Search results for " Sensorineural"
showing 10 items of 62 documents
A genotype-phenotype correlation in Sicilian patients with GJB2 biallelic mutations
2015
The aim of this work was to study the genotype distribution of Sicilian patients with biallelic GJB2 mutations; to correlate genotype classes and/or specific mutations of GJB2 gene (35delG-non-35delG) with audiologic profiles. A total of 10 different mutations and 11 different genotypes were evidenced in 73 SNHL subjects; 35delG (90.36 % of cases) and IVS1+1 (13.69 %) were the most common mutations found in the cohort with a significant difference in the distribution between North and South Sicily. Audiological evaluation revealed a severe (16/73) to profound (47/73) hearing loss (HL) in 86.13 % of cases without significant difference between the degree of HL and the province of origin of t…
Screening e post-screening (Audiologia essenziale)
2014
Newborn hearing screening is a type of screening test for the early detection of hearing loss. It can recognize with good accuracy newborns affected by hearing impairment allowing an early diagnosis and intervention and avoiding cognitive and linguistic deficits [1,2,3,4,5,6]. The incidence of bilateral sensorineural hearing loss (SNHL) in Sicily is 2.35 cases per 1000 newborns; this value increases to 2.95 if we consider also unilateral SNHL [2,3] and to 10 cases per 1000 births among infants at risk [7,8,9]. A correct newborn hearing screening programme is based on different protocols depending on the presence/absence of audiologic risk factors: • Newborns without risk factors: [1,2,3] In…
TINNITUS PATIENTS: ETIOLOGIC, AUDIOLOGIC AND PSYCHOLOGICAL PROFILE
2013
Tinnitus represents one of the most frequent symptoms observed in the general population in association with different pathologies, although often its etiology remains unclear. Objective of this work is to evidence the main aspects concerning epidemiology, causes, audiological characteristics and psychological consequences of tinnitus.
Advantages of binaural hearing provided through bimodal stimulation via a cochlear implant and a conventional hearing aid: a 6-month comparative stud…
2005
Our study data demonstrate the additional benefit derived from continued use of a contralateral hearing aid (HA) post-cochlear implantation for speech recognition ability in quiet and in noise. Postoperative bimodal stimulation is recommended for all subjects who show some speech recognition ability in the contralateral ear as it may offer binaural listening advantages in various listening situations encountered in everyday life.To assess the benefits derived from bimodal stimulation for experienced HA users implanted with a cochlear implant (CI) (score=20% in disyllabic test). The correlation between pre- and postoperative performance on speech perception measures was examined to determine…
Interactions in the network of Usher syndrome type 1 proteins
2004
International audience; Defects in myosin VIIa, harmonin (a PDZ domain protein), cadherin 23, protocadherin 15 and sans (a putative scaffolding protein), underlie five forms of Usher syndrome type I (USH1). Mouse mutants for all these proteins exhibit disorganization of their hair bundle, which is the mechanotransduction receptive structure of the inner ear sensory cells, the cochlear and vestibular hair cells. We have previously demonstrated that harmonin interacts with cadherin 23 and myosin VIIa. Here we address the extent of interactions between the five known USH1 proteins. We establish the previously suggested sans-harmonin interaction and find that sans also binds to myosin VIIa. We …
[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…
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.
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 …
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…
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.