To report the patient's characteristics, preoperative audiological profiles, surgical outcomes, and postoperative performance for children with auditory neuropathy spectrum disorder (ANSD) who ultimately received cochlear implants (CIs).
Prospective, longitudinal study of children with ANSD who received CIs after a stepwise management protocol that included electrophysiologic and medical assessment, documentation of behavioral audiometric thresholds and subsequent fitting of amplification according to Desired Sensation Level targets, auditory-based intervention with careful monitoring of skills development and communication milestones, and finally implantation when progress with the use of acoustic amplification was insufficient.
Of 140 children with ANSD, 52 (37%) received CIs in their affected ears (mean duration of use of 41 mos). Many of these children were born prematurely (42%) and impacted by a variety of medical comorbidities. More than one third (38%) had abnormal findings on preoperative magnetic resonance imaging of the brain and inner ear, and 81% had a greater than severe (>70 dB HL) degree of hearing loss before implantation. Although 50% of the implanted children with ANSD demonstrated open-set speech perception abilities after implantation, nearly 30% of them with >6 months of implant experience were unable to participate in this type of testing because of their young age or developmental delays. No child with cochlear nerve deficiency (CND) in their implanted ear achieved open-set speech perception abilities. In a subgroup of children, good open-set speech perception skills were associated with robust responses elicited on electrical-evoked intracochlear compound action potential testing when this assessment was possible.
This report shows that children with ANSD who receive CIs are a heterogeneous group with a wide variety of impairments. Although many of these children may ultimately benefit from implantation, some will not, presumably because of a lack of electrical-induced neural synchronization, the detrimental effects of their other associated conditions, or a combination of factors. When preoperative magnetic resonance imaging reveals central nervous system pathology, this portends a poor prognosis for the development of open-set speech perception, particularly when CND is evident. These results also show that electrical-evoked intracochlear compound action potential testing may help identify those children who will develop good open-set speech perception. Instead of recommending CI for all children with electrophysiologic evidence of ANSD, the stepwise management procedure described herein allows for the identification of children who may benefit from amplification, those who are appropriate candidates for cochlear implantation, and those who, because of bilateral CND, may not be appropriate candidates for either intervention.
The management of auditory neuropathy spectrum disorder (ANSD) in children remains challenging. Rather than categorically recommending cochlear implants for all children with the disorder, the present study used a stepwise management paradigm that includes medical assessment, documentation of behavioral audiometric thresholds, fitting of amplification, and finally implantation when progress in the use of acoustic amplification is insufficient. Results demonstrate the heterogeneous nature of the disorder and the variability in auditory perceptual skills using cochlear implants. Poor performance was associated with findings on preoperative magnetic resonance imaging and intracochlear compound action potential testing responses.
W. Paul Biggers Carolina Children's Communicative Disorders Program, Department of Otolaryngology-Head and Neck Surgery and the Division of Audiology, University of North Carolina Hospitals, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.
Address for correspondence: Craig A. Buchman, MD, Department of Otolaryngology-Head and Neck Surgery, University of North Carolina at Chapel Hill, G190 Physicians Office Building, 170 Manning Drive, CB#7600, Chapel Hill, NC 27599-7600. E-mail: firstname.lastname@example.org.
Received March 7, 2009; accepted December 3, 2009.