Aetiology of permanent childhood hearing loss at a population level
Karen Liddle1,2, Rachael Beswick3, E Jane Fitzgibbons3
1Child Development Service, Queensland Children's Hospital, Brisbane, Queensland, Australia.
Insights
Aetiological investigations identified causes for 61.3% of permanent childhood hearing loss cases. Genetic and structural factors were the most common causes identified in this population-level study.
Area of Science:
- Genetics
- Audiology
- Pediatrics
Background:
- Permanent childhood hearing loss (PCHL) affects 0.3% of newborns.
- Early identification and aetiological investigation are crucial for intervention.
- Population-level screening programs provide a unique opportunity to study PCHL causes.
Purpose of the Study:
- To evaluate and describe the results of aetiological investigations for PCHL.
- To identify the causes of hearing loss in a large cohort of infants and children.
- To assess the feasibility of a population-level coding scheme for hearing loss aetiology.
Main Methods:
- Descriptive analysis of a statewide newborn hearing screening program cohort (2013-2017).
- Confirmed hearing loss via audiological assessment.
- Medical evaluation by pediatricians/otolaryngologists with stepwise investigations and a coding scheme for aetiology.
Main Results:
- Permanent hearing loss confirmed in 967 children (0.3%).
- An aetiological factor was identified in 61.3% of cases with available data (n=873).
- Genetic (26.8%) and structural (24.9%) causes were most frequent; congenital cytomegalovirus (4.4%) was also noted.
Conclusions:
- A population-level coding scheme for hearing loss aetiology is feasible.
- This approach facilitates data collation from multiple sites.
- The scheme supports outcome mapping and future service planning for hearing loss.
Aim:
To evaluate and describe results of aetiological investigations offered to a population level cohort of babies who had confirmed permanent hearing loss after they either (i) failed universal neonatal hearing screening or (ii) passed newborn screening but were detected with a permanent hearing loss in early childhood.
Methods:
Descriptive analysis of results of investigations offered to neonates and young children in whom permanent hearing loss was detected as part of a statewide newborn hearing screening programme. A total of 306 285 newborns were screened between 2013 and 2017. The failed screening results were confirmed by a diagnostic audiological assessment battery. Medical evaluation for the identification of the cause of the hearing loss was performed by a paediatrician or otolaryngologist, investigations were ordered using a stepwise approach, and aetiology was assigned using a coding scheme.
Results:
Permanent hearing loss was confirmed in 967 children (0.3%). Data were available for 873. An aetiological factor was identified or presumed in 61.3% of cases. Genetic causes were present in 26.8% and structural causes were present in 24.9% of cases. Congenital cytomegalovirus was present in 4.4%.
Conclusions:
Use of a coding scheme is feasible at a population level and allows collation of data from multiple sites and will allow outcome mapping and service planning.
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