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.

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