Search results for "Hearing loss."
showing 10 items of 213 documents
Intratympanic Dexamethasone and Hyaluronic Acid in Patients with Low-Frequency and Ménière's-Associated Sudden Sensorineural Hearing Loss
2005
Steroids are widely used for the treatment of cochleovestibular disorders. Direct steroid application in the middle ear cavity, when combined with a round window membrane permeability-modulating substance, increases the level of the steroid reaching the target cells. We measured hearing in patients with idiopathic isolated low-frequency sensorineural hearing loss and in patients with sudden sensorineural hearing loss and a history of Ménière's disease. Contradictory reports about effectiveness of intratympanic steroid therapy on vertigo control and hearing improvement in patients with Ménière's disease exist in the literature.Eighteen patients with isolated low-frequency idiopathic sudden s…
Intrafamilial variability of the deafness and goiter phenotype in Pendred syndrome caused by a T416P mutation in the SLC26A4 gene.
2004
AbstractPendred syndrome (PS) is the most common cause of syndromic deafness, accounting for more than 5% of all autosomal-recessive hearing loss cases. It is characterized by bilateral sensorineural hearing loss and by goiter with or without hypothyroidism. Mutations in the SLC26A4 gene cause both classical PS and deafness associated with an enlarged vestibular aqueduct without goiter.To investigate a possible genotype-phenotype correlation in PS, we performed a detailed clinical and genetic study in three adult German sibs with typical PS caused by a common homozygous SLC26A4 mutation, T416P. An audiological long-term follow-up of 23 yr showed that the mutation T416P is associated with a …
[Treatment of sensorineural hearing loss in acute viral otitis media with intratympanic dexamethasone and hyaluronic acid in comparison with intraven…
2006
Background: Intratympanic dexamethasone with hyaluronic acid has shown to be an effective treatment of the inner ear damage in sudden hearing loss and Meniere's disease. It is not known yet if the same applies to the toxic inner ear damage in acute viral otitis media. Patients: Retrospectively, 26 patients who suffered from acute viral otitis media with sensorineural hearing loss were examined with regard to the improvement of their inner ear hearing loss. Twelve patients were treated with intravenous therapy only, consisting of hydroxyethylstarch, pentoxifylline and prednisolone. Fourteen patients additionally received intratympanic injections, consisting of dexamethasone and hyaluronic ac…
Otosclerosis associated with type B-1 inner ear malformation
2010
Malformations of bony inner ear are rare anomalies occurring in approximately 20% of patients with congenital sensorineural hearing loss. Conductive hearing loss is usually associated with abnormalities of the external and middle ear. Recent reports of patients with lateral semicircular canal malformations indicate inner ear malformations to be associated with sensorineural or conductive hearing loss. Differential diagnosis of conductive hearing loss should include otosclerosis, isolated ossicular deformities, inner ear anomalies or a combination of these. In this report, a case is described with right vestibule-lateral semicircular canal dysplasia presenting at our centre with bilateral ot…
Intratumoral Haemorrhage Causing an Unusual Clinical Presentation of a Vestibular Schwannoma
2012
We present a case of an elderly woman with no history of audiological disease with sudden onset of visual and hearing deficits associated with systemic clinical signs. On examination she had impairment of right CNs from V to X. CT and MR imaging demonstrated a cystic vestibular schwannoma with a rare intralesional fluid-fluid level correlated to a recent bleed. We include high quality MR images to show the acute impairment of the cranial nerves next to the tumour after acute bleeding. Our case report includes a voxel-based morphometry (VMB) analysis of the tumour that, as far as we know, has never been done before for such a tumour. VBM analysis was performed to calculate the hypothesized …
Distribution and phenotype ofGJB2mutations in 102 Sicilian patients with congenital non syndromic sensorineural hearing loss
2014
Objective: To evaluate the frequency of GJB2 mutations and their correlation with phenotype in Sicilian non-syndromic sensorineural hearing loss (NSHL) patients. Design: Sequencing of the coding region, basal promoter, exon 1, and donor splice site of the GJB2 gene; screening for the presence of the two common GJB6 deletions. Study sample: A cohort of 102 Sicilian NSHL patients. Results: Fifteen different mutations in GJB2 and seventeen different genotypes were detected. No GJB6 mutations were found. The hearing impairment was profound in the 64.72% of probands (mean PTA 0.25 – 4 kHz of 88.82 26.52 dB HL). A total of 81.37% of patients harboured at least one c.35delG allele; c.167delT and c…
Identification of D179H, a novel missense GJB2 mutation in a Western Sicily family
2013
The main purpose of this study was to describe a novel missense mutation (p.D179H) found in a Western Sicily family and to examine the genetic and audiologic profiles of all family members by performing a GJB2 and GJB6 mutations analysis and a complete audiologic assessment. The proband was a 3-month-old infant with a congenital profound sensorineural hearing loss; direct sequencing of the GJB2 revealed the presence of a c.35delG mutation in the heterozygous state and a heterozygous G[C transition at nucleotide 535 in trans; this novel mutation, called p.D179H, resulted in an aspartic acid to histidine change at codon 179. It was also evidenced in the heterozygous state in two members of th…
Speech perception performance as a function of stimulus pulse rate and processing strategy preference for the Cochlear™ Nucleus®CI24RE device: Relati…
2010
Current cochlear implants can operate at high pulse rates. The effect of increasing pulse rate on speech performance is not yet clear. Habituation to low rates may affect the outcome. This paper presents the results of three subsequent studies using different experimental paradigms, applying the Nucleus CI24RE device, and conducted by ten European implant teams. Pulse rate per channel varied from 500 to 3500 pulses per second with ACE and from 1200 to 3500 pps with CIS strategy. The results showed that the first rate presented had little effect on the finally preferred rate. Lower rates were preferred. The effect of pulse rate on word scores of post-linguistic implantees was small; high rat…
Analysis of Fifty-Six Cochlear Implant Device Failures
2008
<i>Objective:</i> Our aim was to present a failure analysis after cochlear implant revision surgery in a large series of children and adults and to assess the outcome and audiologic performance. <i>Methods:</i> Fifty-six cochlear implant failures that occurred in 422 devices implanted between 1990 and 2007 at the Department of Otolaryngology, Head and Neck Surgery at the University of Mainz, Germany, were retrospectively analyzed. The causes of failure were reviewed evaluating the individual history, telemetric and intraoperative findings and manufacturer’s investigation reports. <i>Results:</i> We performed 56 surgical revisions in a series of 422 consec…
Technical Note: Minimal Access Surgery for Cochlear Implantation with MED-EL Devices
2005
<i>Background:</i> Minimally invasive techniques have been described for cochlear implant surgery, but so far this had not been the case for Med-EL devices. <i>Objective:</i> To describe a newly developed minimal access approach for the implantation of Med-EL devices and report our results after up to 1 year of follow-up in 52 patients. <i>Discussion:</i> The use of a minimally invasive approach without raising a flap or extensive drilling of a bony well was feasible in all 52 patients of this series. It shortened the surgical time to an average of 45 min and there were no specific postoperative complications. The average follow-up of 8.4 months was uneve…