Otospongiosis is an osteodystrophy of the temporal bone, characterized by disordered neoformation and deposition of bone, characterized by the presence of a progressive conductive, sensorineural or mixed hearing loss and tinnitus. Typically, otospongiosis presents as a slowly progressive conductive hearing loss in the third to fourth decade of life. Uncommonly children and adolescents may also have conductive or sensorineural hearing loss caused by otosclerosis. We describe a case of an 11-year-old patient, with progressive unilateral conductive hearing loss for 5 years. The otoscopic examination revealed a positive Schwartz's sign in the left ear. Audiometry, impedanciometry and CT scan showed characteristics that suggested otospongiosis. We reviewed clinical aspects, diagnosis and the therapeutic approach for otospongiosis in children. Sensorineural hearing loss, aural fullness and vertigo may eventually ensue. A clinical picture similar to endolymphatic hydrops may be seen in severe cases. The onset of auditory symptoms usually is between ages 20 and 30 years.The diagnosis is made based on the clinical history, the physical examination, and tests such as pure tone audiometry, voice audiometry and immitance testing. Image tests may also provide relevant diagnostic information; computed tomography (CT) is the preferred method.The treatment may be medical (anti-enzyme or anti-bone remodeling drugs) or surgical (stapedotomy or stapedectomy). Personal sound amplification devices (PSAD) are a further option, particularly in patients with surgical contraindications.The aim of this study was to report a case of child otospongiosis and to review the literature on this theme.,,Although otospongiosis is considered a disease of young adults, hearing loss may begin in the first few years of life. 10 Niedermeyer et al. reported signs of this disease in children under age 6 years.,High resolution computed tomography of the temporal bone - which is the image test of choice in this condition - makes it possible to detect abnormalities in the oval window or close to it in 90% of cases with surgical proof of the disease.The differential diagnosis is made with otitis media with effusion, congenital fixation of the stapes, disarticulation of the middle ear bones, tympanosclerosis, osteogenesis imperfecta, Paget's disease and congenital cholesteatoma.A white male patient, aged 11 years, presented at our unit complaining of unilateral progressive hearing loss for 5 years, and moderate, intermittent, whistle-like tinnitus; there were no vestibular symptoms. The patient reported repeated episodes of otitis media with effusion during the past 5 years that was resistant to the usual medical treatment. The patient had a family history of otospongiosis .The otorhinolaryngological exam revealed a reddish hue seen by transparency through the tympanic membrane in the promontory of the left ear (Schwartze's sign) and no right ear alterations. Pure tone audiometry showed mild left ear conductive hearing loss; the right ear hearing thresholds were within normal limits. Immitance testing revealed decreased left tympanic membrane complacency and bilateral absence of the stapedian reflex .Figure 1Computed tomography of the temporal bones showed anterior otosclerosis in the right and left oval windows , bilaterThe clinical picture associated with Schwartze's sign and the CT findings suggest active otospongiosis.Medical treatment with sodium fluoride (10 mg/day) was started, but the patient developed pharmacodermy, which required interrupting the use of this drug.Surgery (stapedotomy) and a hearing aid were suggested. A choice was made for waiting and programmed outpatient monitoring  every six months.,,Conductive hearing loss is frequent in infancy; they are usually secondary to acute or chronic otitis media with or with no effusion.A complete clinical history , an otorhinolaryngological examination and complementary tests are essential for the diagnosis of otospongiosis.In our patient, we found that temporal bone CT contributed significantly to the diagnosis, given that the clinical picture and serial audiometries suggested otitis media with effusion.,,,,,,,Treatment approaches to otospongiosis include anti-enzyme or bone resorption moderating drugs, PSAD, and surgery. Surgery is not recommended in children below age 5 years; in such cases, PSAD are the treatment of choice.Our patient was treated initially with sodium fluoride (10 mg/day); the drug was stopped two days later due to a skin hypersensitivity reaction. Alendronate was not indicated due to the patient's age and the potential risk for bone development. After a careful appraisal of the case and talks with the patient's family, we decided for an expectant approach and outpatient monitoring.A high rate of suspicion is essential for an early diagnosis of infant otosclerosis, making it possible to monitor the disease and to apply the most appropriate treatment. In this study, we found that CT of the temporal bones was extremely important in defining the diagnosis; other tests suggested persistent and/or drug-resistant otitis media with effusion.


Rhizopus spp., Mucor spp., and other mucormycosis with high morbidity and mortalityRhino-orbit-cerebral mucormycosis is an opportunistic infection caused by Rhizopus sp. were detected in tissue samples (A 75-year-old man was hospitalized for COVID-19 pneumonia on June 14, 2021. The patient was referred to the ear-nose-throat clinic due to vision loss in the left eye and persistent maxillary toothache on the left side on day 8 of hospitalization. Left nasal endoscopy detected purple-black discoloration and paresthesia . Magneti samples . TherefoAlthough mucormycosis often develops in diabetic patients, Covid-19 disease causes lymphopenia and T-cell dysfunction


Fun databases, which has attracted high attention in the scientific community. These databases were widely used to gain more information from fungal sequencing datasets by assigning fungal functional traits. More than 1500 publications so far employed FUNGuild and the aim of this study is to compare this successful database with the recent FungalTraits database. Quality and quantity of the assignment by FUNGuild and FungalTraits to a fungal internal transcribed spacer (ITS)–based amplicon sequencing dataset on amplicon sequence variants (ASVs) were addressed. Sequencing dataset was derived from leaves and needles of 12 temperate broadleaved and coniferous tree species. We found that FungalTraits assigned more functional traits than FUNGuild, and especially the coverage of saprotrophs, plant pathogens, and endophytes was higher while lichenized fungi revealed similar findings. Moreover, ASVs derived from leaves and needles of each tree species were better assigned to all available fungal traits as well as to saprotrophs by FungalTraits compared to FUNGuild in particular for broadleaved tree species. Assigned ASV richness as well as fungal functional community composition was higher and more diverse after analyses with FungalTraits compared to FUNGuild. Moreover, datasets of both databases showed similar effect of environmental factors for saprotrophs but for endophytes, unidentical patterns of significant corresponding factors were obtained. As a conclusion, FungalTraits is superior to FUNGuild in assigning a higher quantity and quality of ASVs as well as a higher frequency of significant correlations with environmental factors.Recently, a new annotation tool “FungalTraits” was created based on the previous FUNGuild and FunThe online version contains supplementary material available at 10.1007/s00248-022-01973-2. Fungi play a pivotal role in terrestrial ecosystems and exert important ecological functions including decomposition, transformation, and effective utilization of organic substrates, facilitating the cycling processes . The undFun [Fun database has been developed from FUNGuild by addition of data on various number of traits at the genus and species levels  [In recent years, several relatively efficient and accurate databases or molecular tools that depict and identify fungal functions have been established, for example LIAS , 17, DEEFun , Notes oFun , FUNGuilFun , and FacFun . LIAS foFun , 18. FacFun . Notes oFun . FUNGuilFun . FunFun  traits) . The FUN traits)  has rece traits) –28. The  traits) . Even th traits) . This toFun [Based on the previous fungal functional annotation tools, FUNGuild  and FunFFun , the recFun . They maFun . In addiFun .Until now, more than 1500 publications (last accessed 16.12.2021) have employed FUNGuild to annotate sequencing datasets to ecological functions. Due to the large number of previous studies using FUNGuild, it is necessary to compare the performance and the ecological interpretation provided by the two annotation tools . Our study aimed to compare state-of-the-art methods  for leaf- and needle-associated fungal functional diversity analyses. We investigated the leaf- and needle-associated fungi of 12 temperate tree species in Central Europe forests and validated the consistency of using the two functional annotation tools. We hypothesize that (1) for overall and for all main fungal guilds, FungalTraits outperforms FUNGuild as it contains a higher number of fungal genera in its database; (2) both functional annotation tools provide consistent results for interpreting the richness and community composition of the leaf- and needle-associated fungi across 12 temperate tree species.This study was conducted in the Hainich-Dün region of Thuringia, Germany (51°12' N 10°18'E). Elevations range from 100 to 494 m above sea level, the mean annual precipitations from 600 to 800 mm, and the mean annual temperatures from 6 to 7.5 °C (average temperatures in January = 0.65 °C and July = 17.17 °C). The parent material is Triassic limestone, which is covered by a Pleistocene loess layer of variable thickness (ca. 10–50 cm) at most sites. The litter layer consists mainly of past years foliage (1 to 3 cm). The main soil type is a Cambisol on limestone as bed-rock. The soil pH is weakly acidic .Acer pseudoplatanus, Carpinus betulus, Fagus sylvatica, Fraxinus excelsior, Populus sp., Prunus avium, Quercus robur, and Tilia cordata) and 4 coniferous tree species . Sampling was carried out with gloves and sterilized plastic bags, and leaves and needles from each tree were separately packed and transported under cooled conditions to the lab. In the laboratory, each leaf and needle were frozen at − 80 °C for subsequent molecular approaches.In October 2019, we collected senescing leaves and needles from 60 tree individual , minimum 200-g leaves or needles per tree individual). These tree species include 8 broadleaved  and needles  were subsampled and prepared for DNA extraction. Briefly, we removed loosely adherent dust particles and microbes from leaf and needle samples by vortexing them with a maximum speed for 5 min in sterile Tween solution (0.1% vol/vol), and this step was repeated three times. The samples were then washed three to five times using deionized water. Finally, leaf and needle samples were incubated for 1 h in sterile water at room temperature. Each composite sample was then ground using liquid nitrogen and pestle, homogenized, then stored at − 20 °C for further analysis. Fungal community attached firmly to the leaf and needle samples (~ 120 mg homogenized leaves and needles) was then subjected to DNA extraction using DNeasy PowerSoil Kit  and a Precellys 24 tissue homogenizer  according to the manufacturer’s instructions. The presence and quantity of genomic DNA were checked using NanoDrop ND-1000 spectrophotometer , and the extracts were stored at − 20 °C. Leaf- and needle-associated fungi were characterized by fungal internal transcribed spacer (ITS)–based amplicon sequencing on the Illumina MiSeq sequencing platform, as outlined earlier . For estThe ITS rDNA sequences corresponding to the forward and reverse primers were trimmed from the demultiplexed raw reads using cutadapt . Paired-org was calculated as the difference: Norg = TNb – Nmin. TNb was analyzed using a sum parameter analyzer with high temperature combustion and chemiluminescence detection . For NMin quantification, a flow injection analyzer  with corresponding manifolds for the nitrogen measurement of ammonium Wet leaf and needle samples were shaken for 1 h in falcon tubes with 30 mL milliQ water to leach water-soluble components from their surfaces. The leachates were centrifuged for 5 min at 3500 rpm, decanted, and filtered through pre-flushed 0.45-µm regenerated cellulose syringe filters. The remaining leaf/needle material was dried for two weeks at 40 °C for dry weight determination. All quantification results are given in reference to the dry weight. The pH of the leachates was determined using pH paper with a scale precision of 0.2 pH units. NF-test (for two datasets) and Levene’s test (for more than two datasets). The statistical differences between proportions of functional assignment by FUNGuild and FungalTraits were performed using T-test  and Mann–Whitney U test . Effects of tree species and tree types on fungal community composition were visualized and tested with cluster analysis  and one-way PERMANOVA (based on presence-absence data and the Jaccard distance measure), over 999 permutations were run. The correlation analyses were performed using Pearson’s r  and Spearman’ ρ . The statistical differences of ASV richness among different tree species were performed using one-way ANOVA with Tukey’s post hoc test or KW test with Mann–Whitney U test. All statistical analyses were performed using PAST version 2.17 [The datasets were tested for normality using the Jarque–Bera JB test and for the equality of group variances using ion 2.17 .P > 0.05)  using both FUNGuild and FungalTraits annotation tools Table 1. Overall11Populus sp. revealed the highest proportion of the total fungal functional assignments by FungalTraits, whereas F. sylvatica has the lowest proportion , while the lowest difference was observed for P. abies (7%). While the proportions of overall fungal functional assignments by FungalTraits were much higher in broadleaved tree species compared to coniferous tree species, FUNGuild provided similar proportions across all tree species. The consistent patterns were found in saprotrophs. The proportions of saprotrophic fungal functional assignments by FungalTraits were significantly higher than those by FUNGuild across 12 tree species. The differences between them were also higher in broadleaved tree species (13–24%) compared to coniferous tree species (7–18%).Based on individual tree species, we also found that FungalTraits provided a significantly higher proportion of the total fungal functional assignment compared to FUNGuild Fig. . This paon Figs.  and 2. Rr = 0.62, P < 0.001) , followed by plant pathogens . A significant, but lower correlation, was found for saprotrophs . Contrarily, no correlation was observed for endophytes (P > 0.05).We found a significant correlation between the percentage points of total functional assignments by FUNGuild and FungalTraits (Pearson’s 01) Fig. a. HoweveQ. robur, which exhibited similar richness as compared with L. decidua, P. sylvestris, and P. menziesii. Picea abies and Populus sp. harboured highest and lowest richness. For fungal community composition, we found that almost all needle samples of coniferous trees clustered together into one clade with few broadleaved trees  contained no lichenized fungi and clustered together on the left side of the plot. The effects of tree species  and tree types  on lichenized fungal community composition based on FUNGuild and FungalTraits were almost identical.Likewise, the consistent results of the effect of tree species and tree types (broadleaved vs. coniferous trees) on richness and community composition were highly correlated to fungal guilds Figs. , and 6. ees Fig. . The majPopulus sp. harboured the lowest richness. However, FUNGuild and FungalTraits showed that A. pseudoplatanus and F. excelsior, respectively, harboured the highest saprotrophic richness  and lichenized  fungal richness obtained by FUNGuild and FungalTraits  and two clades belong to coniferous trees (L. decidua separated from other coniferous trees)   and inorganic  N . These values are consistent with the percentage points of functional assignment of FUNGuild reported before in many publications [L. decidua) and lower for the remaining coniferous trees . Another example for the mismatch is the identification of genus Cenangium. Cenangium is classified as “foliar endophyte” in FungalTraits, while it is identified as “saprotroph” with confidence level “probable” in FUNGuild. According to recent research, Cenangium was identified as endophyte, saprotroph, and plant pathogen [Our current study demonstrates that the quality of functional annotations and the resulting interpretations derived from FUNGuild and FungalTraits are relatively similar; however, they are not identical. Furthermore, the degrees of similarity greatly depend on which fungal guilds were considered. While the results on the effect of tree species and tree types on richness and community composition of lichenized fungi are almost identical when FUNGuild and FungalTraits are applied, for saprotrophic fungi, we could detect some discrepancies between these two annotation tools Fig. . Low dispathogen , 50, whipathogen .Pannariaceae, Candelariaceae, and Ramalinaceae, which were annotated as lichenized fungi using FUNGuild (with probable and highly probable confidence levels), but the same ASVs cannot be annotated to any function by FungalTraits. As demonstrated before, FUNGuild and FungalTraits can be applied together to maximize the number of fungal functional annotations and to remove ambiguous annotations. However, based on the results of our datasets, FungalTraits alone already yielded successful functional assignments for high proportions of the fungal community. Adding the functions specifically annotate with FUNGuild to the FungalTraits datasets can increase the percentage points of the total functional assignment by approximately 6%. Apart from the primary lifestyle (function), FungalTraits also provides other interesting information on secondary lifestyle, endophytic interaction capability, plant pathogenic capacity, preferred substrate type, decay type, habitat characteristics, animal biotrophic capacity, hosts, growth form, fruitbody type, Hymenium type, ectomycorrhiza exploration type, ectomycorrhizal lineage, and photobiont.Apart from mismatches, FUNGuild identified two or more functions (with confidence level “probable”) for a single fungal taxon; thus, it is difficult to make decision which function or both functions are most likely fitting to this fungus. Nevertheless, it is quite common to report all functions obtained by FUNGuild for a single taxon when the confidence level is at least “probable”. Another possibility for the discrepancy is that FUNGuild can annotate functions to fungi at a higher taxonomic rank (such as family). For example, we found that four fungal ASVs belonged to r = 0.63, P < 0.001). This is due to the reannotation process of the fungal genera in FungalTraits. Some changes are made to some specific fungal genera by the experts of the field during this process. Nevertheless, we detect similar patterns of factors that shape the saprotrophic community composition derived from both annotation tools. In both datasets, we found that tree species and tree type are the main factors that significantly shape saprotrophic community composition, along with water content and nutrients , Ca, Fe, Mg, and P content), and location. These findings are in line with previously published studies [Saprotrophic fungi are among the most important fungal groups driving important ecosystem functions such as accelerating the decomposition rate, enabling the nutrients accessibility and availability for themselves and other microbes , 52. Fun studies , 54. The studies . These n studies . In contSphaerulina as FUNGuild classifies Sphaerulina as lichenized fungi with the confidence level “highly probable”, while FungalTraits assigns in the primary and secondary lifestyles of this genus as “saprotroph” and “plant pathogen”, respectively. A recent study, however, reports Sphaerulina as lichenicolous fungi [In this study, we revealed a high diversity of lichens associated with senescing leaves and needles assigned by both FUNGuild and FungalTraits. In total, we are able to assign 60 lichenized fungal ASVs that were assigned to 21 genera. The proportion of lichenized fungal assignments obtained from both annotation tools is highly correlated and shared ASVs account to ~ 88% of the total assigned lichenized fungal ASVs. FUNGuild provides six more lichenized fungal ASV assignments compared to those of FungalTraits. Among these, four ASVs are not identified at genus level and functions are only assigned on family level. We found a potential controversial issue for the functional assignment of the genus us fungi , while ous fungi , 57. Theus fungi . This cuPustularia, cannot be assigned to the ecological function by FUNGuild. Members of this genus were previously reported as EcM [Our present study emphasizes on the functional assignments and the performances of the annotation tools, FUNGuild and FungalTraits, in the leaf/needle-associated fungal communities. However, in nature, mycorrhizal fungi are also considered as important functional groups associated with plants and play crucial role in promoting their performances , 60. Thud as EcM , 67. NevFunctional assignment of fungal amplicon sequencing datasets is of pivotal interest to infer a more mechanistic understanding of phylogenetic information and to ease the assessment of ecosystem processes of the respective habitat. The quantity and quality of fungal functional annotations were significantly better in FungalTraits than in FunGuild for evaluating the functional guilds on senescing leaves and needles of 12 temperate tree species. The transferability to other environments and research tasks should be addressed in upcoming studies.Supplementary file1 (PDF 468 KB)Supplementary file2 (XLSX 751 KB)Below is the link to the electronic supplementary material.


With the proliferation of telemedicine during the COVID-19 pandemic, attention began to turn to the risk of health disparities associated with its use among people with disabilities. Therefore, the present study investigates the level of interest in using teleconsultations (TCs) in primary healthcare among people with visual impairments (PVIs) and identifies their motivations and needs. A total of 219 Polish PVIs were surveyed using a combination of closed and open questions. About 50% of the respondents expressed interest in using TCs. The factor most closely related to the willingness to use TCs was age. The predominant reason for using TCs was to obtain a prescription or referral, and the most highlighted need was the possibility to choose between a TC and an in-person visit. The blind and poor-sighted participants differed in some regards. Our study indicates that TCs, under some conditions, may be a beneficial option for PVIs, and provides some directions for its effective implementation. While the series of restrictions and the need for self-isolation associated with the COVID-19 pandemic has distanced people from each other, this distancing has also demonstrated that a lack of direct contact is not always an obstacle in providing healthcare. One particular example of this is the use of telemedicine (TM).The objectives of TM as defined by the World Health Organization (WHO) include the diagnosis, treatment, and prevention of disease and injury by all health professionals using information and communication technology (ICT), where distance is a critical factor, and its primary goal is to improve human health . TM playIt has been proposed that individuals with sensory disabilities, including people with visual impairments (PVIs), may experience particular difficulties when using TCs ,16. The Our study focuses on primary healthcare (PHC), as it is the patient’s first point of contact with the health services ,32,33,34In Poland, TM was authorized in 2015 , and PHCAre PVIs interested in PHC TCs?Is their interest in remote PHC consultations associated with socio-demographic variables, the level of vision deficits, independence in mobility, having NCDs or additional disabilities, previous experience in TM, and digital access and skills?What are the reasons for PVIs to use TCs?Do persons with blindness and low vision differ in their motives for using TCs?What are the needs of PVIs when utilizing TCs?Are there any differences in the needs of the poor sighted and the blind?Taking into account two of the key strategic goals of the Polish health policy defined in 2019, which are the development of TM and the strengthening of care for PwDs , our stuA cross-sectional exploratory study design was used to gather opinions from a significant PVIs’ population and adjust to the diversity of their functioning and difficulties in direct contact arising from pandemic restrictions. A survey was carried out from May to July 2021 among poor-sighted and blind people throughout Poland. The inclusion criteria were as follows: voluntary written or verbal consent to participate in the study, self-declaration of being at least 18 years old, and having VI. Failure to meet any of the inclusion conditions or difficulty understanding items—reported by the respondent or noticed by the interviewer—were exclusion criteria.No register of people belonging to the target population exists in Poland, neither in national institutions nor in clinics. Data collected by non-public organizations were unavailable due to data protection. Therefore, participants were recruited using accidental selection and snowball sampling: the former refers to those respondents who volunteered for the study after encountering an invitation on an internet forum, school, association, sports club, or clinics for PVIs. Information about the study was posted on flyers and websites and distributed by representatives of the institutions listed above. Following this, a snowball method allowed for the inclusion of people who learned about the study from other respondents.Several forms of recruitment and data collection were planned to engage as many people as possible with different levels of VI, digital skills, and place of residence during the planned time of the study. The recruitment protocol also complied with the SARS-CoV-2 pandemic restrictions, minimizing the need for direct contact. Participants responded via Google Form, e-mail, paper, or phone. The paper-pencil questionnaires and print versions of the announcement were prepared in enlarged font and included a QR code to read the information electronically. None of the respondents took the opportunity to use the Braille questionnaire. Participants could register for the telephone survey through a Google Form or the phone number provided in the survey announcement. The telephone interviewer was trained to communicate appropriately with PVIs and not to influence their answers. The respondents were assured of anonymity.in another way” constituted about 20% each of the total. Only two people completed the survey at an eye clinic or department, and eight participants were encouraged by a disability representative at a university.Most participants learned about the study through PVIs associations (37% of study group). Among the other groups, those gathered by internet forums, previous participants, and “The survey used a self-administered questionnaire complied for the purpose of this study. It comprised a set of three structured questions and two open-ended questions on the subject of TM in PHC .The survey also collected sociodemographic data . Furthermore, participants also reported any NCD (yes/no question) or additional disabilities (multiple answers could be selected from yes/no). As only one person reported not using a mobile phone, this variable was not included in the analysis. The participants also assessed their digital skills, such as their computer and internet proficiency on the following scale: lack of ability, low, average, and high level.The respondents indicated whether they had held a TC at the PHC in the six months before the study, and whether they had any experiences in using a cell phone, computer, and the internet . Descriptive, inferential, and predictive methods were used to process the collected data. Descriptive statistics were presented for the whole PVI group and the poor-sighted and blind subgroups. Pearson’s chi2 test of independence was used to compare the frequency of each trait variation in the study groups. The partially sighted and blind individuals were compared with regard to their sociodemographic, health, disability, and digital-related characteristics, as well as their experience in using TCs and their willingness, motives, and needs for doing so. The associations between respondents’ characteristics and their interest in using TCs were assessed firstly with the Pearson’s chi2 test and then with the univariate followed by multivariate logistic analysis. Variables that were previously found to be significant, or near the significance level, were included in these analyses. Therefore, odds ratios (ORs) and 95% confidence intervals (CIs) were estimated for the following independent variables: level of VI, age, sex, education level, having a chronic disease, and internet use. Non-declared subjects, who answered “hard to say” when asked if they were interested in TCs, were excluded from the logistic analysis. It was not possible to conduct a logistic regression analysis among the blind respondents due to underrepresentation (n < 100)  where it would be technically possible and equally effective.”Some participants noted difficulty in arriving at the clinic associated with living far away, and the fact that TCs may be more convenient for someone assisting a PwD. In the answers assigned to the category “time argument,” respondents emphasized situations when they cannot come to an appointment due to work or study and when it is faster to travel to a TC than an inpatient visit. A few respondents wanted to request sick leave during TCs. One participant stated that TCs are cheaper because they eliminate the cost of traveling to the clinic. One respondent expressed a willingness to use TCs “The dominant need for respondents in the context of TCs with PCP was the possibility to choose between an in-person visit and TC (83.6%), followed by the physician’s communication skills (63.9%). For more than half of the respondents, having the appropriate communication equipment  was vital. The least frequently indicated need (28.8%) was the possibility for a family member/friend/assistant or another person to participate in the visit .p = 0.022) and it should be possible for another person to be available if they are not able to notice changes in their body .Blind participants were more likely than poor-sighted ones to propose that TCs should base on accessibility standards  “What else do you think is vital for the blind and partially sighted persons in the context of teleconsultation with primary care physician?” Seven areas were selected by the coders: (1) exact date and time of TC, (2) adequate time for the visit, (3) psychosocial competencies of staff, (4) medical staff competencies related to functioning of PwDs, (5) well-designed TC, (6) awareness of the patient’s health situation, and (7) technological issues of TC. These are listed in A few supplemental areas of needs related to TCs also emerged from the content analysis of the second open-ended question  don’t dominate so that a person with a disability has to leave the house. Through COVID-19 I have trouble remembering different places, my dog (guide) forgets too; I have more stress when leaving the house.”Some of the responses to the open-ended question reiterated a point mentioned earlier in the replies to the closed-ended question: Although we did not ask this explicitly, several reasons for the negative attitude toward using TCs emerged from the responses to the open-ended questions. According to some respondents, TCs do not allow for sufficient conversation or examination:“I’m not a fan of TCs—it’s impossible to examine a patient well remotely, and the interview alone isn’t everything (it’s easy for the doctor to misinterpret what the patient says).”“I prefer a regular stationery visit, during which the doctor devotes more time to the patient, and over the phone sometimes the patient does not say everything he or she would like to.”For others, their health situation is an obstacle:“I think that these types of conditions (VI) require to see a doctor in person.”“(...) I have hearing problems, so I don’t always understand what the doctor is saying, at an inpatient visit it is easier for me to talk.”Finally, also organizational difficulties discourage the use of TCs:“I do not like TCs—first you have to wait tens of minutes to connect to the registration and then wait on the line for tens of minutes to connect to the doctor.”TM has become everyday practice in PHC during the COVID-19 pandemic and will probably be widely used in the future. Hence, there is an urgent need to explore patients’ attitudes and needs towards this form of contact with PCP. This exploration should include the population at risk of exclusion. Our study is the first such study to examine whether Polish patients with VI are interested in using TCs, to determine their reasons for doing so, and to identify their needs during remote contact with PHC.About 50% of the study group expressed interest in using TCs in the future. Similarly, a survey of Polish primary care patients in 2020 found that 43.2% believed that TCs should be one of the forms of contact with a PCP .Among the studied characteristics, age was found to be most closely associated with willingness to use TCs. Likewise in Reed’s study , it decrAmong the sociodemographic characteristics, sex has also been previously described as significant for the interest in TM. Some studies ,54 reporAmong the blind interest in TCs was significantly associated with the presence of another person needing care in their household; this was not observed among the poor sighted. These differences between the two studied groups require further investigations.One of the most commonly cited benefits of TM is its accessibility to people living far from medical facilities . HoweverRegarding health and disability, only having chronic conditions was found to be significantly associated with the interest in TCs: more respondents with NCDs expressed a negative attitude to TCs than those without, as mentioned previously in another Polish publication . In contOur respondents’ previous experience with TCs was not statistically significant for their current interest in using them. Previously, an international study  showed tThe last considered aspects in terms of willingness to use TCs were digital access and skills, as they present barriers for those attempting to use TM. Our findings indicate that only the use of the internet was significantly related with the interest in TC. It is possible that internet users may be more open to new solutions, including telehealth. Computer utilization and self-assessment of digital competencies were not significant in this regard; however, e-health solutions based on the virtual environment are not commonly used in Poland. One study published in August 2020 showed that since the beginning of the pandemic, 81.5% of participants had used TC by phone, and only 0.3% of those surveyed had taken part in a video conference with their PCP . HoweverOnly 10% of participants claimed they had no reason for using TCs, while almost 30% reported having no interest in TCs. It is possible that this reluctance to use TC may be due to the lack of knowledge about its possibilities, as noted in a study of Swiss respondents . PVIs haOur findings of the reasons for TCs utilization are consistent with the motivations described in previous studies, these being “Non-Queue Requests” such as the convenience of e-records, e-referrals, and remotely issued sick leave; time saving; emergencies ; and ecoEnvironmental factors, such as the accessibility barriers caused by adverse weather conditions, reduced PVIs’ mobility, or the lack of support to travel to the clinic, may also influence the attitude to TCs. Adverse weather conditions make it difficult for PVIs to move around and discourage them from leaving their homes ,72. AlthFinally, the blind participants were significantly more interested in monitoring their health condition via TC, and in taking part in consultations with several specialists at the same time than those with poor vision. We attribute this to their greater hardships associated with moving safely and independently.While previous studies have addressed the unmet healthcare needs of patients with disabilities , little For almost all respondents in the present study, the core issue was that TCs should be available as an option in PHCs. This need is consistent with other studies conducted in various group of patients ,76 and wOur respondents with low vision and blindness ex aquae emphasized the im-portance of staff communication skills and psychosocial competence including work with PwDs, as previously spotlighted concerning in-patient visits ,81,82. HThe third highlighted need was to have appropriate communication equipment. Technological solutions are a resource for all individuals; however, in certain groups, like PVIs, they facilitate independent functioning in various aspects of personal life, education, or work ,85. As mMoreover, in the context of this study, it is important to note that peripheral devices ,87 and IWhile accessibility aids may enhance the comfort in using devices by some people with low vision, accessibility equals usability for blind people. Similarly, in some situations, “the use of another person’s eyes” may be their only source of information, not only about external factors but also about themselves. This may be the reason why blind respondents were more likely to specify accessibility standards and the need to receive help from someone, e.g., to notice changes in their bodies, than partially sighted participants.Additionally, in respondents’ answers to an open question, PCP knowing their medical histories was revealed to be essential. Similarly, in another study, TC was more likely to be selected if it was to be performed by the patient’s personal PCP , and phyAn analysis of participants’ responses to the open-ended question explains their need for well-designed and punctual TCs. These are important not only for the sake of comfort, but also to allow adequate preparation for a visit and ensure proper interview conditions, including help of the PwDs assistant.All mentioned aspects should be implemented in the curricula of medical studies to improve staff skills and the coordination of healthcare among PwDs.The study addresses a little-explored topic with significant public health and clinical value, particularly when considering the epidemiology of VI, the proliferation of TM use in PHC, and recent changes in international and national legal health policy.Nevertheless, our survey has some limitations. Due to the chosen sample selection method and its cross-sectional nature, its results should not be generalized, and it is impossible to determine cause-and-effect relationships. However, such non-probabilistic sampling techniques are considered reasonable for exploratory studies such as this one, whose aim is to gain an initial understanding of an issue , and, inMoreover, every effort was made to ensure group heterogeneity with regard to different aspects of disability and social functioning, and to adjust the study to the specific needs connected with VI by including a pilot study, and diverse recruitment and survey methods . AdditioDue to the heterogeneity of the study population and the descriptive purpose of this research, no comparison group was formed; however, the internal comparisons were made with regard to patients’ characteristics, mainly the degree of disability.Only a portion of the group answered the open-ended questions, which also makes it impossible to generalize . NeverthThe survey took place in a specific historical context, during a pandemic that changed the way people and healthcare function. Many respondents had most likely never heard about TM before the pandemic. The next few months of the pandemic brought changes in Ministry of Health recommendations for organizing PHC services in Poland . As suchinvolving PVIs in the process of creating health policies and auditing the accessibility of TM services to monitor if they are suitable for their needs;developing digital competencies and providing financial support to equip PVIs with user-friendly technologies for health monitoring and contact with healthcare ser-vices . The eldincreasing health literacy in PVIs by indicating the potential of TM to make them more aware as consumers of healthcare services, expanding their care options, and thus achieving better health outcomes;improving the psychosocial and communication skills of medical staff, including awareness of biopsychosocial consequences of disability to be more open for heterogeneous needs of people. This also enables patients and increases their engagement with medical procedures ;applying patient-centered care by respecting their preferences, needs, and values, improving their responsibility and a more active role in their health development ,100;performing further qualitative investigations to deepen understanding of the needs of people with various disabilities in healthcare;investigating outcomes, including clinical and cost-effectiveness of TCs used in vulnerable populations like PVIs;addressing the changing needs of vulnerable populations. In response to the current humanitarian crisis in Ukraine, Poland promised free access to healthcare for Ukrainian refugees which requires the formulation of special short and long-term strategies ,102 to iTM tailored to the needs of PVIs may become an essential factor in providing them with equal access to health, ensuring continuity of care and allowing them to adhere to medical appointments. Moreover, it may have positive impact on sustainable changes in healthcare policy as well as in technological, regulatory, and legislative infrastructure . Hence, The key message from our study regarding public health, clinical practice, and patients is that TM can be a beneficial component of healthcare for PVIs if some conditions are met. It has the potential to increase their access to healthcare and improve their social inclusion. A knowledge of the biopsychosocial characteristics of PVIs and their attitudes regarding the use TCs in PHC is essential for planning health policies, healthcare coordination, and health expenditure. It provides an insight into which groups are best suited to TM, and who is best served; it also indicates what changes and conditions are necessary to engage PVIs more with TM utilization.Some of the needs and reasons for using TCs presented by the studied PVIs may also be shared with the general population, but they are worth noting in the broader context of functioning with sensory disability. Among the study group, the blind and poor-sighted people have found several motives for using TCs in PHC, some strictly connected with the impact of disability on their functioning. However, some are still not interested in using TCs, and there is a need to find reasons for it, overcome existing barriers and increase their awareness of its benefits.In the light of our results, special attention should be paid to older persons, those with lower education level, internet non-users, and PVIs with NCDs as they were more reluctant to use TCs. In addition, the blind and poor-sighted groups demonstrated different preferences, and these should be respected. PVIs indicated three key areas for consideration regarding the use of TCs: the optionality of TCs, the communication skills of the PCP, and appropriate equipment for communication. Failure in these areas may result in TM becoming another barrier preventing access to healthcare by PVIs. Some recommendations for implementing TM among persons with blindness and low vision, including patient-centered care, have been outlined.In short, to answer the question posed by the title, the use of TC in PHCs appears to be beneficial for PVIs when it is optional and meets their needs. This is true not only during the pandemic, but also afterwards.


Treacher Collins syndrome (TCS) is one of the rare genetic syndromes which is specified by symmetrical craniofacial dysmorphism without growth abnormalities or neurological disorders. The inheritance is usually autosomal dominant but sometimes it is a sporadic mutation. Prenatal diagnosis could be realized by genetic testing of a chorionic villus sample or amniocentesis if one of the parents is affected. At birth, the most common features are downward‐sloping palpebral clefts, small badly hemmed and folded ears, and mandibular hypoplasia which could lead to respiratory distress. All of these clinical features exist in our case. Goldenhar syndrome shares with TCS some facial features which are not symmetrical and it is also associated with vertebral abnormalities. Some patients with TCS are exposed to many complications and they require multi‐disciplinary medical care. But all of them need psychiatric care to fight social rejection. The aim of our report is to describe the most common features of TCS and similar syndromes. Also, report the involved genetic mutations, some associated complications, and their management. Faced with facial dysmorphism, the clinician should describe clearly the features in order to be oriented to the diagnosis. Some explorations in particular the genetic study and specialized clinical examination are necessary to: confirm diagnosis, reveal complications, and make easier its management and follow‐up. It was described often as an autosomal dominant disorder with a variable degree of phenotypic expression.2A male newborn whose parents are non‐consanguineous. The mother was 27 years old. She is smoking and addicted to cannabis. The pregnancy was poorly monitored. She was cesarized at 37th weeks of amenorrhea for suspected retroplacental hematoma. At birth, the newborn had respiratory distress requiring oxygenotherapy for only 2 days. The examination found hypotrophy in the 10th percentile and facial dysmorphia. He had a triangular face, receding chin, micrognathia, sunken cheekbones, downward slanting palpebral fissures, absent eyelashes of the lower eyelid, the nose large and beak‐like, microtia with badly hemmed ears folded and auditory external canal stenosis.  examination found a cracked tongue and an unseen uvula. Also, there is only an anthelix, a tragus, and a closed auditory external canal. The ophthalmologist described a telecanthus, good palpebral statics, normal oculomotricity, clear cornea, a normal iris, and a round pupil. The back of the eyes was normal. The scan showed diffuse enlargement of the spaces under the arachnoid, concave mandibular branches, and agenesis of the zygomatic process of the left temporal bone. He had also agenesis of the two external auditory canals and hypoplasia of the cavities of the two middle ears Figure . The gen3The estimated incidence of TCS is 1/50000. There is no predominance of gender or race.Meizner described a fetus in which prenatal sonography showed polyhydramnios, micrognathia, a low‐set ear, and a large cleft lip and palate. The biometric measurements were appropriate for gestational age.Goldenhar syndrome or vertebral oculo‐auricular dysplasia shares some craniofacial abnormalities with TCS.At birth, some patients with TCS presented respiratory distress because of choanal stenosis or atresia, retropositionned tongue, and obstruction of hypophryngeal spaces by mandibular hypoplasia. Others have suffered from obstructive sleep apnea.Actually, our patient suffered from developmental delay and hearing loss which required hearing aids. His parents are accepting his dysmorphism as long as he had no functional complaints.4Treacher Collins syndrome is rare and characterized by symmetrical facial anomalies unlike other craniofacial syndromes. It could be evoked in the preterm stage with ultrasound imaging. It did not associate developmental delay or neurological impairment. Patients with their appearance are faced with problems in integration into social life. Taking care of these patients requires a multidisciplinary team.Nadia Kolsi: Conceptualization. Fatma Boudaya: Writing – original draft. Afef Ben Thabet: Visualization. Manel Charfi: Resources. chiraz Regaieg: Investigation. Amira Bouraoui: Validation. Ridha Regaieg: Supervision. nedia Hentati: Supervision. Amel Ben Hamad: Writing – review and editing. Abdellatif Gargouri: Supervision.No conflict of interest in this abstractYes."Consent was obtained from the parent of the patient to publish this report in accordance with the journal's patient consent policy”


Threshold concepts (TCs) are increasingly used in health professions education (HPE) research. TCs are claimed to be conceptual gateways which are often traversed with substantial difficulty. In this paper, we report on a scoping review investigating the following research question: What is the scope and nature of the currently available research on threshold concepts in health professions education literature? We employed Arksey and O’Malley’s model for scoping reviews. A search for literature on TCs in HPE research between 2003 and 2020 yielded 999 records of which 59 were included in the review. The data set was subject to quantitative descriptive analysis of article characteristics as well as qualitative thematic analysis of the scope of research on TCs. Among the 59 articles selected for review, there were 30 empirical, 26 conceptual and three reviews. A majority were published in 2015 or later. Almost half of the included articles attempted to identify possible TCs within HPE. Others investigated how TCs can be traversed or suggested how TCs could influence curriculum design. Some critically appraised the framework of TC. Although TCs are increasingly utilised in HPE, the present review identified how researchers came across methodological challenges related to identifying possible TCs and definitional challenges around identifying the essential characteristics of TCs. Before embracing TCs as the next go-to theory for learning in HPE, we acknowledge the need for methodological stringeny and rigour as well as more data to support TCs. Until then, any implementation of TCs in HPE curricula should be done cautiously.The online version contains supplementary material available at 10.1007/s10459-022-10127-5. Enhancing Teaching–Learning Environments in Undergraduate Courses project launched in the UK, where Meyer and Land further developed the idea of troublesome knowledge and postulated that:“A threshold concept can be considered as akin to a portal, opening up a new and previously inaccessible way of thinking about something. It represents a transformed way of understanding, or interpreting, or viewing something without which the learner cannot progress. As a consequence of comprehending a threshold concept there may thus be a transformed internal view of subject matter, subject landscape, or even world view”. have recently gained broader acceptance and traction in health professions education (HPE) research and there are now a substantial number of research publications exploring TCs both conceptually and empirically  conducted searches on November 3, 2020, in what we identify as three central databases for HPE researchers: Medline (Ovid), Web of Science Core Collection (Clarivate) and CINAHL (EBSCO). Thesaurus terms were used for database-specific keywords by using combinations of the following truncations: “threshold* concept*” in title, abstract and/or keywords. The documentation of search strategies can be found in online Appendix 2. A supplementary literature search was performed on March 25, 2021, to identify late and deferred publications in 2020. No hand-searching of specific journals were performed.After duplicates were identified and removed, all search records were imported to a reference management software , the articles referred to and how TCs were applied. After the first iterative round, details in the spreadsheet were revised to include all articles in the data set. Articles using some form of original data (review articles excluded) were categorised as In the last stage, data were analysed in two distinct ways: (1) through a quantitative descriptive analysis providing insight into the extent, nature and distribution of the included studies, and (2) through a qualitative thematic analysis informed by the research question  were empirical, 26 (44%) conceptual and three (5%) were reviews. Of the empirical articles, 23 (76%) were qualitative, two (7%) were quantitative, three (10%) used a mixed-method approach, and two (7%) described educational development initiatives.The data collection methods used in the empirical articles were mainly qualitative individual interviews (n = 12), questionnaires (n = 10), focus group interviews (n = 8), texts (n = 5), or a combination of these. There were also examples of field observations (n = 1), Delphi method (n = 1), and nominal group technique (n = 1). The empirical work included data from the UK (n = 12), Australia (n = 5), the USA (n = 4), Canada (n = 4), New Zealand (n = 3), China (n = 1), Sweden (n = 1), Switzerland (n = 1), and Taiwan (n = 1). Two studies collected data in two countries. The authors of the conceptual work and reviews were, with few exceptions, based in Australia, Canada, New Zealand, UK or USA.All levels of HPE were represented in the articles, with the majority at the undergraduate level (n = 44). Nine articles referred to postgraduate education, two concerned health care professionals, and in five the level of education was not explicitly stated.Literature concerning seven different health care professions was found: medicine (n = 18), occupational therapy (n = 10), nursing (n = 8), dentistry (n = 4), physiotherapy n = 2), pharmacy (n = 1) and midwifery (n = 1). Fifteen articles were multiprofessional or interprofessional. A total of 31 articles were published in HPE journals, 21 were published in general medicine or health journals, and seven were found in higher education journals. The large majority (n = 48) were published in 2015 or later , both in undergraduate and postgraduate education. A vast majority of articles employed qualitative methods to identify TCs . These articles concerned the process of learning a TC and thus empirically evaluated . Some introduced TCs to their respective fields and presented it as a promising framework for curriculum reformation and elucidated what distinguished TCs from core competencies or core curricula; Neve et al.  introducOthers focused on how the introduction of TCs could reform curricula and proposed it as a novel perspective of learning in HPE. The fields in which TCs are suggested to influence curricula include midwifery students’ learning about infant feeding , also offered detailed suggestions on how these could change existing curricula . In 2017, Barradell and Peseta  was the Other work within this theme critically appraised TCs with a basis in their empirical studies or simply from a conceptual perspective in form of, e.g., commentary. Barradell and Peseta  challengIn contrast to the articles primarily focusing on identifying TCs, the articles in this theme were characterised by their concern with the concept itself and elaborating on the very nature of TCs and the relationship between TCs and core concepts or competencies. Bhat and Goldszmidt  discusseTCs influencing curriculum design there were articles mainly focusing on identifying TCs that also discussed their nature and how one should go about investigating them  Which informants should be invited and involved in TC identification?  Can learners articulate the types of troublesome knowledge they are encountering, or are the TCs unknowable until they have overcome them?  In this regard, what learners perceive as TCs may, in fact, resemble vague symptoms or approximations, and drawing far-reaching conclusions may be unwise. The challenges that investigators possibly encountered when exploring TCs made them call into question the very TC they had identified and construed. While the literature, on the whole, does not provide any clear guidance on this matter, it is apparent that there is a need for further methodological recommendations  suggested TCs as a novel and suitable model for learning in their field, as a large part of the literature questions the very existence of TCs. In the articles promoting TC as a framework for influencing curricula, definitional challenges were rarely addressed; rather, it seemed to be a pedagogical tool that was taken for granted. While there seems to be value in using the TC as an analytical lens for discussions about learning in HPE, implementing it without reflection upon its definitional challenges and empirical shortcomings seems unwise.Our results convey a main concern about the TC discourse in HPE research, thus, pertaining to demarcation and definitional issues, and which elements or characteristics need to be satisfied for a concept to be viewed as a TC. Likewise, the proximity and relationship to other similar notions such as core concepts, and troublesome or difficult knowledge, appeared in several of the examined articles Supplementary file2 (PDF 207 kb)Below is the link to the electronic supplementary material.


In ideal listening situations, subjects with normal hearing can easily understand speech, as can many subjects who have a hearing loss.To present the validation of the Word Recognition Test in a Free Field in the Presence of Noise in normal-hearing adults.Sample consisted of 100 healthy adults over 18 years of age with normal hearing. After pure tone audiometry, a speech recognition test was applied in free field condition with monosyllables and disyllables, with standardized material in three listening situations: optimal listening condition (no noise), with a signal to noise ratio of 0 dB and a signal to noise ratio of −10 dB. For these tests, an environment in calibrated free field was arranged where speech was presented to the subject being tested from two speakers located at 45°, and noise from a third speaker, located at 180°.All participants had speech audiometry results in the free field between 88% and 100% in the three listening situations.Word Recognition Test in Free Field in the Presence of Noise proved to be easy to be organized and applied. The results of the test validation suggest that individuals with normal hearing should get between 88% and 100% of the stimuli correct. The test can be an important tool in measuring noise interference on the speech perception abilities. To understand speech satisfactorily, some auditory tasks are necessary, including: attention, analysis, synthesis, memory, among others. Such skills, when combined, promote auditory recognition, which implies deriving meaning from what is heard. Thus, the understanding of speech is a very complex activity that depends directly on the peripheral hearing mechanisms, central auditory processing and cognition.In ideal listening situations, that is, acoustically comfortable environments, individuals with normal hearing easily can perform the auditory recognition. However, when the environment is degraded, due to the competitive noise or reverberation,In individuals with hearing loss and hearing aid users  this difficulty is greater.4Among other uses, the speech perception test in the presence of noise has been developed and used in audiological diagnosisBecause of this, a low-cost, easy to install audiology laboratory was designed, consisting of an acoustically treated booth and a free field system attached to three speakers, to perform speech recognition testing in the presence of noise.The aim of this paper is to present the validation of the Free Field Word Recognition Test in the Presence of Noise in normal-hearing individuals.This is an experimental, self-controlled study, aimed to verify the accuracy of the Free Field Word Recognition Test in the Presence of Noise. The study was approved by the Institutional Ethics Committee under protocol 937 031/15.One hundred individuals who agreed to participate were randomly selected. All of them were oriented about the study and signed the Informed Consent.The study included persons 18 years of age or greater on the date of the tests, who had normal hearing and no hearing complaints. We excluded individuals with speech problems.For data collection a booth, a two-channel audiometer, conventional open field equipment (for speech stimulus output) and auxiliary equipment (for noise emission) were used.The auxiliary equipment called “third channel” for free field has been developed specifically to control and amplify a third sound source used as “competitive sign” inside the booth. This one has the circuit composed of the following blocks: input preamplifier, calibration circuit with gain adjustment of 0–40 dB, linear output attenuator with 5 dB steps, and full range of 0–100 dB SPL, 50-W class-T digital power amplifier, PIC18F2550 MICROCHIP microcontroller, a 2-lines by 20 characters Display, and a keyboard.The system operation allows the adjustment of the external sound source, which in this project was a Samsung mobile, with an SG (Sound Generator) application; the application was set to generate (broadband) white noise. The professional can calibrate the correct signal level used by the “calibration mode” of the equipment by viewing the signal on the display, and adjusting it to “0 dB” on the VU. Once adjusted, the signal can be displayed in the selected intensity of the attenuator through the stimulus button that turns the presentation of this noise on or off.Test environment consisted of two speakers located at 45°  and noise in a third one, located 180° from the subject from where competitive noise is produced .Figure 1The conventional free field used is Oto Sonic CL30-V model, with no series number, calibrated on October 17, 2013, with certificate No. 415-2013F, according to ISO8253-3 and IEC645-2:1993, the standards used for calibration were Larson Davis Sound Pressure Meter, Mod. 824, series No. 824A2867 (Certificate No. 50381/2013), Larson Davis Sound Calibrator, mod. CAL 250, series no. 4128 (Certificate No. 50378/2013), Larson Davis Microphone, Mod. 2575, series no. 1698 (Certificate No. 50379/2013).The “third channel” equipment was calibrated on July 31, 2014, with certificate no. 425a-2014-F, according to ISO8253-3 and IEC645-2:1993, the standards used for calibration were Bruel & Kjaer Sound Level Meter, Mod. 2250, series No. 3006245 (Certificate No. CBR1400264/2014), Bruel & Kjaer Acoustic Calibrator, mod. 4231, series No. 3007539 (Certificate No. CBR1400268/2014) and Larson Davis Microphone, Mod. 2575, series no. 2119 (Certificate No. 60381/2014).After completing a form of identification, all research subjects underwent pure tone audiometry to determine the auditory thresholds. Those with hearing within normal limits1.Monosyllabic word lists presented without competitive noise (control);2.Monosyllabic word lists presented in signal/noise ratio of 0 dB (study);3.Monosyllabic word lists presented in signal/noise ratio of −10 dB (study).The recognition test was applied in three situations:The word lists were presented at 40 dB NS, or 40 dB above the mean tritone levels previously obtained in pure tone audiometry. In the signal/noise ratio of 0 dB, speech and noise were presented at the same intensity. With a signal/noise ratio of −10 dB, the noise was 10 dB stronger than speech. The speech was presented in the conventional free field systems (speakers at 45°) and the competitive noise was presented at the “third channel” (180°).Data were recorded in a special record protocol, and were statistically analyzed. We used the chi-square test at the 0.05 significance level.The following variables were analyzed and compared: test results with no noise (control) with test results with noise at 0 dB and −10 dB ratio (study).After this, the study subjects were divided into two groups: G1 – people under 40 years of age, and G2 – people with 40.1 years or more, and data were compared to see if the scores varied with increasing age.One hundred normal-hearing subjects were evaluated for purposes of this study, 83 being females and 17 males. The minimum age of the sample was 19 years and the maximum 64 years. The mean age was 34.1 years, with a standard deviation of 10.8.Given the standard deviation found, and to verify if age factor interfered with the results of auditory perception, the sample was divided into two groups, G1 composed of 72 individuals under 40 years and G2 formed by 28 subjects over 40.1 years.All subjects of the sample demonstrated free field speech recognition scores between 88% and 100% accuracy on the three listening conditions .Table 1DSpeech perception performance obtained in the Free Field Word Recognition Test in the Presence of Noise was compared between the two groups . There wThe ability to understand speech in the presence of competitive noise is the object of the study of audiology,When analyzing the mean score of respondents  in the F15When the respondents were divided into two subgroups  for the Normally, the broad spectrum noise tends to hinder the task of auditory discrimination because speech consists of sounds of different frequencies that have continuously varying intensities,The competing noise proposed in our test was presented at an angle of 180°, in accordance with Brazilian researchIt is noteworthy that, in audiology clinics, it is extremely important to establish reference values obtained in normal-hearing subjects in order that the difficulties encountered by the individual with hearing disorder complaints can be compared. Considering the findings reported in this study, values between 90% and 100% accuracy can be inferred as normal standard of the Free Field Word Recognition Test in the Presence of Noise in subjects with no hearing complaints.The test proposed here consisted of a low-cost, easy to install system that provided reliable results that were consistent with the literature. It can be a valuable tool in the investigation of auditory processing, selection and indication of hearing aids, as well as in the evaluation of performance of patients using sound amplification and implantable prostheses, and can provide a framework for research and testing of different populations in the future.The Free Field Word Recognition Test in the Presence of Noise proved to be easy to organize and apply. The results of the test validation suggest that subjects with normal hearing should get between 90% and 100% of the stimuli presented correct, even in the presence of noise. The test can be an important tool in measuring noise interference on speech perception skills in different populations.The authors declare no conflicts of interest.


Among cochlear implant candidates, an increasing number of patients are presenting with residual acoustic hearing. To monitor the postoperative course of structural and functional preservation of the cochlea, a reliable objective biomarker would be desirable. Recently, impedance telemetry has gained increasing attention in this field. The aim of this study was to investigate the postoperative course of the residual acoustic hearing and clinical impedance in patients with long electrode arrays and to explore the applicability of impedance telemetry for monitoring residual hearing.We retrospectively analyzed records of 42 cochlear implant recipients with residual hearing covering a median postoperative follow-up of 25 months with repeated simultaneous pure tone audiometry and impedance telemetry. We used a linear mixed-effects model to estimate the relation between clinical electrode impedance and residual hearing. Besides the clinical impedance, the follow-up time, side of implantation, gender, and age at implantation were included as fixed effects. An interaction term between impedance and follow-up time, as well as subject-level random intercepts and slopes, were included.p < 0.001).Loss of residual hearing occurred either during surgery or within the first 6 post-operative months. Electrode contacts inserted further apically  had higher impedances, independent of residual hearing. The highest impedances were measured 1 month postoperatively and gradually decreased over time. Basal electrodes were more likely to maintain higher impedance. Follow-up time was significantly associated with residual hearing. Regardless of the time, we found that a 1 kΩ increase in clinical impedance was associated with a 4.4 dB deterioration of residual hearing (Pure tone audiometry is the current gold standard for monitoring postoperative residual hearing. However, the association of clinical impedances with residual hearing thresholds found in our study could potentially be exploited for objective monitoring using impedance telemetry. Further analysis including near-field related impedance components could be performed for improved specificity to local immune responses. After implantation, patients with successfully preserved hearing can perform better in complex listening environments , perceive music more naturally, and have improved spatial perception –3. Approstanding , 7. In cstanding –11. Poststanding , intracostanding , intracostanding , or a prTo better understand the postoperative course of inner ear function and to enable frequent monitoring, an objective intracochlear biomarker would be most appropriate. Impedance telemetry could be practicable and recently gained attention in this context , 16. TheSudden increases in postoperative impedance values  were linked with inner-ear events leading to loss of residual hearing and vertigo , 19. In N = 704). Of these, only cases were included, who (i) received a MED-EL  implant, (ii) had a measurable residual hearing of at least 5 dB between 125 and 1,000 Hz in the preoperative pure tone audiogram, and (iii) had a well-documented postoperative follow-up with two or more pure tone audiograms and concurrent impedance telemetry for at least 6 months.This retrospective study was conducted with the approval of the local institutional review board . We reviewed all adult patients who received a CI at our tertiary referral hospital between January 2009 and June 2021  at 125, 250, 500, 1,000, 2,000, 4,000, and 8,000 Hz using either headphone or insert earphones. Impedance telemetry was performed during the same sessions as pure tone audiometry, according to the clinical standard procedure at our institution. For the analysis, we exported the clinical impedance (in kΩ) from the manufacturer's telemetry software . The clinical electrode impedance includes near-field interface impedance and far-field tissue resistance and can be found from the diagonal elements of the measured voltage matrix . In addiR environment (v4.0.3) and the lme4 package (v1.1) for the statistical analysis (audiometry package (v0.3.).Descriptive statistics were used to summarize demographic data and functional outcomes. We used linear mixed-effects models to assess the relation between CI electrode impedance and residual hearing over time. We calculated separate models accounting for the impedance of all active electrodes as well as only the apical (contacts 1–4), middle (contacts 5–8), and basal electrodes (contacts 9–12). The models employed residual hearing (in dB HL) as the dependent variable and electrode impedance (in kΩ) as the independent variable. The follow-up time (in months), side of implantation, gender, and age were additionally included as fixed effect variables. An interaction term between impedance and follow-up time was also included to account for time-dependent variations of the impedance. Subject-level random intercepts were used to account for repeated measurements. Random slopes of impedance were included to enable varying effects for individual subjects. Using the random slope mixed-effects model, subjects may have individual intercepts (random intercept) and individual slopes  around common effects in the association between residual hearing and impedance. We used the analysis . Audiogr28 electrode arrays were implanted (N = 26). Eight patients had a partial electrode insertion, with up to three extracochlear electrodes. Six months post activation, the median word recognition score in quiet was 58%  for the German Freiburg monosyllabic word test and 100%  for the German Freiburg numbers test. Patients had a median follow-up over 25 months  with pure tone audiograms and simultaneous impedance telemetry.Of the 704 entries in our database, 119 patients had a post-operative audiogram available and measurable residual hearing of at least 5 dB between 125 and 1,000 Hz. Among these, 79 were implanted with a MED-EL device. Forty-two patients had two or more pure tone audiograms with concurrent impedance telemetry for at least 6 months and were included in our analysis . Most commonly, CIs with Flexcomplete hearing preservation (category 1), 20 patients had partial hearing preservation (category 2), 10 patients presented minimal hearing preservation (category 3), and five patients had complete loss of residual hearing (category 4). Most commonly, the transition from categories 1–2 occurred within the first 3 months after surgery. On average, cochlear implantation worsened the unaided hearing thresholds by 13.6 dB HL after 3 months and by 19.5 dB HL after 6 months .In general, all linear mixed-effects models used  showed comparable results and significance. The most prominent effects, which are presented herein, were observed for the model including basal electrodes . The results for the additional models including either all, middle, or apical electrodes are summarized in the We found no dependence of the electrode impedance on gender, age, or implantation side. However, there was a time dependence of impedance, with the lowest values observable immediately after the insertion month 0; . Impedanp < 0.001).Loss of residual hearing was significantly correlated with impedance changes on basal electrodes, independently of the follow-up time . The assPure tone audiometry is state-of-the-art when assessing residual hearing. As a subjective, behavioral measurement method, it requires the active participation of the patient and inconsistent responses can occur. In contrast, objective measures could provide additional information independent of the patient's behavior. This attribute would be desirable for postoperative monitoring of the residual inner ear function. Thereby, impedance telemetry could be a promising objective measurement tool, as it can be performed with little effort and can even be self-initiated by the patient. Furthermore, impedance measurements could still be informative in patients with absent residual hearing, e.g., impedance spikes as a surrogate marker of inner-ear events .In our study sample, 64% of the study participants had full or partial hearing preservation at implant activation, 1 month after surgery. This number was further decreased to 48% at the 6-month follow-up. Consequently, most losses of residual hearing occurred intraoperatively or in the early postoperative phase. Reasons can be a traumatic electrode insertion , 23, 24 Our measurements showed a noticeable time dependence of the impedance values. Intraoperatively, impedances were lowest; 1 month after implantation, the impedances reached peak values and subsequently decreased in most cases. Parreño et al.  showed wBesides follow-up time, the position of the electrode inside the cochlea has a substantial influence on impedance values. In the intraoperative measurements, impedances decreased from apical toward basal electrode positions. This is a well-known finding, that can even be exploited to estimate the linear and angular insertion depth of the electrode contacts  and poteWith regard to impedance spikes, in our cohort, only one noteworthy event could be observed in our data (subject 12 after 6 months). According to the medical record, this impedance spike was not related to a clinical symptom . However, most often, the previously described phenomenon usually occurs at a later stage after implantation and only in a subset of patients  .We could observe a significant association between the course of residual hearing and impedance values. The highest impedance values and at the same time the most frequent loss of acoustic hearing was in the first three months after implantation. Jia et al.  demonstrOur study population is too small to draw general conclusions. The choice of a linear model represents a simplification of the more complex hidden processes caused by implantation. In particular, we expect the postoperative short-term responses to exhibit nonlinear behavior. However, in the longer term, individual associations, as shown in The pure tone audiogram remains the gold standard for measuring postoperative residual hearing. Additional objective bio-markers would be desirable to complement these measurements with information about the inner ear independent of the patients' performance. In our study sample, we found an association of residual hearing with clinical impedances indicating its potential use. As this study is exploratory, our results need to be confirmed in larger samples. Analysis of near-field related subcomponents of the electrode impedance could further improve the applicability of impedance telemetry for residual hearing monitoring.The original contributions presented in the study are included in the article/The studies involving human participants were reviewed and approved by Kantonale Ethikbehörde Bern. The patients/participants provided their written informed consent to participate in this study.WW: conceptualization, methodology, formal analysis, and writing—original draft. LS: investigation and data curation. MC: supervision, project administration, and funding acquisition. SW: conceptualization, methodology, project administration, and writing—original draft. All authors contributed to the article and approved the submitted version.This study was funded by the ENT Department, Bern University Hospital. Open access funding provided by University of Bern.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


The aim of this study was to evaluate the intracochlear position of the Slim Modiolar Electrode (SME) after insertion via the extended Round Window (eRW) approach, and to correlate this with residual hearing preservation and speech perception outcomes.Twenty-three adult participants, consecutively implanted with the SME via the eRW approach, were included in this prospective, single-center, observational study. Electrode position was evaluated intra-operatively using X-ray fluoroscopy and TIM measurement, and post-operatively using ultra-high resolution CT. Residual hearing  and speech perception were evaluated at 2 and 12 months after surgery.In each of the 23 participants, complete scala tympani positioning of the electrode array could be achieved. In one participant, an initial tip fold-over was corrected during surgery. Average age at implantation was 63.3 years  and mean preoperative residual hearing was 81.5 dB. The average post-operative PTA threshold shift was 16.2 dB (SD 10.8) at 2 months post-operatively, corresponding with a RHP% score of 44% (SD 34.9). At 12 months, the average RHP% score decreased to 37%. Postoperative phoneme scores improved from 27.1% preoperatively, to 72.1% and 82.1% at 2 and 12 months after surgery, respectively.Use of the eRW approach results in an increased likelihood of complete scala tympani insertion when inserting the SME, with subsequent excellent levels of speech perception. However, residual hearing preservation was found to be moderate, possibly as a result of the extended round window approach, emphasizing that it is not an all-purpose approach for inserting this particular electrode array. With the expansion of the indication criteria for cochlear implantation (CI), preservation of residual hearing has become an important objective . An incrCurrently available CI electrode arrays can be categorized into two main types: ‘straight’ or ‘pre-curved’. Straight electrode arrays essentially follow the lateral wall of the cochlea once implanted. Pre-curved, or (peri)modiolar electrode arrays on the other hand, are designed to curl around the medial wall and position the electrode contacts in proximity to the modiolus and spiral ganglion cells, thus leading to a reduced spread of excitation, lower stimulation thresholds and improved stimulation specificity –11. HoweHowever, a recent study by Heutink et al. in 23 adult participants implanted with a SME via the cochleostomy (CS) approach revealed a translocation from the scala tympani to the scala vestibuli in 36.4% of the participants [Several studies, therefore, advocate the use of either the round window (RW) or extended round window (eRW) insertion approach when using the SME, as this has been shown to decrease the probability of scalar excursion and thus improve audiological outcomes and hearing preservation , 12, 14.This was a prospective, single-center observational study in twenty-three adult patients consecutively implanted with the Slim Modiolar Electrode  using the extended Round Window (eRW) approach. Participants were included between July 2019 and July 2020. Exclusion criteria were (1) pre-lingual onset of deafness, (2) anatomical variations of the cochlea possibly influencing electrode insertion and (3) inability to undergo CT-scanning. Preoperatively, demographic data including the history of hearing loss and audiometry were collected. X-ray fluoroscopy and trans-impedance matrix (TIM) measurements were performed intra-operatively to rule out tip fold-overs. Electrode position was evaluated 1 week post-implantation using an ultra-high resolution computed tomography (UHRCT) scan. Residual hearing was evaluated routinely 2 months post-operatively, whilst speech perception was measured routinely 2 and 12 months post-operatively. Several participants included in the current study were also reported on in a proof-of-concept article by Klabbers et al. on the use of TIM measurements for initial tip fold-over detection. However, no clinical outcomes were evaluated in the previous study. All procedures performed in this study were in accordance with the 1964 Helsinki Declaration and its later amendments . InstituSurgery was performed by three experienced CI-surgeons, and the standard mastoidectomy and facial recess approach was used to gain access to the cochlea in all cases. The bony overhang of the round window niche was carefully removed and the scala tympani was opened using the extended round window technique, in which a so-called round window margin cochleostomy is created by carefully drilling the anterior–inferior bony margin and crista fenestrae Fig. . All 23 Directly following electrode insertion and packing, TIM measurements and fluoroscopy were performed to rule out electrode array tip fold-over. In the event of a tip fold-over, the array was retracted and repositioned in the same surgical setting. One week post-operatively, electrode array position was evaluated using a UHRCT-scan . For scalar position analysis, the UHRCT-images were reconstructed using multiplanar reconstruction (MPR) and filtered back projection (bone filter kernel FC81), with a field of view of 90 mm, 1024 × 1024 matrix and 0.25 mm slice thickness. Mid-modiolar sections of the reconstructed UHRCT-scan images were evaluated by an experienced head and neck radiologist , defined as the average threshold at 250, 500 and 1000 Hz, was calculated for each patient. If a patient failed to respond to the stimulus at a certain frequency, the maximum stimulation level (MSL) was recorded . For th 1000 Hz .RHP percentages for each participant were categorized as either (1) complete preservation (> 75%), (2) partial preservation (25–75%) or (3) minimal preservation (0–25%). Additionally, mean threshold shifts in LFPTA between pre- and post-operative testing were evaluated. If necessary, these values were corrected for the natural progression of hearing loss, as previously described by Snels et al. ((CL)ear) .Speech perception in quiet was evaluated routinely at 2 and 12 months post-operatively. The standard test to assess speech perception in the Netherlands is issued by the Dutch Society of Audiology (NVA) and consists of a list of phonetically balanced, monosyllabic consonant–vowel-consonant (CVC) words presented at 65 dB SPL through a speaker which is placed in front of the participant in a soundproofed booth. The average percentage of three CVC lists was calculated.T tests. P values < 0.05 were deemed statistically significant.Statistical evaluation was performed using SPSS . Continuous data are reported as mean values with standard deviations and comparisons between the audiological outcomes of the current and previous study were performed using independent samples Twenty-three patients were consecutively implanted with the SME at our center. Of these patients, 11 (47.8%) were male and 12 (52.2%) female. Fourteen patients (60.9%) were implanted in the right ear, whilst nine (39.1%) were implanted in the left ear. The average age at implantation was 63.3 years  and participants had a mean preoperative residual hearing (LFPTA) of 81.5 dB (SD 11.9).Upon post-operative UHRCT scan evaluation, all of the electrode contacts for each of the 23 patients included in this study were found to be located fully within the scala tympani: no translocation had occurred. Intraoperative fluoroscopy and TIM measurement revealed a tip fold-over in 1 of the 23 patients (4.3%). In this patient, subsequent repositioning of the electrode array in the same surgical setting led to a satisfactory intracochlear position as confirmed by repeated fluoroscopy and TIM measurements, as well as a complete ST positioning of the array as confirmed with UHRCT 1 week post-operatively. Postoperative UHRCT evaluation did not reveal a tip fold-over in any of the other participants.Postoperative residual hearing thresholds and speech perception scores for all twenty-three participants are presented in Table According to the HEARRING classification, 7 out of 22 (31.8%) participants had complete hearing preservation (RHP > 75%) at activation, whilst 6 out of 22 (27.3%) had partial preservation (RHP 25–75%). 9 out of 22 participants (40.9%) had minimal or no preservation of residual hearing (RHP < 25%). Of the 7 participants who had a preoperative LFPTA of less than 80 dB, only 3 maintained a LFPTA lower than 80 dB at activation.Long-term residual hearing preservation (RHP%) was evaluated at 12 months post-operatively and was found to have deteriorated by an average of 7% (from 44 to 37%), indicating a progression of residual hearing loss over time.The mean post-operative CVC-phoneme score at 2 months was 72.1%, and this further improved to 81.1% at 12 months Table .Table 3MThe post-operative threshold shift in LFPTA in the participant with an intraoperative tip fold-over was 10 dB, whilst the relative hearing preservation score according to the HEARRING classification was 0%. Speech perception scores at 2 and 12 months were 93% and 93%, respectively.This study was initiated to evaluate the intracochlear electrode position and audiological outcomes of patients with the slim modiolar electrode, implanted exclusively using the extended round window approach. This is currently the approach of choice at our tertiary referral hospital for inserting this particular electrode, as a previous study (conducted at our center) revealed translocations of the SME from scala tympani to scala vestibuli in 36% of the patients implanted via cochleostomy. The electrode arrays of all participants included in the current study were found to be located fully within the scala tympani following extended round window insertion, resulting in excellent levels of speech perception scores.Implantation of the SME via the extended round window approach, therefore, seems to invariably lead to correct positioning within the scala tympani. These results are an evident improvement compared to previously reported implantations via a cochleostomy approach at our center, and generally comparable to what is reported in the literature, although studies adequately reporting on the CT-evaluated scalar position by surgical approach for the SME are still relatively scarce. The studies in which this was determined using high-resolution imaging result in a combined average translocation risk of 4.8% (22/461) –24. Of tMoreover, our results further confirm that this in turn gives rise to excellent levels of speech perception with this particular electrode. We found the CVC-phoneme score to improve from 27.1% preoperatively to 72.1% at 2 months and 82.1% at 12 months post-operatively. These scores are comparable to, if not better than, what is reported in other studies , 23, 25.n = 6) [However, the residual hearing preservation results obtained in this study using the eRW approach are suboptimal. We found an average loss of 16.2 dB at implant activation, corresponding to a RHP% of 43.9%. These values approximate the results found for patients with cochleostomy-related translocations in whom all contacts were located within the scala vestibuli , although again, the number of patients in that study was limited (n = 6) . The mecn = 6) . Furthern = 6) . The autn = 6) .n = 148, range: 27.5–95%) using the Freiburg monosyllable word test. However, the fact that so little studies report on ‘pure round window’ insertions or do not exactly describe the procedure leading up to insertion of the electrode emphasizes the exceptionality of the anatomical circumstances necessary to insert the SME directly through the round window membrane. On the other hand, so-called ‘round window enlargement’, in which the posterosuperior lip of the bony overhang covering the round window niche is extensively drilled without additional intracochlear drilling, seems to deliver good results, as described by Ramos et al. and Iso-Mustajärvi et al., who reported on 18 SME insertions via a ‘round window membrane’ approach, but add to their description of surgical technique that the superior bony overhang of the round window niche was largely drilled away to achieve an optimal view of the round window membrane [125-500 < 80 dB) in 14/17 ears (82%).Besides insertions via the extended round window- or cochleostomy approaches, SME insertions via so-called ‘pure round window approach’ and ‘round window enlargement’ have also been described, which do not require drilling the crista fenestrae , 21, 30.membrane , 22. TheIn summary, each of the surgical techniques has its advantages and limitations. To achieve correct positioning of the SME with the highest chances of ST placement, the different round window techniques seem to be favored over the cochleostomy technique. Between the insertions through the round window, the pure round window approach would be the technique of choice if the individual anatomy and silicone sheath would allow it. However, in the majority of the cases insertion directly through the RW is not possible and for those cases, round window enlargement or round window extension will also provide excellent ST positioning and speech perception scores. On the other hand, the extended round window approach showed suboptimal results for preservation of residual hearing in this study, with a level of loss comparable to that of patients implanted via cochleostomy with translocation to the SV . The besThe main limitation of the current study is that the number of participants is relatively small. The sample size was chosen to be able to compare to our previous study, in which we investigated CI outcomes using the SME implanted via cochleostomy. However, the direct comparison to this previous study was hampered by differences between the two patient populations, as the current study was conducted solely in participants with post-lingual hearing loss, with better preoperative pure tone audiometry thresholds and speech perception, and the surgery was conducted by three different surgeons. Additionally, residual hearing has been found to deteriorate beyond 24 months after surgery . Therefowith residual hearing should be implanted via ‘pure round window’ or ‘round window enlargement’ approach, whilst patients without residual hearing should be implanted via an extended round window approach. In cases where the anatomy is not favorable, i.e. in patients in whom round window insertions are not possible due to inadequate visibility of the RW membrane, an inferiorly placed cochleostomy may offer a solution and result in good levels of both residual hearing preservation and speech perception if the electrode is placed within the scala tympani.In this prospective study, we showed that correct positioning of the SME within the scala tympani can consistently be achieved using the extended round window approach, resulting in excellent levels of speech perception. However, the results also indicate that extending the round window opening can be suboptimal for the residual acoustic hearing, emphasizing that with this particular electrode there is no ‘all-purpose’ insertion approach for cochlear implantation. Where the anatomy is favorable, an insertion via the round window should be considered; if surgically feasible, patients