenotype in an Atp1a3 knock-in mouse (Mashl+/−) to determine the sequence of events in seizure-related cardiac death.ResultsNinety-eight patients with AHC, 9 with RDP, and 3 with CAPOS (63 female, mean age 17 years) were included. Resting ECG abnormalities…
Facial nerve schwannoma involving middle cranial fossa: When the unilateral sensorineural hearing loss guide to the correct diagnosis
2011
The Facial Nerve Schwannoma is a rare tumor and it seldom involved the middle cranial fossa. Facial nerve schwannoma has various manifestations, including facial palsy but unfortunately facial nerve is very resistant to compression and often facial nerve paralysis or a facial weakness are not present. We present a case of giant facial nerve schwannoma involved the middle cranial fossa without facial nerve paralysis. In these cases the unilateral hearing loss (if present) guide to a correct diagnosis.
Minimal Reporting Standards for Active Middle Ear Hearing Implants.
2017
There is currently no standardized method for reporting audiological, surgical and subjective outcome measures in clinical trials with active middle ear implants (AMEIs). It is often difficult to compare studies due to data incompatibility and to perform meta-analyses across different centres is almost impossible. A committee of ENT and audiological experts from Germany, Austria and Switzerland decided to address this issue by developing new minimal standards for reporting the outcomes of AMEI clinical trials. The consensus presented here aims to provide a recommendation to enable better inter-study comparability.
Eustachian tube dysfunction in OSMF- often present seldom discovered
2014
Objectives: To evaluate the effect of OSMF on the eustachian tube function and to correlate it with various grades of the disease. Study Design: Twenty OSMF patients (40 ears) and 20 healthy controls (40 ears) were evaluated for eustachian tube function by eustachian tube function test, tympanometry and audiometry. Results: The audiometric and tympanometric analysis showed no significant differences in hearing abilities of OSMF patients and controls and between various grades of OSMF, indicating no hearing impairment. However, eustachian tube function test revealed a statistically significant difference in eustachian tube function in OSMF patients and controls. Further, there was a signific…
Clinical and logopaedic results of simultaneous and sequential bilateral implants in children with severe and/or profound bilateral sensorineural hea…
2015
This article carries out a literature review of the advantages and limitations of the simultaneous bilateral cochlear implantation (SCI) compared to those of the sequential bilateral cochlear implantation (SBCI) and the unilateral cochlear implantation (UCI). The variables analysed in said comparison are: safety and surgical technique, SCI incidence, effectiveness, impact of the inter-implant interval, costs and financing, impact on brain plasticity, impact on speech and language development, main benefits, main disadvantages and concerns, and predictive factors of prognosis. Although the results are not conclusive, all variables analysed seem to point towards observable benefits of SCI in …
Mechanisms underlying noise-induced hearing loss
2006
Noise-induced hearing loss (NIHL) is the worldwide leading occupational disease and presents an important socio-economic factor. Despite numerous identified details about its etiology, the underlying mechanisms, which induce NIHL, have been only partially identified. In the present article, we shall discuss possible mechanisms focusing on failure in cellular calcium regulation, leading to a rise in mitochondrial NO production and reactive oxygen species formation. In cases where radical production is significantly elevated, pathological concentrations lead to alterations of cell physiological conditions and finally contribute to NIHL. A more detailed knowledge about the induction of free ra…
Otoacoustic emissions: a new method to diagnose hearing impairment in children.
1993
Otoacoustic emissions (OAEs) are epiphenomena of sensitive, amplifying processes during hearing which can be detected in persons with normal inner ear function. They originate from the cochlea and are interpreted as an energy leakage of cochlear processes, perhaps resulting from active outer hair cell movements. OAEs travel from the cochlea through the middle ear to the external auditory canal where they can be detected using sensitive miniature microphones. Transient evoked (TEOAE) tests allow to otoacoustic emissions non-invasively check the integrity of the cochlea. In the neonatal period, registration of OAEs can be accomplished during natural sleep. In infants and neonates TEOAEs can b…
Case report supporting that the Barber-Say and ablepharon macrostomia syndromes could represent one disorder.
2009
We report on a 7-year-old girl with unequivocal features of Barber-Say syndrome (BSS): generalized hypertrichosis especially at the back, dry lax skin, macrostomia, thin lips, cup-shaped ears, bulbous nose, hypoplastic nipples, and abnormal external genitalia. She also demonstrated conductive hearing impairment and microblepharon. BSS has been reported with ectropion (not present in our patient), but ablepharon and microblepharon (i.e., absent or hypoplastic eyelids) have always been considered as hallmarks of ablepharon macrostomia syndrome (AMS). This is the first report of microblepharon in BSS. Other authors have discussed that BSS and AMS could possibly represent one syndrome, and our …
Detrimental noise effects on brain's speech functions.
2009
Background noise has become part of our everyday life in modern societies. Its presence affects both the ability to concentrate and communicate. Some individuals, like children, the elderly, and non-native speakers have pronounced problems in noisy environments. Here we review evidence suggesting that background noise has both transient and Sustained detrimental effects on central speech processing. Studies on the effects of noise on neural processes have demonstrated hemispheric reorganization in speech processing in adult individuals during background noise. During noise, the well-known left hemisphere dominance in speech discrimination became right hemisphere preponderant. Furthermore, l…
Changes on blood filterability in patients with fluctuant hearing loss
2006
Abstract We conducted a two-year follow-up of thirteen subjects with fluctuating sensorineural hearing loss, measuring their blood filterability (BF) and correlating it to the onset of episodes of deafness. We observed a decrease in the BF levels of all the patients when they experienced hearing loss. When the patients recovered their normal level of hearing, the BF also increased. The use of reactive techniques to avoid these decreases in BF could potentially prevent or correct fluctuating deafness.