Cochlear implantation in auditory neuropathy spectrum disorders: role of transtympanic electrically evoked auditory brainstem responses and serial neural response telemetry

Abstract

Objective To evaluate the utility of pre-operative transtympanic electrically evoked auditory brainstem responses and post-operative neural response telemetry in auditory neuropathy spectrum disorder patients. Methods Four auditory neuropathy spectrum disorder patients who had undergone cochlear implantation and used it for more than one year were studied. All four patients underwent pre-operative transtympanic electrically evoked auditory brainstem response testing, intra-operative and post-operative (at 3, 6 and 12 months after switch-on) neural response telemetry, and out-patient cochlear implant electrically evoked auditory brainstem response testing (at 12 months). Results Patients with better waveforms on transtympanic electrically evoked auditory brainstem response testing showed superior performance after one year of implant use. Neural response telemetry and electrically evoked auditory brainstem response measures improved in all patients. Conclusion Inferences related to cochlear implantation outcomes can be based on the waveform of transtympanic electrically evoked auditory brainstem responses. Robust transtympanic electrically evoked auditory brainstem responses suggest better performance. Improvements in electrically evoked auditory brainstem responses and neural response telemetry over time indicate that electrical stimulation is favourable in auditory neuropathy spectrum disorder patients. These measures provide an objective way to monitor changes and progress in auditory pathways following cochlear implantation.

Systematic Literature Review and Early Benefit of Cochlear Implantation in Two Pediatric Auditory Neuropathy Cases

“…In most publications, it was stated that there was a trial period with a conventional hearing aid before cochlear implantation, because this was part of the indications to be considered for a CI in the first place [ 6 , 7 , 8 , 12 , 21 , 29 , 31 , 32 , 33 , 34 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 ] with ANSD. If there was no improvement in speech understanding or language development with these interventions, the children were fitted with a CI.…”

“… Results for all included publications. AZBio = AZBio sentence test; BKB = Bamford–Kowal–Bench sentence test; CAEP = cortical auditory evoked potential, CAP = Categories of Auditory Performance; CID = Central Institute of Deafness test; CNC = Consonant–Nucleus–Consonant words; CVC = Consonant–Vowel–Consonant words; ESP = Early Speech perception test; HA = Hearing aid; HSM = Hochmair–Schulz–Moser sentence test; IT-MAIS = Infant–Toddler Meaningful Auditory integration scale; LNT = Lexical Neighbourhood Test; MAPTB = Mandarin Auditory Perception Test Battery; MLNT = Multisyllabic Lexical Neighbourhood Test; MSP = Melbourne Speech Perception; MUSS = Meaningful Use of Speech Scale; MWT = Monosyllabic Word Test; NU-CHIPS = Northwestern University—Children’s Perception of Speech; PBK = Phonetically Balanced Kindergarten word list; SADL = Satisfaction with Amplification in Daily Life; SIR = Speech Intelligibility Rating, SRT = Speech Recognition Threshold [ 4 , 5 , 6 , 9 , 12 , 15 , 16 , 17 , 18 , 19 , 20 , 22 , 23 , 24 , 25 , 29 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , …”

“…Apart from this outlier, it is clearly visible in Table 3 that an improvement was achieved with the use of CIs for the ANSD patients. Even though it is a big decision for parents because a CI implantation is an invasive procedure, most papers [ 6 , 7 , 8 , 12 , 22 , 29 , 31 , 32 , 45 , 46 , 47 , 48 , 51 , 53 , 54 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 65 , 66 , 68 , 69 , 71 , 79 ] advocate for a CI for children with ANSD because of the improved speech recognition or sound recognition.…”

Audiogram Configuration, Molecular Etiology, and Outcome of Cochlear Implantation in Postlingual Auditory Neuropathy Spectrum Disorder

“…Previously, Ji et al (32) reported that intraoperative NRT thresholds were only retrievable in 42.9% of ANSD patients, compared with 95.2% from SNHL patients. Dutt et al (33) reported the individual intraoperative NRT results of their ANSD cohort; NRT was obtained across all electrodes in only one patient and was not obtained on any electrode in half of their patients. However, these previous studies comprised ANSD patients who were clinically heterogeneous and genetically undiagnosed.…”

A Rare Case of Perrault Syndrome with Auditory Neuropathy Spectrum Disorder: Cochlear Implantation Treatment and Literature Review

“…Previous studies showed that individuals with ANSD in which the cochlear sensory system and the synapse are affected, which are bypassed by the CI, have optimal post-implantation outcomes. Besides, also some patients with ANSD due to neural damage benefit from implantation because the stimulus provided by the implant is synchronous and regular, but others show poor performance with CI because of the poor neural transmission of the electrical signal [ 26 , 27 ]. ANSD can also be assessed by electrocochleography showing an absent or abnormal compound action potential, even at high stimulations, and the presence of a robust cochlear microphonic.…”