Search results for "Hearing lo"
showing 10 items of 220 documents
Epidemiology of Usher Syndrome in Valencia and Spain
2004
<b>Objective:</b> To obtain epidemiological data on the prevalence of the different types of Usher syndrome (US) in Spain, since these data were missing; to estimate the proportion of sporadic cases among simplex families, and calculate the prevalence of the Usher syndrome in a homogeneous population from Eastern Spain (3,875,234 inhabitants) that is representative of the Spanish population. <b>Methods:</b> Otological, ophthalmological and genetic studies were performed in 89 US patients from 46 families and subjected to statistical and segregation analysis. <b>Results:</b> 41.6% of them suffered US type I, 46.1% type II, and in 12.3% the classification r…
Síndrome de Susac de presentación ocular atípica
2007
espanolCaso clinico: Mujer de 25 anos de edad afecta de encefalopatia y perdida auditiva neurosensorial, remitida a nuestro servicio para confirmar un sindrome de Susac por presentar una disminucion de agudeza visual bilateral. En la exploracion funduscopica se aprecio una microangiopatia retiniana periferica. Discusion: El sindrome de Susac se caracteriza tipicamente por la triada encefalopatia, perdida auditiva neurosensorial, y oclusiones de ramas arteriales retinianas. En nuestro caso las alteraciones oculares eran atipicas, apreciandose amputacion de vasos terminales con cumulo de microaneurismas en retina periferica de ambos ojos. EnglishCase report: A 25-year-old woman with encephalo…
The role of fissula ante fenestram in unilateral sudden hearing loss
2016
The cause of unilateral sudden sensorineural hearing loss (SNHL) remains unclear in many clinical cases. Perilymphatic leakage through a fissula ante fenestram (FAF) fistula is one possible reason. We present four clinical cases with proven FAF fistula, discovered during surgical exploration. All patients experienced partial hearing recovery after surgical coverage of the fistula. We suggest FAF as a possible site for perilymphatic leakage, representing an anatomical correlate for sudden unilateral SNHL. We recommend early exploratory tympanotomy with special attention to the bony region, anterior to the oval window, in cases of severe sudden SNHL and suspected FAF.
Subarachnoid Hemorrhage Revealing Moyamoya Syndrome in a Patient With May-Hegglin Anomaly.
2017
Etude de cas; Introduction: Moyamoya syndrome is a rare progressive cerebrovascular occlusive disease for which several associated conditions have been described. Case Report: We report the case of a 76-year-old woman with a history of May-Hegglin anomaly who presented with an isolated unusual diffuse headache. Initial laboratory investigations showed only thrombocytopenia (platelet count 95000/mu L). Unenhanced brain computed tomography scan revealed a small subarachnoid hemorrhage in the left frontal lobe. Computed tomography angiography showed occlusion of the terminal portion of the left internal carotid artery (ICA) and narrowing of the terminal portion of the right ICA with abnormal c…
Imaging of inner ear malformations: a primer for radiologists
2021
AbstractIn the multidisciplinary management of patients with inner ear malformations (IEMs), the correct diagnosis makes the differences in terms of clinical and surgical treatment. The complex anatomical landscape of the inner ear, comprising several small structures, makes imaging of this region particularly challenging for general radiologists. Imaging techniques are important for identifying the presence and defining the type of IEM and the cochlear nerve condition. High-resolution magnetic resonance imaging (MRI) sequences and high-resolution computed tomography (HRCT) are the mainstay imaging techniques in this area. Dedicated MRI and HRCT protocols play an important role in the diagn…
302 Hearing Abnormalities in Children with Fabry Disease: Data from FOS - the Fabry Outcome Survey
2005
Background: Fabry disease is an X-linked glycosphingolipid storage disorder due to a deficiency of the enzyme alpha-galactosidase A. Accumulation of substrate results in a progressive and life-threatening multisystemic disease. Early clinical manifestations include pain and gastrointestinal symptoms. Sensorineural hearing loss and vertigo are well-recognized features of the disorder, occurring in approximately 50% of adults with Fabry disease. We have investigated the audiological symptoms of Fabry disease in children using pure-tone and impedance audiometry. Methods: Symptom history was obtained using a standardized questionnaire from FOS − the Fabry Outcome Survey. Hearing was measured us…
Beidseitige progrediente Hörminderung als Erstmanifestation eines metastasierenden Pankreaskopfkarzinoms - Kasuistik -
2001
We report the history of a patient who presented first with a progressive unilateral hearing loss and later with a bilateral deafness and an unilateral facial nerve palsy as first and only symptoms of a pancreatic adenocarcinoma. By means of magnetic resonance tomography tumor-suspect lesions in both internal auditory canals were detected. Referring to the results of further examinations these intracanalicular lesions are most probably due to rarely seen bilateral metastasis of a pancreatic adenocarcinoma in the temporal bone. In addition to this rarely diagnosed localisation of metastasis it is rather uncommon that this kind of primary malignoma had not been detected because of gastrointes…
Effekt der kontralateralen Stimulation auf evozierte otoakustische Emissionen bei akutem Hörverlust*
1994
23 patients with unilateral hearing loss and presence of TEOAE on both ears were examined with regard to the association between contralateral acoustic stimulation and evoked otoacoustic emissions. Contralateral white noise of 40 dB HL, 50 dB HL and 60 dB HL decreases the amplitude of TEOAE both at the ear with and without hearing loss. In comparison with a group of subjects with normal hearing, the decrease in the amplitude of TEOAE was less in the group of patients with hearing loss. Two paradoxical cases were found with an increase in the TEOAE amplitude in the ear with hearing loss during contralateral stimulation. It is assumed that impairment of the efferent synapses on the outer hair…
Cortisol suppression and hearing thresholds in tinnitus after low-dose dexamethasone challenge
2012
Abstract Background Tinnitus is a frequent, debilitating hearing disorder associated with severe emotional and psychological suffering. Although a link between stress and tinnitus has been widely recognized, the empirical evidence is scant. Our aims were to test for dysregulation of the stress-related hypothalamus-pituitary adrenal (HPA) axis in tinnitus and to examine ear sensitivity variations with cortisol manipulation. Methods Twenty-one tinnitus participants and 21 controls comparable in age, education, and overall health status but without tinnitus underwent basal cortisol assessments on three non-consecutive days and took 0.5 mg of dexamethasone (DEX) at 23:00 on the first day. Corti…
Brainstem Auditory Evoked Potentials and Visual Potentials in Kawasaki Disease: An Observational Monocentric Study
2020
Background: Kawasaki Disease is a systemic vasculitis, particularly involving coronary arteries. Rare involvement of other vascular districts is described, as central nervous system arteries, leading to a vasculitic neuropathy. Sensorineural hearing loss and alterations of evoked potentials are uncommonly reported complications.Methods: In an observational monocentric study, 59 children (37 males; 22 females; mean age: 2.7 ± 2.2 years) with documented Kawasaki Disease were enrolled. No risk factors for hearing loss and/or neurological impairment were identified in the cohort. Brainstem auditory evoked potentials and visual evoked potentials were correlated with clinical, hamatological and r…