ABSTRACT: Cochlear implants are the treatment of choice for auditory rehabilitation of patients with sensory deafness. They restore the missing function of inner hair cells by transforming the acoustic signal into electrical stimuli for activation of auditory nerve fibers. Due to the very fast technology development, cochlear implants provide open-set speech understanding in the majority of patients including the use of the telephone. Children can achieve a near to normal speech and language development provided their deafness is detected early after onset and implantation is performed quickly thereafter. The diagnostic procedure as well as the surgical technique have been standardized and can be adapted to the individual anatomical and physiological needs both in children and adults. Special cases such as cochlear obliteration might require special measures and re-implantation, which can be done in most cases in a straight forward way. Technology upgrades count for better performance. Future developments will focus on better electrode-nerve interfaces by improving electrode technology. An increased number of electrical contacts as well as the biological treatment with regeneration of the dendrites growing onto the electrode will increase the number of electrical channels. This will give room for improved speech coding strategies in order to create the bionic ear, i.e. to restore the process of natural hearing by means of technology. The robot-assisted surgery will allow for high precision surgery and reliable hearing preservation. Biological therapies will support the bionic ear. Methods are bio-hybrid electrodes, which are coded by stem cells transplanted into the inner ear to enhance auto-production of neurotrophins. Local drug delivery will focus on suppression of trauma reaction and local regeneration. Gene therapy by nanoparticles will hopefully lead to the preservation of residual hearing in patients being affected by genetic hearing loss. Overall the cochlear implant is a very powerful tool to rehabilitate patients with sensory deafness. More than 1 million of candidates in Germany today could benefit from this high technology auditory implant. Only 50,000 are implanted so far. In the future, the procedure can be done under local anesthesia, will be minimally invasive and straight forward. Hearing preservation will be routine.
Project description:Cochlear implantation, a surgical method to bypass cochlear hair cells and directly stimulate the spiral ganglion, is the standard treatment for severe-to-profound hearing loss. Changes in cochlear implant electrode array design and surgical approach now allow for preservation of acoustic hearing in the implanted ear. Electrocochleography (ECochG) was performed in eight hearing preservation subjects to assess hair cell and neural function and elucidate underlying genetic hearing loss. Three subjects had pathogenic variants in TMPRSS3 and five had pathogenic variants in genes known to affect the cochlear sensory partition. The mechanism by which variants in TMPRSS3 cause genetic hearing loss is unknown. We used a 500-Hz tone burst to record ECochG responses from an intracochlear electrode. Responses consist of a cochlear microphonic (hair cell) and an auditory nerve neurophonic. Cochlear microphonics did not differ between groups. Auditory nerve neurophonics were smaller, on average, in subjects with TMPRSS3 deafness. Results of this proof-of-concept study provide evidence that pathogenic variants in TMPRSS3 may impact function of the spiral ganglion. While ECochG as a clinical and research tool has been around for decades, this study illustrates a new application of ECochG in the study of genetic hearing and deafness in vivo.
Project description:BACKGROUND:Cochlear implantation for single-sided deafness (SSD) is the only treatment option with the potential to restore binaural hearing cues. Significant binaural benefit has been measured in adults by speech in noise and localisation tests, who receive a cochlear implant for SSD, however, little is known on the cortical changes that help provide this benefit. In the present study, detection of sound in the auditory cortex, speech testing and localisation was used to investigate the ability of a cochlear implant (CI) to restore auditory cortical latencies and improve binaural benefit in the adult SSD population. METHODS:Twenty-nine adults with acquired single-sided deafness who received a CI in adulthood were studied. Speech perception in noise was tested using the Bamford-Kowal-Bench speech-in-noise test, localisation ability was measured using the auditory speech sounds evaluation (A?E) localisation test and cortical auditory evoked responses, comparing N1-P2 latencies recorded from the normal hearing ear and cochlear implant were used to investigate the synchrony of the cortical pathway from the CI and normal hearing ear (NHe) with binaural hearing function. RESULTS:There was a significant improvement in speech perception in noise in all spatial configurations S0/N0 (Z = -3.066, p<0.002), S0/NHE (Z = -4.031, p<0.001), SCI/NHE (Z = -3.851, p<0.001). Localization significantly improved when tested with the cochlear implant on (p<0.001) with a shorter duration of deafness correlating to a greater improvement in localisation ability F(1:18) = 6.854; p = 0.017). There was no significant difference in N1-P2 latency recorded from the normal hearing ear and the CI. CONCLUSION:Cortical auditory evoked response latencies recorded from the CI and NHe showed no significant difference, indicating that the detection of sound in the auditory cortex occurred simultaneously, providing the cortex with auditory information for binaural hearing.
Project description:In many countries, a single cochlear implant is offered as a treatment for a bilateral hearing loss. In cases where there is asymmetry in the amount of sound deprivation between the ears, there is a dilemma in choosing which ear should be implanted. In many clinics, the choice of ear has been guided by an assumption that the reorganisation of the auditory pathways caused by longer duration of deafness in one ear is associated with poorer implantation outcomes for that ear. This assumption, however, is mainly derived from studies of early childhood deafness. This study compared outcomes following implantation of the better or poorer ear in cases of long-term hearing asymmetries. Audiological records of 146 adults with bilateral hearing loss using a single hearing aid were reviewed. The unaided ear had 15 to 72 years of unaided severe to profound hearing loss before unilateral cochlear implantation. 98 received the implant in their long-term sound-deprived ear. A multiple regression analysis was conducted to assess the relative contribution of potential predictors to speech recognition performance after implantation. Duration of bilateral significant hearing loss and the presence of a prelingual hearing loss explained the majority of variance in speech recognition performance following cochlear implantation. For participants with postlingual hearing loss, similar outcomes were obtained by implanting either ear. With prelingual hearing loss, poorer outcomes were obtained when implanting the long-term sound-deprived ear, but the duration of the sound deprivation in the implanted ear did not reliably predict outcomes. Contrary to an apparent clinical consensus, duration of sound deprivation in one ear has limited value in predicting speech recognition outcomes of cochlear implantation in that ear. Outcomes of cochlear implantation are more closely related to the period of time for which the brain is deprived of auditory stimulation from both ears.
Project description:Neurodevelopmental changes occur with asymmetric hearing loss, limiting binaural/spatial hearing and putting children at risk for social and educational challenges. These deficits may be mitigated by providing bilateral hearing in children through auditory prostheses. Effects on speech perception and spatial hearing were measured in a large cohort of >450 children who were deaf and used bilateral cochlear implants or bimodal devices (one cochlear implant and a contralateral hearing aid). Results revealed an advantage of bilateral over unilateral device use but this advantage decreased as hearing in the two ears became increasingly asymmetric. Delayed implantation of an ear with severe to profound deafness allowed asymmetric hearing, creating aural preference for the better hearing ear. These findings indicate that bilateral input with the most appropriate device for each ear should be provided early and without delay during development.
Project description:BACKGROUND:For patients with single-sided deafness (SSD), restoration of binaural function via cochlear implant (CI) has been shown to improve speech understanding in noise. The objective of this study was to investigate changes in behavioral performance and cortical auditory responses following cochlear implantation. DESIGN:Prospective longitudinal study. SETTING:Tertiary referral center. METHODS:Six adults with SSD were tested before and 12 months post-activation of the CI. Six normal hearing (NH) participants served as experimental controls. Speech understanding in noise was evaluated for various spatial conditions. Cortical auditory evoked potentials were recorded with /ba/ stimuli in quiet and in noise. Global field power and responses at Cz were analyzed. RESULTS:Speech understanding in noise significantly improved with the CI when speech was presented to the CI ear and noise to the normal ear (p<0.05), but remained poorer than that of NH controls (p<0.05). N1 peak amplitude measure in noise significantly increased after CI activation (p<0.05), but remained lower than that of NH controls (p<0.05) at 12 months. After 12 months of CI experience, cortical responses in noise became more comparable between groups. CONCLUSION:Binaural restoration in SSD patients via cochlear implantation improved speech performance noise and cortical responses. While behavioral performance and cortical auditory responses improved, SSD-CI outcomes remained poorer than that of NH controls in most cases, suggesting only partial restoration of binaural hearing.
Project description:Objectives:This study aimed to assess the effect of hearing and speech rehabilitation in patients with Nurotron® cochlear implants. Design:Ninety-eight paediatric patients with bilateral severe-to-profound sensorineural deafness who received cochlear implantation were divided into three groups according to age: group A (≤3 years), group B (4-7 years), and group C (8-16 years). All patients were followed up for one year for hearing and speech performance after the surgery. The comprehensive Auditory Perception Assessment, MAIS, CAP and SIR hearing and speech assessments and rating materials were used for assessment before the surgery and at 3, 6, and 12 months after implant activation. Results:The scores of patients in the open-set speech assessment, Chinese Auditory Perception Assessment, MAIS, CAP and SIR significantly improved after cochlear implantation in all age groups. The younger the age at implantation, the better the results. Moreover, the hearing and speech performance of cochlear implant recipients gradually improved with the extension of rehabilitation time. Conclusions:Nurotron® Venus™ cochlear implantation can improve the hearing and speech performance of patients with bilateral severe-to-profound sensorineural deafness.
Project description:Children using unilateral cochlear implants abnormally rely on tempo rather than mode cues to distinguish whether a musical piece is happy or sad. This led us to question how this judgment is affected by the type of experience in early auditory development. We hypothesized that judgments of the emotional content of music would vary by the type and duration of access to sound in early life due to deafness, altered perception of musical cues through new ways of using auditory prostheses bilaterally, and formal music training during childhood. Seventy-five participants completed the Montreal Emotion Identification Test. Thirty-three had normal hearing (aged 6.6 to 40.0 years) and 42 children had hearing loss and used bilateral auditory prostheses (31 bilaterally implanted and 11 unilaterally implanted with contralateral hearing aid use). Reaction time and accuracy were measured. Accurate judgment of emotion in music was achieved across ages and musical experience. Musical training accentuated the reliance on mode cues which developed with age in the normal hearing group. Degrading pitch cues through cochlear implant-mediated hearing induced greater reliance on tempo cues, but mode cues grew in salience when at least partial acoustic information was available through some residual hearing in the contralateral ear. Finally, when pitch cues were experimentally distorted to represent cochlear implant hearing, individuals with normal hearing (including those with musical training) switched to an abnormal dependence on tempo cues. The data indicate that, in a western culture, access to acoustic hearing in early life promotes a preference for mode rather than tempo cues which is enhanced by musical training. The challenge to these preferred strategies during cochlear implant hearing (simulated and real), regardless of musical training, suggests that access to pitch cues for children with hearing loss must be improved by preservation of residual hearing and improvements in cochlear implant technology.
Project description:Considerable advancements in cochlear implant technology (e.g., electric acoustic stimulation) and assessment materials have yielded expanded criteria. Despite this, it is unclear whether individuals with better audiometric thresholds and speech understanding are being referred for cochlear implant workup and pursuing cochlear implantation. The purpose of this study was to characterize the mean auditory and demographic profile of adults presenting for preoperative cochlear implant workup. Data were collected prospectively for all adult preoperative workups at Vanderbilt from 2013 to 2015. Subjects included 287 adults (253 postlingually deafened) with a mean age of 62.3 years. Each individual was assessed using the minimum speech test battery, spectral modulation detection, subjective questionnaires, and cognitive screening. Mean consonant-nucleus-consonant word scores, AzBio sentence scores, and pure-tone averages for postlingually deafened adults were 10%, 13%, and 89?dB HL, respectively, for the ear to be implanted. Seventy-three individuals (25.4%) met labeled indications for Hybrid-L and 207 individuals (72.1%) had aidable hearing in the better hearing ear to be used in a bimodal hearing configuration. These results suggest that mean speech understanding evaluated at cochlear implant workup remains very low despite recent advancements. Greater awareness and insurance accessibility may be needed to make cochlear implant technology available to those who qualify for electric acoustic stimulation devices as well as individuals meeting conventional cochlear implant criteria.
Project description:Hearing impairment is the most frequent sensory deficit in humans of all age groups, from children (1/500) to the elderly (more than 50% of the over-75 s). Over 50% of congenital deafness are hereditary in nature. The other major causes of deafness, which also may have genetic predisposition, are aging, acoustic trauma, ototoxic drugs such as aminoglycosides, and noise exposure. Over the last two decades, the study of inherited deafness forms and related animal models has been instrumental in deciphering the molecular, cellular, and physiological mechanisms of disease. However, there is still no curative treatment for sensorineural deafness. Hearing loss is currently palliated by rehabilitation methods: conventional hearing aids, and for more severe forms, cochlear implants. Efforts are continuing to improve these devices to help users to understand speech in noisy environments and to appreciate music. However, neither approach can mediate a full recovery of hearing sensitivity and/or restoration of the native inner ear sensory epithelia. New therapeutic approaches based on gene transfer and gene editing tools are being developed in animal models. In this review, we focus on the successful restoration of auditory and vestibular functions in certain inner ear conditions, paving the way for future clinical applications.
Project description:Introduction:Although rare, cholesteatoma can develop as a late complication of cochlear implantation. The electrode array may then be exposed in the external auditory canal surrounded by cholesteatoma debris. Case Report. The cochlear implant of a child was inadvertently explanted by a clinician during a routine aural toilet procedure. The child had previously reported recurrent ear infections, pain, and unexplained implant function degradation. Reimplantation was carried out 2 days later with good postoperative hearing results. Part of the electrode array was observed to be embedded in cholesteatoma. Postreimplantation recovery was complicated by a breakdown of the blind-sac. Discussion. Clinical indicators that could alert the clinician to the possibility of this late complication include recurrent infections, presence of keratotic debris in the external auditory canal, unexplained implant function degradation, and nonauditory stimulation. Although this patient managed to achieve excellent postreimplantation hearing outcomes, a delay in reimplantation surgery following explantation could possibly compromise successful reinsertion of the electrode array. External ear canal overclosure without mastoid cavity obliteration has merit in facilitating CT scan surveillance, but it may increase the risk of the blind-sac breaking down. This case also illustrated how the electrode array could have facilitated propagation of the cholesteatoma from the middle ear to the mastoid. Conclusion:If aural toilet is required in the implanted ear of a cochlear implant recipient, any complaint of hearing change, pain, or discharge should alert the clinician of the possibility of cholesteatoma developing. It warrants prompt evaluation by an experienced otologist in order to prevent accidental explantation. Keywords. Cochlear implant, cochlear implant complications, chronic suppurative otitis media, cholesteatoma, reimplantation, blind-sac, external auditory canal overclosure, mastoid cavity obliteration.