Objectives/Hypothesis: To compare the identification of safety-relevant environmental sounds between experienced cochlear implant (CI) users and CI candidates.Study Design: Cross-sectional.Methods: A sample of 19 adult, postlingually deaf CI candidates (CI-Cs), 47 experienced CI users (CI-Es), and a control group of 37 age-matched normal-hearing adults were assessed using the Familiar Environmental Sounds Test-Identification (FEST-I). A subset of 11 sounds relevant for safety were selected for analysis in the current study.Results: Analysis of safety-relevant sound identification accuracy revealed no significant difference in safety-relevant environmental sound identification skills between CI-E and CI-C participants, with average scores of 68.1% and 67.9%, respectively. Both performed substantially lower than age-matched normal hearing adults (95.1%). A significant moderate negative correlation (-0.4) was found between safety-relevant sound accuracy and chronological age only in one group, the CI-E group (r = -0.4, P < .005).Conclusions: These findings fail to demonstrate superior performance in safety-relevant environmental sound identification among CI-Es compared with CI-Cs. Although preliminary, these findings suggest that identification of safety-relevant sounds is a significant area of weakness for both CI-Es and CI-Cs, both of whom may benefit from rehabilitation.
Asian Small-Clawed Otters (Aonyx cinerea) are a small, protected but threatened species living in freshwater. They are gregarious and live in monogamous pairs for their lifetimes, communicating via scent and acoustic vocalizations. This study utilized a hidden Markov model (HMM) to classify stress versus non-stress calls from a sibling pair under professional care. Vocalizations were expertly annotated by keepers into seven contextual categories. Four of these-aggression, separation anxiety, pain, and prefeeding-were identified as stressful contexts, and three of them-feeding, training, and play-were identified as non-stressful contexts. The vocalizations were segmented, manually categorized into broad vocal type call types, and analyzed to determine signal to noise ratios. From this information, vocalizations from the most common contextual categories were used to implement HMM-based automatic classification experiments, which included individual identification, stress vs non-stress, and individual context classification. Results indicate that both individual identity and stress vs non-stress were distinguishable, with accuracies above 90%, but that individual contexts within the stress category were not easily separable.
In 1989, one in four (25%) infants born to women living with HIV were infected; by the age of 2 years, there was 25% mortality among them due to HIV. These and other pieces of data prompted the development of interventions to offset vertical transmission, including the landmark Pediatric AIDS Clinical Trial Group Study (PACTG 076) in 1994. This study reported a 67.5% reduction in perinatal HIV transmission with prophylactic antenatal, intrapartum, and postnatal zidovudine. Numerous studies since then have provided compelling evidence to further optimize interventions, such that annual transmission rates of 0% are now reported by many health departments in the US and elimination has been validated in several countries around the world. Despite this success, the elimination of HIV’s vertical transmission on the global scale remains a work in progress, limited by socioeconomic factors such as the prohibitive cost of antiretrovirals. Here, we review some of the key trials underpinning the development of guidelines in the US as well as globally, and discuss the evidence through a historic lens.
The PAX1 gene plays an important role in the development of the parathyroid glands and the thymus. Mouse knockout models of PAX1, PAX3, and PAX9 have been found to have hypoplastic or absent parathyroid glands. To our knowledge, there are no reported cases of PAX1-associated hypoparathyroidism in humans. We present a case of hypoparathyroidism in a 23-month-old boy with a homozygous pathogenic variant in the PAX1 gene ( PAX1 NM_006192.5 c.463_465del variant), predicted to cause an in-frame deletion of asparagine at position 155 (p.Asn155del) of the PAX1 protein. The hypoparathyroidism was unmasked after the patient developed significant hypocalcemia while receiving GoLYTELY (polyethylene glycol 3350, sodium sulfate anhydrous, sodium bicarbonate, sodium chloride, potassium chloride) for bowel cleanout. The patient had mild and asymptomatic hypocalcemia prior to hospitalization. The patient was noted to have inappropriately normal parathyroid hormone (PTH) level at the time of documented hypocalcemia thereby suggesting a diagnosis of hypoparathyroidism. Plain language summary The first human case of hypoparathyroidism associated with a rare genetic disorder: a case report of PAX1 gene mutation The paired box ( PAX) gene family is important for embryo development. One subfamily, PAX1, is necessary for development of the spinal column, thymus (important for the immune system development), and parathyroid (helps regulate the amount of calcium in the body). We present the case of a 23-month-old boy with known PAX1 gene mutation who came in with episodes of vomiting and poor growth. His presentation was thought to be most likely related to constipation. He was started on bowel cleanout medication and intravenous fluids. However, his calcium that had been mildly low subsequently dropped to very low levels. The level of parathyroid hormone (which helps regulate calcium levels) was inappropriately normal, meaning that his body was unable to make more, and was consistent with hypoparathyroidism. He was treated with calcium supplements and vitamin D and calcium levels normalized. He continues to be on calcium and vitamin D and calcium levels have remained stable. Doctors should keep this complication in mind when treating patients with PAX1 gene mutation.
ObjectiveExamine cochlear implant (CI) users' ability to identify safety‐relevant environmental sounds, imperative for safety, independence, and personal well‐being.MethodsTwenty‐one experienced adult CI users completed an Environmental Sound Identification (ESI) test consisting of 42 common environmental sounds, 28 of which were relevant to personal safety, along with 14 control sounds. Prior to sound identification, participants were shown sound names and asked to rate the familiarity and, separately, relevance to safety of each corresponding sound on a 1–5 scale.ResultsOverall ESI accuracy was 57% correct for the safety‐relevant sounds and 55% correct for control sounds. Participants rated safety‐relevant sounds as more important to safety and more familiar than the non‐safety sounds. ESI accuracy significantly correlated with familiarity ratings.ConclusionThe present findings suggest mediocre ESI accuracy in postlingual adult CI users for safety‐relevant and other environmental sounds. Deficits in the identification of these sounds may put CI listeners at increased risk of accidents or injuries and may require a specific rehabilitation program to improve CI outcomes.Level of Evidence4 Laryngoscope, 133:2388–2393, 2023
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