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Analysis of congenital hearing loss after neonatal hearing screening
Gill Verstappen1, Ina Foulon1, Kelsey Van den Houte1
1Department of Otorhinolaryngology-Head and Neck Surgery, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Health Campus, Brussels, Belgium.
Insights
Neonatal hearing screening identifies infants with permanent hearing loss, with genetic causes and anatomical abnormalities being the most common etiologies. Advanced techniques have improved etiological diagnoses over 21 years.
Area of Science:
- Pediatrics
- Audiology
- Genetics
Background:
- Neonatal hearing screening is crucial for early detection of congenital hearing loss.
- Prompt etiological workup is essential for timely and appropriate treatment of confirmed hearing loss.
- Understanding etiologies and risk factors aids in managing permanent hearing loss in neonates.
Purpose of the Study:
- To assess etiologies, risk factors, and hearing outcomes in infants with permanent hearing loss.
- To evaluate the efficacy and consequences of neonatal hearing screening devices over 21 years.
- To analyze the evolution of etiological diagnostic protocols.
Main Methods:
- Retrospective cohort analysis of neonatal hearing screening referrals.
- Etiological workup for infants with confirmed hearing loss.
- Analysis of screening program data over a 21-year period.
Main Results:
- Of 545 referrals, 362 had confirmed hearing loss, and 133 were diagnosed with permanent hearing loss.
- Sensorineural hearing loss (moderate to profound) was predominant, with genetic causes in bilateral and anatomical abnormalities in unilateral cases.
- Familial history of hearing loss was a significant risk factor.
Conclusions:
- Neonatal hearing screening yields a high rate of false positives but is vital for identifying hearing loss.
- Genetic causes and anatomical abnormalities are key etiologies, with diagnostic capabilities improving over time.
- A portion of hearing loss etiologies remain unknown despite extensive investigations.
Introduction:
Neonates undergo neonatal hearing screening to detect congenital hearing loss at an early stage. Once confirmed, it is necessary to perform an etiological workup to start appropriate treatment. The study objective was to assess the different etiologies, risk factors, and hearing results of infants with permanent hearing loss and to evaluate the efficacy and consequences of the different screening devices over the last 21 years.
Methods:
We conducted a single-center retrospective cohort analysis for all neonatal hearing screening program referrals and performed an etiological workup in case of confirmed hearing loss. We analyzed the evolution of the etiological protocols based on these results.
Results:
The governmental neonatal hearing screening program referred 545 infants to our center. Hearing loss was confirmed in 362 (66.4%) infants and an audiological workup was performed in 458 (84%) cases. 133 (24.4%) infants were diagnosed with permanent hearing loss. Ninety infants (56 bilateral and 34 unilateral) had sensorineural hearing loss, and the degree was predominantly moderate or profound. The most common etiology in bilateral sensorineural hearing loss was a genetic etiology (32.1%), and in unilateral sensorineural hearing loss, an anatomical abnormality (26.5%). Familial history of hearing loss was the most frequently encountered risk factor.
Conclusion:
There is a significant number of false positives after the neonatal hearing screening. Permanent hearing loss is found only in a limited number of infants. During the 21 years of this study, we noticed an increase in etiological diagnoses, especially genetic causes, due to more advanced techniques. Genetic causes and anatomical abnormalities are the most common etiology of bilateral and unilateral sensorineural hearing loss, respectively, but a portion remains unknown after extensive examinations.
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