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Rubella virus (RUBV) infection during pregnancy can lead to fetal infection and cause miscarriage, fetal death, or congenital rubella syndrome (CRS), which includes birth defects such as cataracts, sensorineural hearing loss, heart defects, and mental retardation [1]. Despite availability of safe, effective, and inexpensive vaccines, the World Health Organization (WHO) estimated that 103 000 infants with CRS were born in 2010, mainly in developing countries that have not introduced rubella vaccination [1]. In 1998, the Technical Advisory Group on Vaccine-Preventable Diseases of the Region of the Americas recommended that all countries in the region incorporate rubella virus (RUBV)–containing vaccine (RCV) into their childhood vaccination program [2, 3]. In 2011, the WHO recommended that all countries take the opportunity offered by accelerated measles control and elimination activities to introduce RCV [4]. By 2012, 132 (68%) of 194 WHO Member States included RCV in their routine immunization programs [5]. Determining the burden of CRS-related disabilities may inform decisions regarding vaccine introduction. The CRS incidence has been measured in follow-up studies of women infected with RUBV during pregnancy [6, 7], obtained through CRS surveillance targeting children 133 confirmed rubella cases in pregnant women were reported in 1999 and 2000 [23]. Brazil developed a vaccination effort, using measles-rubella vaccine (containing Edmonston-Zagreb measles and RA 27/3 RUBV strains), to accelerate CRS prevention during 2000 and 2001. A second national rubella vaccination campaign, conducted in 2008, interrupted endemic rubella transmission; the last endemic CRS case occurred in 2009. The goal of the current serologic study was to characterize the immune responses of school-aged children with CRS and compare these responses with those of their biological mothers and a group of similarly aged children without CRS. We hypothesized that specific immune responses, including the persistence of low-avidity IgG antibody, the level of the IgG anti-E1 signal, and the level of the IgG anti-C signal, would be associated with CRS.

Patients with diffuse cutaneous systemic sclerosis (dcSSc) have distinct clinical manifestations, usually with more aggressive organ involvement and highermortality than limited cutaneous systemic sclerosis (lcSSC). Hearing loss had not yet been evaluated in this subtype, so our aim was to assess the auditory system of dcSSc patients in a controlled study.Twenty-six patients with dcSSc, according to American College of Rheumatology (ACR) criteria, were evaluated. For each dcSSc patient included in the study, two healthy controls were matched for sex and age (± 2 years). All patients responded to a structured questionnaire regarding otological symptoms and risk factors with regard to hearing loss. A complete ear/nose/throat physical examination was performed followed by speech/pure tone audiometry and impedance measurements.The mean age of the dcSSc patients was 47.0 ± 11.9 years; the mean disease duration was 9.4 ± 9.1 years, and there was a clear female predominance (83%). The questionnaire revealed auditory complaints in 54% of the dcSSc patients (25% hearing loss, 21% aural fullness, 21% tinnitus, and 21% dizziness) and normal otoscopy. Nearly half (46%) of the dcSSc patients were diagnosed as having hearing loss on audiogram compared to only 19% in the control group (p = 0.01). All dcSSc with hearing loss had a sensorineural component, eight (73%) with excellent discrimination on the Percentage Index of Speech Recognition (PISR). Descending configuration and Metz recruitment were observed in 54% of these patients, suggesting cochlear involvement.Patients with dcSSc have a high prevalence of sensorineural audiometric hearing impairment and otological complaints, suggesting that the cochlea is an additional target organ in this disease.

Conclusion: The extended retrolabyrinthine approach (RLA) is a safe and reliable approach for auditory brainstem placement in children. The surgical landmarks to reach cochlear nucleus are adequately exposed by this approach. Objective: To describe a new approach option for auditory brainstem implants (ABIs) in children, highlighting the anatomical landmarks to appropriately expose the foramen of Luschka. Methods: Three prelingually deafened children consecutively operated for ABIs via the RLA. Results: ABI placement via the RLA was successfully performed in all children without any further complications except multidirectional nystagmus in one child. The RLA we employed differed from that used for vestibular schwannoma only in the removal of the posterior semicircular canal. The lateral and superior semicircular canals and the vestibule remained intact, and there was no need to expose the dura of the internal auditory meatus. The jugular bulb was completely exposed to allow adequate visualizatio...

Cochlear implantation is a safe and reliable method for auditory restoration in patients with severe to profound hearing loss.To describe the surgical complications of cochlear implantation.Information from 591 consecutive multichannel cochlear implant surgeries were retrospectively analyzed. All patients were followed-up for at least one year. Forty-one patients were excluded because of missing data, follow-up loss or middle fossa approach.Of 550 cochlear implantation analyzed, 341 were performed in children or adolescents, and 209 in adults. The mean hearing loss time was 6.3 ± 6.7 years for prelingual loss and 12.1 ± 11.6 years for postlingual. Mean follow-up was 3.9 ± 2.8 years. Major complications occurred in 8.9% and minor in 7.8%. Problems during electrode insertion (3.8%) were the most frequent major complication followed by flap dehiscence (1.4%). Temporary facial palsy (2.2%), canal-wall lesion (2.2%) and tympanic membrane lesion (1.8%) were the more frequent minor complications. No death occurred.There was a low rate of surgical complications, most of them been successfully managed. These results confirm that cochlear implant is a safe surgery and most surgical complications can be managed with conservative measures or minimal intervention.

Neurofibromatosis 2 (NF2) is an autosomal dominant disease in which hearing loss is predominant. Auditory restoration is possible using cochlear implants (CI) or auditory brainstem implant (ABI). Objective: To assess the auditory results of CI and ABI in NF2 patients and review the literature. Methods: Four NF2 patients were prospectively evaluated. They were submitted to tumor resection followed by ipsilateral CI or ABI depending on cochlear nerve preservation. Long term auditory results were described for CI (12 months) and ABI (48 months). Results: All patients achieved auditory perception improvements in their hearing thresholds. The CI patient does not recognize vowels or sentences. The 3 ABI patients discriminate 70% of vowels and 86% in the 4-choice test. One of them does not recognize sentences. The other two recognize 100% of closed sentences and 10% and 20% of open sentences. Conclusion: The choice of implant type to restore hearing to NF2 patients will relay on anatomical and functional cochlear nerve preservation during tumor resection surgery. Although our experience was different, the literature shows that if this condition is achieved, CI will offer better auditory results. If not, ABI is recommended.

The Burnout syndrome can be characterized by abnormalities in three domains: emotional exhaustion (EE), depersonalization (DP) and lack of personal realization (PR). In the medical profession, oncologists are especially prone to this syndrome. This study evaluates its prevalence among cancer physicians in Brazil correlating it to their demographic, work related variables and seeks possible solutions to prevent burnout.We mailed three questionnaires (Maslach burnout inventory, general and opinion questionnaires) to all 645 members of the Brazilian Cancer Society and received 136 responses after 10 weeks.The response rate was of 21%. The burnout syndrome was present at moderate or severe levels in all three domains analyzed in 15.7% of the physicians. For each of these the frequency of moderate or severe dysfunction was analyzed and found to be present in 55.8% for EE, 96.1% for DP and 23.4% for RP. Practicing physical activity or having a hobby correlated significantly with lower levels of EE (p = 0.008) while working only for the private sector correlated with higher DP scores (p = 0.021). Cancer physicians pointed out that less paper work (73.5%) and a lower patient load (72.7%) were the most important factors for prevention of this syndrome.Burnout syndrome is frequent among Brazilian cancer physicians and further studies should be conducted to evaluate its prevalence and prevention among other sub specialists.

Characterize the profile of patients under palliative care at this institution and evaluate the prevalence of depression in these patients.Sixty two cancer patients under palliative care, who had answered three questionnaires: one regarding their demographic characteristics, another to evaluate their quality of life and the Beck's depression inventory were surveyed.Of these patients, 68% presented with some degree of depression. Most of them were aware of their diagnosis (87.1%), did not talk to their physicians on other subjects but their disease (81.18%), were satisfied with their treatment (93.33%) and with the support they received (95.70%). Pain, fatigue, weakness and sleep disturbances were the most frequently reported symptoms. There was a significant correlation between presence of depression and not knowing the diagnosis (p=0.008), being admitted to the hospital (p=0.0019) and not having ever received oncologic treatment.Patients under palliative care at this institution, despite being satisfied with the treatment, reported poor communication with their physicians and presented with a high rate of depression. Awareness of their diagnosis and having received prior oncologic treatment (p=0.07) correlated significantly and inversely with having depression.

A 15-YEAR-OLD GIRL PRESENTED WITH A 2-week history of progressive facial palsy on the right side. No other otologic symptoms were identified. Physical examination revealed only a facial palsy, which was classified as House-Brackmann scale grade V. There were no signs of involvement of other cranial nerves. The findings of otoscopy and palpation of the parotid gland were normal. Audiometry showed a normal hearing threshold and an absent stapedial reflex in the right ear. The patient was treated with oral dexamethasone acetate and acyclovir, with no clinical improvement. A reformatted coronal computed tomogram (Figure 1), axial (Figure 2) and coronal (Figure 3) enhanced T1-weighted magnetic resonance images (MRIs), and a coronal T2-weighted MRI (Figure4) were obtained. What is your diagnosis?

8143 Background: The burnout syndrome can be characterized by abnormalities in three domains: emotional exhaustion (EE), depersonalization (DP) and lack of personal realization (RP). In the medical...

Neurofibromatose 2 (NF2) é uma doença autossômica dominante que, invariavelmente, cursa com surdez. Restauração auditiva pode ser realizada por meio do implante coclear (IC) ou implante auditivo de tronco encefálico (ABI). OBJETIVO: Avaliar resultados auditivos de IC e ABI em pacientes com NF2 e revisão de literatura. MÉTODO: Foram avaliados prospectivamente quatro pacientes com NF2 submetidos à exérese tumoral e implantação de IC ou ABI ipsilateral simultâneo, dependendo se o nervo coclear foi preservado ou não cirurgicamente. São descritos resultados auditivos em longo prazo (12-48 meses). RESULTADOS: Todos pacientes implantados obtiveram percepção sonora, com melhora dos limiares tonais. A paciente com IC não discrimina vogais nem sentenças. Os três pacientes com ABI apresentam discriminação média de vogais de 70% e teste 4-choice 86%. Um paciente não discrimina sentenças. Os outros dois discriminam 100% de sentenças em formato fechado e em formato aberto 10% e 20%. CONCLUSÃO: A escolha do tipo de implante usado na restauração auditiva de portadores NF2 dependerá da preservação anatômica e funcional do nervo coclear na cirurgia de ressecção tumoral. Embora nossa experiência mostre o contrário, a literatura mostra que, se essa condição for alcançada, IC fornecerá melhores resultados auditivos. Caso contrário, ABI trará maiores benefícios. Neurofibromatosis 2 (NF2) is an autosomal dominant disease in which hearing loss is predominant. Auditory restoration is possible using cochlear implants (CI) or auditory brainstem implant (ABI). OBJECTIVE: To assess the auditory results of CI and ABI in NF2 patients and review the literature. METHODS: Four NF2 patients were prospectively evaluated. They were submitted to tumor resection followed by ipsilateral CI or ABI depending on cochlear nerve preservation. Long term auditory results were described for CI (12 months) and ABI (48 months). RESULTS: All patients achieved auditory perception improvements in their hearing thresholds. The CI patient does not recognize vowels or sentences. The 3 ABI patients discriminate 70% of vowels and 86% in the 4-choice test. One of them does not recognize sentences. The other two recognize 100% of closed sentences and 10% and 20% of open sentences. CONCLUSION: The choice of implant type to restore hearing to NF2 patients will relay on anatomical and functional cochlear nerve preservation during tumor resection surgery. Although our experience was different, the literature shows that if this condition is achieved, CI will offer better auditory results. If not, ABI is recommended.

A cirurgia de implante coclear (IC) é método seguro e factível de restaurar audição em pacientes com surdez grave a profunda. OBJETIVO: Descrever complicações do IC. MATERIAL E MÉTODOS: Dados de 591 IC com tempo de seguimento de pelo menos 1 ano foram retrospectivamente analisados. Foram excluídos 41 pacientes por dados incompletos, acesso via fossa media ou perda de seguimento. RESULTADOS: Dos 550 IC analisados, 341 foram realizados em crianças e adolescentes e 209 em adultos. O tempo médio de surdez foi de 6,3 ± 6,7 anos para pré-linguais e 12,1 ± 11,6 anos para os pós-linguais. O tempo médio de seguimento foi 3,9 ± 2,8 anos. Complicações maiores ocorreram em 8,9% casos e menores em 7,8%. Problemas durante a inserção do feixe de eletrodos (3,8%) foi a complicação maior mais frequente, seguido pela deiscência de ferida operatória (1,4%). Paralisia facial temporária (2,2%), lesão muro facial (2,2%) e lesão de membrana timpânica (1,8%) foram as complicações menores mais frequentes. Nenhum óbito foi observado. CONCLUSÃO: Complicações cirúrgicas tiveram baixa ocorrência, sendo a maioria solucionada com sucesso. Esses resultados confirmam que o IC é uma cirurgia segura e a maioria das complicações cirúrgicas pode ser manejada com medidas conservadoras ou intervenções menores. Cochlear implantation is a safe and reliable method for auditory restoration in patients with severe to profound hearing loss. OBJECTIVE:To describe the surgical complications of cochlear implantation. MATERIALS AND METHODS: Information from 591 consecutive multichannel cochlear implant surgeries were retrospectively analyzed. All patients were followed-up for at least one year. Forty-one patients were excluded because of missing data, follow-up loss or middle fossa approach. RESULTS: Of 550 cochlear implantation analyzed, 341 were performed in children or adolescents, and 209 in adults. The mean hearing loss time was 6.3 ± 6.7 years for prelingual loss and 12.1 ± 11.6 years for postlingual. Mean follow-up was 3.9 ± 2.8 years. Major complications occurred in 8.9% and minor in 7.8%. Problems during electrode insertion (3.8%) were the most frequent major complication followed by flap dehiscence (1.4%). Temporary facial palsy (2.2%), canal-wall lesion (2.2%) and tympanic membrane lesion (1.8%) were the more frequent minor complications. No death occurred. CONCLUSION: There was a low rate of surgical complications, most of them been successfully managed. These results confirm that cochlear implant is a safe surgery and most surgical complications can be managed with conservative measures or minimal intervention.