The International Standard Set of Outcome Measures for the Assessment of Hearing in People with Osteogenesis Imperfecta.

OBJECTIVE: The aim is to recommend a minimum standard set of clinician-reported outcome measures (CROMs) and patient-reported outcome measures (PROMs) on hearing for people with osteogenesis imperfecta (OI). This project is part of the larger "Key4OI" project initiated by the "Care4BrittleBones foundation" of which the goal is to improve quality of life of people with OI. Key4OI provides a standard set of outcome measures and covers a large set of domains affecting the well-being of people with OI. METHODS: An international team of experts in OI, comprising specialists in audiological science, medical specialists, and an expert patient representative, used a modified Delphi consensus process to select CROMs and PROMs to evaluate hearing problems in people with OI. In addition, focus groups of people with OI identified key consequences of their hearing loss. These criteria were matched to categories of preselected questionnaires to select a PROM that matched their specific hearing-related concerns best. RESULTS: Consensus on PROMs for adults and CROMs for adults and children was reached. The focus of the CROMs was on specific audiological outcome measures and standardized follow-up. CONCLUSIONS: This project resulted in a clear consensus statement for standardization of hearing-related PROMs and CROMs and follow-up management of patients with OI. This standardization of outcome measurements will facilitate comparability of research and easier international cooperation in OI and hearing loss. Furthermore, it can improve standard of care in people with OI and hearing loss by incorporating the recommendations into care pathways.

Characteristic Hallmarks of Aging and the Impact on Carcinogenesis.

Evidence shows that there is a synergistic, bidirectional association between cancer and aging with many shared traits. Age itself is a risk factor for the onset of most cancers, while evidence suggests that cancer and its treatments might accelerate aging by causing genotoxic and cytotoxic insults. Aging has been associated with a series of alterations that can be linked to cancer: i) genomic instability caused by DNA damage or epigenetic alterations coupled with repair errors, which lead to progressive accumulation of mutations; ii) telomere attrition with possible impairment of telomerase, shelterin complex, or the trimeric complex (Cdc13, Stn1 and Ten1 - CST) activities associated with abnormalities in DNA replication and repair; iii) altered proteostasis, especially when leading to an augmented proteasome, chaperon and autophagy-lysosome activity; iv) mitochondrial dysfunction causing oxidative stress; v) cellular senescence; vi) stem cells exhaustion, intercellular altered communication and deregulated nutrient sensing which are associated with microenvironmental modifications which may facilitate the subsequential role of cancer stem cells. Nowadays, anti-growth factor agents and epigenetic therapies seem to assume an increasing role in fighting aging-related diseases, especially cancer. This report aims to discuss the impact of age on cancer growth.

Hearing Loss After Cisplatin-based Chemoradiotherapy for Locally Advanced Head and Neck Cancer: A Prospective Single-institution Study.

BACKGROUND/AIM: A single-institution prospective study was conducted to evaluate hearing loss rate after intensity modulated radiotherapy with concomitant cisplatin-based chemotherapy (CRT) for locally advanced head and neck cancer and identify cochlear dosimetric parameters associated with hearing loss risk. PATIENTS AND METHODS: Hearing assessment, patients' characteristics, tumor-related variables, and cochlear quantitative dosimetric factors for adults with locally advanced head and neck cancer treated with CRT were prospectively collected. Each patient repeated audiometry at baseline (pre-CRT), 1 month after CRT, and then every 3 to 6 months. For each cochlea minimum dose (Dmin), mean dose (Dmean), and maximum dose (Dmax) were extracted from treatment plans. Logistic analysis was used for multivariate modeling. The relation between cochlear dosimetric factors and significant hearing loss was also analyzed with receiver operating characteristic (ROC) curves. RESULTS: Between January 2016 and December 2018, 35 patients (70 cochleae) were included. Most patients (n=29; 82.9%) had primary cancer in a low-risk region (oral cavity, oropharynx, larynx). All patients completed the programmed CRT. During follow-up, significant hearing loss was recorded in 13 cases (37.1%). The ROC areas for significant hearing loss in relation to Dmin, Dmean, and Dmax were 0.70, 0.66, and 0.66, respectively. A dose-dependent relationship was noted between cochlear Dmin and significant hearing loss. CONCLUSION: Dmin <14.4 Gy is associated with reduced rates of significant hearing loss after concomitant cisplatin-based CRT in patients with locally advanced head and neck cancer.

Behavioral dysregulations by chronic alcohol abuse. Motivational enhancement therapy and cognitive behavioral therapy outcomes.

Patients with alcohol use disorder (AUD) do not manifest homogeneous clinical symptoms. Various studies described both cognitive impairments and psychiatric disorders among people with AUD. This disorder is one of the most frequent mental disorders in developed countries, due to excessive alcohol consumption. Alcohol is toxic as it increases the production of reactive oxygen species (ROS) and can cause dependence. This causes negative effects on brain development and cognitive functions that affect the individual's work, health, and social life. Current pharmacology treatment for alcohol addiction is based on direct action against the neurotransmitters involved in alcohol dependence. AUD patients without comorbid psychiatric disorders or severe cognitive deficits are defined as "pure alcoholics". To date, poor is known about effective treatments for this typology of AUD patients. Psychotherapy is largely used in resolving many psychiatric disorders, including substance use disorders. Motivational enhancement therapy (MET) and cognitive-behavioral therapy (CBT) are two psychotherapies used to achieve and maintain abstinence in patients affected by substance use disorders. This short review aims to describe two CBT and MET and to present the advantages and disadvantages of these two psychotherapies in the treatment of AUD.

Anti-inflammatory role of curcumin in retinal disorders (Review).

Curcumin [1,7-bis-(4-hydroxy-3-methoxyphenyl)-hepta-1,6-diene-3,5-dione], the main component of turmeric (Curcuma longa, a flowering plant of the ginger family, Zingiberaceae), is known to possess different pharmacological activities, particularly anti-inflammatory and antioxidant properties. Since an underlying inflammatory process exists in several ocular conditions, such as anterior uveitis, glaucoma, age-related macular degeneration (AMD) and diabetic retinopathy (DR), the aim of the present review was to summarize the pleiotropic effects exerted by this molecule, focusing in particular on its beneficial role in retinal diseases. The anti-inflammatory activity of curcumin has also been described in numerous systemic inflammatory pathologies and tumors. Specifically, the biological, pharmaceutical and nutraceutical properties of curcumin are associated with its ability to downregulate the expression of the following genes: IκBα, cyclooxygenase 2, prostaglandin E2, interleukin (IL)-1, IL-6, IL-8 and tumor necrosis factor-α. According to this finding, curcumin may be useful in the treatment of some retinal disorders. In DR, proliferative vitreoretinopathy and AMD, beneficial effects have been observed following treatment with curcumin, including slowing down of the inflammatory process. Despite the aforementioned evidence, the main disadvantage of this substance is that it possesses a low solubility, as well as poor oral bioavailability due to its reduced absorption, rapid metabolism and rapid elimination. Therefore, several curcumin analogues have been synthesized and tested over the years, in order to improve the possible obtainable therapeutic effects. The purpose of the present review was to identify new aspects that could guide future research on this important traditional medicine, which is a well-tolerated natural product, and is widely considered safe and economical.

The safety of sublingual immunotherapy, can the rare systemic reactions be prevented?

Introduction: The safety of subcutaneous immunotherapy (SCIT), and particularly the dramatic issue of fatal reactions, has been an obstacle that limited the implementation of a therapy with unique characteristics of action on the causes of allergy. The introduction of sublingual immunotherapy (SLIT) was aimed at solving safety problems while maintaining clinical efficacy.Areas covered: For more than 20 years, SLIT has been based on allergen extracts in drops at low average doses. As evidenced by meta-analyses, the typical adverse events (AE) have consisted of local reactions in the mouth and throat. Unlike the injection route, no correlation was observed between the administered dose and AEs. The development of SLIT products in tablets, based on higher doses than drops, has somewhat changed the concept of SLIT safety. Although large trials, performed to obtain regulatory agency approval, have shown overall high safety, rare anaphylactic reactions have been described.Expert opinion: SLIT is globally safe, and no fatal reactions have ever been reported, but with currently available high biological potency products it is necessary to follow prudential rules, such as the administration of the first dose under medical supervision and the thorough education of patients to avoid taking of higher doses than recommended.

The impact of white matter hyperintensities on speech perception.

BACKGROUND: The presence of white matter hyperintensities (WMHs) can impact on normal brain function by altering normal signal transmission and determining different symptoms. AIM: To evaluate the relationship between the presence of brain WMHs and the scores of speech perception test (SPT) in a sample of normal-hearing patients under 70 years of age. MATERIAL AND METHOD: Prospective study. One hundred eleven patients underwent audiological screening with pure tone audiometry (PTA), tympanometry, speech perception testing (SPT), and brain magnetic resonance imaging (MRI). T2 sequences were analyzed to identify the presence of WMH that, if identified, were scored using the Fazekas score. Statistical multiple regression analysis was performed to understand the relationship between PTA and SPT score; the Pearson's and Spearman's tests were used to evaluate the correlation between Fazekas scores and SPT. Chi-square test was used to analyze the difference between gender. RESULTS: The results of PTA were not predictive of the SPT score. A negative statistically significant correlation (Spearman's, p = 0.0001; Pearson's, p < 0.001) was identified between the Fazekas score and the results of SPT. No statistically significant differences were identified in the correlation of WMH and SPT between males and females. CONCLUSION: Multiple WMHs in the brain can worsen word recognition in patients with normal auditory threshold; this may be related to the impact that these lesions have on the memory ability. Spread of lesions into the brain might reduce the brain capacity to remember words, despite the sound is correctly perceived by the ear.

Assistance Dogs for Persons with Hearing Impairment: A Review.

The use of dogs to help people with disabilities has been known for a long time. Assistance dogs carry out a variety of practical tasks for disabled people with appropriate and targeted training, including assisting deaf persons or people with profound hearing loss. The benefits of assistance dogs for persons with hearing impairment (hearing dogs) include a) improved ability to carry out daily tasks through the codified reporting of sounds proper of everyday life and/or of dangerous situations and b) psychosocial aspects such as companionship and sense of protection. The benefits derived from the use of assistance dogs for persons with hearing impairment are less studied compared to those of assistance dogs employed for other disabilities. Moreover, the role of hearing dogs may appear rather controversial considering technological advances in the field of surgical or prosthetic rehabilitation for people with hearing impairment. This article aims to review features and training of hearing dogs, the effects of their employment and legislative aspects for their owners.

Workplace noise exposure and audiometric thresholds in dental technicians.

Noise is a well-known risk factor in occupational medicine. Several studies have been performed in workplaces with noise sources, especially in the industrial field; on the contrary, only a few studies have been carried to evaluate the noise exposure effects in non-industrial workplaces such as small factories, handicraft laboratories, and dental laboratories. The aims of this study were to evaluate workplace noise exposure and hearing thresholds in dental technicians. Four laboratories and 51 dental technicians were included in the study. Noise exposure levels during a nominal eight-hour working day (LEX, 8 h) were assessed in the included laboratories. Audiometric thresholds with pure tone audiometry were performed in 51 dental technicians, and results were compared with those expected in subjects not exposed to noise. The environmental noise measures showed moderate differences of the LEX, 8 h among the four laboratories (range 71.4 to 76.2); average LEX, 8 h was 73.9 ± 2.2 dB(A). The audiometric results showed a progressive increase of hearing threshold values at the frequencies mostly involved in noise-induced hearing loss (3, 4 and 6 kHz) and a correlation with age and working seniority especially in males (p<0.005). Nevertheless, in the 92.1% of subjects the threshold increases were in line with those expected in subjects of the same age and sex not exposed to noise and in the remaining 7.8% were not statistically significant (p>0.05). In 3.9% of the cases the increases were bilateral, typical of noise-induced hearing loss, and only 1.9% showed involvement of several frequencies with worsening of expected thresholds >25 dB. In conclusion, our study showed that exposure to noise in dental laboratories was not sufficient to represent a hazard to hearing, as demonstrated by the LEX, 8 h, which were below 80 dB(A) and therefore below the European exposure limit values and exposure action values for workers.

Adaptation of the STARR test for adult Italian population: A speech test for a realistic estimate in real-life listening conditions.

OBJECTIVES: To introduce the Italian adaptation of the STARR test based on a roving-level adaptive method to mimic challenging real-life listening conditions for use in people with auditory prostheses. DESIGN: Normative data were collected and interlist-variability, as well as learning effects, were investigated using a within-subject design with repeated measures. STUDY SAMPLE: A group of 32 normal-hearing (NH) adults participated in the study. RESULTS: The average speech reception threshold (SRT) for NH subjects was -8.4 dB SNR. The variability of mean SRTs across test lists was relatively small (≤1 dB for all test lists). The statistically significant differences between lists were eliminated after applying correction factors. On the basis of variability for the corrected SRTs within each subject, a difference of 2.8 dB in SRT was meaningful for outcome comparisons using one test list per condition and 2 dB using two lists per condition. Statistical analysis did not show any significant learning effects. CONCLUSIONS: Findings in NH listeners suggested that the Italian STARR test could be a promising supplement to existing speech assessment tools. Further studies in populations with hearing impairment could contribute to cross-language studies.

Adequate formal language performance in unilateral cochlear implanted children: is it indicative of complete recovery in all linguistic domains? Insights from referential communication.

OBJECTIVES: Referential communication (RC) is a key element in achieving a successful communication. This case series aimed to evaluate RC in children with unilateral cochlear implants (CIs) with formal language skills within the normal range. METHODS AND MATERIALS: A total of 31 children with CIs, with language development within the normal range, were assessed using the Pragmatic Language Skills test (MEDEA). RESULTS: Of the children with CIs, 83.9% reached performance levels appropriate for their chronological ages. The results confirmed a positive effect of cochlear implantation on RC development, although difficulties remained in some CI users. CONCLUSIONS: The outcomes emphasize the need to pay greater attention to the pragmatic aspects of language, assessing them with adequate testing in the early phase after cochlear implantation. Clear knowledge of children's communicative competence is the key in optimizing their communicative environments in order to create the basis for future successful interpersonal exchanges and social integration.

Oral versus short-term intratympanic prednisolone therapy for idiopathic sudden hearing loss.

OBJECTIVES: To assess the therapeutic effectiveness of an intratympanic (IT) steroid protocol compared to a systemic steroid protocol. METHODS: A total of 265 consecutive patients presenting unilateral idiopathic sudden sensorineural hearing loss were divided into 2 groups. One group comprised 131 patients enrolled between May 2009 and May 2010, and the other consisted of 134 patients enrolled between June 2010 and June 2011; a total of 48 patients were excluded among the 2 groups. The first group received oral prednisone for 8 days in tapering doses; the second group had IT prednisolone at a dose of 62.5 mg/ml once a day for 3 consecutive days. Audiological examinations were performed at study entry and 30 days after the beginning of therapy. Mean pure tone audiometry (PTA) of both groups and hearing outcomes following the criteria of Furuhashi et al. [Clin Otolaryngol 2002;27:458-463] and Siegel [Otolaryngol Clin North Am 1975;8:467-473] were investigated. RESULTS: The strong efficacy of steroid therapy was evident in both groups, observing both PTA and hearing threshold improvement. The evaluation of the hearing outcomes shows a significantly better result for the short-term IT protocol; this result is ascribable to two types of audiometric curves: down- and up-sloping. CONCLUSION: The results show a significant efficacy of both steroid therapeutic approaches. There was no significant difference in PTA improvement between the 2 study groups; the short-term IT protocol led to better results in the evaluation of the hearing outcomes (following the criteria of Siegel and Furuhashi et al.) for up- and down-sloping audiometric curves.