Cochlear implantation in a Mandarin Chinese-speaking child with auditory neuropathy

Auditory neuropathy (AN) is a hearing disorder characterized by the preservation of outer hair cell function despite the absence of auditory brainstem responses. The pathophysiology and etiology of this condition remain unknown. Recent studies have shown that some patients with AN benefit significantly from cochlear implantation. These patients have all been native speakers of Western languages. A 3-year-old Mandarin-speaking boy was referred to our center because of speech delay. After a series of audiological surveys, retro-cochlear lesion was impressed. During the 2-year period of rehabilitation, poor speech discrimination out of proportion to aided hearing thresholds led to the diagnosis of auditory neuropathy. Because of the limited benefit from amplification, he received a cochlear implant. Significant improvement of speech perception skills assessed by a Mandarin auditory perception test was noted shortly after implantation. The post-implantation performance in this Mandarin-speaking child was consistent with that of reports for implantees speaking Western languages. For Mandarin-speaking children with AN who fail to benefit from conventional treatment, cochlear implantation may be a good alternative choice.