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Hearing loss (HL) is one of the most frequent disorders in Turner syndrome (TS); HL can be present with a wide spectrum of manifestations and also evolve with age. The aim of this paper is to perform a review of the literature on the prevalence of HL in TS patients also analyzing the possible genetic alterations underlying the auditory features. A review of the literature was performed using PubMed/MEDLINE, EMBASE, and Cochrane Library databases, according to the preferred reporting items for systematic reviews and meta-analyses criteria for scoping reviews (from 2000 to December 2023). A total of 17 articles and 2129 patients with TS have been included; the majority of studies focused on young women/girls, with a mean age range from 2 to 43.6 years. External and middle ear problems, inducing conductive and mixed HL, have been reported to be more frequent in childhood, while sensorineural HL has been described since adolescence. Monosomy 45,X and loss of the X chromosome short arm (p) are the alterations most frequently associated with HL. To date, the pathophysiological mechanisms related to HL in TS are still not fully understood; further studies are necessary to clarify these features and to offer therapies or prevention strategies to avoid the progression of HL in TS subjects.

Background : Hearing loss is a highly prevalent condition in the world population that determines emotional, social, and economic costs. In recent years, it has been definitely recognized that the lack of physiological binaural hearing causes alterations in the localization of sounds and reduced speech recognition in noise and reverberation. This study aims to explore the psycho-social profile of adult workers affected by single-sided deafness (SSD), without other major medical conditions and otological symptoms, through comparison to subjects with normal hearing. Methods : This is a cross-sectional, case-control study. Subjects aged between 24 and 65 years, all currently employed and affected by SSD, were enrolled. They were administered both disease-specific and psychometric tests, such as the Hearing Handicap Inventory for Adults (HHIA), the Profile Questionnaire for Rating Communicative Performance, the Psychological General Well-Being Index (PGWBI), and the Social Functioning Questionnaire (SFQ). Results : A total of 149 subjects (mean age = 49.9; SD ± 8.5) were enrolled in the period 2021-2023; 68 were males (45.6%), and 81 were females (54.4%). The normal hearing group was composed of 95 subjects, and the SSD sample was composed of 54 subjects. The results of our study show that the levels of psychological well-being and social functioning in subjects with SSD are statistically worse than in the group of subjects with normal hearing in most subscales. Conclusions : This study definitely outlined evidence for a significantly worse psychological health status and a poorer social attitude of working adults affected by SSD with respect to their normal-hearing counterparts. Understanding the impact of SSD on patients' work environment suggests a multidisciplinary approach to such patients in order to increase their quality of life through adequate counseling, acceptance, and role modeling.

Background The facial nerve (FN) plays a pivotal role in human life; apart from its sensory and parasympathetic functions, it innervates the facial muscles, and it is therefore involved in non-verbal communication, allowing us to express emotions and reactions. Especially in the case of childhood onset, FN dysfunction can severely affect the quality of life. Methods The aim of this review is to analyze the most recent literature, focusing on the acute onset of peripheral FN palsy among pediatric patients, discussing the different etiologies, prognoses, and management strategies. A total of 882 papers were initially identified, but only 7 met the selection criteria. Therefore, data on 974 children in total were pooled and analyzed. Results According to the findings of this review, FN palsy is idiopathic in most cases, while an infective etiology was identified as the second most common. The main pathogen agents identified were Borrelia Burgdorferi, especially in endemic areas, and Herpesviridae. Respiratory tract infections and/or ear infections were also described. Head trauma or direct injury of the FN accounted for 2% of all cases. Conclusions The overall FN recovery rate is high, even though the etiology remains unknown for most patients. Therapeutic indications are still lacking, especially in the case of non-recovering FN palsy. In our opinion, large, prospective studies are necessary for improving our knowledge of this disorder and establishing evidence-based approaches.

Studies about the effects of SARS-CoV-2 on pregnant women and children born to positive women are controversial with regard to possible inner ear-related damage but most of them do not detect the involvement of this virus in auditory function. However, only a few studies on long-term effects on language development are currently available because of the recent onset of the pandemic. The aim of this study was to investigate the impact of SARS-CoV-2 infection on perceptual and expressive abilities and the emerging development of communication in young children. To this purpose, the MacArthur-Bates Communicative Development Inventory-Words and Gestures form (CDI-WG), was administered to parents. In total, 115 children whose mother was infected by SARS-CoV-2 during pregnancy were enrolled in the study and evaluated at the Audiology Service of the Modena University Hospital. All children underwent Otoacoustic Emissions (OAE) at birth: 114/115 had a "pass" result bilaterally, while 1 case had a unilateral "refer" result. Overall, 110/115 newborns (95.65%) underwent audiological evaluation between 10-18 months of age. In 5/110 patients (3.6%), the Pure Tone Average (PTA) result was equal to 35 dB; one case had a hearing threshold of around 50 dB due to a bilateral effusive otitis media. A notable finding was the percentage of children with tubal dysfunction in both evaluations, within 2 months of age and around 12 months of age. Most children revealed normal hearing. The CDI-WG was completed by 56/115 families. The rate of children below the fifth percentile was 8.9% for sentences understood, 12.5% for words understood, and 5.4% for words produced. Concerning CDI-Gestures, only 2 children (3.6%) were below the fifth percentile. A structured audiological follow-up in association with the evaluation of communication skills of children appears fundamental, particularly in the years of maximum neuroplasticity. Long-term studies are still necessary to evaluate the possible consequences of the pandemic.

Introduction: Congenital deafness is a pathological entity that represents an economical and social burden, affecting up to 0.2% of newborns in Europe. Sensorineural hearing loss (SHL) is caused by a variety of factors, including congenital abnormalities, perinatal infectious diseases and genetic syndromes. The inner ear's vestibular system, nestled alongside the auditory organs, is crucial for balance maintenance. Its close connection with the auditory system means that disturbances in one often coincide with disturbances in the other, highlighting their intertwined functions. With this review we aim to describe objective vestibular tests found in literature and to study their use for diagnosis of vestibular disturbances in patients affected by congenital deafness., Methods: The review is conducted with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) 2020 guidelines. The search string used was: [(congenital deafness) OR (congenital hearing loss) OR (congenital hypoacusia)] AND [(vestibular disorders) OR (vertigo)] . An initial abstract reading selection was made, and a subsequent comprehensive full-text reading. For each article, we identified the type of vestibular test utilized and its corresponding outcome., Results: Out of the initial-papers identified through the search string-articles met the eligibility criteria for further analysis through abstract and full-text reading. After further selection-articles were chosen for detailed examination, focusing on the data of patients., Conclusion: Congenital hearing loss profoundly affects a child's development, especially in language and communication skills, and it is frequently associated with a pathological vestibular system. Early identification allows timely intervention with personalized therapies. In current literature, there is still no gold standard test to identify balance disorders in patients with congenital hearing loss. There is considerable variability on the subject due to the inclusion of diverse patients with various diagnoses, alongside a wide range of available technologies. Managing such conditions necessitates collaboration among healthcare providers, ensuring comprehensive care through prompt diagnosis and personalized treatment plans. Ongoing research aims to further improve screening methods and develop precision medicine approaches tailored to individual needs., Competing Interests: 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., (Copyright © 2024 Genovese, Segato, Liberale, Zampieri, Monzani, Apa, Palma and Nocini.)

Background/Objectives : Mitochondrial transfer RNA mutations are one of the most important causes of hereditary hearing loss in humans. In most cases, its presentation is bilateral and symmetrical; however, there are numerous cases of single-sided presentation or asymmetrical onset described in the literature that may represent a diagnostic challenge. The aim of this review is to present the evidence of auditory asymmetry in mitochondrial diseases, highlighting the possible presence of cases with atypical presentation. Methods : A review of the English literature to date on hearing loss and mitochondrial diseases was performed using PubMed, Scopus, and Google Scholar databases. The literature review was performed using the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines for scoping review. Results : A total of 10 full-text articles were included in this review, comprising 25 patients with single-sided or asymmetrical hearing loss associated with mitochondrial disease. Conclusions : Sensorineural hearing loss due to mitochondrial disease can represent a complex diagnostic challenge in cases of asymmetric or unilateral presentation. It is critical to recognize this clinical variant and to diagnose it in daily clinical practice.

Fibrinogen and homocysteine (HCY) are molecules known to play a role in vascular homeostasis, and their blood levels are often elevated in patients with metabolic syndrome. Recent evidence suggests that sudden sensorineural hearing loss (SSHL) may have a vascular origin. This has led many authors to advocate that fibrinogen, homocysteine, and metabolic syndrome (MetS) may play a direct role in SSHL. The aim of this brief review is to examine the role and influence of these molecules and MetS on the mechanisms of SSHL. Elevated fibrinogen levels have been associated with a worse prognosis in SSHL, possibly due to increased blood viscosity and decreased blood flow. Similarly, HCY has been associated with vascular damage, particularly in hyperhomocysteinemia, although the exact association with SSHL remains controversial. MetS has been demonstrated to function both as a causative factor and as a contributor to poorer recovery in cases of SSHL. However, although some studies suggest a possible role for these biomarkers and MetS in the prognosis and treatment of SSHL, specific therapeutic and preventive strategies based solely on these factors have yet to be developed. Given their potential role in prognosis and treatment and the global epidemic of metabolic syndrome, this issue needs to be analyzed comprehensively. Thus, further quality studies need to be conducted, even though it is difficult to determine the actual impact of MetS on the development of SSHL, as it is a multifactorial disease affecting multiple organs.

This study examines the last 10 years of medical literature on the benefits of cochlear implantation in children who are deaf or hard of hearing (DHH) with additional disabilities. The most recent literature concerning cochlear implants (CIs) in DHH children with additional disabilities was systematically explored through PubMed, Embase, Scopus, PsycINFO, and Web of Science from January 2012 to July 2023. Our two-stage search strategy selected a total of 61 articles concerning CI implantation in children with several forms of additional disabilities: autism spectrum disorder, cerebral palsy, visual impairment, motor disorders, developmental delay, genetic syndromes, and intellectual disability. Overall, many children with additional disabilities benefit from CIs by acquiring greater environmental sound awareness. This, in turn, improves non-verbal communication and adaptive skills, with greater possibilities to relate to others and to be connected with the environment. Instead, despite some improvement, expressive language tends to develop more slowly and to a lesser extent compared to children affected by hearing loss only. Further studies are needed to better appreciate the specificities of each single disability and to personalize interventions, not restricting the analysis to auditory and language skills, but rather applying or developing cross-culturally validated instruments able to reliably assess the developmental trajectory and the quality of life of DHH children with additional disabilities before and after CI.

We recently reported the isolation and characterization of an anti-SARS-CoV-2 antibody, called IgG-A7, that protects transgenic mice expressing the human angiotensin-converting enzyme 2 (hACE-2) from an infection with SARS-CoV-2 Wuhan. We show here that IgG-A7 protected 100% of the transgenic mice infected with Delta (B.1.617.2) and Omicron (B.1.1.529) at doses of 0.5 and 5 mg/kg, respectively. In addition, we studied the pharmacokinetic (PK) profile and toxicology (Tox) of IgG-A7 in CD-1 mice at single doses of 100 and 200 mg/kg. The PK parameters at these high doses were proportional to the doses, with serum half-life of ~10.5 days. IgG-A7 was well tolerated with no signs of toxicity in urine and blood samples, nor in histopathology analyses. Tissue cross-reactivity (TCR) with a panel of mouse and human tissues showed no evidence of IgG-A7 interaction with the tissues of these species, supporting the PK/Tox results and suggesting that, while IgG-A7 has a broad efficacy profile, it is not toxic in humans. Thus, the information generated in the CD-1 mice as a PK/Tox model complemented with the mouse and human TCR, could be of relevance as an alternative to Non-Human Primates (NHPs) in rapidly emerging viral diseases and/or quickly evolving viruses such as SARS-CoV-2.

Objective: Validate the Italian version of the Hearing Handicap Inventory for Elderly - Screening version (HHIE-S-It)., Methods: After translation, psychometric properties and attributes were analysed by administering the HHIE-S-It to 167 elderly outpatients together with the Psychological General Well-Being Index (PGWBI)., Results: The Cronbach's α coefficient was 0.908 for the total score, and 0.832 and 0.816 for its two subscales. Significant test-retest reliability was observed (p < 0.001). Moderate to high correlations were found between HHIE-S-It and pure tone average in the better ear (p < 0.001). The ANOVA test confirmed the significant difference in HHIE-S-It scores across groups according to the degree of hearing loss (p < 0.001). Only very low and low significant correlations were observed between HHIE-S-It and PGWBI. The criterion HHIE-S-It > 11 was observed as the best cut-off with highest sensitivity (86.4%), specificity (72.4%), positive predictive value (52.8%), negative predictive value (93.7%) and likelihood ratios (3.12 and 0.19)., Conclusions: Since the HHIE-S-It presented acceptable psychometric properties, its adoption is justified for both clinical and research purposes. Acceptable diagnostic attributes allow its use as a screening tool for age-related hearing loss., (Copyright © 2023 Società Italiana di Otorinolaringoiatria e Chirurgia Cervico-Facciale, Rome, Italy.)

Background and Objectives: Tinnitus affects millions of adults. Many therapies, including complementary and alternative medicine and tinnitus retraining therapies, have been trialed, but an effective option, particularly for chronic subjective tinnitus (CTS), is still lacking., Materials and Methods: This study investigated the effects of alpha-lipoic acid (600 mg. per day for two months) on two groups of patients using a questionnaire. One group (A) was affected by tinnitus associated with likely cochlear dysfunction and metabolic syndrome, and the other (B) was composed of subjects with acoustic nerve lesions. All the patients were asked to complete the Italian version of the tinnitus handicap inventory (THI) to determine the overall degree of perceived annoyance at the beginning and end of therapy. Pure tone averages for speech frequencies and for high frequencies were computed, and psychoacoustic pitch and loudness matches were determined for each subject before and after treatment., Results: The pure tone audiometry, pitch, loudness, and THI scores of both groups were reported. In group A, statistically significant differences were observed for the "functional" and "emotional" subscales. The total score of THI and the loudness of tinnitus were also significantly reduced. No statistically significant differences were observed in group B., Conclusions: These findings suggest a possible contribution of the antioxidant effect to the organ of Corti in subjects with metabolic syndrome and CST.

Cancer patients on chemotherapy have a lower immune response to SARS-CoV-2 vaccines. Therefore, through a prospective cohort study of patients with solid tumors receiving chemotherapy, we aimed to determine the immunogenicity of an mRNA vaccine booster (BNT162b2) among patients previously immunized with an inactivated (CoronaVac) or homologous (BNT162b2) SARS-CoV-2 vaccine. The primary outcome was the proportion of patients with anti-SARS-CoV-2 neutralizing antibody (NAb) seropositivity at 8-12 weeks post-booster. The secondary end points included IgG antibody (TAb) seropositivity and specific T-cell responses. A total of 109 patients were included. Eighty-four (77%) had heterologous vaccine schedules (two doses of CoronaVac followed by the BNT162b2 booster) and twenty-five had (23%) homologous vaccine schedules (three doses of BNT162b2). IgG antibody positivity for the homologous and heterologous regimen were 100% and 96% ( p = 0.338), whereas NAb positivity reached 100% and 92% ( p = 0.13), respectively. Absolute NAb positivity and Tab levels were associated with the homologous schedule (with a beta coefficient of 0.26 with p = 0.027 and a geometric mean ratio 1.41 with p = 0.044, respectively). Both the homologous and heterologous vaccine regimens elicited a strong humoral and cellular response after the BNT162b2 booster. The homologous regimen was associated with higher NAb positivity and Tab levels after adjusting for relevant covariates.

Background: Congenital cytomegalovirus (cCMV) infection is the leading cause of non-hereditary sensorineural hearing loss in children. While about 10% of children reportedly display symptoms at birth, 85-90% of cCMV infection cases are asymptomatic. However, 10-15% of these asymptomatic infants may later develop hearing, visual, or neurodevelopmental impairments. This study aimed to evaluate the impact of cCMV infection on newborns' hearing function with a particular emphasis on progressive and late-onset cases., Methods: This study is a retrospective chart analysis with longitudinal character and was conducted in two Italian centers: Center 1 (from 1 November 2007 to 31 December 2021) and Center 2 (from 1 January 2012 to 31 December 2021). Data collected included newborn hearing screening results, characterization of hearing loss (unilateral/bilateral, degree of impairment), and audiological follow-up., Results: The cohort consisted of 103 children (42% males, 58% females). In total, 28 children presented with hearing impairment; 71.4% (20 out of 28) of the cases of hearing loss were severe/profound, with 35.7% of the cases due to unilateral hearing loss. Out of twenty-eight, six experienced progression of hearing loss and four had late-onset hearing loss., Conclusions: In the absence of universal cCMV screening, hearing screening at birth for cCMV remains a critical factor for early diagnosis. A significant percentage of children affected by cCMV with normal audiological evaluations at birth is easily lost to follow-up. Close collaboration between neonatologists, pediatricians, and audiological services is fundamental to ensure timely diagnosis and treatment of cCMV-related hearing loss.

Background and Aim: Several viruses have previously been reported to be responsible for congenital hearing loss; therefore, since the beginning of the SARS-CoV-2 infection pandemic, various reports have investigated a possible link. The aim of this review is to assess the possible link between maternal COVID-19 infection and congenital hearing loss., Methods: This systematic review was performed using PRISMA criteria, searching Medline and Embase databases from March 2020 to February 2023. A total of 924 candidate papers were identified; however, considering the specific selection criteria, only nine were selected for additional analysis., Results: The overall number of children born from mothers infected with COVID-19 during pregnancy identified through this review was 1687. The confirmed cases of hearing loss were 0.7% (12/1688); a description of its nature (sensorineural vs. conductive) is missing in the selected studies, and the follow-up period is variable across the analyzed papers. Surprisingly, a large proportion of false positives were recorded at the first stage of screening, which resulted normal at the re-test., Conclusions: Currently, a correlation between congenital hearing loss and SARS-CoV-2 infection cannot be definitively established. Further studies are desirable to provide additional evidence on this topic.

Objective: translate and validate the Penn Acoustic Neuroma Quality of Life Scale into Italian language (PANQOL-It)., Methods: the instrument was translated and psychometric properties were subsequently assessed by administering the PANQOL-It to 124 outpatients together with the Depression Anxiety Stress Scale (DASS21) and the Understanding and Communicating domain of the World Health Organization Disability Assessment Schedule II (WHODAS II-D1). The internal consistency, test-retest reliability, construct and criterion-related validity were assessed., Results: Cronbach's alpha coefficient was 0.92 for the total score and ranged from 0.44 to 0.90 in the seven domains. Significant test-retest reliability was observed (intraclass-correlation = 0.75; p < 0.01). Moderate correlation was reported between facial dysfunction domain and objective facial involvement (p < 0.01). Moderate to strong correlations were observed between anxiety, general health domains and all subscales of the DASS21, and between WHODAS II-D1 and general health and energy domains (p < 0.01). These latter results indicated good construct and criterion-related validity respectively., Conclusions: PANQOL-It presented more than acceptable psychometric properties and its adoption is justified for both clinical and research purposes., (Copyright © 2023 Società Italiana di Otorinolaringoiatria e Chirurgia Cervico-Facciale, Rome, Italy.)

Background . Defects of mitochondrial DNA (mtDNA) involved in the function of the mitochondrial electron transport chain can result in primary mitochondrial diseases (PMDs). Various features can influence the phenotypes of different PMDs, with relevant consequences on clinical presentation, including the presence of hearing impairment. This paper aims to describe the hearing loss related to different PMDs, and when possible, their phenotype. Methods. A systematic review was performed according to PRISMA guidelines, searching Medline until December 2022. A total of 485 papers were identified, and based on specified criteria, 7 were included in this study. Results. A total of 759 patients affected by PMDs and hearing loss were included. The age of patients ranged from 2 days to 78 years old, and the male-to-female ratio was 1.3:1. The percentage of subjects affected by hearing loss was 40.8%, (310/759), and in most cases, hearing impairment was described as sensorineural, bilateral, symmetrical, and progressive, with different presentations depending on age and syndrome severity. Conclusions . PMDs are challenging conditions with different clinical phenotypes. Hearing loss, especially when bilateral and progressive, may represent a red flag; its association with other systemic disorders (particularly neuromuscular, ocular, and endocrine) should alert clinicians, and confirmation via genetic testing is mandatory nowadays.

Background: Dizziness and vertigo are among the most prevalent complaints in the elderly and have a major negative influence on (i) the perception of the quality of life; and (ii) the risk of falling. Due to population aging, particularly in wealthy nations, vertigo represents a growing issue and a serious public health concern. In order to approach the patient correctly and to offer the best treatment options, it is mandatory to identify vertigo's underlying causes. The aim of this paper was to identify the different etiologies of vertigo and possibly their frequency in the elderly population, by reviewing the scientific literature of the last decade (2012-2022). Methods: A systematic review was performed according to PRISMA guidelines, searching the Medline database from January 2012 through to December 2022. The search identified 1025 candidate papers, but after the application of specific selection criteria, only five were considered for further analysis. Results: A total of 2148 elderly patients (60-90 y old) presenting with vertigo were reported in the selected papers. A total of 3404 conditions were identified as the cause of vertiginous symptoms, (some patients presented multiple etiologies). All major diagnoses were categorized into different subgroups: the most common origin of vertigo was represented by audio-vestibular disorders (28.4%), followed by cardiovascular (20.4%) and neurological diseases (15.1%). Furthermore, 9.1% of patients were diagnosed with psychiatric conditions, whilst ophthalmologic and musculoskeletal disorders accounted for 7.5% and 6.3% of the cases respectively. Medication adverse effects and metabolic-related diseases were also considered among the causes. For 3.4% of cases the etiology remains unclear. Conclusions: Audio-vestibular disorders represent the most frequent cause of vertigo in the elderly. The etiologies affecting the vertigo patient must be defined in order to identify potential life-threatening conditions, such as cardiovascular and neurological disorders, which according to the data of this review constitute the second and third common causes of vertigo. A multidisciplinary strategy, involving different specialists (such as ENTs, Neurologists, Cardiologists, Geriatricians) is recommended for the correct assessment of these disorders.

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.

Increased noise sensitivity refers to the abnormal subjective response to external sounds, with a prevalence of between 8% and 15.2% in the adult population as suggested by epidemiological studies. The basic neural mechanism of hyperacusis still remains obscure, so therapies for this often-devastating symptom remain elusive. The aim of this study was to assess psychological profiles in patients with presbycusis without tinnitus in a perspective case-control design. All subjects were initially submitted to audiological evaluation (tympanometry, recordings of the acoustic reflex thresholds, pure tone audiometry) and subsequently were administered the following questionnaires: the hyperacusis questionnaire (HQ), the brief symptom inventory (BSI), and the modified somatic perception questionnaire (MSPQ). Patients with hyperacusis reported a total score and subscales (attentional, social, and emotional) of the HQ significantly higher than controls. They also reported higher scores of the MSPQ and significantly higher mean values with concern to the somatization, obsessive-compulsive, interpersonal sensitivity, depression, and anxiety subscales of the BSI. These results show that psychological distress, as expressed by higher level of somatic attention, somatization, anxiety, and depression, is a significant factor to consider for a complete diagnosis and effective treatment of hyperacusis. For a correct diagnosis of patients seeking help for hyperacusis, their psychological distress should also be assessed, regardless of their hearing abilities. Further studies are required to investigate the pathological mechanisms that are involved in the onset of hyperacusis in patients with normal hearing and those with sensorineural hearing loss.

Background. Otosclerosis can occur during childhood, resulting in the early onset of conductive hearing loss. The approach to a child with otosclerosis can present some difficulties in terms of diagnosis and treatment, and the literature on juvenile otosclerosis (JO) is still relatively limited. Aim. To explore the current approaches to JO, in order to clear the management of this condition and evaluate the outcomes and the possible complications of surgical treatment. Methods. A systematic review was performed according to PRISMA guidelines, searching Medline and Embase from January 2002 through to 30 September 2022. A total of 759 papers were identified but based on specified criteria, nine were included in this study. Results. There were 94 children affected by JO and treated by stapes surgery. According to the available data, Male: Female ratio was 1:3−4, whilst the mean ages ranged from 10 to 16.3 years at the time of stapes surgery. After stapes surgery, the target of ABG < 10 dB was achieved in most of the patients. Overall, the 4 complications were reported (4/94= 4%): stenosis of the external ear canal, deterioration of hearing, anacusis with vertigo, tinnitus. Conclusions. The heterogeneity of the available studies does not allow us to draw straight conclusions on this topic, currently. More data about the natural history of the disease in children could help in approaching the treatment correctly, and possibly in drawing guidelines. Studies with a prolonged follow-up could be helpful for assisting clinicians and families in taking the most favorable decision about treatment.

Background: The survival of extremely low birth weight infants (ELBW) has increased worldwide. Even in the absence of major disabilities, ELBW infants show difficulty in simple language functions. It is relevant to assess early abilities, which are the base of early linguistic skills, in order to implement customized intervention programs in ELBW infants. Aims: To evaluate communication precursors of language development in ELBW infants at 12 and 24 months of correct age (C.A). To investigate the correlation of linguistic and communicative prerequisites with mental development outcome at 24 months CA. Method: 52 ELBW neonates (mean gestational age 26.6 weeks, mean birth weight was 775 g) who were admitted to the neonatal intensive care unit of the University Hospital of Modena, were enrolled. Data were collected from archived audio-video recordings of neurodevelopmental follow-up visits. Video analysis of communicative and linguistic developmental was performed at 12 and 24 months CA. Neurodevelopmental outcome was evaluated with Mental Developmental Scales (GMDS-R). Results: The video-analysis showed that infants at 12 months CA used predominantly eye contacts and gestural turns, while vocal turns were scant. At 24 months CA, a significant change in eye contacts, vocal turns, gestural turns, and utterances (p < 0.001) occurred. The total number of utterances (p = 0.036) and eye contacts (p = 0.045) were significantly correlated to the Development Quotient (DQ) of Hearing and Language scale. Moreover, a significant correlation was found with the Personal-Social scale vocal turns (p = 0.009) and the total number of utterances (p = 0,02). Finally, the Global Quotient of the GMDS-R was related to the Vocal Turns (p = 0.034) and the total number of Utterances (p = 0.013). Conclusions: ELBW infants at 12 months CA use predominantly eye contacts and gestural turns to communicate with adults. At 24 months CA, the child’s communicative intention evolves from gestural to verbal communication. The latter is characterized by an increase in both vocal turns and the number of utterances produced during interaction. The video analysis we implement appears to be a sensitive tool for early assessment of communication and language development and to refine early intervention

Older adults with hearing loss have difficulties during conversation with others because an elevated auditory threshold reduces speech intelligibility, especially in noisy environments. Listening and comprehension often become exhausting tasks for hearing-impaired elders, resulting in social isolation and depression. The aim of the present study was to investigate the advantages of hearing aid use in relation to relief from listening-related fatigue, which is still controversial. Participants included a sample of 49 hearing-impaired elders affected by presbycusis for whom hearing aids were prescribed. The Modified Fatigue Impact Scale was used to assess cognitive, physical and psychosocial fatigue. The vitality subscale of the Short Form Health Survey 36 and a single item of the multi-dimensional Speech, Spatial and Quality Hearing Scale ("Do you have to put a lot of effort to hear what is being said in conversation with others?") were also used. The Cognitive Failures Questionnaire was used to investigate daily errors related to lack of memory and reduced mindedness. Hearing aids rehabilitation resulted in improved speech intelligibility in competing noise, and a significant reduction in cognitive and psychosocial fatigue and listening effort in conversation. Vitality was also improved and a significant reduction in the Cognitive Failures Questionnaire scores was observed. Findings from the study indicate that the use of hearing aids in older impaired-listeners provide them not only with an increased auditory function but also with a reduction in listening-related fatigue and mindedness.

Vestibular migraine (VM) is the most common cause of episodic vertigo in children. Vertigo, nausea, dizziness and unsteadiness are often complained of by children with migraine, which can precede, follow or be present simultaneously with headache. The aim of this study was to use posturography to investigate the visually evoked postural responses (VEPRs) of children with VM and compare them to data obtained from children with primary headache (M) and controls (C). Twenty children diagnosed as affected by VM, nineteen children with M without aura and twenty healthy subjects were recruited in this cross-sectional study. Posturography was performed by a standardized stabilometric force-platform (Svep-Politecnica) in the following conditions: open eyes (OE), closed eyes (CE) and during full-field horizontal optokinetic stimulation (OKN-S). Electronystagmography was performed simultaneously to analyze optokinetic reflex parameters. In the OE condition, no difference was found between groups with respect to body sway area. In contrast, this parameter increased in the two pathological groups with respect to controls in the CE condition. The optokinetic stimulations also induced a similar increase of body sway area in the M group relative to controls, but a further increase was elicited in the VM group. Electronystagmographic recording also revealed different optokinetic reflex parameters in the latter groups. This study disclosed an abnormal sensitivity of children with M and VM to full-field moving scenes and a consequent destabilization of posture, as documented by the abnormal VEPRs. Children with VM were particularly exposed to this risk. Possible clinical implications of these findings are discussed.

Background: Vertigo and dizziness are relatively infrequent in paediatric patients, but specific data on the prevalence of these disorders are limited and influenced by various factors, including the age of the examined population. These conditions often have a significant impact on patients' and parents' quality of life. The aim of this paper is to investigate the prevalence of different aetiologies of vertigo in the paediatric population through a systematic review., Methods: According to PRISMA guidelines, a systematic review of the literature was performed. Medline and Embase were searched from January 2011 through to 10 September 2021. The search yielded 1094 manuscripts, which were reduced to 7 upon the application of inclusion criteria., Results: A total of 2470 paediatric patients were evaluated by the selected papers. Vestibular Migraine was the most frequently diagnosed condition, occurring alone or in association with other diseases. Overall, audio-vestibular disorders represented the second cause of vertigo, and the prevalence appears to increase according to age growth. Over the years, even though we assisted in the amelioration of diagnostic rates, partially related to an improvement in diagnostic tools, the aetiology of vertigo remains still unclear in a variable percentage of patients., Conclusion: Vertigo in children, despite being an uncommon symptom, requires a multidisciplinary approach, often involving Paediatricians, Neurologists and Otorhinolaryngologists. A comprehensive evaluation of children suffering from vertigo is crucial for establishing a successful therapy and reducing parental worries.

Hearing loss is one of the most common congenital sensory disorders. It can be associated with several comorbidities, in particular developmental disabilities (DD). In Emilia-Romagna (ER), a region in Northern Italy, Child and Adolescent Mental Health Services (CAMHS) provide the diagnostic framework and treatment for these conditions. The aim of the present study is to evaluate the prevalence of hearing loss, both isolated or in association with comorbidities, in the juvenile population. The study draws its data from the ER Childhood and Adolescent Neuropsychiatry Information System (SINPIAER), an Administrative Healthcare Database collecting the clinical data of all those who have attended CAMHS since 2010. The most frequent type of hearing loss was bilateral sensorineural hearing loss, which was present in 69-72% of the cases, while bilateral conductive hearing loss was the second most common type, ranging from 8 to 10%. Among DD, congenital malformations, mental retardation, visual impairment, and cerebral palsy were the most common. In particular, autism spectrum disorders show increasing incidence and prevalence among CAMHS users in ER region. In-depth knowledge of hearing loss epidemiology and related conditions, such as developmental disabilities, in the juvenile population is crucial for disease prevention, health planning, and resource allocation.

Previous research has shown that the production of third-person singular accusative object clitics (3DO clitics) might be taxing in Italian-speaking pre-school children with cochlear implants (CIs). We investigated this topic by assessing 3DO clitic production in 14 children with an average age of 8 years, who had received CI between age 1 and 4. The first goal of the study was to analyze whether school-aged children with CIs exhibit atypical behavior in 3DO clitic production. The second goal was to analyze whether children with CIs are prone to agreement errors in case of gender mismatch between the subject and the 3DO clitic, as has been shown for normal-hearing, typically developing children. To achieve this, we used two tasks in which subject and object clitic grammatical genders were manipulated so that they would or would not match. As for the first goal, the majority of children with CIs had good performance on the clitic tasks. However, some participants' performance was poor. The pattern of deviant responses differed among the poor performers. We believe that children with CIs showing impairments in 3DO clitic production need careful individual analysis in order to plan effective speech therapy. As for the second goal, children with CIs were more prone to agreement errors in the mismatch condition compared to the match condition; this dimension needs to be considered when assessing and eventually rehabilitating clitic production.

A 32-week preterm-born male with symptomatic congenital cytomegalovirus infection was treated with valganciclovir. He was also affected by congenital severe bilateral hearing loss and, unexpectedly, a normalization of the hearing threshold was reached at one year of age. The improvement of hearing level in relationship with both the late development of the auditory system and the administration of antiviral therapy is discussed. This case also highlights the importance of early diagnosis of congenital cytomegalovirus associated with close follow-up., (This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Background: In many countries, neonatal hearing screening programs (NHS) have been available for many years; however, because of the presence of hearing loss at late onset, early hearing detection programs (EHDP) have been implemented. The aim of this study was to evaluate all cases of infantile hearing loss under the care of two different provinces of a regional health service since the introduction of NHS., Methods: Clinical data (the presence of audiological risk factors, age at which children are placed under the care of health service, entity of hearing loss, treatment, and exposure to bilingualism) were retrospectively collected during the period from 1 January 2012 to 31 December 2018, starting from the IT management system used in all of the regional neuropsychiatric services., Results: In total, 124 children were included-116 cases failed the screening, 1 case had an untraceable result, and 7 cases (5.6%) had hearing screening that passed. Most of the children were placed under the care of a neuropsychiatric infantile and adolescence (NPIA) service within the first year of life. The main differences across the two provinces concerned the percentages of audiological risk factors and the number of unilateral hearing loss cases., Conclusion: In order to plan and manage hearing rehabilitation programs for children in the best way, it is very important to know the local clinical-epidemiological features of the population.

Since January 2012, babies born in the province of Modena, Italy, have routinely undergone hearing testing as part of a two-stage screening programme. Newborn hearing screening (NHS) has been based on an integrated hospital and community care system and this study aims to assess screening coverage, referral rates, the prevalence, type and extent of hearing loss several years into the programme. Data were collected from January 1, 2012 to December 31, 2015. Coverage was over 99% in all five facilities of the province. The ratio of "fails of the screening" to the total number of infants tested varied over the period from 1.2% to 0.9% in the third level facility, and from 0.8% to 0.4% in the other four. Although hearing loss was mainly associated with dysmorphic\syndromic diseases or a family history of hearing loss, some 23% of cases were identified with no known risk factors. We highlight the importance of the NHSP and the need for strong support from healthcare administrators to ensure high coverage. This is especially true since although the prevalence of hearing loss was higher among infants with audiological risk factors, several cases of hearing loss were found in newborns with no known risk factors., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Congenital cytomegalovirus (cCMV) infection is a major cause of hearing loss in children. A few cases of cCMV twin pregnancies are reported in the literature. Twins can react differently to maternal infection, but hearing loss is rarely evaluated. Two couples of twins with cCMV infection and different audiologic outcomes are reported. The first couple of twins was composed by two male twins, both affected by cCMV infection. The first born had normal hearing function, and the second born had sensorineural hearing loss (SNHL). In the second couple, a male and a female twin, only the male twin was affected by cCMV infection, and both had normal hearing function. In this case series, an interesting finding was the association between the presence of viral DNA in liquor and hearing loss in one newborn. Further research is needed to better understand the pathophysiology of SNHL caused by cCMV infection.

The family of maize Kip-related proteins (KRPs) has been studied and a nomenclature based on the relationship to rice KRP genes is proposed. Expression studies of KRP genes indicate that all are expressed at 24 h of seed germination but expression is differential in the different tissues of maize plantlets. Recombinant KRP1;1 and KRP4;2 proteins, members of different KRP classes, were used to study association to and inhibitory activity on different maize cyclin D (CycD)-cyclin-dependent kinase (CDK) complexes. Kinase activity in CycD2;2-CDK, CycD4;2-CDK, and CycD5;3-CDK complexes was inhibited by both KRPs; however, only KRP1;1 inhibited activity in the CycD6;1-CDK complex, not KRP4;2. Whereas KRP1;1 associated with either CycD2;2 or CycD6;1, and to cyclin-dependent kinase A (CDKA) recombinant proteins, forming ternary complexes, KRP4;2 bound CDKA and CycD2;2 but did not bind CycD6;1, establishing a differential association capacity. All CycD-CDK complexes included here phosphorylated both the retinoblastoma-related (RBR) protein and the two KRPs; interestingly, while KRP4;2 phosphorylated by the CycD2;2-CDK complex increased its inhibitory capacity, when phosphorylated by the CycD6;1-CDK complex the inhibitory capacity was reduced or eliminated. Evidence suggests that the phosphorylated residues in KRP4;2 may be different for every kinase, and this would influence its performance as a cyclin-CDK inhibitor., (© The Author 2017. Published by Oxford University Press on behalf of the Society for Experimental Biology.)

Background: Multiple clinical trials have demonstrated the benefits of adjuvant 5-fluorouracil-based chemotherapy for patients with resectable colon cancer (CC), especially in stage III., Aim: To describe the clinical characteristics of a cohort of CC patients treated at a single university hospital in Chile since 2002, and to investigate if chemotherapy had an effect on survival rates., Material and Methods: Review of a tumor registry of the hospital. Medical records of patients with CC treated between 2002 and 2012 were reviewed. Death certificates from the National Identification Service were used to determine mortality. Overall survival was described using the Kaplan-Meier method. A multivariate Cox proportional hazard regression model was also used., Results: A total of 370 patients were treated during the study period (202 in stage II and 168 in stage III). Adjuvant chemotherapy was administered to 22 and 70% of patients in stage II and III respectively. The median follow-up period was 4.6 years. The 5-year survival rate for stage II patients was 79% and there was no benefit observed with adjuvant chemotherapy. For stage III patients, the 5-year survival rate was 81% for patients who received adjuvant chemotherapy, compared to 56% for those who did not receive chemotherapy (hazard ratio (HR): 0.29; 95% confidence interval (CI): 0.15-0.56). The benefit of chemotherapy was found to persist after adjustment for other prognostic variables (HR: 0.47; 95% CI: 0.23-0.94)., Conclusions: Patients with colon cancer in stage III who received adjuvant chemotherapy had a better overall survival.

Third-person direct object (DO) clitic pronoun production is examined through an elicited production method in pre-school- and primary school-aged groups of Italian children with specific language impairment (SLI) to establish whether there is an improvement from age 5 years to age 7 years and whether there are qualitative differences in the two groups' responses. It was found that 5- and 7-year-old Italian children with SLI produce fewer third-person DO clitics than same-age peers. The kind of responses they provide changes: at 5 years, children with SLI tend to omit clitics, while at 7 years, they use a full noun. Production of third-person DO clitics is a persistent challenge for children with SLI and is confirmed to be a good clinical marker both at 5 and 7 years of age.

Hearing loss secondary to diabetes remains under debate. In our study, we used Zucker Diabetic Fatty (ZDF) rats as an animal model of type 2 diabetes to investigate whether (1) hearing ability impairment and structural alterations of the inner ear occur in diabetes and (2) an angiotensin II receptor blocker (losartan) can protect rats from diabetic damage. Homozygous mutants were treated with a placebo or losartan and heterozygous animals served as non-diabetic controls. All animals underwent immunohistochemical and electronmicroscopical analysis. Functional testing of hearing ability was performed by click-evoked auditory brainstem responses. The present study showed significant sensorineural hearing impairment in placebo-treated diabetic rats (hearing threshold, 45.0 ± 2.1 dB SPL) compared to both non-diabetic controls (34.7 ± 4 dB SPL) and losartan-treated diabetic rats (36.1 ± 7.4 dB SPL). Concurrently, the functional decline in the placebo-treated rats was associated with significant morphological abnormalities, particularly in the intermediate cells of the stria vascularis and with strial dysfunction. These degenerative changes were indicated by the down-regulation of several pumps, ionic and cellular channels, which are involved in the cycling of K(+) and the maintenance of the endocochlear potential essential for the hearing process. Thus, the inner ear can be regarded as a target organ during hyperglycemic disorders and a metabolically induced "diabetic otopathy" may be added to angiopathy, nephropathy and neuropathy as a specific complication of diabetes mellitus. Blockade of the angiotensin II receptor can prevent this "diabetic otopathy" despite hyperglycemic serum levels.

Background: Acute otitis media (AOM) is one of the most common childhood infectious diseases. The recent Italian Pediatric Guidelines for the treatment of AOM constitutes a step forward in the management of children with uncomplicated AOM. The aim of this study was to evaluate antibiotic prescription patterns for AOM in a Pediatric Emergency Department (PED) after those guidelines were introduced and to assess the relationship between implementation of the "watchful waiting" strategy and the incidence of acute mastoiditis in the PED., Methods: This retrospective study was conducted between 1st January 2007 to 31st December 2013 at the PED of the University of Modena and Reggio Emilia in Modena (Italy). All children between 0 and 14 years who were examined because of symptoms and/or signs of AOM and acute mastoiditis were enrolled. Pearson's chi-squared test was used to evaluate if introduction of the Italian Paediatric Guidelines was associated with a reduction in the antibiotic prescription pattern in children with AOM and/or with an increase in mastoiditis frequency., Results: 4,573 (89.4%) patients were included in our analysis, antibiotics were prescribed to 81% cases of the children diagnosed with AOM. The frequency of antibiotic prescribing continued to be stable after the Italian guidelines were introduced (82% versus 81%). Forty children were admitted to hospital with a diagnosis of acute mastoiditis. Our study did not find any association between the number of cases of acute mastoiditis and the percentage of patients treated with antibiotics; the annual incidence of mastoiditis before and after the new guidelines were published was, in fact, stable., Conclusions: Despite the diffusion of clinical guidelines recommending a "watchful waiting" approach for children with AOM, the antibiotic prescription rate continues to be high. It appears to be more difficult to impact the percentage of cases for which antibiotics are prescribed than the type of antibiotic that is utilized. In view of these findings, a close follow-up control by the primary care paediatrician or a scheduled follow-up appointment at the PED and incisive campaigns to promote parents' awareness of proper antibiotic use appear to be warranted.

Aim: The aim of the study is to report the new and rare GJB2 variants identified in individuals with nonsyndromic sensorineural hearing impairment (HI) in a retrospective study based on 498 patients referred to the Otolaryngology and Medical Genetics Units of the Modena University Hospital, Italy, with the purpose of building new genotype/auditory phenotype correlations for the GJB2 gene., Results: A total of eight variants identified in HI patients under study were considered rare for their frequency below 1% in the general population and in the HI databases. Of those, four (I20T, V95M, N206S, c.-22-2A>C) were in compound heterozygosity with known mutations resulting in a range of phenotypes from mild to profound, whereas four (W3R, C218Y, K221N, c.-22-6T>C) were found in simple heterozygosity (for those only in silico prediction of pathogenicity was possible due to the absence of a second GJB2 or GJB6 mutation)., Conclusion: Based on patients' phenotype, reported frequency, and in silico prediction analysis, we suggest the prognostic value of eight rare and new GJB2 alleles, which may be of help to the clinician in counseling patients who carry such variants.

Emotional distress (ED) is greater for oncology patients in comparison with the general population, and this has implications for the quality of life of the patient and his/her family, adherence to the treatment, and eventually, survivorship. In general, the detection of these symptoms is low, which explains the need for detection systems appropriate to the clinical reality of the oncology team. The objective of this study is to evaluate for the first time the usefulness of an ultra-brief screening instrument [distress thermometer (DT)], in a group of Chilean oncology patients. A total of 166 outpatients were evaluated at the Cancer Center of the Pontificia Universidad Católica de Chile, before starting chemotherapy. Two screening instruments were applied: Hospital Anxiety and Depression Scale (HADS) and DT. The application of HADS resulted in a prevalence of 32.7% of anxiety symptoms (HADS-A ≥ 8), 15.7% of depression symptoms (HADS-D ≥ 8), and 39.8% had a total score of HADS-T ≥ 11. The DT resulted in the prevalence of 32.5% of distress or ED (DT ≥ 5). The validity of the DT was evaluated as a screening tool in comparison with HADS, observing, in relation to the anxiety scale (HADS-A), a sensitivity of 88.9% and specificity of 78.4% (DT ≥ 4); depression (HADS-D), a sensitivity of 69.2% and specificity of 74.3% (DT ≥ 5); and in relation to the total scale (HADS-T), a sensitivity of 68.2% and specificity of 73.0% (DT ≥ 4). This study demonstrates the elevated prevalence of emotional symptoms in Chilean oncology patients, before the start of chemotherapy, and confirms the potential of the DT as a brief screening instrument with easy application. The DT will allow the clinician to increase the detection threshold in the Chilean oncology population, intervene in a timely manner, and contribute to the comprehensive handling of the oncology patient without affecting the time needed for assistance.

The importance of cell proliferation in plant growth and development has been well documented. The majority of studies on basic cell cycle mechanisms in plants have been at the level of gene expression and much less knowledge has accumulated in terms of protein interactions and activation. Two key proteins, cyclins and cyclin-dependent kinases (CDKs) are fundamental for cell cycle regulation and advancement. Our aim has been to understand the role of D-type cyclins and type A and B CDKs in the cell cycle taking place during a developmental process such as maize seed germination. Results indicate that three maize D-type cyclins-D2;2, D4;2, and D5;3-(G1-S cyclins by definition) bind and activate two different types of CDK-A and B1;1-in a differential way during germination. Whereas CDKA-D-type cyclin complexes are more active at early germination times than at later times, it was surprising to observe that CDKB1;1, a supposedly G2-M kinase, bound in a differential way to all D-type cyclins tested during germination. Binding to cyclin D2;2 was detectable at all germination times, forming a complex with kinase activity, whereas binding to D4;2 and D5;3 was more variable; in particular, D5;3 was only detected at late germination times. Results are discussed in terms of cell cycle advancement and its importance for seed germination.

Background: The 5 G/5 G genotype of PAI-1 polymorphism is linked to decreased plasminogen activator inhibitor-1 (PAI-1) levels and it has been suggested that lower PAI-1 levels may provide protective effects on inflammation, local microcirculatory disturbance, and fibrotic changes, which are likely associated with development of sudden sensorineural hearing loss (SSNHL)., Methods: The association of the 4 G/5 G PAI-1 polymorphism with the development and clinical outcome of SSNHL is evaluated via a case control study. 103 patients with SSNHL and 113 age and sex-matched controls were enrolled at University of Ferrara, Italy and hearing loss outcome was measured at least 3 months after the onset of hearing loss. DNA was isolated from peripheral blood using the QIAamp kit and the 4 G/5 G polymorphism in the -675 promoter region was genotyped with an allele-specific PCR. Genotype distribution was tested in patients and compared to controls by chi-square and odd-ratio analysis. The codominant and recessive models were used for the multiple logistic regression analyses of the PAI-1 gene allele., Results: In this population, 5 G/5 G genotype had a two-time lower frequency in SSNHL patients compared to healthy controls (15.5% vs 30.1%) and was associated with decreased odds compared to 4 G/5 G genotype (OR 0.37, 95% CI 0.19-0.75, p = 0.005). In addition, the patients with 5 G/5 G genotype showed a trend of more than 2 times higher ratio of hearing recovery (> 20 dB) after systemic corticosteroid treatment compared to 4 G/5 G genotype (OR 2.3, 95% CI 0.32 - 16.83, p = 0.39), suggesting a better clinical outcome., Conclusions: The 5 G/5 G genotype of PAI-1 may be associated with a reduced risk of SSNHL in the Italian population.

Objective tinnitus refers to a tinnitus that can also be heard by the examiner. It is a relatively rare condition, and can be misdiagnosed or neglected. Some causes of objective tinnitus are head and neck vascular malformations, or muscular myoclonus of the tensor tympani, stapedial, or palatal muscles. The case of an 11-year-old girl with an objective tinnitus lasting from 1 year is herein presented, and the diagnostic workup performed in this unusual case is described., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Objective: Low frequency of effective contraceptive use remains a challenging problem. This article examines the frequency of effective postpartum contraception and the methods used before discharge in public hospitals in Guatemala. It also discusses the need to implement best practices in providing family-planning and contraceptive services., Methods: In March 2006, a surveillance system was implemented to collect data on the initiation of effective contraceptive methods. Postpartum women were monitored in 34 public hospitals. Univariate and bivariate analyses were performed, and a chi-square test for linear trends was used to compare female surgical sterilization rates after vaginal delivery and cesarean section., Results: Between 1 March 2006 and 31 December 2008, of the 218 656 women who had a postpartum event, 31% received an effective contraceptive method before hospital discharge. The frequency of initiation of effective postpartum methods varied across hospitals. Hospital results were consistent with national data on women of reproductive age. Among women who underwent surgical sterilization, differences between those who had delivered vaginally and those who had a cesarean section were statistically significant., Conclusions: The overall frequency of initiation of effective postpartum contraceptive use is low in public hospitals in Guatemala. It is higher, however, in hospitals at lower health care levels with strong community ties. Routine data collection revealed specific areas for improvement, particularly the need to enhance health providers' knowledge of medical eligibility criteria for effective contraceptive use postpartum. The priority is to promote the provision of high-quality family-planning and contraceptive services in Guatemala's public health system.

We report on a 3-year-old child who presented a de novo rearrangement of chromosome 4, detected on GTG banding and characterized by array CGH and FISH, as a complex intrachromosomal rearrangement with three deletions: del(q32.1q32.2), del(q33q34.1), del(q35.2), one tandem duplication dup(q34.3q35.1) and short normal regions in between. The study of karyotype-phenotype correlations in this and other patients with deletions of 4q suggests 4q33q34.1 as a candidate region for 4q-syndrome and for craniofacial development., ((c) 2007 Wiley-Liss, Inc.)

Introduction and Objectives: Several guidelines on the treatment of cardiovascular risk factors base their recommendations on the assertion that diabetes mellitus (DM) is a coronary heart disease (CHD) or cardiovascular disease (CVD) risk equivalent. To date, no systematic review of studies substantiating this assertion has been carried out., Methods: A systematic search of the PubMed database up to February 2006 was performed to identify prospective studies meeting the following criteria: a) follow-up was >5 years; b) groups of subjects with DM and without CHD (i.e., DM+CHD-), without DM and with CHD (DM-CHD+), and without either DM or CHD (DM-CHD-) were all included; and c) data on CHD or CVD mortality was reported. The characteristics of the studies were assessed, and data were combined separately for men and women using a random effects model and taking the DM-CHD- group as a reference., Results: In total, 13 studies met the inclusion criteria. Overall, CHD mortality was non-significantly lower in DM+CHD- men than in DM-CHD+ men, hazard ratio [HR] (95% confidence interval [CI]), 3.06 (2.45-3.83) vs 4.28 (3.24-5.66), respectively (P=.066); as was CVD mortality, HR (95% CI), 2.55 (2.00-3.26) vs 3.61 (2.81-4.62), respectively (P=.051). In women, there was no significant difference between the DM+CHD- and DM-CHD+ groups with regard to either CHD mortality, HR (95% CI), 4.68 (3.40-6.45) vs 3.51 (1.75-7.04), respectively (P=.42), or CVD mortality, HR (95% CI), 4.70 (4.23-5.22) vs 3.39 (1.51-9.02), respectively (P=.59)., Conclusions: The findings of this meta-analysis support the view that women in the DM+CHD- group have similar CHD and CVD mortality to those in the DM-CHD+ group, whereas men in the DM+CHD- group demonstrated a non-significant trend towards lower CHD and CVD mortality than those in the DM-CHD+ group.

Background: Cantabria has the highest prevalence of alcohol consumption among women in Spain. Patterns of alcohol consumption before pregnancy were assessed as a determinant of alcohol cessation in pregnant women in Cantabria., Methods: Survey on a random sample of women delivering for the period 1998-2002 (n = 1510). Information was obtained from personal interview (data on alcohol consumption), clinical charts and prenatal care records. Relative risks (RR) and 95% confidence intervals (CI) were estimated. Multivariable analyses were carried out using logistic regression., Results: Nearly half (49.5%) of the women drank regularly before pregnancy and 22.7% during pregnancy. Sociodemographic variables favouring alcohol cessation were: high education level and smoking cessation, whereas high social class, advanced maternal age and employment outside of home decreased the rate of alcohol cessation. Cessation decreased with the amount of alcohol consumed on weekdays (P < 0.001), but not with intake during weekends only. In women with alcohol use only during weekends, only the consumption of spirits increased the rate of alcohol cessation (adjusted RR = 1.40, 95% CI: 1.13-1.60). Pre-pregnancy binge drinking (> or =4 drinks on one occasion) decreased alcohol cessation in pregnancy (adjusted RR = 0.66, 95% CI: 0.40-0.97)., Conclusions: Drinking patterns influenced the rate of alcohol cessation: the heavier the alcohol consumption on weekdays, the lower the rate of alcohol cessation.

Merkel cells carcinoma (MCC) is an uncommon skin lesion, considered a malignancy of the neuroendocrine system, which is found mainly in elderly people. Its incidence is highly correlated with sun exposure or immunodeficiency syndromes. MCC is often an aggressive tumour with high tendency for local recurrence, lymph node involvement and distant metastasis. To our best knowledge 20 cases originated from the auricle have been described, 2 of them arising from external ear canal. The authors report a case of the ear canal characterized by two others synchronous tumours and the occurrence of a malignant high grade lymphoma, in which contribute of the pathologist was essential for a critical review. MCC diagnosis is not always easy for its pathological and clinical features and it should always be considered in presence of lymphoma. A multidisciplinary approach is basic.

Background: Cantabria (Spain) has one of the highest prevalence of smoking among women of the European Union. The objectives are to assess the trend of smoking during pregnancy in a five-year period and the determinants of smoking cessation during pregnancy in Cantabria., Methods: A 1/6 random sample of all women delivering at the reference hospital of the region for the period 1998-2002 was drawn, 1559 women. Information was obtained from personal interview, clinical chart, and prenatal care records. In the analysis relative risks and 95% confidence intervals were estimated. Multivariable analysis was carried out using stepwise logistic regression., Results: Smoking prior to pregnancy decreased from 53.6% in 1998 to 39.4% in 2002. A decrease in smoking cessation among women smoking at the beginning of pregnancy was observed, from 37.3% in 1998 to 20.6% in 2002. The mean number of cigarettes/day (cig/d) before pregnancy remained constant, around 16 cig/d, whereas a slight trend to increase over time was seen, from 7.7 to 8.9 cig/d. In univariate analysis two variables favoured significantly smoking cessation, although they were not included in the stepwise logistic regression analysis, a higher education level and to be married. The logistic regression model included five significant predictors (also significant in univariate analysis): intensity of smoking, number of previous pregnancies, partner's smoking status, calendar year of study period (these four variables favoured smoking continuation), and adequate prenatal care (which increased smoking cessation)., Conclusion: The frequency of smoking among pregnant women is very high in Cantabria. As smoking cessation rate has decreased over time, a change in prenatal care programme on smoking counseling is needed. Several determinants of smoking cessation, such as smoking before pregnancy and partner's smoking, should be also addressed by community programmes.

Background: The role of alcohol as a risk factor for motor vehicle crashes is long known. Yet, reports on the prevalence of drinking and driving suggest values between 20%-30% when the adult driving population is interviewed. We wondered whether these values hold true among European educated citizens and whether there are any significant differences in prevalence by age, gender, type of profession and other lifestyle indicators., Methods: Cross-sectional analyses of baseline data from a cohort of university graduates in Spain (SUN study). Answered questionnaires contained items on current drinking and driving practices, together with data on socio-demographic characteristics and lifestyle habits. Chi square, Fisher test, and multivariate logistic regression were used to investigate the impact of several variables on drinking and driving practices. Analyses were stratified by gender., Results: Almost 30% of the participants reported "sometimes" drinking and driving. This percent increased to 47% when "almost never" was also included as a positive answer to the drinking and driving practice question. These percentages varied significantly by gender, with up to 64% of men reporting "sometimes" or "almost never" vs. 36% of women doing so. Drinking and driving practices also differed by overall alcohol consumption habits, smoking, use of safety belts, and notably, type of profession., Conclusion: Our findings are amongst the first on the high prevalence of drinking and driving among Spanish. Particularly worrisome is the fact that health professionals reported this habit even at higher rates. Multidisciplinary interventions (e.g., legal, educational, economic) are needed to reduce this serious health risk.

A positive linear trend (P<.001) between the National Noscomial Infection Surveillance system (NNIS) risk index and mortality was observed in 2,848 general surgery patients followed up 6 years after discharge. In stratified analyses, the NNIS risk index predicted mortality in patients with chronic disease (P=.007, by test for trend) but not in the remaining patients.