Cochlear implantation for single-sided deafness and tinnitus suppression

Objective

To quantify the potential effectiveness of cochlear implantation for tinnitus suppression in patients with single-sided deafness using the Tinnitus Handicap Inventory.

Predicting speech perception outcomes following cochlear implantation in adults with unilateral deafness or highly asymmetric hearing loss

Objective: Unilateral deafness and highly asymmetric hearing loss can impair listening abilities in everyday situations, create substantial audiological handicap, and reduce overall quality of life. Preliminary evidence suggests that cochlear implantation may be effective in reversing some of these detrimental effects. Patient-level data from existing studies were re-analysed to explore potential factors that may be predictive of improved speech perception scores following implantation.

Methods: Logistic regression modelling examined whether improved speech perception following implantation under various listening conditions was related to the duration of deafness of the severe-to-profoundly deaf ear and/or the level of hearing in the better ear.

Results: Patients with a shorter duration of deafness were more likely to improve in listening conditions that created a less favourable SNR at the implanted ear than the non-implanted ear. Those with more residual hearing in the better ear were more likely to improve in the listening condition that created a less favourable SNR at that ear.

Discussion: The analysis suggests that characteristics of both ears may be relevant when seeking to identify those candidates who are likely to obtain benefit to speech perception following cochlear implantation.     

Cortical auditory evoked potential (CAEP) and behavioural measures of auditory function in a child with a single-sided deafness

Objectives: To study the changes in behavioural and cortical responses over time in a child with single-sided deafness fitted with a cochlear implant (CI).

Methods: Cortical auditory evoked potentials (CAEPs) in noise (+5?dB signal-to-noise ratio) were recorded and auditory skills were assessed using tests of sound localization, spatial speech perception in noise, and self-ratings of auditory abilities (Listening inventory for education, LIFE and Speech, spatial and qualities of hearing questionnaire, SSQ parental version). Measures were obtained prior to and after a CI fitting, including one, six, and 12 months after the CI switch on.

Results: Spatial speech recognition improved over time. At 12 months post-CI, word recognition scores were similar to those of normal hearing children. Signal-to-noise ratios for sentences decreased (i.e. improved) over time post-CI. Sound localization markedly improved at 12 months post-CI compared to baseline. Self-perception of difficulty scores decreased over time. Parental ratings of hearing abilities improved compared to baseline for all subscales. There were changes in the P1–N1–P2 complex at 12 months post-CI, which were clearer frontally across stimuli. Further research is needed to understand the significance of such changes after CI fitting for single-sided deafness.

Conclusion: Although the changes observed could reflect maturational changes, the clinically significant improvement in recognition of speech in noise and improved questionnaire results suggest that the CI was beneficial, consistent with the feedback from the participant.     

Cochlear implantation in adults with unilateral deafness: A review of the assessment/evaluation protocols

Objectives: Cochlear implantation is becoming widely used outside the tertiary research centers for treatment of unilateral deafness (UD). No consensus exists, however, on the most suitable assessment/evaluation protocols for this group of adult patients. This paper aims to review the assessment and evaluation protocols used by various research groups and to propose a protocol for the use in the clinical setting.

Methods: The PubMed, Embase, and Cochrane Library databases were searched with the keywords ‘cochlear’, ‘implant’, ‘single-sided’, ‘deafness’, ‘adults’, ‘unilateral’, and ‘deafness’. The words were either used individually, combined in pairs, or in groups of 5. All articles reporting on prospective studies, retrospective studies, or case studies were included.

Results: Sixteen published studies met the inclusion criteria. Measures of hearing performance, tinnitus, subjective quality of hearing, and quality of life varied greatly among studies.

Discussion: Adaptive speech in noise testing, localization, tinnitus measurement questionnaires, and self-rated hearing improvement are widely used among the research groups. These tools in conjunction assess and evaluate the main issues associated with UD.

Conclusion: The test battery most commonly used to assess and evaluate adult cochlea implant users with UD consists of (a) a subjective self-rating of hearing performance, (b) localization testing, and (c) the adaptive speech in noise testing conducted in at least the following three spatial configurations: speech and noise presented from the front (S0/N0), speech presented from the front and noise presented to the good hearing ear (S0/Nhe), and speech presented to the implanted ear and noise presented to the hearing ear (Sci/Nhe).     

The psychological and social consequences of single-sided deafness in adulthood

Objectives: This study examined the subjective psychological and social effects of highly asymmetric hearing loss (single-sided deafness [SSD]) in adults. Design: Three group interviews were conducted using the critical incident technique and analysed using an inductive thematic analysis. Study sample: Eight adults with a clinical diagnosis of a moderately severe hearing loss or greater in one ear and normal or near-normal hearing in the other ear. Results: A range of functional hearing difficulties associated with SSD including impaired speech in background noise and reduced spatial awareness were reported to affect social and psychological well-being. Social consequences of SSD resulted from activity limitations and participation restrictions including withdrawal from and within situations. Participants reported psychological effects including worrying about losing the hearing in their other ear, embarrassment related to the social stigma attached to hearing loss and reduced confidence and belief in their abilities to participate. Conclusions: Single-sided deafness can be associated with many negative consequences. Counselling may help overcome the psychological consequences of hearing loss regardless of whether technological support such as a hearing aid is prescribed. The audiological management of these individuals should support the development of listening strategies and set appropriate expectations for participation in everyday listening situations.     

Duration of unilateral auditory deprivation is associated with reduced speech perception after cochlear implantation: A single-sided deafness study

Objective: Examine the relationship between duration of unilateral deafness and speech perception outcomes after cochlear implantation in adults with single-sided deafness.

Methods: A systematic review of PubMed articles containing individual speech perception and duration of deafness data from single-sided deaf adults. Studies were selected for detailed review and duration of deafness and speech perception outcomes were extracted, with speech scores reported as percent correct. A linear regression as a function of study and length of deafness was performed.

Results: A statistically significant negative effect of duration of unilateral deafness on speech perception was found, but there was substantial uncertainty regarding the strength of the effect.

Discussion: Existing data make it difficult to either support or reject a hard 5- or 10-year unilateral auditory deprivation limit on cochlear implant (CI) candidacy for patients with single-sided deafness. This is because the totality of available data are consistent with a very small effect, perhaps negligible in practical terms, and just as consistent with a very large effect. Regardless of effect size, the present results have important basic implications. They suggest that unilateral sound deprivation may have a deleterious effect on auditory processing even though more central parts of the auditory system have continued to receive input from a contralateral normal ear.

Conclusions: Speech perception scores in SSD patients are negatively correlated with duration of deafness, but the limited amount of data from cochlear implant users with long-term single-sided deafness leads to substantial uncertainly, which in turn precludes any strong clinical recommendations. Further study of SSD CI users with long-term deafness will be necessary to generate evidence-based guidelines for implantation criteria in this population.     

Cochlear implantation as a successful rehabilitation for radiation-induced deafness

Radiotherapy of the head and neck can be associated with conductive and/or sensori-neural hearing loss. We report the case of a 67-year-old man who developed complete bilateral deafness caused by labyrinthitis and radiation-induced neuritis of the acoustic nerve after postoperative radiotherapy of a nasopharyngeal carcinoma. Two years postoperatively extensive clinical workup including computed tomography and magnetic resonance imaging showed no recurrence or secondary brain tumors. To facilitate sound perception a Combi 40 cochlear implant was implanted. Because of fibrosis the insertion depth of the stimulating electrode into the scala tympani was limited and therefore a “short electrode version” was used. Six months after implantation the patient had achieved an excellent enviromental sound recognition and moderate speech intelligibility. Present experiences has shown that although radiotherapy can cause damage to the labyrinth and acoustic nerves and central hearing pathways, there may still be surviving auditory nerve fibers that can be stimulated successfully by a cochlear implant. Received: 30 July 1997 / Accepted: 28 October 1997     

Bone conductive implants in single-sided deafness

Conclusion: Bone conduction implants (BCIs) have been shown to partially restore some of the functions lost when binaural hearing is missing, such as in subjects with single-sided deafness (SSD). The use of a single BCI needs to be recommended by a clinician based on thorough counselling with the SSD subject. Objectives: To perform an overview of the present capabilities of BCIs for SSD and to evaluate the reliability of the audiological evaluation for assessing speech recognition in noise and sound localization cues, which are major problems related to the loss of binaural hearing. Methods: Nine subjects with SSD who received BCI implants underwent a preoperative audiological evaluation that included sound field speech audiometry, word recognition score (WRS) testing and sound localization testing in quiet and in noise. They were also tested for the accuracy of their directional word recognition in noise and their subjective perceptions of their hearing difficulties using the APHAB questionnaire. Results: The mean maximum accuracy of word discrimination was 65.5% in the unaided condition and 78.9% in the BCI-aided condition. Sound localization in noise was better with the BCI than in the unaided condition, especially when the stimulus and noise were presented on the same side as the implanted ear. The accuracy of directional word recognition showed an improvement with the BCI with respect to the unaided condition on the BCI side, with either the stimulus in the implanted ear and the noise in the contralateral ear or with both the stimulus and noise presented to the implanted ear.     

Re-training the deaf ear: Auditory training for adult cochlear implant users with singlesided deafness

Objective: While cochlear implant (CI) provision for adults with single-sided deafness (SSD) is now an accepted treatment option, auditory training programs specific to this group of CI users have not been described. This paper details the auditory training protocol and critical factors required to rehabilitate CI users with post-lingual SSD.

Outcomes and Results: Several key factors are integral to the success of the rehabilitation program; these include 1) CI users with SSD require a map that is balanced as closely as possible to their normal hearing ear and has optimal mapping levels; 2) the auditory training program needs to be stimulating, rewarding, and directly stimulate the implanted ear via Direct Auditory Input (DAI); 3) CI users need to achieve some success in the early post-implantation stages to maintain or increase their motivation; 3) CI users need to be fully committed to the auditory training; and 5) a well-defined structured auditory training program with immediate feedback and markers of success helps ensure optimal communication outcomes. As an indication of success, from the foundation of the program in 2008 until the present all adults with SSD who have received a CI at our clinic (N?=?114) only 5 have elected to stop using their device.

Conclusion: The auditory training program described herein has been developed to optimize hearing and quality of life outcomes for adult CI users with SSD.     

The effects of cochlear implantation in Japanese single-sided deafness patients: five case reports

Conclusion Cochlear implantation (CI) for Japanese single-sided deafness patients resulted in improved speech perception, increased sound localization accuracy, and reduced tinnitus handicap. Objectives This study reports results for five adult SSD cases with CI, focusing on the benefits they obtained in terms of speech recognition, sound localization, and tinnitus handicap. Methods Five Japanese patients meeting the eligibility criteria were included in this study. All patients were implanted with a fully inserted MED-EL Concerto FLEX28® implant (MED-EL, Innsbruck, Austria). Speech perception outcomes in noise, as well as sound localization and tinnitus disturbance, were assessed pre-surgically and at 1, 3, 6, and 12 months after CI activation. Results The Japanese monosyllable test score in noise improved gradually after implantation. In some cases, speech perception ability appeared unstable, particularly in the first 1–6 months after implantation. The sound localization ability showed marked improvement in all cases, with the disturbance to daily life caused by tinnitus also decreasing in all cases from the early post-operative period.     

Cochlear implant in patients with residual hearing

Objective: The postoperative speech perception abilities of severely hearing-impaired patients with multi-channel cochlear implant were compared with preoperative speech perception performance with conventional hearing aids. Methods: Cochlear implantation was performed in six severely to profoundly hearing-impaired patients. They had unaided pure-tone thresholds of 70–100-dB HL and aided thresholds of 35–90-dB HL in the better ear, but were not able to perceive speech sounds well with hearing aids. Results: Postoperatively, all the patients had significantly improved speech perception performance, exceeded the average skills of profoundly deaf cochlear implant users, and were able to communicate without writing. Conclusion: These results imply that cochlear implant may be indicated for severely to profoundly deaf subjects, if they receive little or no benefit from conventional hearing aids.     

Etiology of single-sided deafness and asymmetrical hearing loss

Abstract

Conclusions: The present study revealed that various etiologies are involved in single-sided deafness (SSD), and that the cause of SSD and asymmetrical hearing loss (AHL) differed greatly between congenital/early-onset cases and adult cases. Clarification of the etiology is the first step toward providing appropriate intervention.

Objectives: The study aimed to clarify the etiology of SSD and AHL patients.

Methods: The etiology of a total of 527 SSD or AHL patients who visited Shinshu University Hospital between 2006 and 2016 were analyzed by imaging as well as serological tests for mumps virus, and CMV DNA testing.

Results: In our cohort of congenital/early-onset SSD (n?=?210), the most prevalent cause in children was cochlear nerve deficiency (43.7%; 87 of 199 patients undergoing CT and/or MRI), followed by CMV infection, mumps infection, anomalies of the inner ear, ANSD, and other rare etiologies. In contrast, half of the adult SSD patients presented with idiopathic sensorineural hearing loss, followed by various types of otitis media, cerebellopontine angle tumor and other rare etiologies.     

What can we learn from adult cochlear implant recipients with single-sided deafness who became elective non-users?

Objective: This retrospective study investigates the incidence of elective cochlear implant (CI) non-use amongst a cohort of adult CI recipients with single-sided deafness (SSD), identifies the causes that led to non-use, and assesses how non-use could be prevented.

Methods: All adults with SSD who received a CI between 2008 and 2018 and who became elective CI non-users were included. Elective non-users were defined as CI recipients who decided to stop using their CI or, if explantation was necessary, refused reimplantation.

Results: 5/114 (4.4%) adults with SSD who received a CI became elective non-users. The 5 non-users were a mean 44.2 years old (range 33–70 years) at implantation, had a mean duration of deafness of 7.1 years (range 0.5–20 years) at implantation, and used their CI for a mean 11.5 months (range 1.5–60 months) before (fully) discontinuing use. The primary cause of elective non-use was postoperative discouragement due to unrealistic expectations (4 participants) regarding sound perception with the CI or about the greater than expected level of commitment necessary for rehabilitation.

Conclusions: Elective non-use among adult CI recipients with single-sided deafness was very rare and could be further prevented by comprehensive counselling to ensure that candidates have realistic expectations about the rehabilitation requirements and the outcomes with the CI.     

Cochlear implantation in patients with substantial residual hearing

OBJECTIVES: Cochlear implantation is an effective means for providing auditory rehabilitation in adult patients with severe to profound sensorineural hearing loss. It has been hypothesized that patients with substantial, preoperative residual hearing would be excellent cochlear implant candidates because of surviving neural populations and a lack of auditory deprivation. The purpose of this study is to describe the outcomes of patients with substantial residual hearing who have undergone cochlear implantation. STUDY DESIGN: Retrospective chart review of patients with substantial preoperative residual hearing who underwent cochlear implantation. METHODS: Chart reviews were completed for patients with substantial residual hearing who underwent cochlear implantation (City University of New York Sentence Test [CUNY] > 60%, Hearing in Noise Test sentences presented in quiet [HINTQ] > 50%, or Consonant-Nucleus-Consonant [CNC] > 20% in the ear to be implanted). Preoperative and postoperative measures of audiologic performance as well as complications were assessed. RESULTS: All 12 patients who met inclusion criteria ultimately surpassed their preoperative aided performance level after implantation and gained significant benefit from their cochlear implant. At 6 months postimplantation, mean CUNY, HINTQ, and CNC scores were 93%, 78%, and 48% in the implant ear alone, respectively. However, progress was slower than expected for many patients, and at least one patient took 1 year to surpass his preoperative performance level. There were no complications from surgery in this selected group of patients. CONCLUSIONS: Patients with some degree of residual hearing do benefit from cochlear implantation. However, there may be an initial decline in performance as compared with preoperative levels. This decline is overcome in time in this patient population. These patients need to be counseled accordingly.     

Cochlear implantation for children with GJB2-related deafness

OBJECTIVES/HYPOTHESIS: Mutations in GJB2 are a common cause of congenital sensorineural hearing loss. Many children with these mutations receive cochlear implants for auditory habilitation. The purpose of the study was to compare the speech perception performance of cochlear implant patients with GJB2-related deafness to patients without GJB2-related deafness. STUDY DESIGN: Retrospective case review. METHODS: Pediatric cochlear implant recipients who have been tested for GJB2 mutation underwent chart review. All patients received cochlear implantation at a tertiary referral center, followed by outpatient auditory habilitation. Charts were reviewed for cause and duration of deafness, age at time of cochlear implantation, intraoperative and postoperative complications, duration of use, and current age. Results of standard tests of speech perception administered as a part of the patients' auditory habilitation were reviewed. RESULTS: Twenty patients with GJB2 mutations were compared with 27 patients without GJB2 mutations. There was no statistical difference between patients with and without GJB2-related congenital sensorineural hearing loss with regard to open-set and closed-set speech recognition performance at 12, 24, and 36 months after cochlear implantation. Surgical complications were uncommon. CONCLUSION: Pediatric patients with congenital sensorineural hearing loss without other comorbid conditions (eg, developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. The presence or absence of GJB2 mutation does not appear to impact speech recognition performance at 12, 24, and 36 months after implantation.