1,720,999 research outputs found
Recent and future developments in cochlear implant technology: Review of the literature
No full textCochlear implant (CI) procedure and CIs have a long history filled with innovations, that have resulted in the high performing devices currently available. The development has been both rapid and remarkable and technological developments have yielded substantial gains in the possibility of communication, development of language skills, spoken words recognition and media enjoyment for the users. Multidisciplinary research among medical and engineering fields will continue to be critical in the development of new technology to increase the number candidates for this kind of hearing devices, to improve usability of the device and the results. The future of implantable auditory devices should place an emphasis on making devices accessible to patients with progressively more residual hearing, improving the safety profile and optimizing the electro-neural interface and neural stimulation, improving the usability of the device, finally leading to an improvement of the outcome and of the quality of life. The following are the main research topics and areas of development for cochlear implant technology, addressed in the paper: totally implanted devices, remote CI programming, minimizing surgical trauma, improved neural health and survival through targeted drug therapy and delivery, intraneural electrode placement and methods to enhance the neural-prosthesis interface
Enlarged vestibular aqueduct and Mondini Malformation: audiological, clinical, radiologic and genetic features
Full text linkPurpose: When referring to enlarged vestibular aqueduct (EVA) we should differentiate between nonsyndromic enlarged vestibular aqueduct (NSEVA) and Pendred Syndrome (PDS), a disease continuum associated with pathogenic sequence variants of Pendrin’s Gene (SLC26A4) in about half of the cases. The study was aimed to analyse the clinical and audiological features of a monocentric cohort of Caucasian patients with NSEVA/PDS, their genetic assessment and morphological inner ear features. Methods: We retrospectively reviewed the audiologic, genetic and anamnestic data of 66 patients with NSEVA/PDS followed by our audiology service. Results: SLC26A4 mutations was significantly correlated with the presence of PDS rather than NSEVA (p < 0.019), with the expression of inner ear malformations (p < 0.001) and with different severity of hearing loss (p = 0.001). Furthermore, patients with PDS showed significantly worse pure tone audiometry (PTA) than patients with NSEVA (p = 0.001). Anatomically normal ears presented significantly better PTA than ears associated with Mondini Malformation or isolated EVA (p < 0.001), but no statistically significative differences have been observed in PTA between patients with Mondini Malformation and isolated EVA. Conclusion: NSEVA/PDS must be investigated in all the congenital hearing loss, but also in progressive, late onset, stepwise forms. Even mixed or fluctuating hearing loss may constitute a sign of a NSEVA/PDS pathology. Our findings can confirm the important role of SLC26A4 mutations in determining the phenotype of isolated EVA/PDS, both for the type/degree of the malformation, the hearing impairment and the association with thyroid dysfunction
Exploring the genetic landscape of otosclerosis: current understanding and future perspectives
No full textOtosclerosis is characterised by abnormal bone remodelling in the otic capsule, leading to progressive hearing loss. Unlike many genetic disorders, the causative genes for otosclerosis remain largely unidentified despite extensive research using linkage analysis and genome-wide association studies (GWAS). Inheritance patterns in otosclerosis suggest a multifactorial model involving genetic predisposition and environmental triggers, a model applied to other common diseases, such as age-related hearing loss, coronary artery disease, and Alzheimer’s disease. Linkage analysis has identified nine loci associated with monogenic forms of otosclerosis, yet the specific causative genes and variants remain elusive. Promising insights have emerged from GWAS, with strong associations identified for novel candidate regions, including the RELN gene. Recent studies using next generation sequencing have identified several candidate genes such as SERPINF1, ACAN, and MEPE. SERPINF1, encoding pigment epithelium-derived growth factor, is linked to regulation of angiogenesis in bone remodelling. ACAN, associated with the OTSC1 locus, encodes aggrecan a crucial component of the extracellular matrix in cartilage, showing a range of variants with varied effect sizes and frequencies. MEPE, involved in bone homeostasis, has been significantly associated with otosclerosis in large family-based and case-control cohorts. While considerable progress has been made in identifying potential genetic contributors, the precise genetic architecture of otosclerosis remains to be fully elucidated. An integrated approach combining genetic data and clinical information, such as audiometric testing and temporal bone imaging, is essential for a comprehensive understanding of otosclerosis
Prognosis after acute unilateral vestibulopathy: Usefulness of the suppression head impulse paradigm (SHIMP)
No full textOBJECTIVES: This cross-sectional study aims to describe the features of the suppression head impulse paradigm (SHIMP) in acute unilateral vestibulopathy (AUV) and to define its role in predicting the recovery of patients. METHODS: Thirty patients diagnosed with AUV were retrospectively analyzed. The dizziness handicap inventory score and video head impulse test parameters performed 4-8 weeks from the AUV onset constituted the main outcome measures. Patients with a worse recovery (Group 1) and patients who recovered spontaneously (Group 2) were compared. RESULTS: The SHIMP vestibulo-ocular reflex (VOR) gain was statistically significantly lower than the conventional head impulse paradigm (HIMP) VOR gain (P < 0.001). The SHIMP VOR gain was negatively correlated with the DHI (P < 0.001) and was positively correlated with the HIMP VOR gain (P < 0.001) and the SHIMP overt saccades (%) (P < 0.001). Patients with a worse recovery exhibited the following: higher DHI (P < 0.001), lower SHIMP and HIMP VOR gain (P < 0.001 and P = 0.007, respectively), and lower SHIMP and greater HIMP overt saccade prevalence values (P = 0.007 and P = 0.032, respectively). CONCLUSIONS: The SHIMP and HIMP help in improving our approach to AUV. SHIMP appears to better identify the extent of the vestibular damage in patient suffering from AUV than HIMP and could provide interesting information about the course of the disease. Particularly, the analysis of SHIMP VOR gain and overt saccade prevalence would provide useful information about the recovery of patients
The role of the functional head impulse test with and without optokinetic stimuli in vestibular migraine and acute unilateral vestibulopathy: Discovering a dynamic visual dependence
Full text link(1) Background: Visually induced vertigo (i.e., vertigo provoked by moving visual scenes) can be considered a noticeable feature of vestibular migraines (VM) and can be present in patients suffering from acute unilateral vestibulopathy (AUV). Hypersensitivity to moving or conflicting visual stimulation is named visual dependence. (2) Methods: Visuo-vestibular interactions were analyzed via the functional Head Impulse Test (fHIT) with and without optokinetic stimulation (o-fHIT) in 25 patients with VM, in 20 subjects affected by AUV, and in 20 healthy subjects. We calculated the percentage of correct answers (Ê) without and with the addition of the optokinetic background (OB). (3) In VM groups, the Ê on the fHIT was 92.07% without OB and 73.66% with OB. A significant difference was found between Ê on the deficit side and that on the normal side in AUV, both without OB and with OB. (4) Conclusions: The fHIT results in terms of Ê with and without OB could be useful to identify the presence of a dynamic visual dependence, especially in patients suffering from VM. The difference in Ê with and without OB could provide instrumental support to help correctly identify subjects suffering from VM. We propose the use of the fHIT in clinical practice whenever there is a need to highlight a condition of dynamic visual dependence
Atypical antipsychotics: Drop-out as efficacy indicator
No full textIntroduction. The drop-out rate studies can be considered among the most reliable methods to estimate the effectiveness of drugs in everyday clinical practice. Aim. Estimation of effectiveness of the atypical antipsychotics through the study of drop-out rates; examination of the causes of drop-out and response predictors. Material and Method. The data concerning 793 patients who have benefited from the direct distribution of atypical antipsychotics at the Mental Health Department (DSM) of Massa Carrara from the 01/01/2000 to the 31/12/2002. Results. The data of 56 patients in treatment with clozapine, 339 with risperidone, 289 with olanzapine and 109 to you with quetiapine was examined. 40.9% of the patients have interrupted the treatment. The patients treated with quetiapine and clozapine have demonstrated the lowest drop-out rate. In patients treated with olanzapine, quetiapine and risperidone the onset age was lower in the drop-out group. The level of instruction was more elevated in the drop-out group of patients treated with olanzapine and risperidone. The bipolar patients continue the therapy with olanzapine and risperidone for longer, while quetiapine and clozapine result more effective in patients affected by disorders of schizophrenic spectrum. The percentage of ulterior interruptions of therapy for specific side-effects are similar in the four groups. Conclusions. The tallied differences, given the heterogeneity of the sample, the absence of standardized data collection systems, can only be sprones for hypotheses that need confirmation. Anyhow this research, carried out in authentic conditions, outside of the rigid protocols of the randomized clinical research, has supplied new information on profile of effectiveness and tolerability of atypical antipsychotics
Sequential bilateral cochlear implant: long-term speech perception results in children first implanted at an early age
Full text linkPurpose: The study aims to assess the benefit of sequential bilateral cochlear implantation in children with congenital bilateral profound hearing loss, submitted to the first implant at an early age. Methods: We enrolled all the bilateral sequential cochlear implanted children who received the first implant within 48 months and the second within 12 years of age at our Institution. The children were submitted to disyllabic word recognition tests and Speech Reception Threshold (SRT) assessment using the OLSA matrix sentence test with the first implanted device (CI1), with the second implanted device (CI2), and with both devices (CIbil). Furthermore, we measured the datalogging of both devices. Then we calculated the binaural SRT gain (b-SRTgain) and checked the correlations between speech perception results and the b-SRTgain with the child’s age at CI1 and CI2, DELTA and the datalogging reports. Results: With the bilateral electric stimulation, we found a significant improvement in disyllabic word recognition scores and in SRT. Moreover, the datalogging showed no significant differences in the time of use of CI1 and CI2. We found significant negative correlations between speech perception abilities with CI2 and age at CI2 and DELTA, and between the SRT with CI1 and the b-SRTgain. Conclusions: From this study we can conclude that in a sequential CI procedure, even if a short inter-implant delay and lower ages at the second surgery can lead to better speech perception with CI2, children can benefit from bilateral stimulation independently of age at the second surgery and the DELTA
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