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Objectives: Age of cochlear implantation (CI) is an important predictor of language development in those with congenital sensorineural hearing loss. Despite universal newborn hearing screening initiatives and the known benefits of early CI, a subset of congenitally deaf children continue to be evaluated for cochlear implants later in childhood. This study aims to identify the barriers to early cochlear implantation in these children.
Methods: A retrospective review was conducted for all pediatric cochlear implants aged 3 years or older performed at a single academic institution between 2013 and 2017. Children implanted before the age three, those with a prior unilateral cochlear implant, and those with progressive or sudden hearing loss were excluded. Variables included newborn hearing screen results, age at hearing loss diagnosis, time of initiation and documented benefit of hearing aids, age of implantation, pre/post-implantation evaluation scores, and reason for delayed referral for cochlear implantation.
Results: Thirty-one patients were identified meeting these inclusion criteria. Twenty-one children were subject to UNBS in the U.S. Fourteen of those children failed their newborn hearing screening. Average age at implantation was 6.2 years. Four reasons were identified for increased age at cochlear implantation. Two categories represent delays related to (1) Amplification continually prescribed even though the range of hearing loss and speech development assessment suggests CI may have been more appropriate well before referral (N = 13) (2) Patients were not subject to newborn hearing screening and/or timely diagnosis of their hearing loss (N = 8). In other cases, patients were appropriately fit with hearing aids until evidence that they derived limited benefit and then referred for CI (N = 8). Lastly, in a few cases, records were indeterminate with regards to the timing and appropriate diagnosis of their hearing loss (N = 2).
Conclusion: Understanding the reasons for delayed cochlear implantation in congenitally deaf children might allow the development of targeted interventions to improve outcomes. Specifically, those children who were not referred before age 3 despite use of amplification with limited benefit offer one potential target population for earlier CI.
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http://dx.doi.org/10.1016/j.ijporl.2022.111086 | DOI Listing |
Hum Genet
September 2025
College of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Chinese PLA Medical School, 28 Fuxing Road, Beijing, 100853, China.
Recessive variants in TWNK cause syndromes arising from mitochondrial DNA (mtDNA) depletion. Hearing loss is the most prevalent manifestation in individuals with these disorders. However, the clinical and pathophysiological features have not been fully elucidated.
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September 2025
MED-EL-GmbH, Innsbruck, Austria.
Introduction: Age related hearing loss is in the top ten contributors to the global burden of disease and one of the largest modifiable risk factors for age-related dementia. However, awareness of the consequences of untreated hearing loss is poor and many adults do not seek hearing assessment. Despite World Health Organisation recommendations, no EU country currently has a national adult screening programme.
View Article and Find Full Text PDFBackground: Cytomegalovirus (CMV) viremia is a critical concern and known by the presence of the virus DNA in the blood, which poses sever risks and develops many complications in immuno-compromised patients. When CMV is untreated, it can cause pneumonitis, colitis, hepatitis, and encephalitis. Current diagnosis relies on molecular methods with qPCR as the preferred method.
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