Characteristics of hearing loss in HDR (hypoparathyroidism, sensorineural deafness, renal dysplasia) syndrome

Haploinsufficiency of the zinc finger transcription factor GATA3 causes the triad of hypoparathyroidism, deafness and renal dysplasia, known by its acronym HDR syndrome. The purpose of the current study was to describe in detail the auditory phenotype in human HDR patients and compare these to audiometrical and histological data previously described in a mouse model of this disease. Pure tone audiometry, speech audiometry, speech in noise, auditory brainstem responses and transiently evoked otoacoustic emissions were measured in 2 patients affected by HDR syndrome. Both patients were affected by a moderate-to-severe sensorineural hearing loss. Speech reception thresholds were shifted and speech recognition in noise was disturbed. No otoacoustic emissions could be generated in either patient. Auditory brainstem response interpeak intervals were normal. The human and murine audiological phenotypes seem to correspond well. Hearing loss in HDR syndrome is moderate to severe, seems to be slightly worse at the higher end of the frequency spectrum and may be progressive with age. The absence of otoacoustic emissions and the loss of frequency selectivity suggest an important role for outer hair cells in causing the hearing los

Age-specific and family-centered information modalities to prepare children at home for day-care surgery

Background: Surgery induced stress and anxiety in children and parents can be reduced by providing preoperative information adapted to their needs. Aim of this study was to evaluate the effects of three different information modalities (coloring page, mobile application and videos) to prepare children and their parents for day-care surgery on preoperative anxiety and postoperative pain experienced by the child, and preoperative anxiety and satisfaction of parents. Methods: Prospective observational study including children and their parents that were offered specifically developed information modalities to prepare for day-care surgery. Results were compared between children and their parents that used none (i.e., control group) versus one or more information modalities (i.e., intervention group). Primary outcomes were preoperative anxiety measured using PROMIS v2.0 Anxiety and postoperative pain in children. Secondary outcomes were preoperative parental anxiety (STAI questionnaire) and family satisfaction with information and communication (modified PedsQL Healthcare Satisfaction questionnaire). Subgroup analyses were performed between preschoolers (0-5 years) and school-aged (≥5) children. Results: 93 patients (male 53%) were included in the intervention (n=56) and control group (n=37). Levels of children's preoperative anxiety and postoperative pain, and parental anxiety did not differ between both groups. Families of prepared children were more satisfied with information and communication about preoperative surgical information (8 vs. 6.6, p=0.004) and satisfaction with how parents (7 vs 8, p=0.019) and children (8 vs 6, p=0.018) were prepared for surgery. Conclusions: Preoperative anxiety did not differ between prepared and unprepared children. The use of specifically developed family-centered and age-appropriate information modalities to prepare children for day-care surgery at home results in superior family satisfaction. Level of Evidence: II

No COVID-19 in Patients With Sudden Sensorineural Hearing Loss (SSNHL)

Background: Various case reports have described sudden sensorineural hearing loss (SSNHL) in patients with the 2019 novel coronavirus disease (COVID-19) caused by the severe acute respiratory syndrome coronavirus 2 (SARSCoV-2). Our aim was to determine the incidence of COVID-19 in patients with SSNHL. Methods: All consecutive patients with audiometric confirmed SSNHL between November 2020 and March 2021 in a Dutch large inner city teaching hospital were included. All patients were tested for COVID-19 by polymerase-chain-reaction (PCR) and awaited the results in quarantine. Results: Out of 25 patients, zero (0%) tested positive for COVID-19. Two patients had previously tested positive for COVID-19: at three and eight months prior to the onset of hearing loss. Conclusions: This is the largest series to date investigating COVID-19 in SSNHL patients. In this series there is no apparent relationship between SSNHL and COVID-19

No COVID-19 in patients with sudden sensorineural hearing loss (SSNHL)

Background: Various case reports have described sudden sensorineural hearing loss (SSNHL) in patients with the 2019 novel coronavirus disease (COVID-19) caused by the severe acute respiratory syndrome coronavirus 2 (SARSCoV-2). Our aim was to determine the incidence of COVID-19 in patients with SSNHL. Methods: All consecutive patients with audiometric confirmed SSNHL between November 2020 and March 2021 in a Dutch large inner city teaching hospital were included. All patients were tested for COVID-19 by polymerase-chain-reaction (PCR) and awaited the results in quarantine. Results: Out of 25 patients, zero (0%) tested positive for COVID-19. Two patients had previously tested positive for COVID-19: at three and eight months prior to the onset of hearing loss. Conclusions: This is the largest series to date investigating COVID-19 in SSNHL patients. In this series there is no apparent relationship between SSNHL and COVID-19