BACKGROUND: differential diagnosis between auditory neuropathy spectrum disorder andsteeply sloping cochlear hearing loss in children with presence of cochlear microphonics for click-ABR. PROCEDURES: this case report describes the results of the audiological evaluation for two children assisted at Centro "Audição na Criança" of Divisão de Educação e Reabilitação dos Distúrbios da Comunicação of Pontifícia Universidade Católica of São Paulo (CeAC/DERDIC/PUCSP) with presence of cochlear microphonics for click-ABR. The children are assessed by click-ABR, otoacoustic emissions and pure tone audiological evaluation, using the Visual Reinforcement Audiometry technique. RESULTS: the test battery showed that children have sensorineural hearing loss (cochlear) with steeply sloping configuration, so that the presence of cochlear microphonics in the click-ABR was probably generated by the preservation of the cochlea in the low frequencies. CONCLUSION: the cases demonstrated that the absence of the otoacustic emissions, and presence of cochlear microphonics should not be interpreted as Auditory Neuropathy Spectrum Disorder, since that these conditions are similar to steeply sloping cochlear hearing loss.
Evoked Potentials; Hearing Loss; Sensorineural; Cochlea
