Global Regulatory Review Needed for Cochlear Implants: A Call for FDA Leadership.

INTRODUCTION: Using the United States Food and Drug Administration (FDA) as example, we argue that regulatory agencies worldwide should review their guidance on cochlear implants (CIs). METHODS: This is a position paper, thus the methods are strictly argumentation. Here we give the motivation for our recommendation. The FDA's original approval of implantation in prelingually deaf children was granted without full benefit of information on language acquisition, on childcaregiver communication, and on the lived experience of being deaf. The CI clinical trials, accordingly, did not address risks of linguistic deprivation, especially when the caregiver's communication is not fully accessible to the prelingually deaf child. Wide variability in the effectiveness of CIs since initial and updated approval has been indicated but has not led to new guidance. Children need to be exposed frequently and regularly to accessible natural language while their brains are still plastic enough to become fluent in any language. For the youngest infants, who are not yet producing anything that could be called language although they might be producing salient social signals (Goldstein et al. Child Dev 80:636-644, 2009), good comprehension of communication from caregiver to infant is critical to the development of language. Sign languages are accessible natural languages that, because they are visual, allow full immersion for deaf infants, and they supply the necessary support for this comprehension. The main language contributor to health outcomes is this combination of natural visual language and comprehension in communication. Accordingly, in order to prevent possible language deprivation, all prelingually deaf children should be exposed to both sign and spoken languages when their auditory status is detected, with sign language being critical during infancy and early childhood. Additionally, all caregivers should be given support to learn a sign language if it is new to them so that they can comprehend their deaf children's language expressions fully. However, both languages should be made accessible in their own right, not combined in a simultaneous or total communication approach since speaking one language and signing the other at the same time is problematic. RESULTS: Again, because this is a position paper, our results are our recommendations. We call for the FDA (and similar agencies in other countries) to review its approval of cochlear implantation in prelingually deaf children who are within the sensitive period for language acquisition. In the meantime, the FDA should require manufacturers to add a highlighted warning to the effect that results with CI vary widely and CIs should not be relied upon to provide adequate auditory input for complete language development in all deaf children. Recent best information on users' experience with CIs (including abandonment) should be clearly provided so that informed decisions can be made. The FDA should require manufacturers' guidance and information materials to include encouragement to parents of deaf children to offer auditory input of a spoken language and visual input of a sign language and to have their child followed closely from birth by developmental specialists in language and cognition. In this way parents can align with providers to prioritize cognitive development and language access in both audio-vocal and visuo-gestural modalities. DISCUSSION: The arguments and recommendations in this paper are discussed at length as they come up.

Decompression retinochoroidopathy: a unique presentation post-trabeculectomy.

PURPOSE: To present a unique case of decompression retinochoroidopathy presenting with intraretinal, subretinal and preretinal hemorrhages. METHODS: A 65-year-old hypertensive female presented with diminution of vision in right eye of 1-week duration. She had been a known case of primary open-angle glaucoma and had undergone trabeculectomy with mitomycin C in right eye 1 week back. Her day 1 postoperative intraocular pressure was 6 mmHg. Her left eye had undergone Ahmed glaucoma valve surgery for the same 2 years back. RESULT: Right eye vision at 1 week of presentation was 6/36, N36 and left eye hand movements. Both eyes were pseudophakic. Intraocular pressure in right eye was 35 mmHg with nonfunctional bleb and in left eye 15 mmHg. Right eye fundus shows multiple subretinal, intraretinal, preretinal and some white-centered blot hemorrhage in all the four quadrants including the posterior pole. Disk had glaucomatous cupping with no dilatation or tortuosity of retinal blood vessels. Left eye had total glaucomatous optic atrophy. CONCLUSION: Old hypertensive females may not tolerate hemodynamic changes in retinal and choroidal vasculature so well, and if the autoregulation of retinal capillaries and choriocapillaris fails because of IOP spikes or rise of IOP to high levels in a relatively short duration, sudden lowering of IOP after trabeculectomy may cause decompression retinopathy and choroidopathy.

Discourses of prejudice in the professions: the case of sign languages.

There is no evidence that learning a natural human language is cognitively harmful to children. To the contrary, multilingualism has been argued to be beneficial to all. Nevertheless, many professionals advise the parents of deaf children that their children should not learn a sign language during their early years, despite strong evidence across many research disciplines that sign languages are natural human languages. Their recommendations are based on a combination of misperceptions about (1) the difficulty of learning a sign language, (2) the effects of bilingualism, and particularly bimodalism, (3) the bona fide status of languages that lack a written form, (4) the effects of a sign language on acquiring literacy, (5) the ability of technologies to address the needs of deaf children and (6) the effects that use of a sign language will have on family cohesion. We expose these misperceptions as based in prejudice and urge institutions involved in educating professionals concerned with the healthcare, raising and educating of deaf children to include appropriate information about first language acquisition and the importance of a sign language for deaf children. We further urge such professionals to advise the parents of deaf children properly, which means to strongly advise the introduction of a sign language as soon as hearing loss is detected.

VITREORETINAL COMPLICATIONS IN EYES WITH BOSTON KERATOPROSTHESIS TYPE I.

PURPOSE: To describe the spectrum of vitreoretinal complications in eyes with Boston keratoprosthesis type I and evaluate the treatment outcomes. METHODS: This was a retrospective interventional case series of 23 of 45 eyes that underwent Boston keratoprosthesis from April 2003 to December 2013 and developed vitreoretinal complications. Types of vitreoretinal complications, surgical techniques, and anatomical and visual outcomes were analyzed. RESULTS: Vitreoretinal complications in eyes with Boston keratoprosthesis included retroprosthetic membranes (n = 11), retinal detachment (n = 6), endophthalmitis (n = 4), epiretinal membrane (n = 4), vitreous hemorrhage (n = 2), and choroidal detachment (n = 1). Twenty of 23 eyes (87%) underwent surgical intervention. Retinal reattachment was achieved in 5 eyes (83%) with significant visual improvement in 3 (50%). Mean preoperative visual acuity improved from 1.84 ± 0.89 logMAR to 1.5 ± 0.87 logMAR (P = 0.01) at the last follow-up. Mean 1.4 surgical procedures were performed per eye. Mean follow-up was 28 months (median: 28 months, range: 5-57 months). CONCLUSION: Retroprosthetic membrane and retinal detachment are most common vitreoretinal complications in eyes with Boston keratoprosthesis. Vitreoretinal complications can be managed by appropriate intervention in such eyes with encouraging anatomical and functional results.

Bilingualism: A Pearl to Overcome Certain Perils of Cochlear Implants.

Cochlear implants (CI) have demonstrated success in improving young deaf children's speech and low-level speech awareness across a range of auditory functions, but this success is highly variable, and how this success correlates to high-level language development is even more variable. Prevalence on the success rate of CI as an outcome for language development is difficult to obtain because studies vary widely in methodology and variables of interest, and because not all cochlear implant technology (which continues to evolve) is the same. Still, even if the notion of treatment failure is limited narrowly to those who gain no auditory benefit from CI in that they cannot discriminate among ambient noises, the reported treatment failure rate is high enough to call into question the current lack of consideration of alternative approaches to ensure young deaf children's language development. Recent research has highlighted the risks of delaying language input during critical periods of brain development with concomitant consequences for cognitive and social skills. As a result, we propose that before, during, and after implantation deaf children learn a sign language along with a spoken language to ensure their maximal language development and optimal long-term developmental outcomes.

4CMenB Vaccination to Prevent Meningococcal B Disease in Vietnam: Expert Review and Opinion.

An advisory board meeting was held with experts in Vietnam (Hanoi, August 2022), to review the evidence on invasive meningococcal disease (IMD) epidemiology, clinical management, and meningococcal vaccines to reach a consensus on recommendations for meningococcal vaccination in Vietnam. IMD is a severe disease, with the highest burden in infants and children. IMD presents as meningitis and/or meningococcemia and can progress extremely rapidly. Almost 90% of deaths in children occur within the first 24 h, and disabling sequelae (e.g., limb amputations and neurological damage) occur in up to 20% of survivors. IMD patients are often hospitalized late, due to mild and nonspecific early symptoms and misdiagnosis. Difficulties related to diagnosis and antibiotic misuse mean that the number of reported IMD cases in Vietnam is likely to be underestimated. Serogroup B IMD is predominant in many regions of the world, including Vietnam, where 82% of IMD cases were due to serogroup B (surveillance data from 2012 to 2021). Four component meningococcal B vaccine (4CMenB) is used in many countries (and is part of the pediatric National Immunization Program in 13 countries), with infant vaccination starting from two months of age, and a 2 + 1 dosing schedule. Experts recommend 4CMenB vaccination as soon as possible in Vietnam, starting from two months of age, with a 2 + 1 dosing schedule, and at least completing one dose before 6 months of age.

Optical coherence tomography characteristics in eyes with optic pit maculopathy.

PURPOSE: To study the optical coherence tomography characteristics of optic pit maculopathy and propose the anatomical pathophysiology of fluid conduit from pit to macula. METHODS: The charts of 32 patients (32 eyes) with the diagnosis of optic pit maculopathy were reviewed. Clinical variables that were studied included duration of visual impairment, initial visual acuity, and associated fundus features. Optical coherence tomography characteristics including communication with pit, layer of retina involved in schisis, and presence of subretinal fluid were noted. RESULTS: The mean age of the patients was 27.8 years, and the mean duration of visual impairment was 21.8 months. Seventeen eyes (53.12%) had combined outer and inner layer schisis with subretinal fluid, whereas 12 eyes (37.5%) presented with outer layer schisis and subretinal fluid. Two eyes (6.25%) had outer layer schisis only, and only 1 eye (3.12%) presented with schisis of both inner and outer layers only. CONCLUSION: The authors propose a possible pathway of fluid movement in optic pit maculopathy: fluid first seeps into the outer retinal layer and then involves the other layers.

Review of the epidemiology, diagnosis and management of invasive meningococcal disease in Vietnam.

Invasive meningococcal disease (IMD), caused by Neisseria meningitidis, is life-threatening with a high case fatality rate (CFR) and severe sequelae. We compiled and critically discussed the evidence on IMD epidemiology, antibiotic resistance and disease management in Vietnam, focusing on children. PubMed, Embase and gray literature searches for English, Vietnamese and French publications, with no date restrictions, retrieved 11 eligible studies. IMD incidence rate (/100,000 population) was 7.4 [95% confidence interval 3.6-15.3] in children under 5 years of age; driven by high rates in infants (e.g. 29.1 [8.0-106.0] in 7-11 month-olds). Serogroup B IMD was predominant. Neisseria meningitidis strains may have developed resistance to streptomycin, sulfonamides, ciprofloxacin, and possibly ceftriaxone. There was a lack of current data on diagnosis and treatment of IMD, which remain challenging. Healthcare professionals should be trained to rapidly recognize and treat IMD. Preventive measures, such as routine vaccination, could help address the medical need.

Language acquisition for deaf children: Reducing the harms of zero tolerance to the use of alternative approaches.

Children acquire language without instruction as long as they are regularly and meaningfully engaged with an accessible human language. Today, 80% of children born deaf in the developed world are implanted with cochlear devices that allow some of them access to sound in their early years, which helps them to develop speech. However, because of brain plasticity changes during early childhood, children who have not acquired a first language in the early years might never be completely fluent in any language. If they miss this critical period for exposure to a natural language, their subsequent development of the cognitive activities that rely on a solid first language might be underdeveloped, such as literacy, memory organization, and number manipulation. An alternative to speech-exclusive approaches to language acquisition exists in the use of sign languages such as American Sign Language (ASL), where acquiring a sign language is subject to the same time constraints of spoken language development. Unfortunately, so far, these alternatives are caught up in an "either - or" dilemma, leading to a highly polarized conflict about which system families should choose for their children, with little tolerance for alternatives by either side of the debate and widespread misinformation about the evidence and implications for or against either approach. The success rate with cochlear implants is highly variable. This issue is still debated, and as far as we know, there are no reliable predictors for success with implants. Yet families are often advised not to expose their child to sign language. Here absolute positions based on ideology create pressures for parents that might jeopardize the real developmental needs of deaf children. What we do know is that cochlear implants do not offer accessible language to many deaf children. By the time it is clear that the deaf child is not acquiring spoken language with cochlear devices, it might already be past the critical period, and the child runs the risk of becoming linguistically deprived. Linguistic deprivation constitutes multiple personal harms as well as harms to society (in terms of costs to our medical systems and in loss of potential productive societal participation).

Systemic associations of anterior uveitis in a tertiary care ophthalmic centre in south India.

Anterior uveitis is the most common form of uveitis in most regions of the world. A recent study reported that 50 % of patients with anterior uveitis have a related systemic illness. The most common cause of anterior uveitis to date remains idiopathic (45-50 %), followed by HLA-B27-positive acute anterior uveitis. Ours is a retrospective study of the clinical records of 42 patients with anterior uveitis seen at a tertiary care eye hospital in south India. To diagnose the associated systemic disease, the investigations carried out were erythrocyte sedimentation rate, red and white blood cell counts, anti-nuclear antibody (ANA) analysis and HLA-B27 antigen determination. Based on clinical features, patients were also investigated for serum angiotensin-converting enzyme, high-resolution computed tomography scan, Mantoux test and QuantiFERON-TB Gold test. American College of Rheumatology criteria were followed to diagnose spondyloarthropathies and juvenile idiopathic arthritis (JIA). Of the 42 patients, 14 (33.33 %) were HLA-B27-positive and the remaining 28 (66.66 %) HLA-B27-negative. Overall, 32 of the 42 had no causative association, three patients were diagnosed with JIA, two with ankylosing spondylitis, two were associated with systemic tuberculosis, two with sarcoidosis and one with Vogt-Koyanagi-Harada syndrome. Our case series shows the incidence of various systemic diseases associated with anterior uveitis. Though the overall incidence of HLA-B27-positive acute anterior uveitis in our series (33 %) is similar to that in the Western literature, only 14 % of such patients were positive for a spondyloarthropathy. A few interesting cases like HLA-B27-positive, ANA-negative JIA presenting with chronic persisting anterior uveitis were also seen.

Deaf Children Need Rich Language Input from the Start: Support in Advising Parents.

Bilingual bimodalism is a great benefit to deaf children at home and in schooling. Deaf signing children perform better overall than non-signing deaf children, regardless of whether they use a cochlear implant. Raising a deaf child in a speech-only environment can carry cognitive and psycho-social risks that may have lifelong adverse effects. For children born deaf, or who become deaf in early childhood, we recommend comprehensible multimodal language exposure and engagement in joint activity with parents and friends to assure age-appropriate first-language acquisition. Accessible visual language input should begin as close to birth as possible. Hearing parents will need timely and extensive support; thus, we propose that, upon the birth of a deaf child and through the preschool years, among other things, the family needs an adult deaf presence in the home for several hours every day to be a linguistic model, to guide the family in taking sign language lessons, to show the family how to make spoken language accessible to their deaf child, and to be an encouraging liaison to deaf communities. While such a support program will be complicated and challenging to implement, it is far less costly than the harm of linguistic deprivation.

Infants and children with hearing loss need early language access.

Around 96 percent of children with hearing loss are born to parents with intact hearing, who may initially know little about deafness or sign language. Therefore, such parents will need information and support in making decisions about the medical, linguistic, and educational management of their child. Some of these decisions are time-sensitive and irreversible and come at a moment of emotional turmoil and vulnerability (when some parents grieve the loss of a normally hearing child). Clinical research indicates that a deaf child's poor communication skills can be made worse by increased level of parental depression. Given this, the importance of reliable and up-to-date support for parents' decisions is critical to the overall well-being of their child. In raising and educating a child, parents are often offered an exclusive choice between an oral environment (including assistive technology, speech reading, and voicing) and a signing environment. A heated controversy surrounds this choice, and has since at least the late 19th century, beginning with the International Congress on the Education of the Deaf in Milan, held in 1880. While families seek advice from many sources, including, increasingly, the internet, the primary care physician (PCP) is the professional medical figure the family interacts with repeatedly. The present article aims to help family advisors, particularly the PCP and other medical advisors in this regard. We argue that deaf children need to be exposed regularly and frequently to good language models in both visual and auditory modalities from the time hearing loss is detected and continued throughout their education to ensure proper cognitive, psychological, and educational development. Since there is, unfortunately, a dearth of empirical studies on many of the issues families must confront, professional opinions, backed by what studies do exist, are the only option. We here give our strongly held professional opinions and stress the need for improved research studies in these areas.

Support for parents of deaf children: Common questions and informed, evidence-based answers.

To assist medical and hearing-science professionals in supporting parents of deaf children, we have identified common questions that parents may have and provide evidence-based answers. In doing so, a compassionate and positive narrative about deafness and deaf children is offered, one that relies on recent research evidence regarding the critical nature of early exposure to a fully accessible visual language, which in the United States is American Sign Language (ASL). This evidence includes the role of sign language in language acquisition, cognitive development, and literacy. In order for parents to provide a nurturing and anxiety-free environment for early childhood development, signing at home is important even if their child also has the additional nurturing and care of a signing community. It is not just the early years of a child's life that matter for language acquisition; it's the early months, the early weeks, even the early days. Deaf children cannot wait for accessible language input. The whole family must learn simultaneously as the deaf child learns. Even moderate fluency on the part of the family benefits the child enormously. And learning the sign language together can be one of the strongest bonding experiences that the family and deaf child have.

Intravitreal gas injection with prone positioning for giant retinal pigment epithelial tear.

We report a 78 year old male with acute RPE tear with sudden vision loss, who underwent intravitreal C3F8 injection for reattaching the RPE. The impact of gas on detached RPE was studied by serial OCTs and Fundus pictures.

Cost-effectiveness of two-dose human papillomavirus vaccination in Singapore.

INTRODUCTION: Cervical cancer is the tenth most common cancer and the eighth most frequent cause of death among women in Singapore. As human papillomavirus (HPV) infection is the necessary cause of cervical cancer, the risk of cervical cancer can be substantially reduced through vaccination. This study was conducted to evaluate the cost-effectiveness of two-dose HPV vaccination as part of a national vaccination programme for 12-year-old girls in Singapore, from the perspective of the healthcare payer. METHODS: A lifetime Markov cohort model was used to evaluate the cost-effectiveness of introducing the AS04-adjuvanted HPV-16/18 vaccine (AS04-HPV-16/18v) to the current cervical screening programme in Singapore. Furthermore, the cost-effectiveness of the AS04-HPV-16/18v was compared with the HPV-6/11/16/18 vaccine (4vHPV). Model inputs were derived from local data, where possible, and validated by clinical experts in Singapore. RESULTS: Introduction of the AS04-HPV-16/18v in Singapore was shown to prevent 137 cervical cancer cases and 48 cervical cancer deaths when compared with screening alone. This resulted in an incremental cost-effectiveness ratio of SGD 12,645 per quality-adjusted life year (QALY) gained, which is cost-effective according to the World Health Organization threshold for Singapore. When discounted at 3%, AS04-HPV-16/18v was dominant over 4vHPV, with cost savings of SGD 80,559 and 28 additional QALYs gained. In the one-way sensitivity analysis, AS04-HPV-16/18v remained cost-effective compared with screening alone and dominant compared with 4vHPV. CONCLUSION: AS04-HPV-16/18v is the most cost-effective choice for reducing the burden of cervical cancer through universal mass vaccination for 12-year-old girls in Singapore.

Comparison of serum sodium and potassium levels in patients with senile cataract and age-matched individuals without cataract.

AIM: The study was to analyze mean serum sodium and potassium levels in cataract patients and age-matched individuals without cataract. METHODS AND MATERIALS: It was a prospective case-control study. Individuals more than 50 years of age who attended our ophthalmic center in the year 2007-2010 were grouped into those having cataract and those without cataract. Mean serum sodium and potassium levels in the cataract groups were calculated and compared with the control group. Statistical software SPSS14 was used for statistical analysis. RESULTS: Mean serum sodium levels in cataract group was 135.1 meqv/l and 133 meqv/l in the control group. Mean potassium was 3.96 meqv/l in the case study group and 3.97 meqv/l in controls. Mean sodium levels among cases were significantly higher than control group. No difference was seen in the PSC group and control. The difference in mean potassium among the two groups was statistically insignificant. CONCLUSION: Diets with high sodium contents are a risk factor for senile cataract formation and dietary modifications can possibly reduce the rate of progression cataract.

Unusual course of interferon-related retinopathy in chronic hepatitis C.

Interferon Alfa retinopathy usually presents as cotton wool spots, superficial hemorrhages and macular edema. We describe a rare case of severe retinopathy involving hard exudates at macula that lead to permanent visual loss in one eye. Elderly male presented with diminution of vision in right eye of 8 month duration. He was a diagnosed case of compensated chronic hepatitis C and had received interferon Alfa therapy before. Fundus examination of both eye showed multiple hard exudates at macula with a plaque involving the foveal center in right eye. OCT and FFA confirmed the findings seen clinically. Patient was advised regular follow up and on 3 months follow up his clinical picture was same. Ours is the first case report where patient had significant visual loss secondary to hard exudate plaque at center of fovea and ischemic fovea in right eye. Presence of hard exudates at any stage of therapy of hepatitis C could be an indicator of the severity of retinopathy with possible indication for stopping the drug.

Development of Human Papillomavirus (HPV) Vaccines: A Review of Literature and Clinical Update.

The casual relationship concerning Human papillomaviruses (HPV) and cervical cancer is already established. Therefore, such HPV-associated malignancies might be prevented by prophylactic HPV vaccines. From 2009, two prophylactic HPV L1 Virus-Like Particle vaccines namely, Gardasil®; - quadrivalent (Merck) and Cervarix™ - bivalent (GlaxoSmithKline) are widely commercially available. By Aug 2014, 58 countries had introduced HPV vaccination in their national immunization program; this has led to numerous publications on safety and real world effectiveness. We have also seen long-term immunogenicity and efficacy data emerging. Data on cross-protection has also evolved. In clinical trials, it is observed that vaccinating adolescents results in higher immunological response than young adults hence to achieve best HPV vaccine efficacy it is advisable to immunize before the onset of sexual activity. Recently we have seen development of 2 dose vaccine schedule for adolescent, and emerging evidences even point that single dose of HPV vaccines can result in high efficacy, this observation is currently under consideration but if accepted will greatly impact vaccination coverage. In terms of safety, pregnancy registry did not find any unexpected patterns in fetal or maternal outcomes. This review only focuses on the efficacy and safety data of both Food and Drug Administration approved vaccines from clinical phase I to phase IV.