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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Late-onset, progressive sensorineural hearing loss in the paediatric population: a systematic review
Virginia Corazzi1, Surina Fordington2, Tamsin Holland Brown3
1ENT and Audiology Unit, Department of Neurosciences and Rehabilitation, University Hospital of Ferrara, Via Aldo Moro, 8, 44124, Ferrara, Cona, Italy.
Insights
Congenital cytomegalovirus (cCMV) infection is a key risk factor for progressive childhood hearing loss, often missed by newborn screenings. Early audiological follow-up is crucial for at-risk infants to ensure timely intervention and treatment.
Area of Science:
- Pediatric Audiology
- Neonatal Health
- Infectious Diseases
Background:
- Delayed-onset, progressive sensorineural hearing loss (SNHL) in children presents diagnostic challenges.
- Standard newborn hearing screenings may not detect all cases of SNHL.
- Identifying risk factors is crucial for early detection and intervention.
Purpose of the Study:
- To review risk factors for permanent delayed-onset, progressive SNHL in children.
- To recommend follow-up protocols for early detection of SNHL.
- To inform audiological screening and management strategies.
Main Methods:
- A PRISMA-compliant systematic review of observational studies.
- Included children up to 16 years old who passed newborn hearing screening.
- Searches conducted in Medline, Embase, Cochrane, and Emcare.
Main Results:
- 21 studies linked late-onset SNHL to congenital cytomegalovirus (cCMV) infection.
- 16 studies associated SNHL with other factors: NICU stay, prematurity, respiratory failure, ECMO, CDH, and gene mutations.
- Hearing loss diagnosis occurred between 2.5 and 204 months of age.
Conclusions:
- Congenital CMV infection is a significant cause of late-onset SNHL, potentially missed by initial screenings.
- Universal screening for cCMV in neonates is supported.
- Recommended audiological follow-up for children with cCMV and other high-risk conditions (e.g., NICU stay >5 days, prematurity ≤34 weeks, severe respiratory issues, ECMO, CDH surgery) up to 3-4 years of age, at least annually.
Purpose:
To review possible risk factors for permanent delayed-onset, progressive sensorineural hearing loss (SNHL) in the paediatric population to recommend follow-up protocols for early detection.
Methods:
PRISMA-compliant systematic review was performed, including observational studies on the paediatric population up to 16 years old who have passed the newborn hearing screening programme (NHSP), investigating the development of late-onset, progressive SNHL. Electronic searches were performed through Medline, Embase, Cochrane, and Emcare.
Results:
37 studies were included. 21 showed an association between late-onset SNHL and congenital cytomegalovirus (cCMV) infection (age at hearing loss diagnosis 0.75 to 204 months, mean 45.6 ± 43.9), while 16 between late-onset SNHL and other congenital or perinatal factors, namely Neonatal Intensive Care Unit (NICU) stay, prematurity, neonatal respiratory failure, mechanical ventilation, extracorporeal membrane oxygenation (ECMO) support, hypocapnia, hypoxia, alkalosis, seizure activity, congenital diaphragmatic hernia (CDH), inner ear malformation, and gene mutations (age at hearing loss diagnosis 2.5 to 156 months, mean 38.7 ± 40.7).
Conclusions:
cCMV infection may cause late-onset SNHL, which can be missed on standard NHSP. There is, therefore, evidence to support universal screening programmes to enable detection in even asymptomatic neonates. Ongoing audiological follow-up for all children with cCMV is advisable, to enable timely treatment. In the paediatric population presenting conditions such as NICU stay > 5 days, prematurity ≤ 34 weeks gestation, severe neonatal respiratory failure, mechanical ventilation, ECMO support, and CDH surgery, an audiological follow-up from 3 months of age up to at least 3-4 years of age, and at least annually, should be recommended.
