Brazilian Society of Otology task force - single sided deafness - recommendations based on strength of evidence.

OBJECTIVE: To make evidence-based recommendations for the treatment of Single-Sided Deafness (SSD) in children and adults. METHODS: Task force members were instructed 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 SSD 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 3 parts: (1) Impact of SSD in children; (2) Impact of SSD in adults; and (3) SSD in patients with temporal bone tumors. CONCLUSIONS: Decision-making for patients with SSD is complex and multifactorial. The lack of consensus on the quality of outcomes and on which measurement tools to use hinders a proper comparison of different treatment options. Contralateral routing of signal hearing aids and bone conduction devices can alleviate the head shadow effect and improve sound awareness and signal-to-noise ratio in the affected ear. However, they cannot restore binaural hearing. Cochlear implants can restore binaural hearing, producing significant improvements in speech perception, spatial localization of sound, tinnitus control, and overall quality of life. However, cochlear implantation is not recommended in cases of cochlear nerve deficiency, a relatively common cause of congenital SSD.

The Use of 99mTc-Mononuclear Leukocyte Scintigraphy for Necrotizing External Otitis Diagnosis.

BACKGROUND: Necrotizing external otitis (NEO) is a severe infectious disease in the external acoustic meatus (EAM) and mastoid that may extend to the cranial base. Due to the lack of a gold standard examination technique, the diagnosis is often difficult and delayed. This study aimed to evaluate the sensitivity and specificity of 99mTc-mononuclear leukocyte scintigraphy associated with 99mTc-phytate in suspected NEO compared to 99mTc-MDP and 67Ga-citrate. METHODS: A prospective study (32 patients) was conducted between 2011 and 2016. RESULTS: At the end, twenty-four patients remained for the study conduction; nineteen had confirmed NEO diagnosis, one had sarcoma, one had EAM cholesteatoma, one had diffuse simple external otitis, and two had an inconclusive diagnosis. 99mTc-mononuclear leukocyte scintigraphy plus 99mTc-phytate was as sensitive as 99mTc-MDP bone scintigraphy (19/19X9/19), and more sensitive than 67Ga scintigraphy (19/19 x 17/19). Regarding specificity, it was superior to bone scintigraphy, 100% × 40% (5/5 × 2/5), and 67Ga scintigraphy, 100% × 20% (5/5 × 1/5). After the infection resolution, all NEO patients had their leukocyte scintigraphy negativized. To the best of our knowledge, this is the first study that evaluates this technique in patients with suspected NEO. CONCLUSIONS: 99mTc-mononuclear leukocyte was revealed to be the best option for NEO because of its specificity.

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.

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.

Frequency of GJB2 mutations in patients with nonsyndromic hearing loss from an ethnically characterized Brazilian population.

INTRODUCTION: In different parts of the world, mutations in the GJB2 gene are associated with nonsyndromic hearing loss, and the homozygous 35delG mutation (p.Gly12Valfs*2) is a major cause of hereditary hearing loss. However, the 35delG mutation is not equally prevalent across ethnicities, making it important to study other mutations, especially in multiethnic countries such as Brazil. OBJECTIVE: This study aimed to identify different mutations in the GJB2 gene in patients with severe to profound nonsyndromic sensorineural hearing loss of putative genetic origin, and who were negative or heterozygote for the 35delG mutation. METHODS: Observational study that analyzed 100 ethnically characterized Brazilian patients with nonsyndromic severe to profound sensorineural hearing loss, who were negative or heterozygote for the 35delG mutation. GJB2 mutations were detected by DNA-based sequencing in this population. Participants' ethnicities were identified as Latin European, Non-Latin European, Jewish, Native, Turkish, Afro-American, Asian and Others. RESULTS: Sixteen participants were heterozygote for the 35delG mutation; 14 participants, including three 35delG heterozygote's, had nine different alterations in the GJB2 gene. One variant, p.Ser199Glnfs*9, detected in two participants, was previously unreported. Three variants were pathogenic (p.Trp172*, p.Val167Met, and p.Arg75Trp), two were non-pathogenic (p.Val27Ile and p.Ile196Thr), and three variants were indeterminate (p.Met34Thr, p.Arg127Leu, and p.Lys168Arg). Three cases of compound heterozygosity were detected: p.[(Gly12Valfs*2)];[(Trp172*)], p.[(Gly12Valfs*2)](;)[(Met34Thr)], and p.[(Gly12Valfs*2)(;)[(Ser199Glnfs*9)]). CONCLUSION: This study detected previously unclassified variants and one case of previously unreported compound heterozygosity.

Laryngocele: a cause of upper airway obstruction.

Laryngoceles are abnormal dilatations of the laryngeal saccule, which rises between the ventricular folds, the base of the epiglottis and the inner surface of the thyroid cartilage. Clinical symptoms are rare, and the find of asymptomatic laryngoceles in pathology studies are frequent. Sometimes it is presented as cervical swelling causing airway obstruction in need of emergency intervention. In this study, we report a case of upper airway obstruction due to laryngocele treated by emergency tracheotomy and we review of the literature. Laryngocele complications include infection (pyocele formation), pathogens aspirations with subsequent bronchitis and pneumonia and upper airway obstruction, as in the case reported. Despite being benign tumors, laryngoceles cause relevant airway obstruction. Correct diagnosis and proper treatment can preclude emergencies as it happened to our patient hereby reported.

The role of new vaccines in the prevention of otitis media.

Otitis media is one of the most common infectious diseases of infancy; a reduction in its incidence would have a significant economic and social impact. Vaccines may play a role in the prevention of otitis media. This report discusses vaccines against pneumococci and influenza viruses. We reviewed the literature for results of studies examining the role of these vaccines in the prevention of otitis media. The 23-valent polysaccharide anti-pneumococcal vaccine did not modify the incidence of otitis media in children aged 2 years less, the age group with the highest incidence of otitis. The heptavalent anti-pneumococcal vaccine did not significantly reduce the incidence of otitis media overall. This vaccine did, however, reduce the number of episodes of otitis media with effusion and the number of recurrences; it also altered the profile of causative microorganisms by increasing otitis caused by different microorganisms. We found the inactivated anti-influenza virus vaccine to be effective in reducing otitis media during peak incidence periods of influenza. As these new vaccines are currently available in Brazil, otolaryngologists must be aware of their potential role and impact in the reduction of otitis media, to counsel patients appropriately.

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

Abstract 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.

Nasal histoplasmosis in the acquired immunodeficiency syndrome.

Disseminated histoplasmosis is a disease with a high case-fatality rate, especially in patients with the acquired immunodeficiency syndrome (AIDS). The disease can occur in various sites, such as the lungs, eyes, oral cavity, larynx, nervous system, gastrointestinal tract and, more rarely, the nasal sinus region. It is a cosmopolitan mycosis with a high prevalence in Brazil. Nasal manifestation of the disease is rare, with only three cases reported in the literature, but it is part of the differential diagnosis for other granulomatous diseases, such as Wegener's granulomatosis, tegumentary leishmaniasis and nasal lymphoma. The authors of this study present a literature review and report a case of nasal histoplasmosis in a patient with AIDS. No record of such an aggressive presentation has been reported previously in the literature.

Brazilian study on substance misuse in adolescents: associated factors and adherence to treatment.

OBJECTIVES: To investigate developmental and environmental factors associated to substance misuse in adolescents seen at a university day-hospital in Brazil and to verify the correlations between those factors and adherence to treatment. To compare factors associated to substance misuse in adolescents with the available scientific literature and to suggest specific preventive interventions for a national policy in Brazil. METHODS: Eighty-six adolescent's guardians were evaluated at admission to the service by using a semistructured interview including sociodemographic data, family relationship, perinatal and pregnancy histories, psychomotor and educational development, social relations, history of previous illnesses and family diseases, including drug abuse. RESULTS: The sample was predominantly male (90%). Adolescents referred from the criminal justice were older than those originating from other sources (16.4 x 15.4 years old p=.00). Forty-four percent of adolescents reported school failure, a level which is two times higher than Brazilian statistics. Forty percent of the sample had criminal involvement, mainly drug dealing. Cannabis was the most prevalent reported drug. Living with both parents was protective, delaying the age of initiation by one year. Domestic violence was more frequent among parents with illicit drugs abuse (38.1% x 12.5%, p<.05). Alcoholism and drug addiction in parents and relatives were about four times higher than those reported for the Brazilian population. No correlation was found between the investigated factors and adherence to treatment. CONCLUSION: Our results indicate that the programs must include treatment of adults and education of parents and parents to be. Withdrawal of treatment occurs in the first month, and seems to be related to factors extrinsic to the adolescent. Substance misuse. Adolescent. Treatment.

Frequency of GJB2 mutations in patients with nonsyndromic hearing loss from an ethnically characterized Brazilian population / Frequência de mutações de GJB2 em pacientes com perda auditiva não sindrômica em uma população brasileira etnicamente caracterizada

Abstract Introduction: In different parts of the world, mutations in the GJB2 gene are associated with nonsyndromic hearing loss, and the homozygous 35delG mutation (p.Gly12Valfs*2) is a major cause of hereditary hearing loss. However, the 35delG mutation is not equally prevalent across ethnicities, making it important to study other mutations, especially in multiethnic countries such as Brazil. Objective: This study aimed to identify different mutations in the GJB2 gene in patients with severe to profound nonsyndromic sensorineural hearing loss of putative genetic origin, and who were negative or heterozygote for the 35delG mutation. Methods: Observational study that analyzed 100 ethnically characterized Brazilian patients with nonsyndromic severe to profound sensorineural hearing loss, who were negative or heterozygote for the 35delG mutation. GJB2 mutations were detected by DNA-based sequencing in this population. Participants' ethnicities were identified as Latin European, Non-Latin European, Jewish, Native, Turkish, Afro-American, Asian and Others. Results: Sixteen participants were heterozygote for the 35delG mutation; 14 participants, including three 35delG heterozygote's, had nine different alterations in the GJB2 gene. One variant, p.Ser199Glnfs*9, detected in two participants, was previously unreported. Three variants were pathogenic (p.Trp172*, p.Val167Met, and p.Arg75Trp), two were non-pathogenic (p.Val27Ile and p.Ile196Thr), and three variants were indeterminate (p.Met34Thr, p.Arg127Leu, and p.Lys168Arg). Three cases of compound heterozygosity were detected: p.[(Gly12Valfs*2)];[(Trp172*)], p.[(Gly12Valfs*2)](;)[(Met34Thr)], and p.[(Gly12Valfs*2)(;)[(Ser199Glnfs*9)]). Conclusion: This study detected previously unclassified variants and one case of previously unreported compound heterozygosity.

Resumo Introdução: Em diferentes partes do mundo, mutações do gene GJB2 estão associadas a perda auditiva não sindrômica e a mutação homozigótica 35delG (p.Gly12Valfs*2) é uma das principais causas de perda auditiva hereditária. No entanto, a mutação 35delG não é igualmente prevalente em todas as etnias, faz com que seja importante estudar outras mutações, especialmente em países multiétnicos, como o Brasil. Objetivo: Identificar diferentes mutações no gene GJB2 em pacientes com perda auditiva neurossensorial grave ou profunda não sindrômica de origem genética putativa e negativos ou heterozigotos para a mutação 35delG. Método: Estudo observacional que analisou 100 pacientes brasileiros caracterizados etnicamente, com perda auditiva neurossensorial grave ou profunda não sindrômica, negativos ou heterozigotos para a mutação 35delG. As mutações de GJB2 foram detectadas por sequenciamento baseado no DNA nessa população. As etnias dos participantes foram identificadas como latino-europeia, não latino-europeia, judaica, nativa, turca, negra, asiática e outras. Resultados: Dezesseis participantes eram heterozigotos para a mutação 35delG e 14, incluindo três heterozigotos para 35delG, apresentaram nove alterações no gene GJB2. Uma variante, p.Ser199Glnfs*9, detectada em dois participantes, não havia sido relatada anteriormente. Três variantes eram patogênicas (p.Trp172*, p.Val167Met, e p.Arg75Trp), duas não patogênicas (p.Val27Ile e p.Ile196Thr) e três indeterminadas (p.Met34Thr, p.Arg127Leu, e p.Lys168Arg). Três casos de heterozigosidade composta foram detectados: p.[(Gly12Valfs*2)];[(Trp172*)], p.[(Gly12Valfs*2)](;)[(Met34Thr)], e p.[(Gly12Valfs*2)(;)[(Ser199Glnfs*9)]). Conclusão: Este estudo detectou variantes não classificadas anteriormente e um caso de heterozigosidade composta ainda não relatada.

Profile of patients assessed for cochlear implants.

INTRODUCTION: Knowledge of the characteristics related to profound hearing loss is a matter of great importance, as it allows for the etiological and prognostic identification and strategic planning for public health interventions. OBJECTIVE: To assess the different etiologies of hearing loss, age at diagnosis of the hearing loss, its relation to language acquisition, and the age at the first consultation in this service for cochlear implant assessment. METHODS: This was a historical cohort, cross-sectional study, using retrospective analysis of the records of 115 patients with confirmed sensorineural hearing loss, who were followed in a university hospital, based on gender, age of hearing loss, age at the first consultation, language, and hearing loss etiology. RESULTS AND CONCLUSION: The majority of patients assessed for cochlear implants attend the first consultation when they are older than one year (an alarming mean of 3.8 years in the prelingual group) in spite of the early diagnosis of hearing loss. This reflects an already deficient health care system, in terms of referral. The idiopathic cause remains the most frequently identified. Among the known causes, the most prevalent are perinatal causes and meningitis.

Antibiotic prophylaxis in otolaryngologic surgery.

AIM: Antibiotic prophylaxis aims to prevent infection of surgical sites before contamination or infection occurs. Prolonged antibiotic prophylaxis does not enhance the prevention of surgical infection and is associated with higher rates of antibiotic-resistant microorganisms. This review of the literature concerning antibiotic prophylaxis, with an emphasis on otolaryngologic surgery, aims to develop a guide for the use of antibiotic prophylaxis in otolaryngologic surgery in order to reduce the numbers of complications stemming from the indiscriminate use of antibiotics.

Nasal septum perforation in patient with pyoderma gangrenosum.

INTRODUCTION: The cocaine is obtained from the leaves of the coca (Erythroxylon coca). It can be used in many ways, but the most common is the drug inhalation. The Cocaine also causes vasoconstriction at nasal mucous membrane and its chronic use can cause necrosis and nasal septum perforation. Pyoderma gangrenosum is an uncommon idiopathic disease characterized by ulcerations, usually observed on the legs. Its diagnosis is most common an exclusion of others diseases. So far, there is no specific treatment based on evidence by randomized controlled trials. OBJECTIVE: Describe the rare association between Pyoderma gangrenosum and cocaine. CASE REPORT: E. A., 27-year-old woman with destruction of nasal septum and palate who has been using a big amount of cocaine, been necessary note the difference from which disease cause de damage. Final Comments: Also there are only three cases of Pyoderma gangrenosum complicated with nasal septum perforation in cocaine users.

Profile of patients assessed for cochlear implants / Perfil dos pacientes em avaliação para implante coclear

INTRODUCTION: Knowledge of the characteristics related to profound hearing loss is a matter of great importance, as it allows for the etiological and prognostic identification and strategic planning for public health interventions. OBJECTIVE: To assess the different etiologies of hearing loss, age at diagnosis of the hearing loss, its relation to language acquisition, and the age at the first consultation in this service for cochlear implant assessment. METHODS: This was a historical cohort, cross-sectional study, using retrospective analysis of the records of 115 patients with confirmed sensorineural hearing loss, who were followed in a university hospital, based on gender, age of hearing loss, age at the first consultation, language, and hearing loss etiology. RESULTS AND CONCLUSION: The majority of patients assessed for cochlear implants attend the first consultation when they are older than one year (an alarming mean of 3.8 years in the prelingual group) in spite of the early diagnosis of hearing loss. This reflects an already deficient health care system, in terms of referral. The idiopathic cause remains the most frequently identified. Among the known causes, the most prevalent are perinatal causes and meningitis. .

INTRODUÇÃO: O conhecimento das características relacionadas à perda auditiva profunda é de extrema importância já que permite a identificação etiológica e de fatores prognósticos e planejamento estratégico para intervenções de saúde pública. OBJETIVO: Verificar as diferentes etiologias da perda auditiva, idade de diagnóstico da perda, relacionada ou não à aquisição de linguagem e idade dos pacientes ao procurar o serviço para a avaliação de implante coclear. MÉTODO: Estudo de casos, de coorte histórica transversal, com análise retrospectiva de arquivos de 115 pacientes com perda auditiva neurossensorial comprovada acompanhados num hospital universitário, com base em sexo, idade da perda, idade na primeira consulta, linguagem e etiologia da perda. RESULTADOS E CONCLUSÃO: os pacientes avaliados para implante coclear chegam à primeira consulta, em sua maioria, com mais de um ano de idade (média preocupante de 3,8 anos no grupo pré-lingual), apesar de o diagnóstico da perda ser precoce, o que reflete um sistema ainda deficiente em termos de referenciamento. A causa idiopática ainda é a mais encontrada. Dentre as etiologias conhecidas, as mais prevalentes são as perinatais e a meningite. .

Gustatory alteration evaluation in patients with chronic otitis media.

UNLABELLED: Many studies have shown the consequent gustatory alteration caused by ear surgeries. However, few have reported this alteration in patients with chronic otitis media (COM), prior to surgical treatment. AIM: to identify gustatory alterations due to chorda tympani nerve involvement in patients with COM without prior surgery. METHODS: Clinical essay, with tests based on 'taste strips' with different concentrations of salt, sweet, bitter, and sour, was performed in 45 patients with unilateral cholesteatomatous or suppurated COM not previously submitted to otological surgery, using the disease-free ear on the contralateral side as control. The score ranged between 0 and 16. RESULTS: A total of 25 patients presented cholesteatoma and 20 had non-cholesteatomatous disease. The mean score was 6.65 for the affected side and 9.93 for the half of the tongue on the side of the healthy ear (p<0.001). No patients had complained of gustatory alterations before the examination. Among the 24 patients with unilateral hypogeusia, eight cases of unilateral ageusia were found on the affected side. There was an association between cholesteatoma (p=0.055), disease duration (p=0.07) and worsening in gustatory sensitivity. CONCLUSION: Patients with COM can have gustatory alterations, even in the absence of complaints.

Antibiotic prophylaxis in otolaryngologic surgery

Aim: Antibiotic prophylaxis aims to prevent infection of surgical sites before contamination or infection occurs. Prolonged antibiotic prophylaxis does not enhance the prevention of surgical infection and is associated with higher rates of antibiotic-resistant microorganisms. This review of the literature concerning antibiotic prophylaxis, with an emphasis on otolaryngologic surgery, aims to develop a guide for the use of antibiotic prophylaxis in otolaryngologic surgery in order to reduce the numbers of complications stemming from the indiscriminate use of antibiotics...

Perfuração de septo nasal em paciente com pioderma gangrenoso / Nasal septum perforation in patient with pyoderma gangrenosum

Introdução: A cocaína é extraída das folhas do arbusto da coca (Erythroxylon coca), podendo ser consumida de várias formas, mas o modo mais comum é pela aspiração da droga, sendo absorvida pela mucosa nasal, causando vasoconstricção, levando o seu uso crônico à perfuração de septo nasal. Pioderma gangrenoso é uma doença inflamatória rara, idiopática que se caracteriza pela presença de úlceras destrutivas principalmente em membros inferiores. Seu diagnóstico clínico é muitas vezes de exclusão. Objetivo: Descrever a raridade de associação entre Pioderma Gangrenoso e cocaína. Relato de Caso: E. A., 27 anos, com apresentação atípica de Pioderma Gangrenoso com perfurações de septo nasal e palato duro usuária de grande quantidade de cocaína, sendo necessário diferenciar qual patologia causou esse dano. Comentários Finais: Além da necessidade dessa diferenciação, apenas existem três casos relatados na literatura, envolvendo o Pioderma Gangrenoso complicado com perfuração de septo nasal em usuários de cocaína...

Introduction: The cocaine is obtained from the leaves of the coca (Erythroxylon coca). It can be used in many ways, but the most common is the drug inhalation. The Cocaine also causes vasoconstriction at nasal mucous membrane and its chronic use can cause necrosis and nasal septum perforation. Pyoderma gangrenosum is an uncommon idiopathic disease characterized by ulcerations, usually observed on the legs. Its diagnosis is most common an exclusion of others diseases. So far, there is no specific treatment based on evidence by randomized controlled trials. Objective: Describe the rare association between Pyoderma gangrenosum and cocaine. Case Report: E. A., 27-year-old woman with destruction of nasal septum and palate who has been using a big amount of cocaine, been necessary note the difference from which disease cause de damage. Final Comments: Also there are only three cases of Pyoderma gangrenosum complicated with nasal septum perforation in cocaine users...