Determination of Benefits of Cochlear Implantation in Children with Auditory Neuropathy

Abstract

BackgroundAuditory neuropathy (AN) is a recently recognized hearing disorder characterized by intact outer hair cell function, disrupted auditory nerve synchronization and poor speech perception and recognition. Cochlear implants (CIs) are currently the most promising intervention for improving hearing and speech in individuals with AN. Although previous studies have shown optimistic results, there was large variability concerning benefits of CIs among individuals with AN. The data indicate that different criteria are needed to evaluate the benefit of CIs in these children compared to those with sensorineural hearing loss. We hypothesized that a hierarchic assessment would be more appropriate to evaluate the benefits of cochlear implantation in AN individuals.MethodsEight prelingual children with AN who received unilateral CIs were included in this study. Hearing sensitivity and speech recognition were evaluated pre- and postoperatively within each subject. The efficacy of cochlear implantation was assessed using a stepwise hierarchic evaluation for achieving: (1) effective audibility, (2) improved speech recognition, (3) effective speech, and (4) effective communication.ResultsThe postoperative hearing and speech performance varied among the subjects. According to the hierarchic assessment, all eight subjects approached the primary level of effective audibility, with an average implanted hearing threshold of 43.8 ± 10.2 dB HL. Five subjects (62.5%) attained the level of improved speech recognition, one (12.5%) reached the level of effective speech, and none of the subjects (0.0%) achieved effective communication.ConclusionCIs benefit prelingual children with AN to varying extents. A hierarchic evaluation provides a more suitable method to determine the benefits that AN individuals will likely receive from cochlear implantation.

Ensino por múltiplos exemplares e integração de comportamentos de ouvinte e falante com unidades sintáticas substantivo-adjetivo em crianças com DENA e IC

“…The inter-participant variability can be identified in this study from the performances that were unstable along consecutive teaching sessions and that did not keep stable between two consecutive sessions, what replicates the results found in the case study of Rique et al (16) . The variability results between participants, in the present study, is similar to the ones found in studies of other centers (8) and suggest that the variability between children with ANSD and CI is recurrent in relation to the learning rhythm, to the number of repetitions and to order of acquisition of the speaker's behavior (tact and echoic).…”

“…Dado has used CI for five months, detected speech sounds (1 of listen) and spoke isolated words (2 of language), while Flor had more than three years of CI use, recognized words in closed set (3 of listen) and constructed short sentences (4 of language). This difference in CI time of using and in the previous hearing and language categories could predict intervention success (5,8) , supposing it is more effective for Flor than for Dado. However, the present study showed that these children with ANSD and CI learned and integrated the listener and speaker tasks, by MEI, regardless the short time of CI use and the variability presented (6) .…”

“…In the cases of ANSD, this device can minimize auditory dyssynchrony, once it partially replaces the functions of the hearing sensorial cells and directly stimulates the auditory nerve (6) . The neural synchrony improvement by means of CI can deeply contribute to the learning of the listening and speaking abilities of children with ANSD (8) .…”

Elongated EABR wave latencies observed in patients with auditory neuropathy caused by OTOF mutation

“…19 Treatment of profound-to-severe hearing loss in patients with AN requires cochlear implantation (CI); however, the efficacy of CI in such cases is controversial. [20][21][22][23][24] On the other hand, AN caused by OTOF mutations is thought to be a better candidate for cochlear implantation because the electrode can stimulate the auditory nerves directly, thus bypassing impaired synapses. Several reports have demonstrated an adequate level of cochlear implant performance in patients with OTOF mutations.…”

Cochlear implant: indications, contraindications and complications

“…In several studies it was reported that patients had some forms of hereditary nonsyndromic autosomal dominant auditory neuropathy progressing to outer hair cell malfunction causing decline in pure-tone audiometer results. Some of the patients in these series benefitted from hearing aids or FM devices but in some a CI is the only way to communicate (10)(11)(12).…”