Auditory neuropathy (AN) is characterized by varying degrees of sensorineural hearing loss, an absent or severely abnormal auditory brainstem response, and normal otoacoustic emissions. The nomenclature for this condition reflects the concept that the site of lesion is proximal to the cochlea (e.g., cochlear nerve). Given this hypothesis, it is reasonable to expect limited benefit from cochlear implantation in patients with AN. However, a growing body of evidence shows the striking benefits of cochlear implantation in AN. To explore this topic, we reviewed our population of children with AN and, specifically, the performance results in those children having undergone cochlear implantation.
A retrospective case review of those patients diagnosed with AN from 1993 to 2001.
A tertiary pediatric referral center.
A diagnosis of AN reported from the Center for Hearing and Deafness Research, Cincinnati, OH, database.
Eighteen patients were diagnosed with AN (11 girls, 7 boys), with 3 sets of siblings, including 1 set of identical twins. Four patients with AN underwent implantation in the previous 5 years. Twelve out of the 18 patients had classic risk factors for AN (e.g., prematurity and hyperbilirubinemia). The degree of hearing loss varied in our patients, with a majority showing severe to profound deficits. All children with implants showed improvement in auditory and verbal development, but this improvement was variable.
The success of cochlear implantation in these patients suggests that some children with AN have an auditory system lesion that can be compensated for by cochlear implantation. This implies either an inner hair cell or inner hair cell–cochlear nerve junctional pathology that can be overcome by direct electrical stimulation.