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The identification of a congenital Zika syndrome (CZS) during the November 2015 microcephaly epidemic in Brazil raised many questions (1). So far, the main phenotypic indicator of CZS is microcephaly, [...]

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. Author summary: The ability to meaningfully quantify the absolute and relative risks of Congenital Zika Syndrome is contingent on the accurate identification of ZIKV infections in pregnant women and the long-term follow-up of children at risk. This study builds on the evidence base on ZIKV in pregnancy by not only examining pregnancy and birth outcomes, but also considering later onset manifestations of Congenital Zika Syndrome, conducting a deep and standardized investigation of infant outcomes. In addition, it used unprecedented repeated testing and the use of multiple diagnostic platforms, including qRT-PCR, IgM and IgG3 ELISAs, and PRNTs to identify ZIKV cases. The absolute risk of microcephaly was 2.9%, of calcifications and/or ventriculomegaly was 7.2%, of additional neurologic alterations was 5.3%, of ophthalmologic abnormalities was 7% and of dysphagia was 1.8%. The manifestations presented more frequently in isolation than in combination (i.e., less than 1% of the children experienced abnormalities across all of the domains simultaneously). 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 [ABSTRACT FROM AUTHOR]

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., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

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.

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.