Electroacoustic Stimulation: Now and into the Future

Cochlear implants have provided hearing to hundreds of thousands of profoundly deaf people around the world. Recently, the eligibility criteria for cochlear implantation have been relaxed to include individuals who have some useful residual hearing. These recipients receive inputs from both electric and acoustic stimulation (EAS). Implant recipients who can combine these hearing modalities demonstrate pronounced benefit in speech perception, listening in background noise, and music appreciation over implant recipients that rely on electrical stimulation alone. The mechanisms bestowing this benefit are unknown, but it is likely that interaction of the electric and acoustic signals in the auditory pathway plays a role. Protection of residual hearing both during and following cochlear implantation is critical for EAS. A number of surgical refinements have been implemented to protect residual hearing, and the development of hearing-protective drug and gene therapies is promising for EAS recipients. This review outlines the current field of EAS, with a focus on interactions that are observed between these modalities in animal models. It also outlines current trends in EAS surgery and gives an overview of the drug and gene therapies that are clinically translatable and may one day provide protection of residual hearing for cochlear implant recipients.

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The Limitations of FDA Criteria: Inconsistencies with Clinical Practice, Findings, and Adult Criteria as a Barrier to Pediatric Implantation

Seminars in Hearing ◽

10.1055/s-0041-1739370 ◽

2021 ◽

Vol 42 (04) ◽

pp. 373-380

Author(s):

Lisa R. Park ◽

Erika B. Gagnon ◽

Kevin D. Brown

Keyword(s):

Clinical Practice ◽

Cochlear Implant ◽

Cochlear Implants ◽

Cochlear Implantation ◽

Spoken Language ◽

Evidence Based ◽

Residual Hearing ◽

Off Label ◽

Single Side ◽

Hearing Children

AbstractChildren require greater access to sound than adults as they are learning to communicate using hearing and spoken language. Yet when it comes to cochlear implant candidacy, currently approved Food and Drug Administration (FDA) criteria for adults are much less restrictive than those for children, allowing for greater levels of residual hearing and aided speech recognition in adults. Cochlear implant guidelines for children have changed very little in the 30 years since cochlear implants have been approved for pediatrics, and this lack of change has proven to be a barrier to implantation. Using evidence-based practice, centers have been providing off-label implantation for children who fall outside of current FDA criteria, including children with more residual hearing, children with single-side deafness younger than 5 years, and infants with bilateral profound loss younger than 9 months. The purpose of this article is to outline how these restrictions impede access to implants for children and describe the evidence supporting cochlear implantation in children who fall outside of current criteria.

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Cochlear Implantation Outcome in Children with DFNB1 locus Pathogenic Variants

Journal of Clinical Medicine ◽

10.3390/jcm9010228 ◽

2020 ◽

Vol 9 (1) ◽

pp. 228 ◽

Cited By ~ 1

Author(s):

Dominika Oziębło ◽

Anita Obrycka ◽

Artur Lorens ◽

Henryk Skarżyński ◽

Monika Ołdak

Keyword(s):

Cochlear Implants ◽

Hearing Aids ◽

Cochlear Implantation ◽

Treatment Method ◽

Wide Frequency Range ◽

Residual Hearing ◽

Frequency Range ◽

Pathogenic Variants ◽

Auditory Development ◽

Shed Light

Almost 60% of children with profound prelingual hearing loss (HL) have a genetic determinant of deafness, most frequently two DFNB1 locus (GJB2/GJB6 genes) recessive pathogenic variants. Only few studies combine HL etiology with cochlear implantation (CI) outcome. Patients with profound prelingual HL who received a cochlear implant before 24 months of age and had completed DFNB1 genetic testing were enrolled in the study (n = 196). LittlEARS questionnaire scores were used to assess auditory development. Our data show that children with DFNB1-related HL (n = 149) had good outcome from the CI (6.85, 22.24, and 28 scores at 0, 5, and 9 months post-CI, respectively). A better auditory development was achieved in patients who receive cochlear implants before 12 months of age. Children without residual hearing presented a higher rate of auditory development than children with responses in hearing aids over a wide frequency range prior to CI, but both groups reached a similar level of auditory development after 9 months post-CI. Our data shed light upon the benefits of CI in the homogenous group of patients with HL due to DFNB1 locus pathogenic variants and clearly demonstrate that very early CI is the most effective treatment method in this group of patients.

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Benefit of Cochlear Implantation in Children with Multiple-handicaps: Parent's Perspective

International Archives of Otorhinolaryngology ◽

10.1055/s-0038-1642607 ◽

2018 ◽

Vol 22 (04) ◽

pp. 415-427 ◽

Cited By ~ 3

Author(s):

Heloisa Nasralla ◽

Adilson Montefusco ◽

Ana Hoshino ◽

Paola Samuel ◽

Ana Magalhães ◽

...

Keyword(s):

Cochlear Implants ◽

Oral Language ◽

Cochlear Implantation ◽

Autism Spectrum ◽

Music Appreciation ◽

Social Emotional ◽

Handicapped Children ◽

Daily Routines ◽

School Activities ◽

The Social

Introduction The cochlear implants centers in the world are studying the cochlear implantation in children with multiple handicaps. Objective To develop a questionnaire to analyze the subjective benefits of the cochlear implantation in multiple handicapped children according to their parent's perspective. Methods A questionnaire was applied to 14 families of multiple handicapped children, aging from 2–12 years old and having from 11 months to 11 years of implant use. Results The social-emotional abilities were improved because of many factors, such as: auditory exposure, which happened in 84% of the children; recognition of their own names, which increased in 56%; and development of eye contact, in 28% of the subjects. Other benefits appeared to be: music appreciation and more attention and adherence to other therapies and school activities. Besides, some children became interested in objects, playing with other children, and more adapted to daily routines. Thirty-five percent of the children acquired oral language, mainly the bilaterally implanted, while 14% of them were engaged in sign language. Although all of the children showed a significant improvement in communication, the emotional issues of some families and the severity of the handicaps negatively impacted the outcomes. In spite of the families' acknowledgement of some benefits, the diagnosis of autism spectrum disorder caused frustration, requiring a readjustment of the expectations. Conclusion The questionnaire turned out to be an adequate tool to reveal the social-emotional benefits of cochlear implantation. Although oral language was not the major outcome in these cases, the cochlear implant benefits involved the whole family. All of the families recommended the implant to other children in a similar situation.

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Hearing Preservation Cochlear Implants: A Review of Potential Mechanisms of Hearing Loss After Implantation

Perspectives of the ASHA Special Interest Groups ◽

10.1044/persp2.sig6.54 ◽

2017 ◽

Vol 2 (6) ◽

pp. 54-63

Author(s):

Conor Kelly ◽

Lina A. J. Reiss

Keyword(s):

Hearing Loss ◽

Cochlear Implants ◽

High Frequency ◽

Music Perception ◽

Low Frequency ◽

Lateral Wall ◽

Hearing Preservation ◽

Acoustic Stimulation ◽

Residual Hearing ◽

Wide Range

Hearing preservation cochlear implants (CIs) are specifically designed to preserve residual low-frequency acoustic hearing for use together with electrically stimulated high-frequency hearing. This combined electro-acoustic stimulation (EAS) provides a promising treatment option for patients with severe high-frequency hearing loss, but with some residual low-frequency hearing, and has been shown to improve speech perception, especially in background noise, music perception, and sound source localization. Thus, preservation of residual hearing should be a priority in treatment. Although residual low-frequency hearing is successfully preserved to varying degrees in many patients, some patients experience a loss of residual hearing following implantation. A wide range of potential causes of, or contributors to, loss of residual hearing in EAS CI users have been proposed. In this paper, we review the evidence for several of the proposed causes of hearing loss with EAS CI. We conclude that its etiology is likely a multifactorial, heterogeneous phenomenon. Furthermore, we suggest that studies to further elucidate effects of ischemia on lateral wall function and maintenance of endocochlear potential in the context of EAS CI implantation and use are needed.

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Hearing Preservation after Cochlear Implantation: UNICAMP Outcomes

International Journal of Otolaryngology ◽

10.1155/2013/107186 ◽

2013 ◽

Vol 2013 ◽

pp. 1-8 ◽

Cited By ~ 4

Author(s):

Guilherme Machado de Carvalho ◽

Alexandre C. Guimaraes ◽

Alexandre S. M. Duarte ◽

Eder B. Muranaka ◽

Marcelo N. Soki ◽

...

Keyword(s):

Speech Perception ◽

Surgical Technique ◽

Hearing Aids ◽

Cochlear Implantation ◽

Hearing Preservation ◽

Acoustic Stimulation ◽

Residual Hearing ◽

Low Frequencies ◽

Great Satisfaction ◽

New Device

Background. Electric-acoustic stimulation (EAS) is an excellent choice for people with residual hearing in low frequencies but not high frequencies and who derive insufficient benefit from hearing aids. For EAS to be effective, subjects' residual hearing must be preserved during cochlear implant (CI) surgery.Methods. We implanted 6 subjects with a CI. We used a special surgical technique and an electrode designed to be atraumatic. Subjects' rates of residual hearing preservation were measured 3 times postoperatively, lastly after at least a year of implant experience. Subjects' aided speech perception was tested pre- and postoperatively with a sentence test in quiet. Subjects' subjective responses assessed after a year of EAS or CI experience.Results. 4 subjects had total or partial residual hearing preservation; 2 subjects had total residual hearing loss. All subjects' hearing and speech perception benefited from cochlear implantation. CI diminished or eliminated tinnitus in all 4 subjects who had it preoperatively. 5 subjects reported great satisfaction with their new device.Conclusions. When we have more experience with our surgical technique we are confident we will be able to report increased rates of residual hearing preservation. Hopefully, our study will raise the profile of EAS in Brazil and Latin/South America.

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Preservation of residual hearing following cochlear implantation: comparison between three surgical techniques

The Journal of Laryngology & Otology ◽

10.1017/s0022215107000254 ◽

2007 ◽

Vol 122 (3) ◽

pp. 246-252 ◽

Cited By ~ 36

Author(s):

S Berrettini ◽

F Forli ◽

S Passetti

Keyword(s):

Cochlear Implant ◽

Cochlear Implantation ◽

Surgical Techniques ◽

Electrode Array ◽

Lateral Wall ◽

Hearing Preservation ◽

Acoustic Stimulation ◽

Residual Hearing ◽

Electrode Arrays ◽

Electrode Insertion

AbstractThe preservation of residual hearing is becoming a high priority in cochlear implant surgery. It allows better speech understanding and ensures long-lasting and stable performance; it also allows the possibility, in selected cases, of combining electro-acoustic stimulation in the same ear.We present the results of a retrospective study of the conservation of residual hearing in three different groups of patients who had undergone cochlear implantation using three different cochlear implant electrode arrays, combined with three different surgical techniques for the cochleostomy. The study aimed to evaluate which approach allowed greater preservation of residual hearing.The best residual hearing preservation results (i.e. preservation in 81.8 per cent of patients) were achieved with the Contour Advance electrode array, using the Advance Off-Stylet technique and performing a modified anterior inferior cochleostomy; this combination enabled reduced trauma to the lateral wall of the cochlea during electrode insertion.

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805 Eluting Electrodes for Hearing Preservation – the Future of Cochlear Implantation?

British Journal of Surgery ◽

10.1093/bjs/znab134.581 ◽

2021 ◽

Vol 108 (Supplement_2) ◽

Author(s):

Y Nikookam ◽

A Radotra ◽

C Metcalfe ◽

J Muzaffar ◽

P Monksfield ◽

...

Keyword(s):

Cochlear Implants ◽

Cochlear Implantation ◽

Web Of Science ◽

Hearing Preservation ◽

Narrative Synthesis ◽

Residual Hearing ◽

Inclusion Criteria ◽

Growth Promoting ◽

Anti Inflammatory ◽

Electrode Insertion

Abstract Introduction Cochlear implants (CIs) utilising eluting electrodes are a relatively new phenomenon. CI surgery can lead to a variety of complications, namely electrode insertion trauma. This affects CI function leading to a loss of residual hearing. To minimise this, electrodes eluting anti-inflammatory or growth-promoting agents can be used to protect and enhance residual hearing through the sustained release of an agent. Eluting agents include glucocorticoids, steroids, growth factors and neurotrophins. Method Systematic review and narrative synthesis. Databases searched MEDLINE, EMBASE, CENTRAL, ClinicalTrials.gov and Web of Science. Results Searches identified 66 abstracts and 37 full texts. Of these, 15 studies met the inclusion criteria, reporting outcomes in 469 animals and 24 humans, with at least 233 implants. Eluting electrodes included dexamethasone (n = 13), growth factor (n = 1) and neurotrophins (n = 1). Audiological outcomes improved across all studies following cochlear implantation with an eluting electrode to a greater extent when compared to a standard electrode. Outcomes improved significantly when a higher concentration of dexamethasone was used. Conclusions Audiological outcomes with eluting electrodes following cochlear implantation are generally superior to the omission of medication following cochlear implantation. An electrode elution enables a sustained, localised release of anti-inflammatory or anti-apoptotic agents, therefore reducing the advent of fibrosis, scarring and subsequent impedance.

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Hearing preservation in children with electric-acoustic stimulation after cochlear implantation

HNO ◽

10.1007/s00106-018-0532-3 ◽

2018 ◽

Vol 66 (S2) ◽

pp. 56-62 ◽

Cited By ~ 2

Author(s):

T. Rader ◽

A. Bohnert ◽

C. Matthias ◽

D. Koutsimpelas ◽

M-A. Kainz ◽

...

Keyword(s):

Cochlear Implantation ◽

Low Frequency ◽

Hearing Preservation ◽

Acoustic Stimulation ◽

Residual Hearing ◽

Short Term ◽

Mean Values ◽

Minimal Trauma ◽

Electric Acoustic Stimulation ◽

The Mean

Abstract Background Cochlear implantation in patients with functional residual low-frequency hearing is performed according to an established hearing-preserving surgical technique in order to cause minimal trauma of inner ear structures. Due to the increasing number of cochlear implants in children, the preservation of residual hearing is becoming increasingly important in this patient collective. Objectives Short- and mid-term hearing preservation outcome in pediatric patients is investigated. Materials and methods A group of 9 children (12 ears) between 5 and 12 years of age were examined after hearing-assisted cochlear implantation with respect to the pure tone audiometric thresholds. Retrospectively, short-term hearing preservation (up to 3 months after surgery) was examined. In a subgroup of 5 children, mid-term hearing preservation (7.5 to 16 months after surgery) was also analyzed. The mean values of hearing preserved (HL%) and hearing loss (HL) due to electrode insertion were calculated as measured values. Results In the whole group, the mean values of the preoperative PTAlow were 29.8 dB and the short-term postoperative PTAlow 42.6 dB. The mean value of the HL% was 73.6%, corresponding to an HL of 9.4 dB. In the subgroup, the mean PTAlow postoperatively was 46.0 dB in the mid-term and the HL% at 80.7% with a HL of 6.6 dB. Conclusions The results in children are consistent with the results in adults. Electric-acoustic stimulation (EAS) should be used in the treatment of children with existing low-frequency residual hearing, as good residual hearing preservation can also be achieved in children after implantation.

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