The literature states that the absence of otoacoustic emissions (OAE) or presence at single frequencies does not exclude the diagnosis of an auditory neuropathy spectrum disorder (DENA). OBJECTIVE: To investigate auditory function (outer hair cells) through OAE in 3 children with DENA in order to identify any loss of cochlear function. CASE REPORT: The research was conducted in 3 children:2 brothers with bilateral profound sensorineural hearing loss and DENA treated at a rehabilitation center. All children underwent newborn hearing screening using OAE and "passed" the criterion of evaluation. Each child was also assessed with an ENT examination as well as tests of the Auditory Brainstem Response (ABR) acoustic reflexes. Two of the 3 children had undergone cochlear implantation (CI) at approximately 2 years of age, with the presence of OAEs noted in both cases. The other child who did not undergo surgery presented no OAEs when assessed several months after birth. CONCLUSION: We observed the presence of OAE at single frequencies in 2 children before cochlear implant surgery, which suggests a chronic loss of cochlear function.
