Objective: Approximately 1 to 2 of every 1000 American newborns has hearing loss identified by newborn screening. This study was designed to determine if infant hearing loss is more common in socioeconomically disadvantaged communities.
Study Design: In this retrospective study, we analyzed electronic medical record data using geostatistical models.
Setting: Infants were residents of Durham County, North Carolina, born in 2 hospitals of the Duke University Health System. This county includes the city of Durham and surrounding suburban and rural communities.
Subjects And Methods: Subjects were hearing-screened newborns, born between 2005 and 2016, whose residential address was in Durham County, North Carolina. This was a retrospective study using medical record data. We used Bayesian regression models with smoothing of coordinate date to identify both spatial and nonspatial predictors of infant hearing loss.
Results: We identified 19,348 infants from Durham County, of whom 675 had failed initial hearing screening and 191 had hearing loss confirmed on follow-up. Hearing loss was significantly associated with minority race (odds ratio [OR], 2.45; 95% confidence interval, 1.97-3.06), as well as lower gestational age and maternal sexually transmitted infections. We identified significant geographic heterogeneity, with a higher probability of hearing loss in poorer urban neighborhoods (local OR range, 0.59-1.39). Neighborhood disadvantage was a significant predictor of hearing loss, as was high local seroprevalence of cytomegalovirus (CMV) among pregnant women.
Conclusions: Urban, low-income neighborhoods have a high prevalence of infant hearing loss compared with more affluent surrounding communities, particularly among minorities. This distribution may be attributable to congenital CMV infection.
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http://dx.doi.org/10.1177/0194599818803305 | DOI Listing |
Worldwide, congenital deafness and pigmentation disorders impact millions with their diverse manifestations, and among these genetic conditions, mutations in the Microphthalmia-associated transcription factor (MITF: OMIM#156845) gene are notable for their profound effects on melanocyte development and auditory functions. This study reports a novel porcupine model exhibiting spontaneous deafness and pigmentation abnormalities reminiscent of human Waardenburg Syndrome Type 2 (WS2: OMIM#193510). Through phenotypic characterization, including coat color, skin, eye morphology, and auditory brainstem response (ABR) assessments, we identified hypopigmentation and complete deafness in mutant porcupines.
View Article and Find Full Text PDFInt J Pediatr Otorhinolaryngol
December 2024
Hacettepe University, Faculty of Medicine, Department of Opthalmology, Ankara, Turkey.
Aims And Objectives: This study aimed to investigate the presence, type, and severity of hearing losses in individuals with Duane Retraction Syndrome (DRS), and to ascertain if there are anomalies in the auditory pathways at the brainstem level in DRS, believed to arise from aberrant interaction between cranial nerves and brainstem nuclei.
Study Design: Cross-sectional observational study.
Setting: Tertiary referral centre.
Zh Nevrol Psikhiatr Im S S Korsakova
December 2024
Bochkov Research Centre for Medical Genetics, Moscow, Russia.
A fifth world case of autosomal recessive Siddiqi syndrome (SIDDIS) related to ene is presented. In a consanguineous Lezgin (a Dagestan ethnicity) family, there were two affected brothers aged 28 yrs (proband, personally examined) and 32 yrs. Whole-exome sequencing followed by familial Sanger sequencing detected a novel missence variant c.
View Article and Find Full Text PDFZh Nevrol Psikhiatr Im S S Korsakova
December 2024
Federal Center of Brain Research and Neurotechnologies, Moscow, Russia.
According to the literature, a number of anti-epileptic drugs (AEDs) have an ototoxic effect. The mechanism of hearing dysfunction due to the use of AEDs is not well known. The main clinical manifestations of the cochleotoxic effect of the drugs are: tinnitus, sensorineural hearing loss, impaired pitch perception, hyperacusis.
View Article and Find Full Text PDFBMC Musculoskelet Disord
December 2024
Physical medicine & rehabilitation research center, School of medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Background: Pompe disease is a glycogen storage disease primarily affecting striated muscles. Despite its main manifestation in muscles, patients with Pompe disease may exhibit non-muscle symptoms, such as hearing loss, suggesting potential involvement of sensory organs or the nervous system due to glycogen accumulation.
Aims: This study aimed to evaluate the presence of concomitant small and large fiber neuropathy in patients with Pompe disease.
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