Quantitative Speech Assessment in Ataxia-Consensus Recommendations by the Ataxia Global Initiative Working Group on Digital-Motor Markers.

Dysarthria is a common and debilitating symptom of many neurodegenerative diseases, including those resulting in ataxia. Changes to speech lead to significant reductions in quality of life, impacting the speaker in most daily activities. Recognition of its importance as an objective outcome measure in clinical trials for ataxia is growing. Its viability as an endpoint across the disease spectrum (i.e. pre-symptomatic onwards) means that trials can recruit ambulant individuals and later-stage individuals who are often excluded because of difficulty completing lower limb tasks. Here we discuss the key considerations for speech testing in clinical trials including hardware selection, suitability of tasks and their role in protocols for trials and propose a core set of tasks for speech testing in clinical trials. Test batteries could include forms suitable for remote short, sensitive and easy to use, with norms available in several languages. The use of artificial intelligence also could improve accuracy and automaticity of analytical pipelines in clinic and trials.

Sensitivity and specificity of an acoustic- and perceptual-based tool for assessing motor speech disorders in French: the MonPaGe-screening protocol.

To respond to the need of objective screening tools for motor speech disorders (MSD), we present the screening version of a speech assessment protocol (MonPaGe-2.0.s), which is based on semi-automated acoustic and perceptual measures on several speech dimensions in French. We validate the screening tool by testing its sensitivity and specificity and comparing its outcome with external standard assessment tools. The data from 80 patients diagnosed with different types of mild to moderate MSD and 62 healthy test controls were assessed against the normative data obtained on 404 neurotypical speakers, with Deviance Scores computed on seven speech dimensions (voice, speech rate, articulation, prosody, pneumophonatory control, diadochokinetic rate, intelligibility) based on acoustic and perceptual measures. A cut-off of the MonPaGe total deviance score (TotDevS) >2 allowed MSD to be diagnosed with specificity of 95% and an overall sensitivity of 83.8% on all patients pulled, reaching 91% when very mildly impaired patients were excluded. A strong correlation was found between the MonPaGe TotDevS and an external composite perceptual score of MSD provided by six experts. The MonPaGe screening protocol has proven its sensitivity and specificity for diagnosing presence and severity of MSD. Further implementations are needed to complement the characterization of impaired dimensions in order to distinguish subtypes of MSD.

First European report of Francisella tularensis subsp. holarctica isolation from a domestic cat.

Francisella tularensis subsp. holarctica is a select agent causing life-threatening tularemia. It has been isolated from humans and animals, mainly lagomorphs and rodents, rarely other wild carnivore species. Increasing numbers of human tularemia cases have been reported during the last 5 years in Switzerland. Here we report the first isolation of Francisella tularensis subsp. holarctica from a domestic cat in Europe and compare its genome sequence with other Swiss isolates. The cat isolate shows a close phylogenetic relationship with a contemporary hare isolate from close geographic proximity, indicating a possible epidemiological link.

Benefit of the UltraZoom beamforming technology in noise in cochlear implant users.

The objectives of the study were to demonstrate the audiological and subjective benefits of the adaptive UltraZoom beamforming technology available in the Naída CI Q70 sound processor, in cochlear-implanted adults upgraded from a previous generation sound processor. Thirty-four adults aged between 21 and 89 years (mean 53 ± 19) were prospectively included. Nine subjects were unilaterally implanted, 11 bilaterally and 14 were bimodal users. The mean duration of cochlear implant use was 7 years (range 5-15 years). Subjects were tested in quiet with monosyllabic words and in noise with the adaptive French Matrix test in the best-aided conditions. The test setup contained a signal source in front of the subject and three noise sources at +/-90° and 180°. The noise was presented at a fixed level of 65 dB SPL and the level of speech signal was varied to obtain the speech reception threshold (SRT). During the upgrade visit, subjects were tested with the Harmony and with the Naída CI sound processors in omnidirectional microphone configuration. After a take-home phase of 2 months, tests were repeated with the Naída CI processor with and without UltraZoom. Subjective assessment of the sound quality in daily environments was recorded using the APHAB questionnaire. No difference in performance was observed in quiet between the two processors. The Matrix test in noise was possible in the 21 subjects with the better performance. No difference was observed between the two processors for performance in noise when using the omnidirectional microphone. At the follow-up session, the median SRT with the Naída CI processor with UltraZoom was -4 dB compared to -0.45 dB without UltraZoom. The use of UltraZoom improved the median SRT by 3.6 dB (p < 0.0001, Wilcoxon paired test). When looking at the APHAB outcome, improvement was observed for speech understanding in noisy environments (p < 0.01) and in aversive situations (p < 0.05) in the group of 21 subjects who were able to perform the Matrix test in noise and for speech understanding in noise (p < 0.05) in the group of 13 subjects with the poorest performance, who were not able to perform the Matrix test in noise. The use of UltraZoom beamforming technology, available on the new sound processor Naída CI, improves speech performance in difficult and realistic noisy conditions when the cochlear implant user needs to focus on the person speaking at the front. Using the APHAB questionnaire, a subjective benefit for listening in background noise was also observed in subjects with good performance as well as in those with poor performance. This study highlighted the importance of upgrading CI recipients to new technology and to include assessment in noise and subjective feedback evaluation as part of the process.

Genetic identification of an oxyurid from a captive, black-handed spider monkey--implications for treatment and control.

Parasites are of major clinical significance in captive primates in zoos, particularly those with direct life cycles. Oxyurid nematodes can be a persistent problem, as infection intensity and environmental contamination with infective eggs are usually high. Observations at the Basel Zoo in Switzerland have revealed that particularly black-handed spider monkeys (Ateles geoffroyi) exhibit continuous oxyurid nematode infection(s), despite regular deworming with anthelmintics. In the present study, using a molecular approach, we were able to identify the nematode (Trypanoxyuris atelis) causing this ongoing problem, and we are now evaluating a practical treatment and control regimen to tackle this parasite problem.

Cochlear implant programming: a global survey on the state of the art.

The programming of CIs is essential for good performance. However, no Good Clinical Practice guidelines exist. This paper reports on the results of an inventory of the current practice worldwide. A questionnaire was distributed to 47 CI centers. They follow 47600 recipients in 17 countries and 5 continents. The results were discussed during a debate. Sixty-two percent of the results were verified through individual interviews during the following months. Most centers (72%) participated in a cross-sectional study logging 5 consecutive fitting sessions in 5 different recipients. Data indicate that general practice starts with a single switch-on session, followed by three monthly sessions, three quarterly sessions, and then annual sessions, all containing one hour of programming and testing. The main focus lies on setting maximum and, to a lesser extent, minimum current levels per electrode. These levels are often determined on a few electrodes and then extrapolated. They are mainly based on subjective loudness perception by the CI user and, to a lesser extent, on pure tone and speech audiometry. Objective measures play a small role as indication of the global MAP profile. Other MAP parameters are rarely modified. Measurable targets are only defined for pure tone audiometry. Huge variation exists between centers on all aspects of the fitting practice.

Investigations on the Potential Role of Free-Ranging Wildlife as a Reservoir of SARS-CoV-2 in Switzerland.

Amid the SARS-CoV-2 pandemic, concerns surfaced regarding the spread of the virus to wildlife. Switzerland lacked data concerning the exposure of free-ranging animals to SARS-CoV-2 during this period. This study aimed to investigate the potential exposure of Swiss free-ranging wildlife to SARS-CoV-2. From 2020 to 2023, opportunistically collected samples from 712 shot or found dead wild mustelids (64 European stone and pine martens, 13 European badgers, 10 European polecats), canids (449 red foxes, 41 gray wolves, one golden jackal) and felids (56 Eurasian lynx, 18 European wildcats), as well as from 45 captured animals (39 Eurasian lynx, 6 European wildcats) were tested. A multi-step serological approach detecting antibodies to the spike protein receptor binding domain (RBD) and N-terminal S1 subunit followed by surrogate virus neutralization (sVNT) and pseudotype-based virus neutralization assays against different SARS-CoV-2 variants was performed. Additionally, viral RNA loads were quantified in lung tissues and in oronasal, oropharyngeal, and rectal swabs by reverse transcription polymerase chain reactions (RT-qPCRs). Serologically, SARS-CoV-2 exposure was confirmed in 14 free-ranging Swiss red foxes (prevalence 3.1%, 95% CI: 1.9-5.2%), two Eurasian lynx (2.2%, 95% CI: 0.6-7.7%), and one European wildcat (4.2%, 95% CI: 0.2-20.2%). Two positive foxes exhibited neutralization activity against the BA.2 and BA.1 Omicron variants. No active infection (viral RNA) was detected in any animal tested. This is the first report of SARS-CoV-2 antibodies in free-ranging red foxes, Eurasian lynx, and European wildcats worldwide. It confirms the spread of SARS-CoV-2 to free-ranging wildlife in Switzerland but does not provide evidence of reservoir formation. Our results underscore the susceptibility of wildlife populations to SARS-CoV-2 and the importance of understanding diseases in a One Health Concept.

Analysis of a European general wildlife health surveillance program: Chances, challenges and recommendations.

In a One Health perspective general wildlife health surveillance (GWHS) gains importance worldwide, as pathogen transmission among wildlife, domestic animals and humans raises health, conservation and economic concerns. However, GWHS programs operate in the face of legal, geographical, financial, or administrative challenges. The present study uses a multi-tiered approach to understand the current characteristics, strengths and gaps of a European GWHS that operates in a fragmented legislative and multi-stakeholder environment. The aim is to support the implementation or improvement of other GWHS systems by managers, surveillance experts, and administrations. To assess the current state of wildlife health investigations and trends within the GWHS, we retrospectively analyzed 20 years of wildlife diagnostic data to explore alterations in annual case numbers, diagnosed diseases, and submitter types, conducted an online survey and phone interviews with official field partners (hunting administrators, game wardens and hunters) to assess their case submission criteria as well as their needs for post-mortem investigations, and performed in-house time estimations of post-mortem investigations to conduct a time-per-task analysis. Firstly, we found that infectious disease dynamics, the level of public awareness for specific diseases, research activities and increasing population sizes of in depth-monitored protected species, together with biogeographical and political boundaries all impacted case numbers and can present unexpected challenges to a GWHS. Secondly, we found that even a seemingly comprehensive GWHS can feature pronounced information gaps, with underrepresentation of common or easily recognizable diseases, blind spots in non-hunted species and only a fraction of discovered carcasses being submitted. Thirdly, we found that substantial amounts of wildlife health data may be available at local hunting administrations or disease specialist centers, but outside the reach of the GWHS and its processes. In conclusion, we recommend that fragmented and federalist GWHS programs like the one addressed require a central, consistent and accessible collection of wildlife health data. Also, considering the growing role of citizen observers in environmental research, we recommend using online reporting systems to harness decentrally available information and fill wildlife health information gaps.

Rehabilitation and release of orphaned Eurasian lynx (Lynx lynx) in Europe: Implications for management and conservation.

Rehabilitation of injured or immature individuals has become an increasingly used conservation and management tool. However, scientific evaluation of rehabilitations is rare, raising concern about post-release welfare as well as the cost-effectiveness of spending scarce financial resources. Over the past 20 years, events of juvenile Eurasian lynx presumably orphaned have been observed in many European lynx populations. To guide the management of orphaned lynx, we documented survival, rehabilitation and fate after the release and evaluated the potential relevance of lynx orphan rehabilitation for population management and conservation implications. Data on 320 orphaned lynx was collected from 1975 to 2022 from 13 countries and nine populations. The majority of orphaned lynx (55%) were taken to rehabilitation centres or other enclosures. A total of 66 orphans were released back to nature. The portion of rehabilitated lynx who survived at least one year after release was 0.66. Release location was the best predictor for their survival. Of the 66 released lynx, ten have reproduced at least once (8 females and 2 males). Conservation implications of rehabilitation programmes include managing genetic diversity in small, isolated populations and reintroducing species to historical habitats. The lynx is a perfect model species as most reintroduced populations in Central Europe show significantly lower observed heterozygosity than most of the autochthonous populations, indicating that reintroduction bottlenecks, isolation and post-release management have long-term consequences on the genetic composition of populations. The release of translocated orphans could be a valuable contribution to Eurasian lynx conservation in Europe. It is recommended to release orphans at the distribution edge or in the frame of reintroduction projects instead of a release in the core area of a population where it is not necessary from a demographic and genetic point of view. Rehabilitation programmes can have conservation implications that extend far beyond individual welfare benefits.

Is electrode-modiolus distance a prognostic factor for hearing performances after cochlear implant surgery?

The aim of this study was to evaluate electrode array position in relation to cochlear anatomy and its influence on hearing performance in cochlear implantees. Twenty-two patients (25 ears) with Med-El cochlear implants were included in this retrospective study. A negative correlation was observed between electrode-modiolus distance (EMD) at the cochlear base and monosyllabic word discrimination 6 months after implantation. We found no correlation between EMD and hearing outcome at 12 months. The insertion depth/cochlear perimeter ratio appeared to negatively influence the EMD at the base. Indeed, deep insertions in small cochleae appeared to yield smaller EMD and better hearing performance. This observation supports the idea of preplanning the surgery by adapting the electrode array to the length of the available scala tympani.

New cochlear implant technologies improve performance in post-meningitic deaf patients.

The objective of the study was to compare the performance of cochlear implantation between post-meningitic and non-meningitic patients, and to evaluate the impact on hearing outcome of technical advances in cochlear implant technology. Retrospective chart review was used as the study design. Twenty adults with post-meningitic profound hearing loss receiving unilateral or bilateral cochlear implants between 1990 and 2008 were tested. Results were compared to a control group of 46 adults implanted for a non-meningitic hearing loss, with the same pre-operative speech scores. Speech scores were poorer in post-meningitic patients compared to those of control group, whatever the duration after implantation (p < 0.0001). Speech scores of subjects implanted and fitted before 2001 were compared to those of subjects implanted after 2001, with the same duration of hearing loss. Performance improved with implants and processors available after 2001, with a magnitude of improvement higher in post-meningitic patients (p < 0.0001 and p < 0.05 in post-meningitic and control groups, respectively, two-way ANOVA). Consequently, speech scores of post-meningitic patients implanted after 2001 achieved those of control subjects (two-way ANOVA). Advances in cochlear implant technology and coding strategy improve hearing outcome in post-meningitic adult patients, who now achieve similar performance as those of non-meningitic patients.

MULTISYSTEMIC EMPHYSEMA (GAS BUBBLE DISEASE)-ASSOCIATED ACUTE MASS MORTALITY IN A FREE-RANGING POPULATION OF COMMON FROG (RANA TEMPORARIA) IN SWITZERLAND.

In April 2020, nearly 5,000 free-ranging common frogs (Rana temporaria) were found dead on the surface of the water in a retention pond in the Swiss Alps. Macroscopic and microscopic lesions revealed multisystem emphysema, affecting multiple organs. The most severe lesions were seen in the skin, eyes, and blood vessels of internal organs and were secondary to the sudden massive distension of the skin and other affected organs. All frogs had similar lesions consistent with those described associated with gas bubble disease. No obvious pre-existing conditions potentially priming the occurrence of the observed lesions could be detected. All the examined frogs were negative by PCR for Batrachochytrium dendrobatidis, Ranavirus and Ranid Herpesvirus 3 (now Batravirus ranidallo 3). The proposed etiology is considered to be an undetermined physical event, leading to an abrupt change in the molecular or physical characteristics of the water (namely pressure and oxygen or other gas supersaturation), resulting in the occurrence of the observed lesions in the frogs. No obvious pumping system malfunction was recorded in the Mägisalp ponds before the mass mortality, but a sudden and temporary undetected change in the water flow, which then quickly rebalanced, cannot be excluded. Other hypotheses include weather conditions, such as lightning strikes in the water, or a device detonating in the water.

Preliminary evaluation of computer-assisted home training for French cochlear implant recipients.

For French cochlear implant (CI) recipients, in-person clinical auditory rehabilitation is typically provided during the first few years post-implantation. However, this is often inconvenient, it requires substantial time resources and can be problematic when appointments are unavailable. In response, we developed a computer-based home training software ("French AngelSound™") for French CI recipients. We recently conducted a pilot study to evaluate the newly developed French AngelSound™ in 15 CI recipients (5 unilateral, 5 bilateral, 5 bimodal). Outcome measures included phoneme recognition in quiet and sentence recognition in noise. Unilateral CI users were tested with the CI alone. Bilateral CI users were tested with each CI ear alone to determine the poorer ear to be trained, as well as with both ears (binaural performance). Bimodal CI users were tested with the CI ear alone, and with the contralateral hearing aid (binaural performance). Participants trained at home over a one-month period (10 hours total). Phonemic contrast training was used; the level of difficulty ranged from phoneme discrimination in quiet to phoneme identification in multi-talker babble. Unilateral and bimodal CI users trained with the CI alone; bilateral CI users trained with the poorer ear alone. Outcomes were measured before training (pre-training), immediately after training was completed (post-training), and one month after training was stopped (follow-up). For all participants, post-training CI-only vowel and consonant recognition scores significantly improved after phoneme training with the CI ear alone. For bilateral and bimodal CI users, binaural vowel and consonant recognition scores also significantly improved after training with a single CI ear. Follow-up measures showed that training benefits were largely retained. These preliminary data suggest that the phonemic contrast training in French AngelSound™ may significantly benefit French CI recipients and may complement clinical auditory rehabilitation, especially when in-person visits are not possible.

Exploring the epidemiological role of the Eurasian lynx (Lynx lynx) in the life cycle of Toxoplasma gondii.

Toxoplasma gondii is a successful coccidian parasite able to infect all warm-blooded animals and humans, causing one of the most common zoonoses worldwide. The Eurasian lynx (Lynx lynx) is one of the feline potential hosts of T. gondii in Switzerland, but little is known about its epidemiological role as a definitive or intermediate host. Serum samples from 183 Eurasian lynx collected from 2002 to 2021 were tested for antibodies to T. gondii by ELISA, IFAT and in case of inconclusive results, immunoblot. Antibodies to T. gondii were found in 150 of 183 (82%) Eurasian lynx. Older age, good health status and a low-altitude habitat were found to be significant predictors for seropositivity. T. gondii oocysts were detected in 3 of 176 (1.7%) faecal samples, indicating the Eurasian lynx as a definitive host. In addition, T. gondii DNA was detected in skeletal muscle (7/88), heart muscle (2/26) and/or brain tissue (2/36) from 10 different lynx by real-time PCR. In one animal, a T. gondii-like tissue cyst was observed in heart muscle and confirmed as T. gondii by immunohistochemistry (1/20) and real-time PCR. With an adapted nested-PCR-multilocus-sequence typing (MLST) and in silico restriction-fragment-length-polymorphism analysis (RFLP) approach two different T. gondii genotypes were detected: a lineage II variant (ToxoDB #3) in three animals (two oocyst samples and one heart muscle sample) and a novel genotype exhibiting both type II and III alleles in a further animal (skeletal muscle). The present results indicate that T. gondii infection is widespread in the Swiss lynx population. The Eurasian lynx may contribute to environmental contamination with oocysts and is able to harbour the parasite in different tissues. Genotyping revealed the presence of both a common T. gondii lineage in Europe and a previously unknown genotype and thus shedding more light on the complex molecular epidemiology of T. gondii.

Patient-reported, health economic and psychosocial outcomes in patients with Friedreich ataxia (PROFA): protocol of an observational study using momentary data assessments via mobile health app.

INTRODUCTION: Friedreich ataxia (FA) is the most common hereditary ataxia in Europe, characterised by progressively worsening movement and speech impairments with a typical onset before the age of 25 years. The symptoms affect the patients' health-related quality of life (HRQoL) and psychosocial health. FA leads to an increasing need for care, associated with an economic burden. Little is known about the impact of FA on daily lives and HRQoL. To fill that gap, we will assess patient-reported, psychosocial and economic outcomes using momentary data assessment via a mobile health application (app). METHODS AND ANALYSIS: The PROFA Study is a prospective observational study. Patients with FA (n=200) will be recruited at six European study centres (Germany, France and Austria). We will interview patients at baseline in the study centre and subsequently assess the patients' health at home via mobile health app. Patients will self-report ataxia severity, HRQoL, speech and hearing disabilities, coping strategies and well-being, health services usage, adverse health events and productivity losses due to informal care on a daily to monthly basis on the app for 6 months. Our study aims to (1) validate measurements of HRQoL and psychosocial health, (2) assess the usability of the mobile health app, and (3) use descriptive and multivariate statistics to analyse patient-reported and economic outcomes and the interaction effects between these outcomes. Insights into the app's usability could be used for future studies using momentary data assessments to measure outcomes of patients with FA. ETHICS AND DISSEMINATION: Ethical approval has been obtained from the Ethics Committee of the University Medicine of Greifswald, (BB096/22a, 26 October 2022) and from all local ethics committees of the participating study sites. Findings of the study will be published in peer-reviewed journals, presented at relevant international/national congresses and disseminated to German and French Patient Advocacy Organizations. TRIAL REGISTRATION NUMBER: ClinicalTrials.gov Registry (NCT05943002); Pre-results.

[Early management of presbycusis: recommendations from the French Society of Otorhinolaryngology and Head and Neck Surgery, the French Society of Audiology, and the French Society of Geriatrics and Gerontology]. / Recommandations de la Société française d'otorhinolaryngologie et de chirurgie de la face et du cou, la Société française d'audiologie et la Société française de gériatrie et gérontologie pour la prise en charge précoce de la presbyacousie.

INTRODUCTION: Presbycusis is the physiological decrease in hearing due to advancing age and begins well before the sixth decade. These recommendations recall the principles of early diagnosis of presbycusis and the means of optimal rehabilitation as soon as the first symptoms appear. MATERIAL AND METHODS: The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of doctors and audioprosthetists from all over France. They are graded A, B, C or expert opinion according to decreasing level of scientific evidence. RESULTS: The diagnosis of presbycusis is more difficult at the beginning of its evolution but a certain number of tools are available for its early diagnosis and its management in face-to-face or even distance learning. CONCLUSION: In case of a clinical profile suggestive of presbycusis in a young subject, especially if there are several family cases, it is recommended to propose a genetic investigation. It is recommended to perform free-field speech audiometry in noise to measure intelligibility in an environment as close as possible to reality. Questionnaires can be used in addition to audiometry to best assess the patient's disability. It is recommended that hearing rehabilitation with a hearing aid or cochlear implant may slow or prevent cognitive decline. Combined auditory and cognitive rehabilitation should be offered regardless of the time elapsed since the fitting. It is recommended to integrate programs accessible via smartphones, tablets or the Internet, integrating different training domains in addition to face-to-face sessions.

Early management of presbycusis: recommendations from the French Society of Otorhinolaryngology and Head and Neck Surgery, the French Society of Audiology, and the French Society of Geriatrics and Gerontology.

INTRODUCTION: Presbycusis is the physiological decrease in hearing due to advancing age and begins well before the sixth decade. These recommendations recall the principles of early diagnosis of presbycusis and the means of optimal rehabilitation as soon as the first symptoms appear. MATERIAL AND METHODS: The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of ENT physicians, audiologists, geriatricians and hearing specialists from all over France. They are classified as grade A, B, C or professional agreement according to a decreasing level of scientific evidence. RESULTS: The diagnosis of presbycusis is more difficult at the beginning of its evolution but a certain number of tools are available for its early diagnosis and its face-to-face or remote management. CONCLUSION: In the case of a clinical profile suggestive of presbycusis in a young subject, especially if there are several family cases, it is recommended to propose a genetic investigation. Free-field speech audiometry in noise is recommended to measure intelligibility in a realistic environment. Questionnaires in addition to audiometric tests would allow the best assessment of the patient's disability. Hearing rehabilitation with a hearing aid or cochlear implant may slow or prevent cognitive decline. Combined auditory and cognitive rehabilitation should be offered regardless of the time since the hearing was fitting. It is recommended to integrate programs accessible via smartphones, tablets or the Internet, that include different training domains to complement face-to-face sessions.

Hearing preservation after cochlear implantation using deeply inserted flex atraumatic electrode arrays.

The aim of this study was to investigate hearing preservation in adults receiving cochlear implants with Flex arrays. Fifteen adults (19 ears) implanted with Flex EAS® (n = 4) or Flex Soft® (n = 15; MedEL, Innsbruck, Austria) were included in this retrospective study. The mean array insertion length was 25 ± 0.9 mm (n = 19), and the mean cochlear coverage was 435 ± 14.5° (n = 19), with no difference between EAS and Soft arrays. Residual low-frequency hearing was preserved in all implanted ears but deteriorated [pure-tone average (125-1000 Hz) 55.1 ± 2.90 dB before vs. 81.0 ± 3.02 dB after surgery (n = 19, p < 0.01)]. Both Flex arrays allow deep insertion with reproducible hearing preservation.

The French version of Speech Handicap Index: validation and comparison with the Voice Handicap Index.

OBJECTIVE: This study aimed to adapt the Speech Handicap Index (SHI) in French, to compare it with the Voice Handicap Index (VHI) and to study its relevance in dysarthric patients. PATIENTS AND METHODS: Two hundred and twenty-nine subjects filled out the French versions of the SHI and VHI. SHI test-retest reliability was performed on 82 subjects. Psychometric properties were analyzed in 112 healthy controls and 89 patients with speech disorders. Mean scores of 89 speech-disordered and 28 dysphonic patients were obtained. RESULTS: For SHI, internal consistency (Cronbach's alpha >0.9) and test-retest reliability (Spearman's r > 0.75) were high. Mean scores were significantly different between healthy subjects (8/120) and patients (52/120) (p < 0.0001). Scores were statistically different between dysphonic (40/120) and speech-disordered patients (52/120) (p < 0.05), and also between patients with oropharyngeal surgery (44/120) and dysarthric patients (57/120) (p < 0.05). Patients with speech difficulties had higher scores on the SHI than dysphonic patients (p < 0.05). CONCLUSION: The French SHI is a reliable and sensitive tool. It can be proposed to all patients with speech disorders. The contrasting results between the SHI and the VHI highlight the importance in giving the appropriate questionnaire tailored to the patient's pathology.

Cochlear implant in immune mediated inner ear diseases: Impedance variations and clinical outcomes.

OBJECTIVE: Immune-mediated inner ear disease (IMIED) might cause severe/profound hearing loss and these patients are considered ideal candidates to cochlear implant (CI) surgery. The aim of the study was to evaluate impedance changes over time. METHOD: The Study Group (SG) was composed of CI IMIED patients (31 ears) and a Control Group (CG) of CI patients with hearing loss not related to their immune system (31 ears). Audiological performance and impedance values were measured and compared amongst groups at 3, 6, 12 and 18 months following the fitting sessions. RESULTS: Speech perception was significantly better for SG in word and sentence recognition in quiet. Impedance values were, on average, significantly higher for apical and middle electrode segments in SG compared to CG at the 3- month follow-up and were maintained over time. Additionally, a subset of SG patients (active patients) experienced significantly greater impedance fluctuation corresponding to clinical symptom reactivation. CONCLUSION: IMIED patients achieve good audiological performance. However, the relapsing inflammation could change the inner ear environment, causing impedance fluctuations and, consequently, more frequent CI fittings. Additionally, impedance evaluation could be utilized as an early warning sign of IMIED recurrence and as an aid to therapeutic decision-making.