Abstract
Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder characterized by the absence of auditory brainstem response (ABR) and presence of otoacoustic emissions (OAE). Although in most patients, ANSD is bilateral, unilateral ANSD patients have also been rarely reported. Recently, OAE and automated ABR (AABR) have been used widely for screening of hearing in newborns. With the advent of these newborn hearing screening tests, we can identify hearing-impaired children early. ANSD cannot be detected by OAE screening alone, because children with ANSD may also have normal cochlear function and a neural hearing loss. Herein, we report the case of a 7 year-old girl who was diagnosed as having unilateral ANSD. Her school hearing screening test was followed by the comment,“refer,” and she was found by further examination to have hearing loss on the right side. Although ABR was absent in the right ear, OAE were present in both ears. At the time of birth, the child had undergone a newborn hearing screening test by OAE, however her hearing loss had been overlooked. From now on, careful follow-up of her hearing is considered to be important, because the hearing loss may vary over time.
