Age-related hearing loss (ARHL) is a complex multifactorial disorder. Studies in animals, including mitochondria-mutator mice, and in human suggest that oxidative stress and mitochondrial disturbance play an important role in the pathoetiology of ARHL. Mitochondrial DNA (mtDNA) haplogroups are populations with genetically similar traits, and they have been reported to affect the mitochondrial function of oxidative phosphorylation. To gain further insights into the relationships between mitochondrial haplotypes and the susceptibility to cochlear aging, in this study, we aimed to elucidate how the differences in mtDNA haplogroups may affect ARHL development in Japanese general population. We focused on early onset ARHL, as the same mtDNA haplogroup can show either a negative or positive effect on systemic co-morbidities of ARHL that appear later in life. A total of 1167 participants of the Iwaki Health Promotion Project were surveyed in 2014, and 12 major haplotype groups  were selected for the analysis. A total of 698 subjects aged 30 to 65 years were included in the statistical analysis, and the hearing loss group consisted of 112 males (40.3%) and 111 females (26.4%). Multiple logistic regression analysis showed that the male subjects belonging to haplogroup A had a significantly increased risk of hearing loss, whereas the female subjects belonging to haplogroup N9 had a significantly decreased risk of hearing loss. These results suggested that the mtDNA haplogroup may be an indicator for future risk of morbidity associated with ARHL. Hearing loss is the most common sensory disorder in the world. Aging is the main cause of hearing loss, affecting tens of millions of people worldwide . In 2008The World Health Organization defined hearing loss as a pure tone average of more than 25 dB thresholds at 500, 1000, 2000, and 4000 Hz , and heaBesides aging, ARHL is also associated with a variety of factors, including noise exposure , ototoxiHuman mtDNA and mtDNA, in general, are maternally inherited and not affected by homologous recombination during gamete formation, unlike nuclear chromosomal DNA , 17. TheIn this study, we aimed to elucidate how the differences in mtDNA haplogroups affect the development of ARHL in Japanese population.The Iwaki Health Promotion Project is an annual, large-scale epidemiological survey performed in Iwaki District, Hirosaki City, Japan. All residents older than 20 years of age living in this district are invited to participate in the project. The data collected during this project in 2014 were used in the study. A total of 1167 participants were surveyed, among which 33 data-deficient subjects and 58 subjects with ear disorders, including acute sensorineural hearing loss, Meniere’s disease, cholesteatoma, and traumatic inner ear disorder, were excluded. To detect early hearing loss with aging, subjects aged only 30‒64 years were enrolled in the present study. Finally, 698 subjects were included in the statistical analysis , and all subjects provided written informed consent before participating in the project.To obtain the participants’ lifestyle information, we used a self-administered questionnaire and conducted face-to-face interviews to determine the medical history, drug information, noise exposure history, smoking habit, and drinking habit of the participants. The pack-years (number of cigarette packs per day × years of smoking) for smoking habit was also calculated. Venous blood samples were obtained early morning with the participants on an empty stomach to examine hemoglobin A1c (HbA1c), triglyceride (TG), high-density lipoprotein cholesterol (HDLC), and low-density lipoprotein cholesterol (LDLC). The diseases that may affect hearing were defined as follows: hypertension was defined according to self-reported physician diagnosis and current use of antihypertensive medicine; diabetes mellitus was defined according to self-reported physician diagnosis and current use of antihyperglycemic medicine or the HbA1c level greater than 6.5% according to the diagnostic criteria of the Japan Diabetes Society; dyslipidemia was defined according to self-reported physician diagnosis and current use of lipid-lowering medicine or serum level of TG ≥ 150 mg/dL and/or HDLC < 40 mg/dL and/or LDLC ≥ 140 mg/dL according to the diagnostic criteria of the Japan Atherosclerosis Society.Pure tone audiometry was conducted by trained doctors and technologists in a quiet room. Using audiometers , they measured air-conducted hearing on both sides at 125, 250, 500, 1000, 2000, 4000, and 8000 Hz. The worse-hearing ear was used for the analysis, depending on the average thresholds at 500, 1000, 2000, and 4000 Hz. As the beginning of hearing loss with aging occurs at high frequencies [Genomic DNA extracted from venous blood samples was subjected to whole-genome sequencing by Takara Bio Inc. . Sequence mapping was performed at the Institute of Medical Science, University of Tokyo. The mitochondrial haplogroup of each BAM file of the mitochondrial genome was assigned using MitoSuite version 1.0.9 .t-test and chi-square test. A comparison of specific mtDNA haplogroup prevalence was performed using the chi-square test.The characteristics of the subjects  and the hearing loss and control groups for each gender were compared using Student’s Risk factors influencing high-frequency hearing loss were evaluated using the multiple logistic regression analysis separately for males and females. The dependent variable was the presence or absence of high-frequency hearing loss, and the independent variables were age, smoking history (pack-years), drinking habit, noise exposure, hypertension, diabetes, and dyslipidemia. Furthermore, each of the 12 haplogroups were added to the independent variable.p < 0.05 were regarded as statistically significant. All statistical analyses were performed using IBM SPSS Statistics .Test results with The characteristics of our subjects by gender are shown in Table The characteristics of subjects in the hearing loss and control groups for each gender are shown in Table p = 0.04) and haplogroup N9 was significantly less common (p = 0.006) in the hearing loss group of female subjects , and 95% confidence interval (CI) for each haplogroup are summarized in Table p = 0.01), with an odds ratio of 4.096 (95% CI: 1.327–12.643) (Table p = 0.02), with an odds ratio of 0.091  m.8794 C > T polymorphism . The polARHL is known to progress faster in males than in females . The higIn this study, differences in the correlation between ARHL and mtDNA haplogroups depending on gender were observed. Some studies have reported that there were differences depending on gender, but the reason for gender differences was not clear. Haplogroup N9a is a protective factor for type 2 diabetes only in females , and hapIn conclusion, multivariate analyses after considering confounding factors were performed to investigate the association between any mtDNA haplogroup and ARHL development. The results indicated that males in mtDNA haplogroup A were more likely to develop ARHL than males in other haplogroups, and females in haplogroup N9 were less likely to develop ARHL than females in other haplogroups. This suggested that the mtDNA haplogroup may be an indicator for future risk of morbidity associated with ARHL.


There seems to be a lack of consensus on the concept and domains of Activities of Daily Living (ADL) measured among children and adolescents with developmental disabilities (DD), despite a significant number of existing measures of ADL and associated constructs, and two prevailing theoretical frameworks .This systematic review (SR) aims to identify articles that systematically reviewed measures of ADL for children and adolescents aged 7–18 years with DD to evaluate the quality of included articles, and describe the measures and domains identified.and Procedures: Searches were conducted in PubMed®, Academic Search Complete® (EBSCOhost), Education Source Search® (EBSCOhost), ERIC® (EBSCOhost), and PsychInfo® (EBSCOhost). 14,931 articles were identified, and two researchers completed title screening, abstract screening, and full-text screening, with disagreements resolved. Out of these 14,931 articles, fourteen were included, which resulted in a total of 163 ADL measures. Out of the 163 ADL measures, forty-eight met the criteria and were included for analysis. PRISMA and COSMIN checklists were used to appraise the methodological quality of the included articles.Results indicated that most of the 14 systematic review articles did not provide information on instrument development and content validity of their included measures. Analysis of the identified 48 measures of ADL and its associated constructs revealed heterogeneity in domains covered, although there were seven domains that were most often included.Implications in terms of practice, research, and policy are further discussed. Developmental disability, Inclusive practices, Life skills. This paper assessed articles that reviewed Activities of Daily Living (ADL) measures applied to children and adolescents with developmental disabilities aged 7–18 years old. Fourteen included studies were appraised for reporting and methodological quality according to recommendations in PRISMA and COSMIN. Key information  were extracted for the identified 48 measures of ADL and its associated constructs. Results suggest a lack of guidelines for quantitatively synthesizing SRs of measures and evaluating their psychometric properties. Using the COSMIN checklist, it was found that most reviews did not provide information on instrument development and details of content validity. Among the identified measures, each had seven domains on average. The key domains are personal/domestic, motor/mobility, academics, social, leisure, community participation, and communication. The most frequently measured outcomes were degree of competence in skills, degree of assistance required, frequency of behavior/activity performance, and degree of difficulty doing a task/activity. Rating scales were the most widely used response format. An evidence-based approach as delineated in this study may be conducive to understanding how ADL has been empirically represented in measurement practices.1Activities of Daily Living (ADL) as a term, was first coined by Sidney Katz in 1950 in his field of gerontology and health services . It refeADL is also closely related to the construct of adaptive behavior or adaptive functioning. The similarity or resemblance can be reflected in one frequently cited definition for adaptive behavior or adaptive functioning - “the level of everyday performance of tasks that is required for a person to fulfill typical roles in society, including maintaining independence and meeting cultural expectations regarding personal and social responsibility” , p. 18. Despite stemming from relatively different disciplinary foci , similar to measures of adaptive behavior, ADL measures aim to assess an individual's level of independence or perceived autonomy in performing different basic or more advanced activities necessary for daily living such as personal hygiene, socialization, communication, and community participation . The key1.1Using ADL measures for populations experiencing disability has considerable implications. From a broader perspective, ADL measures could be used to collect aggregate data on disability populations to inform international policies for a more inclusive society, as envisioned in the United Nations 2030 Agenda for Sustainable Development Goals . The ageConsidering a narrower perspective, measures of adaptive behavior in the field of psychological assessment are used for diagnostic purposes to inform high-stakes decisions on needs and supports for individuals with disability at different developmental stages, such as informing a determination on whether a person can be diagnosed as having intellectual disability . Such crIt is noteworthy that in the field of psychology, measures of adaptive behavior have often been developed under one of two major theoretical frameworks. For one, the American Association on Intellectual and Developmental Disabilities (AAIDD) defines adaptive behavior in terms of three areas – conceptual, which includes language, numeracy, self-direction etc., social, which involves aspects of social problem solving, interpersonal skills, social responsibility etc., and practical skills which includes domains of personal care, vocational skills, community participation, healthcare etc. cf. . AlternaOn the basis of these two top-to-down theoretical frameworks, by far, more than 200 measures of adaptive behavior have been developed for different purposes . MeanwhiCompared to measures of adaptive behavior in the field of psychology with a theoretically driven approach to measure development, there seems to be a lack of theoretical frameworks for measures of ADL, which are used across more various disciplinary fields. Hence, there remains a gray area where it is universally unclear about what domains constitute ADL , within 1.2As an initial step to understand the ADL measures especially for individuals with developmental disabilities (DD), the current study aims to take a down-to-top approach by first identifying ADL measures for children and adolescents with DD via a systematic approach and then analyzing the key attributes of these ADL measures . In particular, we decided to conduct a systematic review (SR) of articles that reviewed ADL measures. This decision was made on basis of two major rationales, (a) to identify the most relevant measures because the measures identified in the existing reviews of ADL measures have been scientifically endorsed to a certain degree in terms of psychometric evidence or practical utility, and (b) to avoid duplicates of existing literature reviews.In accordance with the Centers for Disease Control and Prevention , DD encoThis study aims to (a) describe and assess the quality of reporting and evaluation of measurement properties in existing SRs identified, and (b) identify existing measures pertaining to ADL or its associated constructs for children and adolescents with DD aged 7–18 years to report various domains and outcomes measured. To the best of our knowledge, such an extensive review of measures in ADL or its associated constructs has not been conducted to inform the extent of psychometrically validated measures across multiple specialties in healthcare for school aged children and adolescents with DD.22.1The included SRs had at least one study that described activities required for daily functioning based on self or others' reports  for children and adolescents with DD aged 7–18 years. 2.2This SR of SRs complies with Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) guidelines . PRISMA 2.3The searches were completed in July 2020, with 14,931 articles identified. With the duplicates removed in EndNote 9, there was a total of 12,815 articles for screening. Two researchers independently screened the title and abstract, resulting in 798 articles, with a Cohen's Kappa of 0.94. All disagreements were resolved via discussion, and 86 articles were eligible for full-text screening. We could not locate the full text for four of the 86 articles despite electronic searches and interlibrary loans through three international universities. Two researchers independently conducted full-text screenings of the 82 articles, with all disagreements resolved via discussion. Eventually, 14 articles met the inclusion criteria. Measures from these 14 SRs were extracted, resulting in a total of 163 entries. Following the inclusion and exclusion criteria, 48 measures were included for analysis . The PRI2.4Quality assessments for SRs such as the Assessment of Multiple Systematic Reviews  used as eligibility criteria, with a rationale provided. Eleven articles described all information sources in the search, including the last search date. Eleven articles presented a complete electronic search strategy for at least one database. Ten articles stated the process for study selection. Seven studies described the data collection process . Seven articles listed and defined the variables for which data were coded. Eleven articles described methods used to assess individual studies' measurement properties and how this information could be used in data synthesis. None of the articles stated principal summary measures . Similarly, none of the articles described the methods of handling data and combining results of studies or specified any assessment of risk of bias that may affect cumulative evidence .Within the results, nine articles provided details on the total numbers of studies screened, studies included in the review based on an eligibility assessment, and reasons for exclusions at each stage. Five articles presented a flow diagram delineating the inclusion and exclusion process of the studies . ThirteeFor the discussion section, all 14 articles provided a summary of the main findings; 10 articles discussed limitations at individual study and review levels; 13 articles contained a conclusion giving a general interpretation of the results. Finally, seven articles described sources of funding for the SR.3.33.4M = 6.7, SD = 4.0), with the domains for one measure  not explicitly stated. The number of items ranging from 8 to 502 , and thus the number of items per domain varied widely across the 47 measures, ranging from 2 to 100 . The 313 domains could be further categorized into 19 broader domains. The most frequently occurring domains involved personal/domestic, motor skills/mobility, academics, social, leisure, community participation, and communication.Across the 48 measures, the number of domains across 47 measures totaled 313, ranging from 1 to 24 . Twenty nine out of the 48 measures are freely downloadable or could be requested from the developer(s), while the other 19 measures require to be commercially purchased. Finally, 19 of the 48 ADL measures assessed one outcome while the other 29 measures assessed more than one outcome (range: 2–34). The ADL outcomes assessed included degree of competence in skills, degree of difficulty in completing a task/activity, degree of assistance required, frequency of activity participation, degree of importance, degree of satisfaction, amount spent in activity, etc. The most frequently measured outcomes were degree of competence in skills, degree of assistance required, frequency of behavior/activity performance, and degree of difficulty doing a task/activity. In terms of response formats, rating scales, multiple choice items, and open-ended items were used across the 48 measures. Rating scales were the most widely used response format.4This study conducted an SR of SRs that synthesized measures assessing ADL for children and adolescents with DD aged 7–18 years. Fourteen SRs were identified from the initial search output of 14,931 articles, and 48 measures were further identified among the 163 measures derived from the included studies. Aligned with research literature on ADL measures for children and adolescents with DD, SRs in the current paper reported consistent trends in assessing psychometric properties of the individual measures, with common areas of deficits and strengths . These iThe 14 SRs of measurement instruments could not meet all criteria outlined in the PRISMA checklist. A consistent deficit with reporting results across the 14 SRs was the lack of information on summary measures, the synthesis of results , and the overall risk of bias on the studies reviewed based on PRISMA standards. This could be due to an abundance of statistics in psychometrics, heterogeneity of instruments with different theoretical frameworks as reflected in their varied purposes, and the lack of well-understood methodologies for meta-analysis of psychometrics-related studies. However, when reviewers selectively choose which information to include in a review based on the direction and significance of findings, they risk biasing the evidence base on which healthcare decisions and policies are made. Hence, the next logical and imperative step for research in this area is to employ a well-established criterion, such as the PRISMA checklist, to critically evaluate studies and avoid biases in reporting outcomes within reviews .th edition (DSM-5). Providing details of instrument development would give us crucial information regarding the relevance, comprehensiveness, and comprehensibility of the measures , and the rest used various criteria for evaluating the measurement properties of their included ADL measures. This introduces considerable heterogeneity to their review methodology as well as inherent difficulties in comparing the findings of these studies. The evaluation of SRs using the COSMIN checklist showed that most review articles reported psychometric properties of studies included in terms of reliability, construct validity, and responsiveness but lacked methodological rigor . For insmeasures .Based on our review, this article could be the first of its kind to provide a comprehensive overview of assessment measures pertaining to ADL and its associated constructs for children and adolescents with DD aged 7–18 years. The relevance of this systematic search is three-fold. Firstly, for practitioners working closely with children and adolescents with DD, this article provides an extensive inventory of measures that could be used to assess and monitor practical outcomes for this population. Secondly, this consolidated database could provide a foundation for researchers and scale developers to generate future ADL measures while incorporating newer items or domains across various aspects of daily functioning to keep abreast with universal technological advancements . Finally, for policymakers trying to reach global targets for a more inclusive world, this register of measures (including 29 freely available measures) could help improve accessibility to assessment options for ADLs across countries, families, and children and adolescents with DD where resources are limited.It is noteworthy that the purpose of this paper was not to identify an ideal or perfect ADL measure. Employing a specific instrument may differ considering client contextual variables such as their primary needs , their context's cultural attitudes and beliefs towards disability, support based on national/global policies etc. . The aim4.1Diagnostic Adaptive Behavior Scale . Additionally, as this paper involved an SR of review articles, newer measures assessing ADL that are freely available or commercially purchased were not included. For example, AAIDD's le DABS;  was rece5The findings of this SR of SRs exemplify a systematic approach to identifying measures focusing on ADL, with the aim of providing clarity to its operational definition. Focusing on the population of children and adolescents with DD aged 7–18 years old, the identified measures that primarily rely on others' reports examined ADL from different domains with a consistent purpose of gathering information to inform an individual's functional independence in day-to-day life. Without systematically documenting ADL performance for children and adolescents with DD using appropriate measures, it would be difficult to move forward toward the prospect of a more inclusive society, as outlined in the Sustainable Development Goals by the United Nations.Rahul Nair; Mo Chen; Anuradha SK Dutt: Conceived and designed the experiments; Analyzed and interpreted the data; Contributed reagents, materials, analysis tools or data; Wrote the paper.10.13039/501100006124National Institute of Education, Singapore [DEV 05/19 CM].Mo Chen was supported by Office of Educational research- The authors do not have permission to share data.The authors declare no conflict of interest.No additional information is available for this paper.


Cochlear implants are considered a viable therapeutic option for deaf children and adults who meet the implant criteria. They enable the hearing impaired to deal with the effects of deafness and participate academically, socially, and vocationally with their family and peers, aiding them to live a life with fewer limitations in the world of spoken languages. Most importantly, cochlear implants counteract the impact of profound deafness in early childhood, in which the ability to develop spoken language is severely restricted. For these children, cochlear implants represent the only means of hearing speech, resulting in the development of meaningful oral communication abilities. Over the past decades, there have been intense debates about communication philosophies for deaf or hard-of-hearing children, the nature and concept of deafness, the value and diversity of the deaf culture, the purpose of deaf education, and the best education systems for deaf or hard-of-hearing children. There are two broad camps in the debate over cochlear implantation: those who embrace the technique and those who oppose it. In this paper, we describe my own journey from total deafness to the beginning of a dream career as an otorhinolaryngology resident. We also engage in a discussion about the ethical concerns associated with cochlear implantation. While the first known attempt to use electricity to enable hearing goes back to 1790, when the researcher Alessandro Volta placed metal rods in his own ears and connected them to a 50-volt circuit, it was actually in the 1980s that cochlear implants received the stamp of Food and Drug Administration (FDA) approval for implantation in adults, thereby ushering in wide-ranging advancements in speech processors and implant technology, particularly the miniaturization of the speech processor . The plaThere are two major schools of thought with respect to cochlear implantation, and these comprise the supporters and opponents of the technology.I had never faced any hearing issues prior to my deafness and had no significant medical history. At the age of eight, I began experiencing severe headaches. It started in the early afternoon of March 28, 2001. As time passed, the symptoms progressed to dizziness and retching. I remember walking down the hall with difficulty one day and approaching my mother in tears, informing her that I was ill. My mother called our family doctor and described my symptoms to him. He then advised that if I developed a fever, I should seek medical attention immediately and that I was most likely suffering from a viral infection.That same night, my fever spiked to 37.5 °C, which was quickly reduced with simple antipyretic medications, while I kept a large bowl nearby in case I needed to vomit. The following day, I felt better, but my head felt heavier, and I had no appetite. During the day, the sunlight bothered me, and I expressed my discomfort and confusion through my words and actions. My mother called an ambulance, and during the drive, I experienced epileptic seizures.I was diagnosed with meningoencephalitis caused by pneumococcus based on a thorough lab examination, scanning, and spinal tap. I was moved to isolation and, following a dramatic deterioration of my condition, admitted to the pediatric ICU. The doctors had prepared my mother for the worst-case scenario, as my health was deteriorating and every moment was critical both for my survival and to arrest the disease's progression.I was in the ICU for two weeks in total, and following that, I was eating via nasogastric tube, had poor sphincter control, and was completely unable to walk. The complication of deafness became apparent two weeks later when my family was unable to communicate with me effectively due to my inability to respond appropriately to their questions. My hearing loss primarily affected me psychologically, by causing melancholy, which resulted in social isolation. When I gradually regained my mental capacity, the doctors began looking for a cure for my bilateral neurosensory deafness.Cochlear implantation was proposed following a failed attempt with hearing aids to improve my hearing and speech. Due to my recent meningitis history, I was required to undergo a regular temporal bone MRI to monitor for possible cochlea non-ossification. Finally, on May 12, 2002, I underwent cochlear implantation in the right ear.Following successful implantation, I attended months of intensive speech therapy classes to maximize the implant's capabilities in conjunction with speech therapy intervention. Through speech therapy, I improved my conceptual ability in speech and hearing without the use of lip-reading. Additionally, I engaged in extensive play therapy and sessions with an expert psychologist, which aided me in comprehending the world around me in these new circumstances.I continued my education in a public school without receiving any additional assistance or encountering significant difficulties. I first became acquainted with sign language when I was 17 years old, approximately nine years after I was diagnosed with hearing loss, and was introduced to the deaf community. Initially, I encountered the deaf community's disbelief and suspicion, followed by judgment, as I did not know sign language and was a cochlear implant user. Eventually, in order to communicate with deaf people, I learned sign language.Hundreds of thousands of people of all ages are believed to benefit from cochlear implants, which enable them to hear properly and thereby fully engage with the complexities of their environment and contribute actively and creatively to society. According to the author, cochlear implant technology opens doors previously closed to the deaf, allowing the hearing-impaired to overcome the effects of deafness and participate academically, socially, and professionally with their family and peers, enabling people to live a life with fewer limitations, and maximizing their capacities and opportunities . Most imMany studies have shown that the majority of hearing-impaired children find reading difficult, leading to significantly lower levels of reading attainment than their hearing peers throughout their school years. Moreover, this gap between deaf children and their hearing peers tends to widen with age . HoweverIt is critical to emphasize, however, that supporters of this procedure feel that cochlear implantation should remain a personal choice and should not be made mandatory for all deaf people. Additionally, when patients are minors, the decision to implant a cochlear implant is made by the parents, who have been informed about the procedure and opt to incorporate the child's "hearing culture" -8. MoreoDespite the benefits of cochlear implant technology, there have been persistent debates over the last several decades about communication philosophies for deaf or hard-of-hearing children, the nature and concept of deafness, the value and diversity of deaf culture, the purpose of deaf education, and the best education system for deaf or hard-of-hearing children. These debates revolve around discrepancies between theory and practice; ethical concerns about cochlear implant surgery at an early stage, both medically and socially; informed consent; the parental decision-making process; and the annihilation of residual hearing .Primarily, the sociocultural approach establishes that the deaf community does not view itself as a medically curable disability group. The community has a tendency to regard deafness as an aspect of difference rather than a hearing impairment, portraying it as a natural state. Additionally, the deaf community's principal form of communication, American Sign Language (ASL), which is a language in and of itself with its own grammatical elements and styles of expression, is viewed as their "native language". Also, it has been observed that deafness inspires and sustains a unique language and culture that enables meaningful engagement in all facets of life and society -12.Additionally, members of the deaf activist community believe that trending YouTube videos of cochlear implant activation sensationalize and romanticize cochlear implants, offering a skewed perspective by way of sugarcoating the shock and anguish experienced by many recipients . FurtherCrouch, in a study, further argues that opting out of cochlear implantation for one's child does not doom them to a world of meaningless quiet, but rather exposes them to the deaf community, which is historically, linguistically, and morally a rich and rewarding experience . ResearcGiven the clinical and scientific evidence that cochlear implants minimize the effects of inadequate auditory influences and increase overall verbal development, they are highly recommended . I am a Cochlear implants are considered a viable therapeutic option for deaf children and adults who meet the implant criteria. Over the decades, we have witnessed passionate debates about communication philosophies related to deaf or hard-of-hearing children, the nature and concept of deafness, the value and diversity of deaf culture, the purpose of deaf education, and the best education system for deaf or hard-of-hearing children. There are two broad camps in the debate over cochlear implantation: those who embrace the technique and those who oppose it. In this article, I have described my own journey from total deafness to the beginning of a dream career as an otorhinolaryngology resident.


The voice of hearing-impaired individuals has been described extensively, and exhibits abnormalities in quality, articulation and resonance. Having an understanding of the aspects that may have an impact on voice characteristics of cochlear implant users is important for users and for professionals in this field.To verify the existence of correlation between age, time of device use, voice detection threshold, hearing category score and language category score with acoustic data of voices of cochlear implanted children.Retrospective study. Fifty-one children ranging in age from 3 years to 5 years and 11 months who unilaterally used cochlear implants participated. Acoustic analysis of the sustained vowel/a/, sequential speech and spontaneous speech was performed. The results were correlated with demographic data and hearing test results.p ≤ 0.001) and in the spontaneous speech (p ≤ 0.005).Children with worse voice detection threshold showed higher frequency in the sustained vowel (There was a correlation between the voice detection threshold and the frequency values of the sustained vowel and spontaneous speech of the studied population. The vocal behavior of children can vary considerably, and parameters such as resonance, pitch and loudness are essential for determining this performance and to identify voice abnormalities.In the child with hearing impairment, especially if pre-lingual, there is a flaw in auditory monitoring of the voice resulting in several deviations of vocal production, because in addition to neuromuscular control, hearing is important for good performance in oral communication. The auditory system is able to regulate voice parameters such as intensity, extension and frequency.7Especially when performed early in life, the cochlear implant is a most important advancement in the treatment of children with pre-lingual hearing impairment, and implanted children achieve better auditory perception of speech sounds, incidental appropriation of oral language, and better speech intelligibility and vocal production.It is known that the results with cochlear implants are closely related to the age at which the child received the device.In this study we question whether the vocal characteristics of cochlear implanted children are related to other demographic and hearing performance characteristics. Therefore, the aim of this study was to investigate the correlation between age, time of device use, voice detection threshold, hearing category scoreThis study was approved by the Ethics Committee in Research with Human Beings of the institution where it was held, under process N. 131/2010. All parents/guardians signed the free and informed consent form.Nucleus 24 Contour, Nucleus Freedom Contour, PulsarCI100 or SonataTI100 cochlear implants, participated in the study.Fifty-one children with pre-lingual hearing impairment, who unilaterally used the The inclusion criteria for participation in the study were severe or profound bilateral congenital sensorineural hearing loss, no intellectual or emotional impairments, participation in an auditory habilitation program in the city of origin, cochlear implantation before 36 months of age with full insertion of the electrodes and use of the CI for more than a year.Considering the purpose of the study, speech samples were collected from participants, comprising the utterance of the vowel/a/three times, sequential speech (counting from 1 to 5) and spontaneous speech for subsequent acoustic analysis. The recording program used was Sony Sound Forge  with sampling rate of 44,100 Hz, 16 Bit, mono channel. The participant remained seated during the recording in an acoustically treated audiometric testing room. The sound card M-Audio Fast Track Pro and headset microphone AKG C512 positioned at 45° and with a distance of 3 cm from the mouth were used.f0) and standard deviation;Frequency measurements: fundamental frequency mean (f0 (vf0) and jitter;Long-term and short-term frequency disturbance measures: coefficient of variation of Long-term and short-term amplitude disturbance measures: coefficient of variation of the amplitude (vAm) and shimmer;Noise measurements: vocal turbulence index (VTI), soft phonation index (SPI), harmonics-to-noise ratio (HNR) and degree of sub-harmonics (DSH).Acoustic analysis was performed with the Multi Dimensional Voice Program, model 5105, version 2.5.2 (Kay Elemetrics), and Real Time Pitch, model 5121, version 2.5.2 (Kay Elemetrics). The selected acoustic parameters for analysis of the long vowel were:The selected parameters for analysis of sequential speech and spontaneous speech samples were frequency measurements: frequency mean, frequency extension measured in Hertz (Hz), minimum frequency, maximum frequency, frequency standard deviation and frequency extension measured in semitones.Data regarding age, time of cochlear implant use, free-field voice detection threshold (VDT), hearing category score and language category score were collected on the same day of the voice recording, during routine evaluations by speech therapists from the cochlear implant service of the institution where the study was carried out.The selected variables are continuous and normally distributed. Therefore, the statistical test used for the analysis was Pearson's correlation with a 5% significance level. A classification scale was used, where the correlation of 0–20% is considered poor, 20–40% bad, 40–60% regular, 60–80% good and 80–100% excellent.This study found a correlation between age, time of device use, voice detection threshold, hearing category score and language category score with acoustic data of voices of cochlear implanted children. The data that characterize the study population are shown in f0 in the sustained vowel (p ≤ 0.001) and in spontaneous speech (p ≤ 0.005) increases . Their determinant factors are the natural length of the vocal folds, stretching, mass vibration and the involved tension.It is possible that the lack of auditory feedback by decreased auditory thresholds causes an increase in tension during the glottic cycleIn this study, it was observed that both in the sustained vowel and in spontaneous speech, the participants with worse voice detection threshold had the highest frequency. As the hearing loss increases or a hearing threshold obtained with a hearing aid worsens, the voice parameters tend to be more deviated.f0 increases.25A previous studyThe impact of worsening hearing thresholds on the fundamental frequency measures has also been shown in studies that evaluated vocal production immediately after removing the auditory feedback provided by the CI.The other acoustic measures, including speech signal disturbances, utterance noise level and stability, did not correlate with the assessed data. Acoustic analysis of cochlear implant users demonstrated alterations of frequency and amplitude measures in short term with noise measurements within the normal range, and altered stability measures,Understanding the physiological processes that contribute to vocal development in cochlear implanted children allows us to understand the speech production strategies in this population, attempting to establish goals for an adequate speech production.This study demonstrated the importance of understanding not only the vocal characteristics of cochlear implant users, but also with what factors these characteristics are correlated. This information is of great value for clinicians to increasingly ensure appropriate intervention regarding the vocal production of CI users.For the assessed cochlear implanted children, there was a correlation between the voice detection thresholds and the frequency values for the sustained vowel and also in spontaneous speech. That allows us to affirm that the worse the voice detection threshold is, the more high-pitched is the child's voice. Other acoustic parameters did not correlate with the other assessed variables.The authors declare no conflicts of interest.


The relationship between diabetes mellitus and hearing loss is described as ‘controversial’ in the literature, given that in the clinical realm many patients present dysacusis while others do not.this study aims to investigate the relationship between hearing loss and diabetes mellitus and add to the knowledge being developed in this area.cross-sectional clinical trial.in our study we analyzed the medical charts of 50 adult patients of both genders, aged above 45 years, and diagnosed with diabetes mellitus, seen in our institution in 2006 and 2007, and compared them to the charts of 50 metabolic disease-free individuals.this study found statistically significant worse audiometric thresholds among patients with diabetes mellitus when compared to patients in the control group.the results identified in this study point to a correlation between hearing loss and diabetes mellitus. This possibility should therefore be further investigated by health care workers providing care to patients with diabetes mellitus, in the form of closer follow-up on the auditory health of this patient group. Diabetes mellitus (DM) is a genetically inherited disease in which glucose serum levels are abnormally high due to relative or absolute insulin deficiency. Its clinical manifestation is characterized by metabolic disorders, and vascular and neuropathic complications. DM is yet an incurable disease and its management should be focused on preventing chronic complicationsDiffuse thickening of basal membranes, also observed in the vascular endothelium, is one of the most prevalent morphologic findings in DM, referred to as diabetic microangiopathy. Such thickening is more evident on skin capillaries of skeletal muscles, retina, kidney glomeruli, and renal medulla. The pathogenesis of this morphologic disorder is yet unclear, but it is directly related to hyperglycemia. There also are morphologic disorders related to the involvement of lower extremity motor and sensorial nerves, characterized by injuries to Schwann cells, myelinic degeneration, and axonal damage. The cause of this neuropathy is still controversial, but it may be related to diffuse microangiopathy and the consequent malnourishment of peripheral nerves. Arteriosclerosis, frequently seen in conjunction with DM, may also contribute to the onset of neuropathies, as it interferes with the nutrient transfer rateAngiopathy may occur both directly - interfering with cochlear blood supply and reducing nutrient transportation as a result of capillary wall thickening - and indirectly - reducing flow in a narrowed vasculature or, yet, causing secondary degeneration of the eighth cranial nerveStudies carried out on rats found thickened walls in the vessels of the modiolus, increased basal membrane thickness in the stria vascularis capillaries and increased loss of outer hair cells (this last feature was found in diabetic rats exposed to noise). These studies were disputed, as drugs were given to the rats to induce DM, thus not reflecting the actual physiological and genetic mechanism that leads to the onset of DM in human beingsPathology tests carried out in temporal bones of diabetic individuals of various age ranges have shown that the changes in stria vascularis capillary wall thickness - they were 10 to 20 times thicker than usual - were similar to those found in atherosclerosis, although they were more pronounced and limited to the stria vascularis. The authors have also observed other forms of degeneration, but they were also seen in temporal bones of non-diabetic individuals of the same age ranges. They also reported that these alterations are typically found in DM patients, but that they are not DM-specific, observing that atherosclerosis is reduced peripherally, whereas diabetic angiopathy increases in intensity in small vesselsAnother study performed on temporal bones detected thickened stria vascularis and modiolus capillary thickeningCapillary wall thickening and the consequently reduced vessel lumen will more intensely affect the internal auditory arteryOther pathology test-based studies done in humans described spiral ganglion neuron atrophy and eighth cranial nerve demyelination in diabetic individuals, indicating that demyelination is also an early injury to extremity peripheral nerves in DM and that myelin metabolic disorders may be relevant in the pathogenesis of diabetic neuropathy. The following findings were observed under optic microscopy: auditory nerve demyelination by degeneration of the myelin sheath, with minor axonal alterations and perineurium fibrosis; severe spiral ganglion atrophy with cell loss in the cochlear basal and middle turns, accompanied by decreased number of nerve fibers in the spiral lamina. Other findings are: reduced number of ganglion cells in the ventral and dorsal cochlear nuclei, small loss of ganglion cells in the superior olivary complex, inferior colliculus and medial geniculate body. No DM-specific alteration was found in the auditory centers of either temporal lobesSome authors believe that angiopathy is the primary injury connected to hearing loss, while others credit such role to neuropathy, arguing that vessel wall thickening is quite unspecific and also found in other diseases. Human audiology studies indicate that the thresholds of patients with peripheral neuropathy were always worse than control group individuals in any frequencyHearing loss may also have its origins in congenital or acquired genetic mutations affecting nuclear and mitochondrial genes in both syndromic and non-syndromic manifestations8. Mitochondrial DNA mutations are transmitted through maternal genes, but spontaneous mutations may also occur. Mitochondrial disorders may lead to cell deathIn a study done on a particular genogram in which nine children of mothers with DM also developed the disease, coinciding hearing loss of varied severity was observed in each diabetic individual. The authors also observed that in the third generation of this family diabetes and hearing loss were present only in the offspring of affected mothers. Such finding strongly suggested that inheritance is exclusively maternal, a trait found in diseases associated with mitochondrial DNA mutationOther studies reported that DM may be part of a genetically inherited syndrome in which diabetes mellitus is merely one of the syndrome's symptoms: Wolfram syndrome, also called DIDMOAD Some authors have mentioned DM as a possible cause for sudden hearing loss. In spite of other causing factors such as atherosclerosis and viral infection, DM is seen as the main cause of deafness due to diabetic microangiopathyMany papers have tried to identify the relationship between hearing loss and DM. Such studies are justified as controversy still looms this topic, and given that while many diabetic patients develop hearing loss, many do not. Another fact is that most of the time DM will involve older patients, thus making it even harder to connect hearing loss to DM due to presbycusis.A study compared 51 diabetic patients aged between 8 and 21 years against a control group of 13 patients and found no statistically significant differences between their audiological test resultsHowever, contradictory results were found in other studies on the same topic. Another study looked at 30 diabetic patients and compared them to 30 healthy individuals, all of which aged 50 or more, only to find higher prevalence rates of high frequency hearing loss among diabetic patients. No connections were however established between hearing loss and disease durationOther studies suggest that there could be injuries in various points of the auditory pathways, possibly due to neural defects or cochlear injury. Results indicate that the hearing loss patterns found in DM patients do not comply with what is observed in senility-related hearing loss in terms of frequency distributionAnother study found high-frequency sensorineural hearing loss in 37.5% in the patient population. However, these results were not compared against a control groupThis paper aims to describe the audiological characteristics of diabetes mellitus patients and compare these findings against to the test results of DM-free individuals of similar age groups.This study was carried out in the Clinical Audiology Division of a health care center located in the countryside of the State of São Paulo, Brazil. The study was approved by the Research Ethics Committee of our institution under permit 1494/2006.The medical charts of 50 diabetes mellitus patients of both genders  aged between 45 and 83 years (mean 67.56 years) sent for audiological examination in the years of 2006 and 2007 were examinedWe collected information on the patients' audiological history, identification data and hearing health history; tone threshold audiometry, to assess tone thresholds (air and bone conduction)Audiometry and logoaudiometry tests were performed in a soundproof booth, with audiometer GSI 61 Grason - Stadler. Acoustic impedance test measurements were performed with a GSI 38 Grason - Stadler device.The data sets were analyzed in terms of audiometric findings: hearing loss levelTympanometry results were classified as proposed by JergerThe main results obtained from audiological research in the group of DM patients were the following: dysacusis (74%), high blood pressure (72%), tinnitus (70%), recruitment (46%), aural fullness (44%), otalgia (36%), dizziness (32%), autophony (30%), vertigo (30%), pruritus (24%), and oozing (4%). The main findings gathered from the control group were: dysacusis (50%), tinnitus (46%), recruitment (30%), aural fullness (24%), vertigo (22%), autophony (14%), otalgia (14%), dizziness (14%), and oozing (4%).No statistically significant differences were found between the age mean values and standard deviations for the case (65.50±8.48) and control 67.56±9.55) groups (Anova p=0.256 / a=0.05).In the case group, acoustic reflex was present in 72% and absent in 28% of the patients. Acoustic reflex was present in 41% and absent in 59% of the control group subjects.Tone audiometry tests identified the following types of hearing loss among case group members: sensorineural (38%), mixed (24%), and conductive (1%); 37% of the audiograms in this group were considered as normal. Control group members had the following types of hearing loss: sensorineural (26%), mixed (4%), and conductive (2%); 68% of the audiograms in this group were deemed normal.In order to facilitate analysis, the results for impedance tests and hearing loss levels are presented in the form of tables considering each analyzed variable.Tone audiometry test results were significantly worse among diabetic patients when compared to control group individuals from the statistical standpoint, thus eliciting a correlation between DM and hearing loss in our group of subjectsAccording to JergerA-curves were found in 80% of the DM group members and in 88% of the control group subjects, as they can be related to normal hearing and sensorineural hearing loss. This finding matches the main type of hearing loss diagnosed in our study . The number of B, Ad, and As-curves in the DM group was higher when compared to the control group, as more mixed and conductive hearing loss cases were also seen in the case group.Acoustic reflex is an involuntary contraction of the stapedius muscle in response to sound stimuli. Normal threshold values range between 70 and 100 dB, and are situated usually 85 dB above the hearing thresholdThe widespread presence of high blood pressure in this population points to angiopathy as one of the main etiologies, as described by RobbinsNo statistically significant differences were found in the mean ages of the DM and control group members, as described by FrisinaThe data gathered from the audiological interview indicated that the main complaint from DM group members was dysacusis, followed by high blood pressure, and tinnitus. FukuiA paper published recently showed similar findings, indicating that DM is associated with hearing loss in middle-aged individuals. This paper is the only that seems to present such association at a larger scaleThe findings and results gathered in this study point to the existence of a relationship between hearing loss and diabetes mellitus. Therefore, the auditory health of DM patients is to be more carefully and thoroughly followed up by health care workers dealing with this condition.To the São Paulo State Research Support Foundation (FAPESP - Process # 2006/04810-3) for the grant given to this research project.


The Superficial Siderosis (SS) of the Central Nervous System (CNS) is a rare clinical entity. It was first described in 1908, by Hamill, and there are up to 200 cases described so farIt is secondary to chronic and recurrent hemorrhage in the subarachnoid spaceThere are two types of SS: idiopathic and secondary. The cause for hemorrhage is detected in almost half of the cases, and the most frequent causes are: highly vascularized cord tumors, CNS vascular malformations, surgery in the posterior fossa, head injury or prior intradural surgeryThe classic clinical triad is bilateral sensorineural hearing loss, progressive cerebellum ataxia and pyramidal tract involvementAge ranges between 14 and 77 years, becoming evident around 50 years in average, with the pre-clinical phase identification about 10 years beforeA 54-year-old woman with clinical signs and symptoms of bilateral sensorineural hearing loss of unknown origin, with 15 years of evolution, followed by an ENT, with pure tone audiogram showing mild sensorineural hearing loss (SNHL) in the right ear and severe SNHL in her left ear, associated with changes in gait and balance disorders worsening for 10 years and, recently, memory loss.Upon the objective exam, she had had disorders to her recent memory. The cranial nerves were normal, except for a reduction in hearing acuity and nystagmus in the extreme right gaze. Her motor exam showed bilateral ataxia and cerebellar dysarthria. She had live bone-tendon reflexes on her lower limbs, with Babinsky being positive in both sides.We did the following diagnostic complementary tests: pure tone audiometry - which showed moderate sensorineural hearing loss on her right ear and profound SNHL on the left; brainstem auditory evoked potentials which were inconclusive because of numerous artifacts; analytical study with high ferritin and lumbar punction with xanthochromic CSF. MRI-CE  showed (Thus, we diagnosed idiopathic CNS SS, and the patient is being observed, having been medicated with vitamin E.When the ENT has a patient with progressive, bilateral sensorineural hearing loss, especially a young adult, the differential diagnosis must include various clinical entities, namely: neurosyphilis, multiple sclerosis, autoimmune causes and CNS SSThe eight cranial nerve, is the one with the longest glial component, being particularly vulnerable to hemosiderin depositsThe CSF is altered in about 75% of the cases, being xanthochromic or hemorrhagic, with high levels of protein iron and ferritin, and with siderophagesCNS MRI shows pathognomonic signs which may be detected in the pre-symptomatic phase of the disease.Treatment, in cases of secondary SS, is geared towards the identifiable cause of hemorrhage. In idiopathic SS, treatment is symptomatic only, including Paracetamol and PiracetamThe slow and dragging clinical course of the disease, its rarity and evolution without alarming signs, result in late diagnosis, at which point the CNS lesions are irreversible; therefore, a high level of suspicion is required.


Background: Gestational SARS-CoV-2 infection can impact maternal and neonatal health. The virus has also been reported to cause newborn sensorineural hearing loss, but its consequences for the auditory system are not fully understood. Objective: The aim of this study was to evaluate the impact of maternal SARS-CoV-2 infection during pregnancy on newborn’ hearing function during the first year of life. Methods: An observational study was conducted from 1 November 2020 to 30 November 2021 at University Modena Hospital. All newborns whose mother had been infected by SARS-CoV-2 during pregnancy were enrolled and underwent audiological evaluation at birth and at 1 year of age. Results: A total of 119 neonates were born from mothers infected by SARS-CoV-2 during pregnancy. At birth, five newborns (4.2%) presented an increased threshold of ABR (Auditory Brainstem Evoked Response), but the results were confirmed only in 1.6% of cases, when repeated 1 month later, while the ABR thresholds in all other children returned to normal limits. At the 1-year follow-up, no cases of moderate or severe hearing loss were observed, while concomitant disorders of the middle ear were frequently observed. Conclusions: Maternal SARS-CoV-2 infection, regardless of the trimester in which it was contracted, appears not to induce moderate or severe hearing loss in infants. It is important to clarify the possible effect of the virus on late-onset hearing loss and future research is needed. Since the Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) outbreak spread worldwide, the international scientific community has been particularly concerned about vulnerable populations, such as pregnant women and newborns. SARS-CoV-2 has been shown to be more than a respiratory viral infection, with effects being observed in many other organs and systems . VerticaAs a consequence, the possibility of onset of hearing impairment in newborns, after the mother’s viral exposure during pregnancy has been considered. This topic has been targeted by a few studies. In one of the first papers, neonates born to COVID-19-positive mothers did not seem to have an increased risk of hearing loss ; a few aMost of these authors concluded that more large-scale, multicenter studies of pregnant women were needed and more information concerning long-term follow-up of auditory function in newborns was necessary. Another aspect that is not clear is whether the consequences may be different depending on the trimester of pregnancy in which maternal SARS-CoV-2 infection appears ,20.In our region, a law  has intrAn audiological surveillance program, as recommended in , has beeThe aim of this study was to evaluate the impact of maternal SARS-CoV-2 infection during pregnancy on a newborn’s hearing function during the first year of life.An observational study was conducted from 1 November 2020 to 30 November 2021 at University Modena Hospital when, according to general protocols, all women that accessed the hospital to give birth underwent a nasopharyngeal swab to exclude SARS-CoV-2 infection.All newborns whose mothers had been infected by SARS-CoV-2 during pregnancy were enrolled. Women were considered infected in the case of SARS-CoV-2 positivity confirmed by reverse transcription–polymerase chain reaction (RT-PCR) on nasopharyngeal swabs. Newborns whose mothers had been infected by SARS-CoV-2 up to 14 days before the delivery were also tested with an RT-PCR test on nasopharyngeal swabs. Neonatal positivity and related symptoms were eventually acquired.Newborns with risk factors for hearing loss such as syndromic features , TORCH infections, meningitis, encephalitis, familiar history of hearing loss, aminoglycoside or other ototoxic drugs administration for more than 5 days, hyperbilirubinemia treated with exchange transfusion, neonatal intensive care unit (NICU) admission for more than 5 days, birth weight <1500 g and gestational age <28 weeks, were excluded .Clinical data concerning the general course of pregnancy, age at delivery, trimester of SARS-CoV-2 infection and relative phenotype (asymptomatic vs. symptomatic), weeks of gestational age at birth, birth weight, gender, Apgar score at 1′ and 5′ min, presence of peripartum complications and acute respiratory distress were collected. ® Medical Incorporated, Taastrup, Denmark) was used. As usual, results were binary for each ear, “pass” in the case of the presence of an OAE response in both ears, and “refer” in the case of a repeated unclear unilateral or bilateral response.The study protocol was scheduled within two time-points: the first step at birth in the Neonatal Department and the second step within 3 months-old in the Audiology Department. All neonates underwent NHS by means of otoacoustic emissions (OAE) at birth . For thi® Synergy software. Acoustic wide range click stimuli of 21 pps were applied by headphones. Action potentials were detected using vertex-mastoid ipsilateral derivations. At least two runs were obtained at any stimulus intensity and compared to each other, in order to assess waveform repeatability. The exam was conducted presenting medium intensity stimuli, then decreasing gradually until the V wave peak threshold was obtained; lastly, a high intensity stimulus was presented to detect wave’ latencies. With regards to ABR results, the following parameters were evaluated: identification of I, III and V wave peaks at different stimulus intensity and their replicability; identification of V wave threshold expressed in dB nHL; measurements of peak latencies of I, III and V waves expressed in ms; interaural difference of V wave latency (IT-5) expressed in ms.Then, within the third month of life, every newborn was evaluated in the Audiology Department, where otoscopy, ABR and impedance tests were performed bilaterally. ABR was recorded during spontaneous sleep, using MedelecThresholds of V wave identification ≤30 dB nHL, without pathological delay of latency, were considered indicative of normal results.® Medical Incorporated, Denmark) at 1000 Hz probe tone frequency was used to test acoustic immittance. Tympanograms were classified according to the Jerger classification and acoustic reflex (AR) measures were taken using the same instrument [An acoustic immittance test was conducted in order to rule-out potential overestimations of the auditory threshold caused by middle or external ear dysfunctions. A Madsen Zodiac device  using a two-channel diagnostic audiometer  and an immittance test.The severity of sensorineural hearing loss was defined according to the WHO classification: mild (≥26 to <40 dB), moderate (≥41 to <55 dB), moderate-severe (≥56 to <70 dB), severe (≥71 to <90 dB) and profound (>90 dB) .® database. A descriptive analysis of variables was performed; quantitative and qualitative variables were expressed as means with standard deviations (SD) and rates. IBM SPSS Statistics® software was used for analysis and graphical presentations. A chi-square test for qualitative variables and a one-way ANOVA for quantitative variables were performed. The comparison between the mean V wave threshold of the sample and the value considered normal was performed descriptively, taking into account the number of ears in which a value considered pathological was observed.All data were collected in a Microsoft Excelp-value < 0.01 was considered statistically significant.The casuistry was divided into three groups according to the trimester of maternal SARS-CoV-2. Due to the small size of each group, a bootstrapping resampling procedure for 10,000 sub-samples with Bonferroni’s post hoc analysis was applied. A In the period from 1 November 2020 to 30 November 2021, a total of 3150 neonates were born; 134 (4.25%) were born from mothers infected by SARS-CoV-2 during pregnancy, among which, 15 cases were excluded due to the presence of other risk factors for hearing loss. Thus, a final sample of 119 (3.78%) newborns was identified. Epidemiological data regarding mothers and newborns are reported in All newborns obtained a “bilateral pass” result for OAEs at newborn hearing screening. The mean gestation age at the date of ABR was 50.06 (±7.75) weeks. From the sample of 119 newborns, a total of 238 ears were considered. In 232 cases (97.5%), the V wave threshold was ≤30 dB nHL, whereas in six cases (2.5%), it was considered pathological . The meaA total of five newborns (4.2%) presented a threshold elevated in ABR , globallThe mean V wave threshold was 25.71 (±6.46) dB nHL in neonates born from mother infected by SARS-CoV-2 in the first trimester, whereas it was slightly higher in newborns whose mother was infected in the other two trimesters. Specifically, it was 28.50 (±4.58) dB nHL and 28.81 (±3.84) dB nHL for the second and the third trimester respectively .p = 0.040). Bonferroni’s post-hoc analysis confirmed a non significant difference in fact, p = 0.077 and 0.034 for first-trimester infection versus second-and third-trimester maternal infection, respectively. No difference was observed between the second and the third trimester maternal infection’ subgroups (p = 1.000).According to the one-way ANOVA test, the differences among these subgroups were not significant (p = 0.509) and the weight at birth , as shown in The V wave threshold was not significantly different regarding the gestational age . A frequent occurrence of middle ear disorders was observed  underwent a follow-up examination at 1 year of age . The audobserved . The results of the 1-year follow-up, considering only 30.3% of the sample, reported the frequent presence of concurrent disorders of the outer and middle ear. According to free-field VRA a threshold higher than 32.5 dB was never observed. Only one child with an altered threshold completed the follow-up at 1 year of life, with a good performance.The SARS-CoV-2 pandemic has caused millions of cases worldwide, but it has been evidenced that children infected remain mostly asymptomatic or with mild symptoms. ,26. VariWhether positive pregnant women will affect the hearing of their newborns remains unclear, but it is supposed that the auditory system of the newborn could be affected through intrauterine hypoxia and vertical transmission .Although it seems reasonable to hypothesize that the fetal inner ear cells are damaged after the mother’s viral exposure during pregnancy, with subsequent hearing impairment, the results of our study did not strongly confirm this assumption. In fact, even if around 4% of our cases demonstrated a moderate change in the threshold of ABR, in most cases, it was retrieved, and early clinical pictures characterized by moderate or severe hearing loss in those newborns, were not evidenced.In a recent review it was reported that SARS-CoV-2 might have a greater influence on hearing loss in newborns during the second and third trimesters of pregnancy . In our On the other hand, according to serology studies, the reported number of laboratory-confirmed cases of SARS-CoV-2 infection in children was likely underestimated given the high proportion of mild and asymptomatic cases in which testing may not have been performed ,32. MoreAnother aspect that makes it difficult to clarify the effects of the virus on newborn’ hearing is that early audiological diagnosis is not always reliable and not all children identified as suffering from sensorineural hearing loss will have permanently impaired hearing thresholds . On one At the moment, it is not possible to have studies showing data concerning prolonged follow-up since the pandemic started in 2020. In this way, the monitoring period for newborns with hearing loss should be extended to detect possible late-onset hearing impairments caused by SARS-CoV-2 infection during pregnancy . It mustIn our sample, all children that came for follow-up at 1 year of age demonstrated mild/moderate conductive hearing loss. Unfortunately, only 30 % of the children completed 1 year of follow-up. This could be due to the effect of the pandemic on the national health system as all services, including essential ones, have been impacted , as wellThis study had some other limitations. Data concerning the mothers’ vaccination status were lacking, and the exact serotype of virus responsible for maternal infection was not known. A relatively small number of children completed the audiological surveillance pathway.The strengths of the study are the thorough statistical analysis, the exclusion of newborns with known other risk factors for hearing loss and the 1-year length of follow-up of children. Lastly, this study was mono-centric since only neonates born at the hospital were selected, greatly reducing selective reporting bias.Although it seems reasonable to hypothesize that the fetal inner ear cells are damaged after the mother’s SARS-CoV-2 exposure during pregnancy, with subsequent hearing impairment, the results of our study did not strongly confirm this assumption. No cases of moderate/severe bilateral hearing loss were detected during the 1-year follow-up, while the presence of findings consistent with middle ear dysfunction was concurrently observed. It is important to clarify the possible effect of the virus on late-onset hearing loss and future research is needed.


The soft palate laceration was present in all cases. Surgical therapy was successful in all cases with follow-up. The most common techniques used for the closure of the hard palate defect were bilateral pedicle flaps with lateral releasing incisions , direct apposition of the oral mucosa , bilateral pedicle flaps with lateral releasing incisions and interquadrant splinting , and unilateral pedicle flap with one lateral releasing incision . A tension-free closure by direct apposition of the edges was possible for the soft palate laceration. No oronasal fistulae were identified at follow-up. The only complication was malocclusion. The interquadrant splinting was most often used for PDE extending to the rostral portion of the hard palate (p < 0.05). The cats that suffered postoperative malocclusion were significantly more likely to have sustained temporomandibular joint injury, underwent CT scan, or had a feeding tube placed before discharge. The results of this retrospective study indicate that the early treatment (within 5 days) of the acquired longitudinal defects in the midline of the hard and soft palates is highly successful.Acquired midline palate defects (PDE) affect the hard palate and/or soft palate, and result from trauma, commonly falling from a height or being hit by a motor vehicle. Additional life-threating injuries and costs associated with the treatment may delay the surgical treatment. This retrospective study describes signalment, cause, and extent of the PDE, and treatment in 25 cats. In addition, the outcome of the surgical repair is described in 19 (76%) cats. All defects were repaired within 5 days of the injury. Twenty (80%) cats were 4 years of age or younger. The most common rostral extent of the PDE was to the level of the third premolar tooth ( The acquired midline palate defects (PDE) result from trauma and are frequently diagnosed after falling from a height or road traffic accidents –3. The hEpistaxis and oral bleeding with or without difficulty breathing, pain, facial asymmetry, and malocclusion can be associated with acute midline PDE. When such defects occur after a traumatic event, their assessment and repair may be delayed in favor of first addressing other life-threatening injuries , 5. The Conservative management of a midline PDE has been historically recommended for narrow linear defects . Other aThe main objective of this study was to describe the presentation, location, extent, treatment, outcome, and complications of acquired midline PDE in client-owned cats. A second objective was to make specific recommendations for the surgical management of such PDE.Medical records of client-owned cats with maxillofacial trauma presented to the dentistry and oral surgery service of a specialty teaching hospital from 2000 to 2021 were reviewed. Cats were included in this study if an acquired midline PDE causing oronasal communication was diagnosed by means of an oral examination and surgical repair was attempted. Patients were excluded if the oronasal communication was not located in the midline of the palate, the defect affected only the incisive bones , or the patient presented with an oronasal fistula . Information about signalment and body weight of the cats, cause and extent of the PDE, diagnostic imaging findings by means of dental radiography and/or computed tomography (CT), treatment, and outcome  were retrieved from the medical records.The extent of the PDE was classified from the most rostrally affected aspect of the hard palate to the transition of the hard and soft palate (THSP) or to the soft palate . The most rostral location was leveled with the teeth. The categorization of the defects according to the width (in mm) was attempted, but such data were only available for a few cats; therefore, this information was not assessed. The anatomical functional regions that were fractured were identified in patients with CT according to the classification described in a previous study .The time from trauma to surgical repair was assessed. Treatment was categorized as (a) direct medial mucoperiosteal apposition, (b) medial mucoperiosteal apposition with only one pedicle flap , (c) medial mucoperiosteal apposition with bilateral pedicle flaps , and (d) combinations of these techniques with the addition of the interquadrant splinting to reduce and stabilize the median palatine suture separation. The mucoperiosteum between the defect and the lateral releasing incisions was elevated as deemed necessary by the clinician; thus, allowing a tension-free closure in the midline of the palate. All techniques included a gentle debridement of the soft tissue edges prior to the closure and flushing of the nasal cavity to remove blood clots and debris before and after the PDE repair. The closure of the PDE was classified as one-layer , two-layer  or a three-layer closure . The suture patterns and suture materials were also noted. The lateral releasing incisions had been left to heal by second intention.The outcome was defined as good at the follow-up visits if there was no evidence of oronasal fistula after the healing period upon oral examination of the conscious patient. The evaluation was performed under anesthesia only if the patient needed further treatment . Healing was defined as continuity of the oral mucosa with no evidence of linear inflammation (redness). The inflammation attributed to the suture material in the absence of a midline defect was considered as “healed” at the time of the follow-up evaluation. The lateral defects were considered healed when the bed of granulation tissue was completely covered by epithelium, with no exposed bone. For each cat, only one healing time was recorded, corresponding to the time of healing of the PDE and any lateral releasing incisions made at the time of the repair. Information about the potential postoperative complications  also was retrieved from the medical records.p < 0.05 as the criterion for statistical significance. The descriptive analyses included the computation of medians and range. The categorical variables were reported as frequency counts and percentages. The Spearman rank correlation was conducted to identify significant association between the independent variables with the extent of the PDE and the complications as outcomes considered. In addition, the analysis was conducted to identify whether the use of a CT was correlated with any independent variables. The independent variables studied were gender, breed, age, weight, cause of trauma, time from trauma to repair in days, presence of soft tissue trauma in the head, concurrent temporomandibular (TMJ) injury, mandibular fracture (TMJ excluded), symphyseal separation, orbital or ocular injury , presence of head injury (discussed further in the next paragraph), lesions of other body parts excluding the head, treatment performed, placement of a feeding tube, and diagnostic imaging by means of a CT. To further explore the association between the extent of the PDE and treatment, the variable treatment was split into the following two new variables: Use of interquadrant splinting between the maxillary canine teeth and the type of soft tissue closure .All analyses were conducted with Stata 17MP, StataCorp, College Station TX, with two-sided tests of hypotheses and a For the statistical purposes, head injury was categorized as (1) no injury , (2) fracture of the mandible (excluding TMJ), (3) ocular or orbital injury, (4) concurrent fracture of the mandible and injury of the TMJ, (5) concurrent injury of the orbit/eye and TMJ, (6) concurrent injury of the orbit/eye and fracture of the mandible (excluding TMJ), and (7) concurrent injury of the orbit/eye, fracture of the mandible and injury of the TMJ. For the TMJ, the term injury was chosen instead of fracture because one case had a subluxation of the TMJ. Body injury was grouped as follows: (1) no injury (excluding the head), (2) thoracic injury, (3) orthopedic injury (limbs and spine), and (4) concurrent orthopedic and thoracic injuries. For all pairwise association the p statistic and the spearman rho coefficient were reported.Twenty-five cats were included in the study. The median age was 21 months . Twenty cats (80%) were 4 years of age or younger. The median body weight was 5 kg . Nine cats (36%) were female, of which one was intact, and 16 cats (64%) were male, of which three were intact. There were 23 domestic shorthair cats, one domestic longhair cat, and one Turkish Angora cat.n = 17), animal altercation (n = 2), and hit by car (n = 2). The cause was unknown in four cats, although it was suspected to be a fall from a height in one of them and hit by car in another (based on other concurrent injuries). All cats had other concurrent dental, oral, and maxillofacial injuries. Ten cats (40%) had orbital or ocular injury. Thirteen cats (52%) had injuries in other body parts (excluding the head)  . A full n = 8; 32%) or the incisor teeth  caudally into the soft palate. Five cats (20%) had a PDE extending from the level of the mesial aspect of the maxillary fourth premolar teeth to the soft palate. The least common extension was from the level of the maxillary canine  or the second premolar teeth  to the soft palate and from the maxillary fourth premolar teeth to the THSP . The medical record did not provide accurate description of the rostral extent of the PDE in one cat, but both the hard and soft palate (30-mm long) were affected  . The sepThe treatment was performed within 5 days following the traumatic injury, preceded by general stabilization of the 25 cats. Twenty-two cats received perioperative antibiotic therapy . An esophagostomy feeding tube had been placed in six cats due to the extent of maxillofacial trauma, but not necessarily because of the presence of the PDE.n = 8; 32%)  , and medial mucoperiosteal apposition with a unilateral pedicle flap and lateral releasing incision   (n = 3) were included in the interquadrant splinting if they had sufficient coronal length. The vital pulp therapy at the time of PDE repair and subsequent root canal treatment upon splint removal was performed in one tooth. The other two teeth had no initial treatment and were extracted at the time of splint removal. The laceration in the soft palate was closed by direct apposition of the soft tissue edges after a careful undermining of the oral mucosa to obtain a tension-free closure in all cats.The most common techniques used to repair the PDE were medial mucoperiosteal apposition with bilateral pedicle flaps and lateral releasing incisions  , direct  7; 28%) , medial  4; 16%) . The PDE= 1; 4%) ; Table 2A one-layer closure was used for the PDE repair in 15 cats (60%) . The PDE of seven cats (28%) was repaired with a two-layer closure; a horizontal mattress pattern for the connective tissue of the hard palate and the nasal mucosal–muscular  layer and a simple interrupted pattern for the oral mucosa of the soft palate. The PDE of one cat (4%) was repaired with one layer of sutures in the hard palate and three layers of sutures in the soft palate . There was insufficient information in the medical records about the suture patterns used in two cats (8%). The PDE was closed with 4–0 or 5–0 poliglecaprone 25 in 19 cats (76%) or polydioxanone in four cats (16%). There was an insufficient information in the medical records about the type or size of the absorbable monofilament suture material used in two cats (8%).n = 19; 76%) or provided nutrition through the esophagostomy feeding tube . The pain medication was tailored to each cat depending on the additional injuries, and all cats wore an E-collar to prevent grooming for at least 2 weeks.The patients were discharged with instructions to be fed a soft diet for 2 weeks  with no complications associated with the healing of the PDE. None of the patients was diagnosed with an oronasal fistula at any of the recheck examinations. Malocclusion was noted in two cats (10.5%). One of them had palatoversion of the maxillary premolar/molar teeth due to displacement of multiple bone fragments of the midface causing alveolar mucosal trauma at the ipsilateral mandible. Extraction of the malocclusion maxillary teeth resolved the soft tissue trauma. The malocclusion in the second cat was mild, and further treatment was not necessary.The information about healing rechecks was available for 19 of the 25 cats (76%). A good outcome was observed in all cases that had follow-up visits . A bed of granulation tissue at the lateral releasing incisions was visible in some cats as early as 10 days after surgery.p < 0.05) with the following seven variables: Time to repair, presence of symphyseal separation, head injury, body injury, orthopedic injury, thoracic injury, and extent of the PDE. For the outcome of complications, only the variables TMJ injury, use of a feeding tube, and having a CT performed were significantly associated with the outcome. Having a CT performed showed association with the presence of symphyseal separation, orbital/ocular, TMJ and head injury, use of a feeding tube, and time to repair . Repairing the PDE soon after stabilization of the patient has a good outcome with a low frequency of complications. The data suggest that the treatment chosen for each case was not only dependent on the extent of the PDE but also on the stability and separation of the bone fragments of the palate and the ability to achieve a tension-free closure. The defects extending rostrally in the hard palate benefit from stabilization of the midface with an interquadrant splint.An oral examination is sufficient to diagnose the presence of a PDE. If the separation of the tissue edges and the oronasal communication are not readily evident, a PDE can be verified by gentle evaluation of the wound with a periodontal probe under sedation or general anesthesia. An abnormal interdental space between the maxillary first incisors and instability of the midface can be easily evaluated by inspection and palpation. A complete dental and maxillofacial assessment must be performed to establish a plan of treatment for the other maxillofacial injuries. In the previous studies, ~70% of dogs and cats with maxillofacial trauma also sustained dental trauma , 2, 9. SMany of the cats of this study sustained pulmonary contusion or other lesions that involved intensive care, additional diagnostic tests, multiple surgical procedures, or more involved management. A head CT might not have been prioritized due to reasonably good health status of the patient, minimal clinical signs related to the fractures of the midface  and additional cost. The recent imaging studies have shown that midface fractures tend to occur simultaneously with a median of eight regions of the face affected at the same time , 10. TheA conservative treatment with antibiotic therapy and feeding a soft diet for at least a month was recommended in a study with 22 high-rise syndrome cats presenting with an acquired midline PDE, stating that all cases healed by second intention without signs of oronasal fistula. However, the authors also mentioned that they had previously seen chronic oronasal fistulae secondary to a PDE that were not surgically repaired . The epiA study conducted in rats showed that when the palatal mucosa incision is left to heal by second intention, an oronasal fistula would result in up to 85% of the animals when the distance between the bony edges was 2 mm and in up to 33% of the animals when the bony defect was as small as 1 mm. During healing by second intention, the nasal mucosa may grow into the space of the bony defect, eventually contacting and fusing with the palatal mucosa and forming an oronasal fistula . That stThe surgical methods for the PDE repair after wound debridement include direct medial mucoperiostal apposition of the soft tissue edges, medial mucoperiosteal apposition with one or two pedicle flaps  with or without interquadrant splinting or a tension band with a pin and wire , 3, 5, 8The surgical technique should adhere to the following general principles of palate surgery: (1) Avoid tension on the suture line by creating flaps that are larger than the defect; (2) Maintain the vascular supply of the flaps by preserving the major palatine artery, which in cats arises from the major palatine foramen under the palatal mucosa at the level of the mesial aspect of the maxillary fourth premolar tooth halfway between the dental arch and median plane; (3) Suture only fresh edges of soft tissue together; (4) Avoid electrosurgery or cauterization when controlling hemostasis; and (5) handle the tissues gently to minimize trauma. An attempt should be made to keep the suture lines over connective tissue and bone instead of over a void .vs. no healing) between the use of a simpler technique  vs. a more complicated one  because each technique was carefully selected on a case-by-case basis, trying to follow the general principles of palate surgery. The separation of the median palatine suture with more severe displacement and instability likely warranted a more aggressive treatment protocol .Successful outcome of a PDE surgery can be expected if the trauma was acute, the defect in the midline, the tissue edges nicely vascularized, the major palatine arteries intact, and surgical repair performed without delay. The techniques used in this retrospective study were based on the judgment of the primary clinician with the intention of avoiding tension in the suture line. There was no significant difference in the outcome  may not reflect the outcome of all cases with acquired midline PDE. Comminuted fractures of the bones of the hard palate may cause wider bone gaps and decreased bone support for the soft tissues used for the closure, increasing the risk of dehiscence or development of secondary lateral PDE if lateral releasing incisions are used for a tension-free closure in the midline. If loose bone fragments are retrieved from the PDE, the addition of a barrier material  between the remaining bone and palatal mucoperiosteum could facilitate successful outcome. Therefore, full characterization with oral examination and head CT and careful surgical planning of each case should be made before attempting the PDE repair with the techniques described in this study.On the statistical analysis, the only treatment-related variable that was associated with the extent of the PDE was interquadrant splinting between the maxillary canine teeth. Interquadrant splinting may reduce and stabilize the separation of the median palatine suture in the more rostral part of the hard palate at the level of the incisor, canine, and second premolar teeth. Therefore, based on the cases evaluated in this study, PDE extending into the rostral half of the hard palate may benefit from interquadrant splinting. However, splinting may not be necessary if the PDE is located in the caudal half of the hard palate  unless the separation of the median palatine suture extends farther rostrally. In addition, the number of lateral releasing incisions is subjectively decided by the primary clinician based on PDE width and the ability to achieve a tension-free closure. There was insufficient information in the medical records to determine the cut-off value of PDE width.The only complication observed was malocclusion, which was associated with concurrent injury of the TMJ. The TMJ injury was treated conservatively. Even a mild displacement of bone fragments can cause malposition of the canine or premolar/molar teeth. This, in addition to the malalignment of the injured midface, could result in malocclusion and difficulty to fully close the mouth. The prevalence of malocclusion may be underestimated if some teeth were extracted at the time of the PDE repair or interquadrant splint removal, eliminating the possible tooth-to-tooth contact or tooth-to-soft tissue contact. Malocclusions secondary to other maxillary and mandibular fractures may be more common, but if they did not cause any obvious clinical signs, they may not have been recorded in the medical record or observed as such by the clinician. The cats sustaining a fall from a height tend to hit the ground with their limbs first, followed by the thorax, and then the head. Several mechanisms have been suggested to explain the resulting maxillofacial injuries . The impIt is possible that more cats in this study could have had complications that were simply not identified because of the lack of medium to long-term follow-up. The presence of complications also was significantly associated with CT performed and feeding tube placed. As suggested before, this may reflect the decision made by the primary clinician to better manage cats with worse injuries diagnosed. The TMJ injury is frequently treated with maxillomandibular fixation, muzzling, sutures through labial buttons, or elastic chains , 6, 15. The average healing time of the PDE in this study was 20 days. However, this number does not reflect the actual time needed for palatal mucoperiosteum to heal, as the healing time assessed was based on recheck examinations at follow-up visits. The cats were typically evaluated by means of an awake oral examination 2 weeks post-surgery or in 4–8 weeks for the removal of the interquadrant splint and further dental treatment. Healing of the oral mucosa has been extensively studied in cleft palate models in dogs, where the midline defect healed quickly within 1 week by first intention after accurate apposition of the soft tissue edges, while the lateral incisions healed in ~ 3–5 weeks by second intention with clot formation followed by granulation tissue, epithelialization, and scar formation –18. MucoIn acute midline PDE described in this study, there is usually minimal soft tissue loss; thus, the amount of denuded bone, if one or two pedicle flaps are made, is minimal compared to the studies on cleft palate models. Therefore, healing  of the lateral incisions typically occurs within 2 weeks which is consistent with our clinical observations. In experimental studies in dogs, incisions made in the palatal mucosa without elevating the mucosa from the periosteum or without causing bone loss healed by second intention quickly in a similar fashion to first intention, leaving a small scar of 0.5 mm wide , 18. TheThis study has some limitations. The medical records may not have contained all the details we would have liked to include in the study, and the follow-up visits were not standardized. The surgical technique used to repair the PDE was biased due to clinician's experience and preference for one technique or another. Computed tomography imaging was not used in all patients; therefore, some injuries of the midface or the TMJ may have been missed. Small oronasal fistulae may not have been diagnosed because healing by means of probing the surgical site was not assessed under general anesthesia in every patient.This retrospective study showed that treatment of an acquired midline PDE with the techniques reported here was highly successful. Cats with a PDE extending rostrally to the level of the maxillary third premolar tooth may benefit from the addition of an interquadrant splint constructed between the maxillary canines. The maximum width at which the PDE can be left to heal by second intention and the number of releasing incisions required based on PDE width still needs to be determined. Evaluation of complete medical records, with accurate measurements of defects in the mucoperiosteum and bone, of cats with and without primary closure of the PDE may elucidate this information.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Ethical review and approval was not required for the animal study because the retrospective study used information retrieved from medical records. Written informed consent for participation was not obtained from the owners because it was not deemed necessary due to the retrospective nature of the study.AC-G: study concept and design, acquisition of data, analysis and interpretation of data, drafting of manuscript, and revision of the manuscript. DS: analysis and interpretation of data and statistical analysis. AR: study concept and design, acquisition of data, and critical revision of the manuscript for important intellectual content. All authors contributed to the article and approved the submitted version.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.