The relatively new term "auditory neuropathy spectrum disorder" (ANSD) describes auditory characteristics of people with normal outer hair cell function (as demonstrated via OAEs and/or cochlear microphonics) in tandem with aberrant/disordered neural conduction along the auditory pathway (as demonstrated by ABR). Children with ANSD are heterogeneous and, thus, treatment/management/habilitation of these children has remained controversial. Although, in general, professionals agree audiological and educational management of children with ANSD must be uniquely tailored to the needs of the individual child.
In their comprehensive article, Teagle et al (2010) reviewed 140 children with ANSD. Of the 140, 58 children received CIs. Of the 58 who received CIs, 50 of the children had bilateral ANSD, 8 of the children had bilateral hearing loss with unilateral ANSD, 52 of the children received their CI in their affected ear, and 22 of the children were born prematurely (mean age of gestation = 29 weeks). Complete electrode insertion was achieved in all except one primary implantations.
Teagle et al note that CIs can be an effective intervention strategy for children with ANSD. Of the children who received CIs, half have some degree of open-set speech perception ability and nearly three-fourths demonstrate substantial auditory skills (> 30 percent correct on PB-K words). Nonetheless, children with ANSD are heterogeneous and have a "wide variety" of comorbidities. Some children with ANSD will benefit from CIs, while others may not. Of those who do not, their lack of benefit may be due to a lack of electrically induced neural synchronization.
For More Information, References, and Recommendations
Teagle HFB, Roush PA, Woodard JS, Hatch DR, Zdanski CJ, Buss E, Buchman CA. (2010) Cochlear Implantation in Children with Auditory Neuropathy Spectrum Disorder. Ear & Hearing 31(3):325-335.