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Published on: September 7, 2022
[Prevalence of auditory neuropathy: prospective study in a tertiary-care center]
Francisco Javier Rodríguez Domínguez1, José Domingo Cubillana Herrero, Nieves Cañizares Gallardo
1Sección de Otorrinolaringología Infantil, Servicio de Otorrinolaringología, Hospital Universitario Virgen de la Arrixaca, Murcia, España. franrodrguez@yahoo.es
Insights
Auditory neuropathy is not rare in infants, affecting 5.26% of sensorineural hearing loss cases. Delays in diagnosing idiopathic auditory neuropathy (AN) occur due to screening limitations.
Area of Science:
- Pediatric Audiology
- Neonatal Screening
- Neurology
Context:
- Auditory neuropathy (AN) presents diagnostic challenges in infants.
- Current neonatal hearing screening methods may miss idiopathic AN cases.
- Early detection of AN is crucial for timely intervention.
Purpose:
- To determine the frequency and prevalence of auditory neuropathy in an infant population.
- To identify diagnostic delays associated with idiopathic AN.
- To evaluate the effectiveness of current screening programs for AN.
Summary:
- A prospective study at a Spanish hospital (2000-2006) screened 114 infants with sensorineural hearing loss.
- Auditory neuropathy accounted for 5.26% of these cases, with a prevalence of 14.06 per 10,000 children.
- Idiopathic AN cases caused diagnostic delays, with a mean age of diagnosis at 11.5 months.
Impact:
- Auditory neuropathy is more common than previously thought.
- Current universal newborn hearing screening may result in false negatives for AN.
- Improved screening protocols are needed to reduce diagnostic delays and improve outcomes for infants with AN.
Objective:
The primary goal of this study is to determine the frequency of onset of this disorder in our infant population. Its clinical importance is due, among other reasons, to the fact that idiopathic cases constitute false negatives in the auditory screening programmes based on the performance of otoacoustic emissions to all newborn children and brainstem auditory evoked response only to those presenting these risk factors, for which reason another goal is to establish the prevalence of these pure cases and the diagnostic delay they cause.
Patients And Method:
Prospective study of all newborn children at the Virgen de la Arrixaca Mother and Child Hospital (Murcia, Spain) in the period between June 1, 2000 and June 30, 2006.
Results:
Our screening programme, with a coverage of 95.68 %, detected 114 patients with unilateral or bilateral sensorineural hearing loss, with 6 presenting hearing loss attributable to auditory neuropathy.
Conclusions:
The estimated prevalence of auditory neuropathy in our infant population turned out to be 1406 for every 10 000 children, ie 5.26 % of all sensorineural hearing loss diagnosed. Another significant finding was the delay in diagnosis, since the mean age at the moment of diagnosis was of 11.5 months, mainly due to idiopathic cases not detected during neonatal screening. Two clear conclusions can be inferred from these data: a) auditory neuropathy does not constitute an extraordinarily rare disorder, and b) idiopathic cases constitute false negatives in the universal auditory screening programmes based on the performance of otoacoustic emissions, which habitually leads to a delay in diagnosis and treatment.
