Search results for "sensorineural hearing lo"
showing 10 items of 64 documents
[Paediatric cochlear implantation in the critical period of the auditory pathway, our experience].
2009
Numerous experimental and clinical studies have suggested a critical or sensitive period in which the auditory pathway develops its greatest potential in terms of plasticity and learning. Early cochlear implantation performed in prelingual deaf children in this period provides a better prognosis for language acquisition. The aim of this study is to show the importance of cochlear implantation before this critical period ends.We conducted an observational, longitudinal, retrospective study of 57 children suffering profound prelingual bilateral sensorineural hearing loss who had received Advanced Bionics implants at our ENT department between June, 1998, and November, 2006. Data on their audi…
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.
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 …
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.
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.
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…
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…