[Auditory neuropathy in childhood]

The combination of transient otoacoustic emissions (TOAE) and auditory brainstem responses (ABR) permits us to identify a group of patients with auditory neuropathy. The clinical and therapeutic aspects of these patients are a challenge for otological practice. The patients underwent ENT, neurological, and otological examination using audiometry, tympanometry, stapedial reflex, OAE, or ABR, depending on the patient. TOAE and tympanograms were normal in all cases. The stapedial reflex and ABR were absent in all cases. Logoaudiometry showed a disproportionate decrease in speech comprehension in relation to each patient's audiogram. These audiograms revealed mild-to-moderate hearing loss. These findings suggest that the lesion is sensorineural with normal function of the external hair cells. However, we could not determine if the lesion lay in the internal hair cells, the eighth cranial nerves, or both sites. The causes of hearing loss were: hyperbilirubinemia, perinatal asphyxia, or idiopathic. All the patients were treated by a speech therapist: The results obtained with hearing aids vary. Successful speech rehabilitation depends on early treatment.