Cochlear implantation in children with hypo- and aplasia of the auditory nerve

Introduction:

The diagnostic and therapeutic management of children with hypo- (HH) or aplasia (HA) of the auditory nerve confronts the treating center with a challenging task.

Methods:

Focusing on HH or HA, we performed a retrospective examination of all children ≤6 years, who received a cochlear implant (CI) preexamination including imaging (CT scan of the temporal bone/cranial MRI scan) in the period from 01/2011 to 07/2018 (n = 296). The findings, the medical treatment recommendation and the measures implemented for hearing rehabilitation were evaluated.

Results:

28 patients (9.5%) had the findings of HH (n = 21) or HA (n = 7). Twenty-five patients (89.3%) presented due to conspicuous neonatal hearing screening (NHS), 1 patient (3.6%) for performing a NHS and 2 patients (7.1%) presented due to deficient language development. In each of the 28 patients, the option of cochlear implantation was discussed and considered. In 16 patients (57.1%) the decision against cochlear implantation was made after a differentiated counseling, whereby 11 of these patients (39.3%) were provided with a hearing aid. In 7 patients (25.0%), the parents asked for time to think and did not return. Of the 28 patients (17.9%), 5 were finally implanted, with 80% demonstrating good acceptance of the implant.

Conclusion:

Cochlear implantation may be recommended in children with HH/HA in selected cases. However, acceptance and benefit are highly dependent on factors such as comorbidity, family environment and ability to rehabilitate. Differentiated preoperative evaluation and consultation is therefore of crucial importance.

Publication History

Publication Date:
23 April 2019 (online)