Five-Year Longitudinal Cohort Study Determines the Critical Intervals for Periodic Audiometric Testing Based on 5070 Tests of Metallurgical Workers Exposed and Nonexposed to Noise.

OBJECTIVES: To compare the progression of 3-, 4-, and 6-kHz thresholds (pure-tone average) over 5 years and determine the most critical period for occupational risk among workers exposed and nonexposed to noise. DESIGN: Metallurgical workers were divided into 2 groups: noise-exposed and non-noise-exposed groups. The 6 initial audiometric tests of each worker were analyzed as baseline test and annual tests 1 to 5. RESULTS: A total of 845 workers were included, 748 in the noise-exposed group and 97 in the non-noise-exposed group, resulting in 5070 tests analyzed. The nonexposed group showed no significant difference in the mean pure-tone averages between any of the annual tests in either ear. In the exposed group, a significant difference was observed in mean pure-tone averages between baseline and Test1 (p = 0.001 right ear; p = 0.001 left ear), between Test3 and Test4 (p = 0.002 right ear; p = 0.005 left ear), and between Test4 and Test5 (p = 0.003 right ear; p = 0.001 left ear). There was no difference between Test1 and Test2 or between Test2 and Test3 in either ear. CONCLUSIONS: The progression of pure-tone averages at 3, 4, and 6 kHz differed between workers exposed and nonexposed to noise. Noise-exposed workers had a significant progressive worsening of audiometric thresholds after 3 years of employment. This study identified, in an unprecedented way, two critical periods of noise exposure: in the first year and after the third year of employment in a noisy environment.

Effect of Noise Exposure on 1,382 Metallurgical Workers in Periodic Audiometric Evaluation: A Cohort Study.

INTRODUCTION: Noise-induced hearing loss is the most preventable cause of auditory impairment. Periodic audiometric evaluations are essential to monitor the hearing health of noise-exposed workers. OBJECTIVE: To compare the evolution of audiometric thresholds in the initial three evaluations at frequencies of 3, 4, and 6 kHz in groups of workers exposed or not to noise. METHODS: In this historical cohort study, audiometric evaluations were obtained from male workers between 18 and 40 years of age at six different metallurgical companies in Brazil. The workers were separated into noise-exposed and non-noise-exposed groups. The mean thresholds for 3, 4, and 6 kHz were calculated for both ears at baseline and the first and second periodic evaluations. The non-parametric Wilcoxon test was used for statistical analysis. RESULTS: A total of 1,382 metallurgical workers were evaluated (1,199 noise-exposed and 183 non-noise-exposed). There was a significant difference between baseline and the first periodic evaluation (right ear - effect size = 0.62; p = 0.0030 and left ear - effect size = 0.74; p = 0.0063) and between baseline and the second periodic evaluation (right ear - effect size = 0.85; p = 0.004 and left ear - effect size = 0.96; p = 0.0002). In the non-noise-exposed group, there was no difference between baseline and the first periodic evaluation (right ear - effect size = 0.18; p = 0.2703 and left ear - effect size = 0.12; p = 0.7907) and between baseline and the second periodic evaluation (right ear - effect size = 0.29; p = 0.4475 and left ear - effect size = 0.41; p = 0.6381). CONCLUSION: In noise-exposed workers, there was a significant worsening of audiometric thresholds between baseline and the initial periodic evaluation, but there was no difference between the two post-baseline evaluations. This shows that noise exposure can quickly affect hearing, despite protective measures.

Cochlear implants: Insertion assessment by computed tomography.

BACKGROUND AND OBJECTIVES: Imaging exams play a key role in cochlear implants with regard to both planning implantation before surgery and quality control after surgery. The ability to visualize the three-dimensional location of implanted electrodes is useful in clinical routines for assessing patient outcome. The aim of this study was to evaluate linear and angular insertion depth measurements of cochlear implants based on conventional computed tomography. METHODS: Tools for linear and angular measurements of cochlear implants were used in computed tomography exams. The tools realized the insertion measurements in an image reconstruction of the CIs, based on image processing techniques. We comprehensively characterized two cochlear implant models while obviating possible changes that can be caused by different cochlea sizes by using the same human temporal bones to evaluate the implant models. RESULTS: The tools used herein were able to differentiate the insertion measurements between two cochlear implant models widely used in clinical practice. We observed significant differences between both insertion measurements because of their different design and construction characteristics (p = 0.004 and 0.003 for linear and angular measurements, respectively; t-test). The presented methodology showed to be a good tool to calculate insertion depth measurements, since it is easy to perform, produces high-resolution images, and is able to depict all the landmarks, thus enabling measurement of the angular and linear insertion depth of the most apical electrode contacts. CONCLUSION: The present study demonstrates practical and useful tools for evaluating cochlear implant electrodes in clinical practice. Further studies should measure preoperative and postoperative benefits in terms of speech recognition and evaluate the preservation of residual hearing in the implanted ear. Such studies can also determine correlations between surgical factors, electrode positions, and performance. In addition to refined surgical techniques, the precise evaluation of cochlear length and correct choice of cochlear implant characteristics can play an important role in postoperative outcomes.

Analysis of mitochondrial alterations in Brazilian patients with sensorineural hearing loss using MALDI-TOF mass spectrometry.

BACKGROUND: Mutations in the mitochondrial DNA (mtDNA) have been associated with aminoglycoside-induced and nonsyndromic deafness in different populations. In the present study, we investigated the contribution of mutations in mitochondrial genes to the etiology of hearing loss in a Brazilian sample. METHODS: Using mass spectrometry genotyping technology, combined with direct sequencing, 50 alterations previously described in 14 mitochondrial genes were screened in 152 patients with sensorineural hearing loss and in104 normal hearing controls. RESULTS: Fifteen known mitochondrial alterations were detected (G709A, A735G, A827G, G988A, A1555G, T4363C, T5628C, T5655C, G5821A, C7462T, G8363A, T10454C, G12236A, T1291C, G15927A). Pathogenic mutations in MT-RNR1 and MT-TK genes were detected in 3 % (5/152) of the patients with hearing loss. CONCLUSIONS: This study contributed to show the spectrum of mitochondrial variants in Brazilian patients with hearing loss. Frequency of A1555G was relatively high (2.6 %), indicating that this mutation is an important cause of hearing loss in our population. This work reports for the first time the investigation and the detection of the tRNA(Lys) G8363A mutation in Brazilian patients with maternally inherited sensorineural hearing loss.

Screening of genetic alterations related to non-syndromic hearing loss using MassARRAY iPLEX® technology.

BACKGROUND: Recent advances in molecular genetics have enabled to determine the genetic causes of non-syndromic hearing loss, and more than 100 genes have been related to the phenotype. Due to this extraordinary genetic heterogeneity, a large percentage of patients remain without any molecular diagnosis. This condition imply the need for new methodological strategies in order to detect a greater number of mutations in multiple genes. In this work, we optimized and tested a panel of 86 mutations in 17 different genes screened using a high-throughput genotyping technology to determine the molecular etiology of hearing loss. METHODS: The technology used in this work was the MassARRAY iPLEX® platform. This technology uses silicon chips and DNA amplification products for accurate genotyping by mass spectrometry of previous reported mutations. The generated results were validated using conventional techniques, as direct sequencing, multiplex PCR and RFLP-PCR. RESULTS: An initial genotyping of control subjects, showed failures in 20 % of the selected alterations. To optimize these results, the failed tests were re-designed and new primers were synthesized. Then, the specificity and sensitivity of the panel demonstrated values above 97 %. Additionally, a group of 180 individuals with NSHL without a molecular diagnosis was screened to test the diagnostic value of our panel, and mutations were identified in 30 % of the cases. In 20 % of the individuals, it was possible to explain the etiology of the HL. Mutations in GJB2 gene were the most prevalent, followed by other mutations in in SLC26A4, CDH23, MT-RNR1, MYO15A, and OTOF genes. CONCLUSIONS: The MassARRAY technology has the potential for high-throughput identification of genetic variations. However, we demonstrated that optimization is required to increase the genotyping success and accuracy. The developed panel proved to be efficient and cost-effective, being suitable for applications involving the molecular diagnosis of hearing loss.

Task force of the Brazilian Society of Otology - evaluation and management of peripheral facial palsy.

OBJECTIVE: To review key evidence-based recommendations for the diagnosis and treatment of peripheral facial palsy in children and adults. METHODS: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on peripheral facial palsy were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. RESULTS: The topics were divided into 2 main parts: (1) Evaluation and diagnosis of facial palsy: electrophysiologic tests, idiopathic facial palsy, Ramsay Hunt syndrome, traumatic peripheral facial palsy, recurrent peripheral facial palsy, facial nerve tumors, and peripheral facial palsy in children; and (2) Rehabilitation procedures: surgical decompression of the facial nerve, facial nerve grafting, surgical treatment of long-term peripheral facial palsy, and non-surgical rehabilitation of the facial nerve. CONCLUSIONS: Peripheral facial palsy is a condition of diverse etiology. Treatment should be individualized according to the cause of facial nerve dysfunction, but the literature presents better evidence-based recommendations for systemic corticosteroid therapy.

Optimization of simultaneous screening of the main mutations involved in non-syndromic deafness using the TaqMan® OpenArray™ Genotyping platform.

BACKGROUND: Hearing loss is the most common sensory deficit in humans, affecting approximately 10% of the global population. In developed countries, one in every 500 individuals suffers from severe to profound bilateral sensorineural hearing loss. For those up to 5 years old, the proportion is higher, at 2.7 in 1000 individuals, and for adolescents the average is 3.5 in 1000. Among the causes of hearing loss, more than 50% are related to genetic factors. To date, nearly 150 loci and 64 genes have been associated with hearing loss. Mutations in the GJB2 gene, which encodes connexin 26, constitute the main genetic cause. So far, more than 300 variations have been described in this gene.As a response to the clinical and genetic heterogeneity of hearing loss and the importance of correct molecular diagnosis of individuals with hereditary hearing loss, this study worked in the optimization for a diagnostic protocol employing a high-throughput genotyping technology. METHODS: For this work, was used the TaqMan® OpenArray™ Genotyping platform. This is a high performance, high-throughput technology based on real-time PCR, which enables the evaluation of up to 3072 SNPs (Single Nucleotide Polymorphisms), point mutations, small deletions, and insertions, using a single genotyping plate. For the study, were selected the layout allowing to analyze 32 alterations in 96 individuals simultaneously. In the end, the generated results were validated by conventional techniques, as direct sequencing, Multiplex PCR and RFLP-PCR. RESULTS: A total of 376 individuals were analyzed, of which 94 were healthy controls, totaling 4 plates in duplicate. All 31 of the changes analyzed were present in the nuclear genes GJB2, GJB6, CRYL1, TMC1, SLC26A4, miR-96, and OTOF, and in the mitochondrial genes MT-RNR1 and MT-TS1. The reactions were subsequently validated by established techniques (direct sequencing, multiplex PCR, and RFLP-PCR) that had previously been used to perform molecular screening of hearing loss at the Human Genetics Laboratory of the Center for Molecular Biology and Genetic Engineering (CBMEG), at the State University of Campinas (UNICAMP). In total, 11,656 genotyping reactions were performed. Of these, only 351 reactions failed, representing approximately 3.01% of the total. The average accuracy of genotyping using the OpenArray™ plates was 96.99%. CONCLUSIONS: The results demonstrated the accuracy, low cost, and good reproducibility of the technique, indicating that the TaqMan® OpenArray™ Genotyping Platform is a useful and reliable tool for application in molecular diagnostic testing of hearing loss.

Etiologic and diagnostic evaluation: algorithm for severe to profound sensorineural hearing loss in Brazil.

OBJECTIVE: Evaluation of the effectiveness of imaging and genetic testing, and establishment of a cost-effective diagnostic protocol for the etiologic diagnosis of sensorineural hearing loss (SNHL) in Brazil. DESIGN: Prospective cohort study. STUDY SAMPLE: Analysis of 100 unrelated Brazilian patients with severe to profound bilateral SNHL submitted to cochlear implant (CI) between 2002 and 2010 at the University of Campinas hospital. The study was based upon three groups: individuals with congenital, progressive, and sudden SNHL. RESULTS: After the diagnostic investigation, the number of cases with unknown etiology was reduced from 72 to 42 (a 42% reduction); 25% of cases were due to environmental factors, 19% to genetic causes, and 14% to inner-ear abnormalities or other clinical features. The genetic and imaging findings contributed to the diagnosis of SNHL in 19% and 20% of the cases analysed, respectively. Molecular testing mainly contributed to the diagnosis of patients with congenital SNHL, while the contribution of radiologic examination was higher for individuals with progressive or sudden SNHL. A sequential diagnostic protocol was proposed based on these data. CONCLUSIONS: The proposed diagnostic workup algorithm could provide better optimization of etiologic diagnosis, as well as reduced costs, compared to a simultaneous testing approach.

Are hearing protection devices used in the workplace really efficient? A systematic review.

BACKGROUND: The most common current hearing protection devices (HPDs) on the market include earplugs and earmuffs. A variety of materials can be used to manufacture these devices, and each offers a level of noise attenuation that is informed by the manufacturer although it does not always correspond to the attenuation observed in real-world use. OBJECTIVE: To evaluate the noise attenuation of HPDs available to workers exposed to noise. METHODS: The most relevant studies originally published in English, Portuguese, or Spanish that investigated the noise attenuation effectiveness of HPDs used by workers exposed to noise were analyzed. The following electronic databases were searched by 2 independent reviewers for studies published from 1999 to 2019: MEDLINE (PubMed), Scopus, Web of Science, EMBASE, Cochrane Library (OVID), ProQuest, and BVS-Bireme. Different combinations of the following search terms (MeSH terms) were used for all databases: "Hearing Loss, Noise-Induced", "Ear Protective Devices" (Efficacy OR Effectiveness)", "Noise, Occupational". RESULTS: The search strategy yielded a total of 326 potentially relevant studies. After the removal of duplicates, 156 remained for the screening of titles and abstracts. After reviewing titles and abstracts, 46 studies were selected for full-text reading. Of these, six were included in this systematic review. CONCLUSION: Hearing protection devices reduced the noise exposure and were effective in all included studies in different countries, types of activity, and sound pressure exposure.

Association of Clinical Aspects and Genetic Variants with the Severity of Cisplatin-Induced Ototoxicity in Head and Neck Squamous Cell Carcinoma: A Prospective Cohort Study.

BACKGROUND: Cisplatin (CDDP) is a major ototoxic chemotherapy agent for head and neck squamous cell carcinoma (HNSCC) treatment. Clinicopathological features and genotypes encode different stages of CDDP metabolism, as their coexistence may influence the prevalence and severity of hearing loss. METHODS: HNSCC patients under CDDP chemoradiation were prospectively provided with baseline and post-treatment audiometry. Clinicopathological features and genetic variants encoding glutathione S-transferases (GSTT1, GSTM1, GSTP1), nucleotide excision repair (XPC, XPD, XPF, ERCC1), mismatch repair (MLH1, MSH2, MSH3, EXO1), and apoptosis (P53, CASP8, CASP9, CASP3, FAS, FASL)-related proteins were analyzed regarding ototoxicity. RESULTS: Eighty-nine patients were included, with a cumulative CDDP dose of 260 mg/m2. Moderate/severe ototoxicity occurred in 26 (29%) patients, particularly related to hearing loss at frequencies over 3000 Hertz. Race, body-mass index, and cumulative CDDP were independent risk factors. Patients with specific isolated and combined genotypes of GSTM1, GSTP1 c.313A>G, XPC c.2815A>C, XPD c.934G>A, EXO1 c.1762G>A, MSH3 c.3133A>G, FASL c.-844A>T, and P53 c.215G>C SNVs had up to 32.22 higher odds of presenting moderate/severe ototoxicity. CONCLUSIONS: Our data present, for the first time, the association of combined inherited nucleotide variants involved in CDDP efflux, DNA repair, and apoptosis with ototoxicity, which could be potential predictors in future clinical and genomic models.

Task force Guideline of Brazilian Society of Otology - hearing loss in children - Part II - Treatment.

OBJECTIVES: To provide an overview of the main evidence-based recommendations for the diagnosis of hearing loss in children and adolescents aged 0-18 years. METHODS: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on childhood hearing loss were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. RESULTS: The topics were divided into 2 parts: (1) treatment of sensorineural hearing loss: individual hearing aids, bilateral cochlear implants, cochlear implants in young children, unilateral hearing loss, and auditory neuropathy spectrum disorder; and (2) treatment of conductive/mixed hearing loss: external/middle ear malformations, ventilation tube insertion, and tympanoplasty in children. CONCLUSIONS: In children with hearing loss, in addition to speech therapy, Hearing AIDS (HAs) or implantable systems may be indicated. Even in children with profound hearing loss, both the use of HAs and behavioral assessments while using the device are important.

Task force Guideline of Brazilian Society of Otology ‒ hearing loss in children - Part I ‒ Evaluation.

OBJECTIVES: To provide an overview of the main evidence-based recommendations for the diagnosis of hearing loss in children and adolescents aged 0 to 18 years. METHODS: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on childhood hearing loss were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. RESULTS: The evaluation and diagnosis of hearing loss: universal newborn hearing screening, laboratory testing, congenital infections (especially cytomegalovirus), genetic testing and main syndromes, radiologic imaging studies, vestibular assessment of children with hearing loss, auditory neuropathy spectrum disorder, autism spectrum disorder, and noise-induced hearing loss. CONCLUSIONS: Every child with suspected hearing loss has the right to diagnosis and appropriate treatment if necessary. This task force considers 5 essential rights: (1) Otolaryngologist consultation; (2) Speech assessment and therapy; (3) Diagnostic tests; (4) Treatment; (5) Ophthalmologist consultation.

Brazilian Society of Otology task force - Vestibular Schwannoma ‒ evaluation and treatment.

OBJECTIVE: To review the literature on the diagnosis and treatment of vestibular schwannoma. METHODS: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on vestibular schwannoma were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. RESULTS: The topics were divided into 2 parts: (1) Diagnosis - audiologic, electrophysiologic tests, and imaging; (2) Treatment - wait and scan protocols, surgery, radiosurgery/radiotherapy, and systemic therapy. CONCLUSIONS: Decision making in VS treatment has become more challenging. MRI can diagnose increasingly smaller tumors, which has disastrous consequences for the patients and their families. It is important to develop an individualized approach for each case, which highly depends on the experience of each surgical team.

Brazilian Society of Otology task force - Otosclerosis: evaluation and treatment.

OBJECTIVES: To review and provide evidence-based recommendations for the diagnosis and treatment of otosclerosis. METHODS: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on otosclerosis were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. RESULTS: The topics were divided into 2 parts: 1) Diagnosis - audiologic and radiologic; 2) Treatment - hearing AIDS, pharmacological therapy, stapes surgery, and implantable devices - bone-anchored devices, active middle ear implants, and Cochlear Implants (CI). CONCLUSIONS: The pathophysiology of otosclerosis has not yet been fully elucidated, but environmental factors and unidentified genes are likely to play a significant role in it. Women with otosclerosis are not at increased risk of worsening clinical condition due to the use of contraceptives or during pregnancy. Drug treatment has shown little benefit. If the patient does not want to undergo stapedotomy, the use of hearing aids is well indicated. Implantable systems should be indicated only in rare cases, and the CI should be indicated in cases of profound deafness.

Is it necessary to perform occupational audiometric testing at 6-months of employment?

INTRODUCTION: Current Brazilian legislation requires that all workers exposed to noise are to be given an audiogram upon hiring, after 6 months of employment (first periodic test), and annually after the first periodic test. In other countries, the regulations of hearing conservation programs do not include the requirement for audiometric testing at 6 months of employment, but only annually. There is no evidence that the periodicity adopted by Brazilian legislation is the most appropriate. OBJECTIVE: The present study aimed to evaluate the first 3 occupational audiometric tests of workers exposed to noise. METHODS: Historical cohort study with cross-sectional analysis. Participants were all male metallurgy workers aged up to 40 years. The first 3 audiograms of each worker were analyzed: pre-employment audiometric test, periodic audiometric test 1, and periodic audiometric test 2. For each worker, mean frequency thresholds were calculated at 3, 4, and 6 kHz in the left and right ears for each test. Statistical analysis was performed using the nonparametric Wilcoxon test. RESULTS: A total of 988 workers were included. There was a significant difference in auditory thresholds between the pre-employment test and the 2 subsequent periodic tests for the right and left ears. There was no significant difference between Test1 and Test2 in either ear. CONCLUSION: Given the lack of difference between the first 2 periodic tests, we believe that they could be merged into a single test, i.e., first periodic audiometric testing could be performed at 12 months of employment without compromising workers' health.

SARS-CoV-2 infection with bilateral intralabyrinthine hemorrhage.

A 47-year-old woman presented with the complaint of sudden hearing loss associated with vertigo. Serological testing was positive for IgM and negative for IgG COVID-19 antibodies, with no other associated factors. Magnetic resonance imaging of the brain showed bilateral intralabyrinthine hemorrhage.

Bilateral facial paralysis secondary to temporal bone trauma: A case report and literature review.

When temporal bone fractures are associated with facial palsy (FP) evolution the medical team have no much time to make a decision: to do or not to do a surgical approach? How to evaluate the necessity? When to do it and when is the correct time?

Conservative management of petrous apex abscess and Gradenigo's syndrome in a diabetic patient: Case report and literature review.

The case reported evidences the possibility of nonsurgical treatment in patients with abscesses located in regions difficult to reach surgically due to surgical limitations or possible sequelae related to surgical procedure.

Progressive Asymmetry in Occupational Noise-Induced Hearing Loss: A Large Population-Based Cohort Study With a 15-Year Follow-Up.

BACKGROUND: To evaluate interaural differences between the right and left ears at frequencies from 0.25 to 8 kHz in 3 groups of workers from metallurgy companies. METHODS: This study is a cross-sectional cohort study. Workers were divided into 3 groups: (1) workers without occupational noise exposure and normal audiometric testing; (2) workers with 10 years of occupational noise exposure; and (3) workers with 15 years of occupational noise exposure. The interaural difference from 0.25 to 8 kHz was measured in each group. RESULTS: A total of 2103 workers were included. Of these, 483 workers had been exposed to noise in the workplace for 10 years and 216 workers for 15 years. Group 1, only at 4 and 6 kHz, the difference was statistically significant. Group 2, only at 3 , 4 , and 6 kHz, the difference was statistically significant. Group 3, the difference was statistically significant at the frequencies from 2 to 8 kHz. CONCLUSION: Asymmetry between the right and left ears was observed in all groups, with higher air-conduction thresholds in the left ear. It is important for otolaryngologists be aware that NIHL can also cause or accentuate asymmetry between the right and left ears over time.

Thorax necrotizing fasciitis following Bezold's abscess.

Bezold's abscess is a rare complication of acute otitis media, but it should be recognized and aggressively treated. Otolaryngologists must be aware of this diagnosis, and multidisciplinary care should be provided as soon as possible.