Partial Labyrinth Removal Restricted to the Superior Semicircular Canal in Focal Combined Transpetrosal Approach: Description and Illustrative Cases.

BACKGROUND AND OBJECTIVES: The labyrinthine structures obstruct the surgical view of the deep petroclival region in the transpetrosal approach. Historically, labyrinthectomy and removal of all 3 semicircular canals, with resultant deafness, was used in patients with ipsilateral functional hearing deficits to improve access. The advent and systematization of superior and posterior semicircular canal removal (transcrusal approach) with good rates of hearing preservation has allowed a redefinition of the possibility of partial labyrinthectomy in patients without previous hearing deficits. The present manuscript is intended to describe a technical refinement of partial labyrinthectomy during focal combined petrosectomy, offering a customization of the approach through the selective removal of the superior semicircular canal for specific types of tumors. METHODS: The use of the technique is demonstrated through surgical drawings, pictures, and videos. The rationale to indicate this new approach is discussed based on clinical cases. RESULTS: Three illustrative clinical cases (petroclival meningiomas) are demonstrated. Functional hearing on the approach side has been preserved in all of them. CONCLUSION: The focal combined transpetrosal approach associated with the superior semicircular canal resection has been a promising surgical technique in the treatment of selected petroclival tumors. It has the potential to further decrease the risks of postoperative auditory and vestibular dysfunctions associated with labyrinthectomies.

Cochlear radiation dose and hearing loss in patients with vestibular schwannoma undergoing radiosurgery: systematic review

Abstract Objectives: To determine the cut-off point of the cochlear radiation dose as a risk factor for hearing loss in patients with vestibular schwannoma treated with radiosurgery. Methods: A systematic review of the literature was performed without language or publication year restrictions in the MEDLINE/PubMed, EMBASE, Web of Science, LILACS/VHL and Cochrane Library databases. Studies that met the following criteria were included: 1) population: adults of both sexes who underwent radiosurgery for vestibular schwannoma treatment; 2) exposure: cochlear radiation; 3) outcome: hearing loss; 4) type of study: cohort. Two independent reviewers conducted the entire review process. The registration number in PROSPERO was CRD42020206128. Results: From the 333 articles identified in the searches, seven were included after applying the eligibility criteria. There was no standardization as to how to measure exposure or outcome in the included studies, and most studies did not present sufficient data to enable meta-analysis. Conclusion: It was not possible to determine a cut-off point for high cochlear dose that could be considered a risk factor for hearing loss.

Cochlear radiation dose and hearing loss in patients with vestibular schwannoma undergoing radiosurgery: systematic review.

OBJECTIVES: To determine the cut-off point of the cochlear radiation dose as a risk factor for hearing loss in patients with vestibular schwannoma treated with radiosurgery. METHODS: A systematic review of the literature was performed without language or publication year restrictions in the MEDLINE/PubMed, EMBASE, Web of Science, LILACS/VHL and Cochrane Library databases. Studies that met the following criteria were included: 1) population: adults of both sexes who underwent radiosurgery for vestibular schwannoma treatment; 2) exposure: cochlear radiation; 3) outcome: hearing loss; 4) type of study: cohort. Two independent reviewers conducted the entire review process. The registration number in PROSPERO was CRD42020206128. RESULTS: From the 333 articles identified in the searches, seven were included after applying the eligibility criteria. There was no standardization as to how to measure exposure or outcome in the included studies, and most studies did not present sufficient data to enable meta-analysis. CONCLUSION: It was not possible to determine a cut-off point for high cochlear dose that could be considered a risk factor for hearing loss.

Otomycosis caused by the cryptic and emerging species Aspergillus sydowii: two case reports.

Two cases of otomycosis have been reported in patients undergoing tympanomastoidectomy. The first one had chronic otitis media, hypertrophic concha and nasal septum deviation, tympanic perforation and otorrhea. The second had otalgia, pruritus, chronic otitis media and cholesteatoma. Direct examination showed mycelial septate filaments with a branch at an angle close to 45°, later identified as Aspergillus sydowii by sequencing the BenA and CaM genes. Susceptibility testing showed low MIC of amphotericin B, itraconazole, ketoconazole and ciclopirox olamine. In both cases, ketoconazole was instituted for 10 days. Otomycosis is a challenge as it is primarily recurrent in patients undergoing surgery. The clinical implication, the identification of the emerging pathogen and the determination of MIC were necessary for the knowledge of the epidemiological profile and establishment of the treatment.

Aspergillus are fungi that can cause ear disease. In severe infections, these fungi can be present for long periods inside the ear, and commonly belong to the species Aspergillus section Nigri and Aspergillus flavus. In this work, we present two cases of ear infections by a different species, Aspergillus sydowii. Patients had obstructed nasal cavities, crooked internal separation of the nose and complaints of secretion in the ear. The efficient diagnosis allowed a treatment that resulted in the death of the fungus and the cure of the patient.

Audiological follow-up of children with congenital Zika syndrome.

How does the auditory function of children with congenital Zika syndrome present during the first three years of life? To determine the auditory function of children with congenital Zika syndrome during the first three years of life and estimate the frequency and long-term presentation of hearing loss in this syndrome, an auditory assessment with screening and diagnostic tests was conducted. The screening test consisted of measuring the short latency ABR using click stimuli. If the ABR click indicated hearing loss, confirmation was obtained with a frequency-specific ABR (FS-ABR), in which the stimuli were tone bursts at frequencies of 500 and 2000 Hz by bone and air conduction. This case series included 107 children with confirmed congenital Zika syndrome, and the cumulative incidence of sensorineural hearing loss in the first three years of life was 9.3% (10/107). There were no cases of delayed-onset or progressive deficits in hearing. Early presentation of sensorineural hearing loss seems to occur with a higher frequency in children with congenital Zika syndrome than in the general population. Sensorineural hearing loss resulting from congenital Zika virus infection does not appear to present with delayed onset or with progressive deficits.

The Microcephaly Epidemic Research Group Paediatric Cohort (MERG-PC): A Cohort Profile.

This cohort profile aims to describe the ongoing follow-up of children in the Microcephaly Epidemic Research Group Paediatric Cohort (MERG-PC). The profile details the context and aims of the study, study population, methodology including assessments, and key results and publications to date. The children that make up MERG-PC were born in Recife or within 120 km of the city, in Pernambuco/Brazil, the epicentre of the microcephaly epidemic. MERG-PC includes children from four groups recruited at different stages of the ZIKV microcephaly epidemic in Pernambuco, i.e., the Outpatient Group (OG/n = 195), the Microcephaly Case-Control Study (MCCS/n = 80), the MERG Pregnant Women Cohort (MERG-PWC/n = 336), and the Control Group (CG/n = 100). We developed a comprehensive array of clinical, laboratory, and imaging assessments that were undertaken by a 'task force' of clinical specialists in a single day at 3, 6, 12, 18 months of age, and annually from 24 months. Children from MCCS and CG had their baseline assessment at birth and children from the other groups, at the first evaluation by the task force. The baseline cohort includes 711 children born between February 2015 and February 2019. Children's characteristics at baseline, excluding CG, were as follows: 32.6% (184/565) had microcephaly, 47% (263/559) had at least one physical abnormality, 29.5% (160/543) had at least one neurological abnormality, and 46.2% (257/556) had at least one ophthalmological abnormality. This ongoing cohort has contributed to the understanding of the congenital Zika syndrome (CZS) spectrum. The cohort has provided descriptions of paediatric neurodevelopment and early epilepsy, including EEG patterns and treatment response, and information on the frequency and characteristics of oropharyngeal dysphagia; cryptorchidism and its surgical findings; endocrine dysfunction; and adenoid hypertrophy in children with Zika-related microcephaly. The study protocols and questionnaires were shared across Brazilian states to enable harmonization across the different studies investigating microcephaly and CZS, providing the opportunity for the Zika Brazilian Cohorts Consortium to be formed, uniting all the ZIKV clinical cohorts in Brazil.

Zika-related adverse outcomes in a cohort of pregnant women with rash in Pernambuco, Brazil.

BACKGROUND: While Zika virus (ZIKV) is now widely recognized as a teratogen, the frequency and full spectrum of adverse outcomes of congenital ZIKV infection remains incompletely understood. METHODS: Participants in the MERG cohort of pregnant women with rash, recruited from the surveillance system from December/2015-June/2017. Exposure definition was based on a combination of longitudinal data from molecular, serologic (IgM and IgG3) and plaque reduction neutralization tests for ZIKV. Children were evaluated by a team of clinical specialists and by transfontanelle ultrasound and were classified as having microcephaly and/or other signs/symptoms consistent with congenital Zika syndrome (CZS). Risks of adverse outcomes were quantified according to the relative evidence of a ZIKV infection in pregnancy. FINDINGS: 376 women had confirmed and suspected exposure to ZIKV. Among evaluable children born to these mothers, 20% presented with an adverse outcome compatible with exposure to ZIKV during pregnancy. The absolute risk of microcephaly was 2.9% (11/376), of calcifications and/or ventriculomegaly was 7.2% (13/180), of additional neurologic alterations was 5.3% (13/245), of ophthalmologic abnormalities was 7% (15/214), and of dysphagia was 1.8% (4/226). Less than 1% of the children experienced abnormalities across all of the domains simultaneously. Interpretation: Although approximately one-fifth of children with confirmed and suspected exposure to ZIKV in pregnancy presented with at least one abnormality compatible with CZS, the manifestations presented more frequently in isolation than in combination. Due to the rare nature of some outcomes and the possibility of later manifestations, large scale individual participant data meta-analysis and the long-term evaluation of children are imperative to identify the full spectrum of this syndrome and to plan actions to reduce damages.

The Prevalence of Adenoid Hypertrophy among Children with Zika Related Microcephaly.

Upper respiratory obstruction is a common sequela in children with Zika-related microcephaly (ZRM). As a cross-sectional analysis nested in a cohort study, this study aims to investigate the prevalence of adenoid hypertrophy (AH) in children with ZRM and symptoms of respiratory obstruction. The data were collected in the first three years of life from children with ZRM who were followed in two reference centers for otorhinolaryngological care of patients with congenital Zika syndrome. Out of 92 children with confirmed ZRM, 57 were evaluated by nasopharyngoscopy after presenting with upper respiratory obstruction symptoms. In this study, 31 of the 57 (54%) children with ZRM who were evaluated had obstructive AH. Thirteen children with obstructive AH were submitted to surgery, which resulted in the complete resolution of symptoms for 11, partial resolution in 1, and no improvement in 1. No evidence of direct involvement by Zika virus (ZIKV) infection in the adenoid tissues was demonstrated by histology or immunohistochemistry. Our results suggest that there is a high prevalence and early presentation of AH in children with ZRM, with consequent upper airway obstruction causing upper airway obstructive disorder, secretory otitis media, and dysphagia.

Hearing preservation middle fossa approach for intracanalicular vestibular schwannoma in a NF2 patient.

Hearing preservation is a cornerstone in the management of intracanalicular vestibular schwannomas. This video demonstrates a middle fossa approach to an intracanalicular schwannoma and highlights some technical and anatomical nuances relevant to the procedure. The patient had sustained hearing preservation in the postoperative period. There are potential benefits in favor of the middle fossa when the tumor reaches the fundus of the internal auditory canal, but the surgeon's individual experience plays a decisive role in the choice of approach. The video can be found here: https://stream.cadmore.media/r10.3171/2021.7.FOCVID21121.

Characteristics of Dysphagia in Infants with Microcephaly Caused by Congenital Zika Virus Infection, Brazil, 2015.

We summarize the characteristics of dysphagia in 9 infants in Brazil with microcephaly caused by congenital Zika virus infection. The Schedule for Oral Motor Assessment, fiberoptic endoscopic evaluation of swallowing, and the videofluoroscopic swallowing study were used as noninstrumental and instrumental assessments. All infants had a degree of neurologic damage and showed abnormalities in the oral phase. Of the 9 infants, 8 lacked oral and upper respiratory tract sensitivity, leading to delays in initiation of the pharyngeal phase of swallowing. Those delays, combined with marked oral dysfunction, increased the risk for aspiration of food, particularly liquid foods. Dysphagia resulting from congenital Zika virus syndrome microcephaly can develop in infants >3 months of age and is severe.

Hearing Loss in Infants with Microcephaly and Evidence of Congenital Zika Virus Infection - Brazil, November 2015-May 2016.

Congenital infection with Zika virus causes microcephaly and other brain abnormalities (1). Hearing loss associated with other congenital viral infections is well described; however, little is known about hearing loss in infants with congenital Zika virus infection. A retrospective assessment of a series of 70 infants aged 0-10 months with microcephaly and laboratory evidence of Zika virus infection was conducted by the Hospital Agamenon Magalhães in Brazil and partners. The infants were enrolled during November 2015-May 2016 and had screening and diagnostic hearing tests. Five (7%) infants had sensorineural hearing loss, all of whom had severe microcephaly; however, one child was tested after receiving treatment with an ototoxic antibiotic. If this child is excluded, the prevalence of sensorineural hearing loss was 5.8% (four of 69), which is similar to that seen in association with other congenital viral infections. Additional information is needed to understand the prevalence and spectrum of hearing loss in children with congenital Zika virus infection; all infants born to women with evidence of Zika virus infection during pregnancy should have their hearing tested, including infants who appear normal at birth.

Achados otomicroscópicos e histológicos da miringoesclerose induzida em ratos: estudo crítico de um modelo experimental / Otomicroscopic and histologic findings of induced myringosclerosis in rats: a critical study of an experimental model

A miringoesclerose é caracterizada por hialinização da lâmina própria da membrana timpânica. Trabalhos experimentais têm utilizado a otomicroscopia ou a histologia para estudar a miringoesclerose em animais, porém sem correlacionar precisamente estes dois métodos de avaliação. OBJETIVO: Este estudo procura avaliar a acurácia da otomicroscopia no diagnóstico de miringoesclerose em modelo experimental com ratos. FORMA DE ESTUDO: experimental. MATERIAL E MÉTODO: Foi induzida miringoesclerose por inoculação transtimpânica de Streptococcus pneumoniae em 25 ratos Wistar, que foram examinados quinzenalmente por otomicroscopia e sacrificados após oito semanas, tendo suas membranas timpânicas examinadas por histologia. RESULTADOS: Da confrontação das alterações otomicroscópicas com os achados histológicos, houve uma sensibilidade de 80 por cento e especificidade de 75 por cento. CONCLUSÃO: Pelos resultados obtidos neste estudo, a otomicroscopia não representou um bom método para avaliar a miringoesclerose neste modelo experimental.

Otomicroscopic and histologic findings of induced myringosclerosis in rats: a critical study of an experimental model.

UNLABELLED: Myringosclerosis is characterized by hyaline changes of the lamina propria of the tympanic membrane. Experimental studies have used otomicroscopy or histology to evaluate myringosclerosis in animals, but they do not correlate precisely these two methods. AIM: The present study evaluates the accuracy of otomicroscopy in the diagnosis of myringosclerosis in rats. STUDY DESIGN: Experimental. MATERIAL AND METHOD: Myringosclerosis was induced by transtympanic inoculation of Streptococcus pneumoniae in 25 Wistar rats, which were examined weekly through otomicroscopy and sacrificed after eight weeks for histologic study of their tympanic membranes. RESULTS: From the comparison of the otomicroscopic data in relation to the histologic findings, we could observe sensibility of 80% and specificity of 75% for the otomicroscopy. CONCLUSION: Considering the results in this study, otomicroscopy did not represent a good method to evaluate myringosclerosis in this experimental model.

Music perception in adult cochlear implant recipients.

OBJECTIVE: To evaluate musical perception in adult cochlear implant (CI) recipients, i.e. perceptual accuracy for pitch, timbre, rhythmic patterns and song identification. MATERIAL AND METHODS: Twenty-nine adult patients were included in this transverse single-center study. Evaluative measures included tests assessing ability to discriminate pitch, rhythm and timbre and to identify nursery songs with and without verbal cues. Performance scores were correlated with duration of deafness, duration of implantation, speech discrimination and musical perception skills. RESULTS: A total of 38% of patients reported that they did not enjoy listening to music with their device and 86% presented lower scores of listening habits after implantation. We found positive correlations between musical background and pitch identification and identification of nursery songs played by piano. We also found positive correlations between speech discrimination and rhythm, timbre and identification of nursery songs with verbal cues. CONCLUSION: Trends in the patterns of correlation between speech and music perception suggest that music patterns are differentially accessible to CI users. New processing strategies may improve this.

Transtornos factícios por procuraçäo: discussäo de um caso / Factitious disorders by proxy: discussion of one case

Condrossarcoma é o sarcoma mais freqüente da laringe. Sua incidência é maior na cartilagem cricóide do que nas outras cartilagens da laringe, sendo raro que ele se origine na epiglote. Relatamos no texto um caso de condrossarcoma originado na epiglote, no qual foi realizada laringectomia subtotal com crico-hioidopexia - e realizamos revisão da literatura

Características de recém-nascidos portadores de hemoglobina S detectados através de triagaem em sangue de cordäo umbilical / Hemoglobin S positive newborn detected by cord blood and its characteristics

Objetivo: verificar a prevalência de Hemoglobina "S" (Hb"S") em recém-nascidos (RN) no Instituto Materno Infantil de Pernambuco (IMIP) e sua ocorrênciaa segundo o sexo, peso e Apgar. Metodologia: Foram analisados 1.988 RN no período de outubro/96 a março/97 para detecçäo de Hb "S" em sangue de cordäo umbilical através de eletroforese de Hb em pH acalino. Näo houve nenhum critério de exclusäo. Os dados sobre variáveis dos RN foram obtidos através de consulta aos prontuários médicos. A análise dos dados foi feita através do "software" EPI-INFO 6.0. Resultados: Foram encontrados 105 (5,3 por cento) RN com presença de Hb "S". Destes, 102 (5,1 por cento) apresentavam-se na forma de traço falciforme (Hb "FAS") e 3 (0,2 por cento) sob a forma de doença falciforme (Hb "SC"). Näo foi encontrado nenhum RN com hemozigose para Hb "S". Näo foi observada diferença estatisticamente significante entre os RN portadores ou näo de Hb "S" quanto ao sexo, peso e Apgar. Conclusöes: Sugerimos a realizaçäo de triagaem neonatal de hemoglobinopatias em todos os RN na cidade de Recife com posterior seguimento dos casos positivos e suspeitos e subseqüênte aconselhamento genético às famílias acometidas