1,720,964 research outputs found
Cochlear implant outcomes and genetic mutations in children with ear and brain anomalies
No full textIntroduction. Cochlear implantation (CI) was a significant surgical innovation in the 20th century and represented the first artificial sensory organ that was applied in clinical medicine. Currently, CI is still one of the most effective medical procedures. Nonetheless, cochlear implantation in adults and children represents a controversial issue from an economic, clinical and ethical point of view, especially in specific clinical conditions that could compromise the CI outcome and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. Objectives. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We analysed the effects of ear malformations and brain anomalies on the CI outcomes. Finally, we described the genetic mutations that we found in the study group. A control study group of implanted patients without ear and brain anomalies was obtained (virtually) from clinical and literature data for statistical purposes.
Materials and methods. The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations) on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests.
Results. Between 1 January 1996 and 1 April 2012 at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years). Among these, 143 patients (64 females and 79 males) presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children) ranged from 9 months and 16 years (average = 4.4; median = 3.0). The most common inner ear malformation is represented by an enlarged vestibular aqueduct; brain lesions are usually represented by white matter disorders. The 35delG in the GJB2 gene remain the most common mutation.
Discussion and Conclusions. Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities, even if central nervous system anomalies represent a negative prognostic factor that is made worse by the concomitant presence of cochlear malformations. Common cavity and stenosis of the internal auditory canal (less than 2 mm) are negative prognostic factors even if brain lesions are absent. Because the cochlear implantation is an invasive and expensive surgical procedure, the identification of predictive factors, even in hearing-impaired patients with cochlear and brain anomalies, is one of the most important goals, because it can help to guide rehabilitation programs that are tailored to meet the expectations of clinicians, teachers and parents. Our findings suggest that cochlear implantation (CI) is a safe and effective procedure even for patients with brain and inner ear abnormalities. Nonetheless, specific conditions, such as a common cavity, or in general, the absence of modiolus and the stenosis of the internal auditory canal, can increase the risk of post-operative complications and prevent the achievement of acceptable perceptual categories. For the aforementioned conditions, it is strictly recommended that cochlear implant indications, neuroimaging and surgery are performed in experienced hospitals
Auricular involvement of a multifocal non-AIDS Kaposi's sarcoma: a case report [Un caso di sarcoma di Kaposi, multifocale non-HIV correlato, dell’orecchio esterno]
No full textIl Sarcoma di Kaposi (KS) è una patologia neoplastica maligna su base vascolare, che principalmente coinvolge la cute e le mucose, caratterizzata dalla proliferazione di cellule endoteliali. L’eziologia del KS è ancora sconosciuta, sebbene sia stato riportato che fattori epidemiologici, ambientali e virali (es Herpes virus umano 8, HHV-8) possano avere un ruolo nella patogenesi di tale affezione. Ad oggi, sono state descritte quattro forme principali di KS: classico (sporadico), africano (endemico), iatrogeno (associato a stati di immunosoppressione) ed AIDS-relato (epidemico). Gli Autori presentano un caso di KS con coinvolgimento del condotto uditivo esterno, in una paziente HIV-negativa con storia di linfoma non-Hodgkin e tubercolosi.Kaposi's sarcoma (KS) is a multicentric, malignant neoplastic vascular disease, mainly involving skin and mucosae, characterised by the proliferation of endothelial cells. The aetiology of KS still is unknown. Nonetheless, it has been reported that several epidemiological and environmental factors may play a role in its pathogenesis. Viral factors (i.e. human herpes virus 8, HHV-8) have also been claimed to play a role in the onset of KS. Four main clinical presentations of KS have been described: classic (sporadic), African (endemic), iatrogenic (immunosuppression-associated) and AIDS-associated (epidemic). The authors present a case of KS involving the external ear of a HIVnegative patient with a history of non-Hodgkin lymphoma and tuberculosis
Ear and brain neuroradiological findings and outcomes in cochlear-implant candidate children
No full textCT and MRI are complementary for exhaustive evaluation of cochlear implant candidates. In pre-operative protocol we suggest to study not only the ear and petrous mastoid structures, but oalso the brain. In fact whole MRI can demonstrate anomalies in children without neurological signs or symptoms, and can explain the wide variation of performance across individual cochlear implant user
The universal newborn hearing screening program at the University Hospital of Ferrara: focus on costs and software solutions
No full textIn the present paper, the authors report the results of the Universal Newborn Hearing Screening (UNHS) project at the University Hospital of Ferrara. A total of 6,759 full-term newborns and a total of 1,016 NICU babies were tested at the University Hospital of Ferrara, from January 2000 to December 2006. The paper presents information from clinically acceptable screening procedures developed and tested during the 6 years of the program and addresses two questions pertinent to hearing screening: (i) the cost-estimate of a UNHS program based on European economical and administration premises and (ii) the development of a database-structure for the evaluation of the UNHS/NHS performance and the individual patient tracking
Hearing threshold assessment in young children with Electrocochleograpy (EcochG) and auditory brainstem responses (ABR): Experience at the University Hospital of Ferrara
Full text linkObjective: Electrophysiological evaluation is a fundamental procedure for the diagnostic assessment of hearing loss during infancy; in these cases, information concerning threshold level and auditory perception is particularly useful to establish a correct hearing rehabilitation program (hearing aids and cochlear implants). Purpose of this study is to underline the role of auditory brainstem responses (ABR) and electrocochleography (EcochG) in the definition of hearing loss in a selected group of children, referred to the Audiology Department of the University Hospital of Ferrara, for a tertiary level audiological assessment.
Methods: A retrospective study of the paediatric patient database at the Audiology Department of the University Hospital of Ferrara has been performed. In a period between January 2000 and December 2007, a total of 272 paediatric cases have been identified (544 ears). An EM 12 Mercury apparatus has been used for the electrophysiological threshold identification (ABR and EcochG). Recordings were carried out under general anaesthesia, in a protected enviroment.
Results: In 19 of the 272 paediatric cases selected—38 ears (7%), the results of threshold evaluation through ABR were uncertain. The Ecochg recording resulted crucial for the final diagnosis in terms of definition of the hearing threshold level, and it was then possible to ensure the better hearing rehabilitation strategy.
Conclusions: ABR has to be considered the first choice in hearing assessment strategy, either for screening or for diagnosis in newborns as well as in non-collaborating children; ECochG still may be considered a reliable diagnostic tool
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Appropriate Similarity Measures for Author Cocitation Analysis
Full text linkWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
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