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Screening for congenital hearing impairment with brainstem evoked response audiometry in isolated orofacial cleft
Y Lill1, W V Cespedes2, B K Benitez1
1Oral and Craniomaxillofacial Surgery, University Hospital Basel and University of Basel, Basel, Switzerland; Department of Clinical Research, University of Basel, Basel, Switzerland; Department of Biomedical Engineering, University of Basel, Allschwil, Switzerland.
Insights
Brainstem evoked response audiometry (BERA) is crucial for diagnosing hearing loss in infants. For infants with orofacial clefts, BERA is recommended only for those with syndromic conditions, as they show a higher incidence of sensorineural hearing loss.
Area of Science:
- Audiology
- Pediatrics
- Genetics
Background:
- Brainstem evoked response audiometry (BERA) is the standard for diagnosing infant hearing impairment.
- The hearing risk status of infants with orofacial clefts remains unclear.
- Objective audiometry is essential for early detection of congenital hearing loss.
Purpose of the Study:
- To evaluate the necessity of diagnostic BERA in infants with cleft palate +/- cleft lip.
- To assess the predisposition to and diagnosis of congenital hearing impairment in this cohort.
- To determine if infants with orofacial clefts meet high-risk criteria for BERA screening.
Main Methods:
- Retrospective cohort study of 122 infants treated at a single cleft center.
- BERA performed during palate repair surgery (4-6 months of age).
- Clinical follow-up up to 4 years, analyzing syndrome presence as a risk factor.
Main Results:
- Four out of 122 infants had congenital sensorineural or mixed hearing loss requiring hearing aids.
- All affected infants had syndromes associated with their clefts.
- Transient conductive hearing loss was common; most suspected sensorineural hearing loss was not confirmed.
Conclusions:
- Congenital hearing impairment requiring intervention is primarily associated with syndromic clefts.
- Diagnostic BERA is recommended for infants with syndromic clefts at the time of initial surgery.
- Routine BERA for all infants with orofacial clefts may not be necessary, but syndromic association warrants screening.
Abstract:
Brainstem evoked response audiometry (BERA) is the most established and recommended objective audiometric method for the clinical diagnosis of hearing impairment in high-risk infants. It is unclear whether infants with orofacial clefts meet the criteria for the high-risk group. This retrospective cohort study evaluated the need for diagnostic BERA in infants with cleft palate with or without cleft lip by assessing the predisposition to and diagnosis of congenital hearing impairment. Data from 122 patients treated at a single cleft centre were evaluated. BERA was conducted at the time of palate repair at 4-6 months of age. Clinical follow-up was analysed up to 4 years. The presence of a syndrome was examined as a risk factor for congenital hearing impairment. Among the 122 patients, four had congenital sensorineural or mixed hearing loss requiring hearing aids. All affected patients had syndromes in addition to the cleft. Most patients with elevated hearing thresholds had transient conductive hearing loss. Most suspected sensorineural hearing loss initially diagnosed was refuted. However, a higher incidence of sensorineural hearing loss was found in patients with syndromic clefts, supporting the diagnostic use of BERA with initial surgery only in patients with syndromic clefts.
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