Performance of Cochlear Implants in Pediatric Patients with Auditory Neuropathy Spectrum Disorder

Abstract

OBJECTIVE:To describe the performance and results of CIs (cochlear implant) in patients with AN (auditory neuropathy) and to present a medical literature review. MATERIALS and METHODS:Retrospective chart review of patients with AN who were treated with CI. The mesh terms used for the review in the Pubmed and Scopus databases were as follows: "hearing loss, cochlear implants, rehabilitation of persons with hearing impairment, auditory neuropathy". Statistical Analyses: The Mann-Whitney test was performed. RESULTS:The sample consisted of 10 patients. The mean age at surgery was 4.3 years, range 2-16 years. The average length of CI use was 5.2 years. The comparison of hearing levels before and after CI use showed a significant improvement in all patients, with p<0.05. All of them also reported an increase in overall satisfaction 1 year after the procedure. A CI is the standard treatment for the hearing rehabilitation of patients with severe profound hearing loss who do not benefit from conventional hearing aids. There are diseases such as AN that also invoke a discussion in the literature regarding CI benefits. CONCLUSION:Individuals with an demonstrated a significant gain in hearing levels and language use with CI. KEYWORDS:Auditory neuropathy spectrum disorder, evoked auditory brainstem response, otoacoustic emissions, GJB2, hearing loss, cochlear implants Guilherme Machado de Carvalho, Priscila Ramos, Castilho Arthur, Alexandre Guimarães, Edi SartoratoDepartment of Ear Nose Throat Head and Neck Surgery, UNICAMP (Campinas University, São Paulo, Brazil), Campinas, Brazil (GMDC, CA, AG) Centro de Biologia Molecular e Engenharia Genética, UNICAMP (Campinas University, São Paulo, Brazil), Campinas, Brazil (PR, ES) 8 Cochlear implants (CI) currently has ample evidence for its efficacy in the treatment of various forms of deafness, from unilateral to bilateral cases with severe to profound hearing loss. It also has been shown as an alternative for hearing rehabilitation in many conditions, such as in deafness with residual hearing at low frequencies and AN. In AN cases, CI is an option when the patient has not had a good response to conventional hearing aids and an adequate development of speech [1,3,5,10,11] .Although it has been demonstrated that the earlier use of CI in children with AN with severe or profound hearing loss leads to an important benefit in most cases, the situation is still challenging in cases when the hearing loss is moderate [1,3] . Thus, CI has shown conflicting results worldwide, probably because AN must be heterogeneous and have different etiologies [5] .A recent systematic review evaluated the improvement of speech development in children with AN treated with CIs and showed favorable results after CI regarding hearing and speech parameters but concluded that the evidence for CI is still weak and further studies are needed to clarify where and when CI could be applied as a good option for pediatric patients with AN [12] .Regarding the etiologic heterogeneity of AN, it is not surprising that ...

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 , …”

The audiovestibular profile of Brown-Vialetto-Van Laere syndrome

“…8 The benefit of cochlear implantation in auditory neuropathy spectrum disorder is well documented and has been shown to be comparable with outcomes in other paediatric implant patients. [27][28][29] More recently it has been suggested that the predicted outcomes from cochlear implantation in auditory neuropathy spectrum disorder depend on whether the pathology is pre-or post-synaptic, with pre-synaptic auditory neuropathy spectrum disorder benefitting comparably to those with cochlear sensorineural hearing loss whereas the outcomes for post-synaptic auditory neuropathy spectrum disorder are more variable. 30 It has been proposed that the poorer performance in post-synaptic auditory neuropathy spectrum disorder could be because of the effects on neural transmission of the cochlear implant electrical signal.…”