Have newborn hearing screening programs been affected by the COVID-19 pandemic?

Introduction: Due to the vital importance of early diagnosis and intervention of congenital hearing loss, the aim of this study was to compare the rate of newborn hearing screening before and after the COVID-19 pandemic quarantine. Material(s) and Method(s): In this study, the data related to 6144 newborns during two 18-month time periods before and after the onset of the COVID-19 pandemic quarantine, was analyzed. Result(s): From all visited newborns, 3770 and 2374 referred to the centre before and after the COVID-19 pandemic quarantine, respectively. There were statistically significant differences in the results of screening and the referral rate of neonates before and after the pandemic (p = 0.00). The results showed that both the residents of urban and rural regions visited for newborn hearing screening less frequently after the onset of the pandemic, but no statistically significant difference was shown between the number of boys and girls before and after the pandemic (p = 0.72). Conclusion(s): Our results help gain new insights regarding the impacts of disruptive events such as pandemics on the implementation of health screening programs. Boosting public knowledge by providing families with proper education appears to be helpful in preventing the negative effects of delayed implementation of health screening programs.Copyright © 2022 International Association of Physicians in Audiology.

Prenatal COVID-19 Infection Does Not Increase the Risk of Congenital Hearing Loss

Introduction: The global pandemic caused by the novel coronavirus (SARS-CoV-2), commonly known as COVID-19, has had profound effects at every level of society. Other known viral illnesses during pregnancy, such as cytomegalovirus, can affect the developing fetus and cause congenital hearing loss. The long-term effects of COVID-19, including the risk of congenital hearing loss, are not currently established. Method(s): A retrospective cohort study was performed at a quaternary care facility. Sixty-four consecutive patients with a COVID-19 infection during pregnancy were identified. Charts were reviewed for trimester of positive COVID-19 test, maternal and neonatal comorbidities, symptomatology, vaccination status, and results of newborn screening and follow-up hearing testing, if performed. Result(s): Sixty-four women with positive COVID-19 test during pregnancy (6 in second trimester, 59 in third trimester) were identified. Most (n=54, 84.4%) patients were asymptomatic at time of positive test and 29.7% (n=19) had received at least 1 dose of a COVID-19 vaccine. Newborn hearing screening was performed in 58 patients (90.6%). Failed initial screening occurred in 3 (5.2%) right ears and 4 (7.0%) left ears. Two patients passed on repeat screening, and the remaining 1 patient was found to have a mild conductive hearing loss in the right and middle ear dysfunction on the left. Conclusion(s): This study demonstrates no significant increased risk of congenital hearing loss in prenatal COVID-19 infection. Additional investigation into timing of infection and effect of vaccination status will be performed to provide guidance to women navigating pregnancy during the pandemic.

IMMUNE SYSTEM IN MOTION: A CASE OF ADULT-ONSET STILL'S DISEASE

CASE: A 23-year-old female with a history of congenital deafness and HLAB27 positivity presented for two weeks of diffuse arthralgias, fever, and nausea. She had a history of an erythematous rash around her eyes and upper chest that had resolved with prednisone;however, her other symptoms persisted. She denied known tick exposures, drug use, sick contacts, or travel, but had recently been hiking. On presentation, she was febrile to 38.8°C and tachycardic to 130 beats/min. Her labs were notable for an AST of 232 U/L, ALT of 266 U/L, LDH of 680 U/L, haptoglobin <10 mg/dL, and ferritin of 12,230 ng/mL, with no cytopenias or leukocytosis. Her CRP was 127 mg/dL and ESR was normal. Her troponin and BNP were both elevated, to 54 ng/L and 468 pg/mL respectively. ANA and RF titers was negative. Viral studies including EBV, CMV, and SARSCoV-2 as well as bacterial studies were negative. She was started on doxycycline for possible tick-borne infection, but titers returned negative. Echocardiography and chest x-ray were unremarkable. CT scan demonstrated nonspecific para-aortic and mesenteric lymphadenopathy. The patient's presentation and labs were consistent with adult-onset Still's disease (AOSD), meeting the Yamaguchi criteria for diagnosis. She was started on IV hydrocortisone and anakinra with symptomatic improvement. Her liver function testing worsened due to concerns for macrophage activation syndrome (MAS). She was treated with ruxolitinib with gradual improvement in her liver function, followed by tofacitinib. She made a full recovery on discharge. IMPACT/DISCUSSION: Due to its rarity, AOSD can be challenging to diagnose. This case highlighted the key manifestations and distinguishing characteristics of the disorder. The patient presented with fever, rash, and polyarthralgias. While the location of the rash in AOSD varies, the upper chest as seen in this case is typical. While this patient did not have cytopenia or leukocytosis, she did have elevated transaminases and a disproportionately elevated ferritin, a hallmark of AOSD. Importantly, ANA and RF titers were negative, which helps to differentiate AOSD from other autoimmune disorders. The case also demonstrated a feared complication of AOSD, MAS, a form of hemophagocytic lymphohistiocytosis that occurs in 10-15% of patients with AOSD. This case highlighted the importance of remaining vigilant for MAS, as the patient's liver function continued to decline despite symptomatic improvement. While MAS is difficult to diagnose, hyperferritinemia and pancytopenia are thought to be relatively strong indicators. CONCLUSION: AOSD is a rare and debilitating disease, with an illness script that has significant overlap with other diseases. In addition to the combination of polyarthralgias, rash, and fevers, a markedly elevated ferritin is a strong indicator of AOSD. ANA and RF titers will be negative. It is crucial to remain vigilant for complications of the disease, such as MAS.

COMMUNICATION CONUNDRUMS OF COVID-19- BARRIERS TO COMMUNICATING WITH DEAF AND HARD OF HEARING PATIENTS DURING A PANDEMIC

CASE: A 53-year-old female with a past medical history of neonatal meningitis complicated by congenital deafness was admitted for evaluation of disorientation. Collateral history provided by family revealed progressive fatigue and labial itching for 1 month. Physical examination revealed excoriated, irregular nodules of the labia with partial obstruction of the vaginal canal. CT of abdomen and pelvis revealed a large mass involving the posterior wall and floor of the bladder with extension to the vagina and vulva, inguinal lymphadenopathy, and multiple liver masses. The admitting team attempted communicating with the patient using a virtual American Sign Language (ASL) interpreter via an iPad and written communication, but both were limited by the patient's vision impairment. The patient was able to lip-read, but this approach was complicated by the need for mask-wearing during the COVID-19 pandemic. The care team was reluctant to remove their masks to avoid putting the patient's health at risk, as her vision impairment would have required them to stand close. The team attempted to arrange for family to assist with communication, but this was limited by hospital policy restricting visitors due to COVID-19. Eventually, an interdisciplinary goals-of-care meeting was held with the patient and her family to discuss diagnosis with the assistance of in-person ASL interpreters. The patient had a better understanding of her diagnosis with in-person interpretation but expressed feeling excluded from her care plan and lack of empathy in communication from her providers. IMPACT/DISCUSSION: This care team was faced with multiple barriers to conveying a life-changing diagnosis and holding a meaningful goals-of-care discussion with the patient. The goal was to convey a diagnosis and create a plan through shared decision-making as promptly and empathetically as would be done for a patient without these barriers to communication. Despite exhausting the available resources for communicating with the deaf population, the ability to communicate at the same standard as patients without their medical condition remained limited. It is important to identify the limitations to communication in this case to improve communication with deaf patients. Unavailability of in-person ASL interpreters during the night, lack of staff education regarding resources available to facilitate communication between providers and patients who are deaf, prevention of communicating emotion with ASL due to mask-wearing, and prevention of family member visitation, who often develop their own effective ways of communicating, due to hospital policy were all barriers to effective communication in this case. CONCLUSION: This case illustrates the challenges imposed by COVID-19 safety precautions on communicating with patients who experience deafness as well as the need for new resources and staff education on current resources available, both inside and outside of their institutions, to assist with communicating with deaf patients.