Longitudinal speech perception and language performance in pediatric cochlear implant users: the effect of age at implantation.

OBJECTIVES: Few studies have examined the long-term effect of age at implantation on outcomes using multiple data points in children with cochlear implants. The goal of this study was to determine whether age at implantation has a significant, lasting impact on speech perception, language, and reading performance for children with prelingual hearing loss. DESIGN: A linear mixed-model framework was used to determine the effect of age at implantation on speech perception, language, and reading abilities in 83 children with prelingual hearing loss who received cochlear implants by the age of 4 years. The children were divided into two groups based on their age at implantation: (1) under 2 years of age and (2) between 2 and 3.9 years of age. Differences in model-specified mean scores between groups were compared at annual intervals from 5 to 13 years of age for speech perception, and 7 to 11 years of age for language and reading. RESULTS: After controlling for communication mode, device configuration, and preoperative pure-tone average, there was no significant effect of age at implantation for receptive language by 8 years of age, expressive language by 10 years of age, reading by 7 years of age. In terms of speech-perception outcomes, significance varied between 7 and 13 years of age, with no significant difference in speech-perception scores between groups at ages 7, 11, and 13 years. Children who used oral communication (OC) demonstrated significantly higher speech-perception scores than children who used total communication (TC). OC users tended to have higher expressive language scores than TC users, although this did not reach significance. There was no significant difference between OC and TC users for receptive language or reading scores. CONCLUSIONS: Speech perception, language, and reading performance continue to improve over time for children implanted before 4 years of age. The present results indicate that the effect of age at implantation diminishes with time, particularly for higher-order skills such as language and reading. Some children who receive cochlear implants after the age of 2 years have the capacity to approximate the language and reading skills of their earlier-implanted peers, suggesting that additional factors may moderate the influence of age at implantation on outcomes over time.

Tinnitus retraining therapy: mixing point and total masking are equally effective.

OBJECTIVES: Habituation to tinnitus cannot occur with total masking, an argument made by proponents of "tinnitus retraining therapy." We also compared the effectiveness of retraining therapy with mixing-point masking, total masking, and with counseling alone. DESIGN: Forty-eight tinnitus patients were randomly assigned to one of three groups: counseling, counseling plus bilateral noise generators set to completely mask the tinnitus, or counseling plus bilateral noise generators with a focus on the mixing point (partial masking just below total masking). A picture-based counseling protocol was used to assist in providing similar counseling among all three groups. The Tinnitus Handicap Questionnaire was administered before and after about 12 months of treatment. RESULTS: After 12 months, in the counseling group, three of 18 patients benefited significantly, in the mixing-point group, six of 19 patients benefited, and in the total masking group, four of 11 patients benefited from the treatment. The average decrease in the questionnaire was 16.7% for the counseling group, 31.6% for the retraining group, and 36.4% for the total masking group. No significant average differences among groups were observed. CONCLUSIONS: One premise of retraining therapy is incorrect; a focus on mixing-point masking is not required for habituation.

Tinnitus Activities Treatment with Total and Partial Masking.

BACKGROUND: There are many counseling and sound therapy approaches to treat tinnitus. Counseling approaches range from providing information using directive or collaborative approaches. Sound therapies include strategies that use background sounds to totally or partially mask tinnitus to reduce the prominence of or decrease the loudness or annoyance of the tinnitus. PURPOSE: We evaluated the effectiveness of tinnitus activities treatment (TAT) in two groups, those without hearing aids (HA) and those who were provided with HA. In both groups, comparisons were made among those receiving (1) counseling only, (2) counseling and partial masking, and (3) counseling and total masking. RESEARCH DESIGN: Participants were provided with HA or not, based on their choice, and then randomly assigned to one of the three groups. The Tinnitus Handicap Questionnaire (THQ) was used as the primary measure. RESULTS: For those without HA, significant benefits were obtained for 8 out of 22 participants in the counseling group, 8 of 13 in the total masking group, and 8 of 24 in the partial mask group. The average decrease in the THQ was 15% for the counseling group, 25% for the total mask, and 14% for the partial masking group. For those with HA; significant benefits were obtained for 5 of 16 in the counseling group, 3 of 14 in the total mask group, and 6 of 13 in the partial mask group. The average decrease in the THQ score was 12% for counseling, 13% for total masking, and 16% for partial masking. No significant differences among groups were observed. CONCLUSION: Individual differences were large. Many benefited from their treatment, but some did not. We believe this was likely influenced by their expectations.

Bilateral and unilateral cochlear implant users compared on speech perception in noise.

OBJECTIVE: Compare speech performance in noise with matched bilateral cochlear implant (CICI) and unilateral cochlear implant (CI only) users. DESIGN: Thirty CICI and 30 CI-only subjects were tested on a battery of speech perception tests in noise that use an eight-loudspeaker array. RESULTS: On average, CICI subject's performance with speech in noise was significantly better than the CI-only subjects. CONCLUSION: The CICI group showed significantly better performance on speech perception in noise compared with the CI-only subjects, supporting the hypothesis that CICI is more beneficial than CI only.

Performance over time on adults with simultaneous bilateral cochlear implants.

BACKGROUND: Maximum performance and long-term stability of bilateral cochlear implants has become an important topic because there has been increasing numbers of recipients of bilateral cochlear implants. PURPOSE: To determine the performance over time (up to 6yr) of subjects with simultaneous bilateral cochlear implants (CI+CI) on word recognition and localization. RESEARCH DESIGN: Over-time investigation of word recognition in quiet (CNC) and sound localization in quiet (Everyday Sounds Localization Test). STUDY SAMPLE: The subjects were 48 adults who simultaneously received their cochlear implants at the University of Iowa. RESULTS: For word recognition, percent correct scores continuously improved up to 1 yr postimplantation with the most benefit occurring within the first month of implantation. In observing up to 72 mo, the averaged scores reached to the plateau of about 63% correct in CNC after 2 yr (N = 31). But, when we followed 17 subjects who have complete data set between 12 mo and 48+ months, word recognition scores were significantly different from 12 mo to 48 + months, which implies binaural advantages need more time to be developed. Localization test results suggested that the root mean square (RMS) error scores continuously improved up to 1 yr postimplantation with most benefits occurring within the first 3 mo. After 2 yr, the averaged scores reached to the plateau of about 20 degrees RMS error (N = 27). When we followed 10 subjects who have complete data set between 12 mo and 48+ months, localization scores were not improved from 12 mo to 48+ months. There were large individual differences in performance over time. CONCLUSIONS: In general, substantial benefits in both word recognition and localization were found over the first 1-12 mo postimplantation for subjects who received simultaneous bilateral cochlear implants. These benefits were maintained over time up to 6yr postimplantation.

Validation of the Spatial Hearing Questionnaire.

OBJECTIVES: Subjective questionnaires are informative in understanding the difficulties faced by patients with hearing loss. Our intent was to establish and validate a new questionnaire that encompasses situations emphasizing binaural hearing. The Spatial Hearing Questionnaire is a self-report assessment tool with eight subscales representing questions; pertaining to the perception of male, female, and children's voices; music in quiet; source localization; understanding speech in quiet; and understanding speech in noise. DESIGN: The Spatial Hearing Questionnaire, composed of 24 items, is scored from 0 to 100. It was administered to 142 subjects using one or two cochlear implants. Speech perception and localization abilities were measured, and the Speech, Spatial, and Other Qualities questionnaire was completed to evaluate validity of the questionnaire. Psychometric tests were performed to test the reliability and factor structure of the Spatial Hearing Questionnaire. RESULTS: Results showed high internal consistency reliability (Cronbach's alpha = 0.98) and good construct validity (correlations between the Spatial Hearing Questionnaire and other test measures, including the Speech, Spatial, and Other Qualities, were significant). A preliminary factor analysis revealed scores loaded on three factors, representing the following conditions: localization, speech in noise and music in quiet, and speech in quiet, explaining 64.9, 13.0, and 5.3% of the variance, respectively. Most of the questionnaire items (12/24) loaded onto the first factor that represents the subscale related to source localization. Mean scores on the Spatial Hearing Questionnaire were higher for subjects with bilateral cochlear implants than for subjects with a unilateral cochlear implant, consistent with other research and supporting construct validity. CONCLUSIONS: The Spatial Hearing Questionnaire is a reliable and valid questionnaire that can be completed independently by most patients in about 10 minutes. It is likely to be a valuable tool for clinicians and researchers to measure spatial hearing abilities.

The relationship between tinnitus pitch and the audiogram.

We studied the relationship between tinnitus pitch and the audiogram in 195 patients. Patients with tone-like tinnitus reported a higher pitch (mean = 5385 Hz) compared to those with a noise-like quality (mean = 3266 Hz). Those with a flat audiogram were more likely to report: a noise-like tinnitus, a unilateral tinnitus, and have a pitch < 2000 Hz. The average duration of bilateral tinnitus (12 years) was longer than that of unilateral tinnitus (5 years). Older subjects reported a less severe tinnitus handicap questionnaire score. Patients with a notched audiogram often reported a pitch or=8000 Hz. We failed to find a relationship between the pitch and the edge of a high frequency hearing loss. Some individuals did exhibit a pitch at the low frequency edge of a hearing loss, but we could find no similar characteristics among these subjects. It is possible that a relationship between pitch and audiogram is present only in certain subgroups.

Identifying tinnitus subgroups with cluster analysis.

PURPOSE: We believe it is important to uncover tinnitus subgroups to identify subsets of patients most likely to benefit from different treatments. We review strategies for subgrouping based on etiology, subjective reports, the audiogram, psychoacoustics, imaging, and cluster analysis. METHOD: Preliminary results of a 2-step cluster analysis based on 246 participants from whom we had 26 categorical and 25 continuous variables were determined. RESULTS: A 4-cluster solution suggested the following subgroups: (a) constant distressing tinnitus, (b) varying tinnitus that is worse in noise, (c) tinnitus patients who are copers and whose tinnitus is not influenced by touch (somatic modulation), and (d) tinnitus patients who are copers but whose tinnitus is worse in quiet environments. CONCLUSIONS: Subgroups of tinnitus patients can be identified by using statistical approaches. The subgroups we identify here represent a preliminary attempt at identifying such patients. One next step would be to explore clinical trials of tinnitus treatments based on subgroup analyses or on using subgroups in the selection criteria.

Survey on the Effectiveness of Dietary Supplements to Treat Tinnitus.

PURPOSE: We surveyed the benefit of dietary supplements to treat tinnitus and reported adverse effects. METHOD: A website was created for people with tinnitus to complete a variety of questions. RESULTS: The 1,788 subjects who responded to questionnaires came from 53 different countries; 413 (23.1%) reported taking supplements. No effect on tinnitus was reported in 70.7%, improvement in 19.0%, and worsening in 10.3%. Adverse effects were reported in 6% (n = 36), including bleeding, diarrhea, headache, and others. Supplements were reported to be helpful for sleep: melatonin (effect size, d = 1.228) and lipoflavonoid (d = 0.5244); emotional reactions: melatonin (d = 0.6138) and lipoflavonoid (d = 0.457); hearing: Ginkgo biloba (d = 0.3758); and concentration Ginkgo biloba (d = 0.3611). The positive, subjective reports should be interpreted cautiously; many might have reported a positive effect because they were committed to treatment and expected a benefit. Users of supplements were more likely to have loudness hyperacusis and to have a louder tinnitus. CONCLUSIONS: The use of dietary supplements to treat tinnitus is common, particularly with Ginkgo biloba, lipoflavonoids, magnesium, melatonin, vitamin B12, and zinc. It is likely that some supplements will help with sleep for some patients. However, they are generally not effective, and many produced adverse effects. We concluded that dietary supplements should not be recommended to treat tinnitus but could have a positive outcome on tinnitus reactions in some people.

Manganese and Lipoflavonoid Plus(®) to Treat Tinnitus: A Randomized Controlled Trial.

BACKGROUND: Several tinnitus sufferers suggest that manganese has been helpful with their tinnitus. PURPOSE: We tested this in a controlled experiment where participants were committed to taking manganese and Lipoflavonoid Plus(®) to treat their tinnitus. RESEARCH DESIGN: Randomized controlled trial. STUDY SAMPLE: 40 participants were randomized to receive both manganese and Lipoflavonoid Plus(®) for 6 months, or Lipoflavonoid Plus(®) only (as the control). DATA COLLECTION AND ANALYSIS: Pre- and postmeasures were obtained with the Tinnitus Handicap Questionnaire, Tinnitus Primary Functions Questionnaire, and tinnitus loudness and annoyance ratings. An audiologist performed the audiogram, the tinnitus loudness match, and minimal masking level. RESULTS: Twelve participants were dropped out of the study because of the side effects or were lost to follow-up. In the manganese group, 1 participant (out of 12) showed a decrease in the questionnaires, and another showed a decrease in the loudness and annoyance ratings. No participants from the control group (total 16) showed a decrease in the questionnaires ratings. Two participants in the control group reported a loudness decrement and one reported an annoyance decrement. CONCLUSIONS: We were not able to conclude that either manganese or Lipoflavonoid Plus(®) is an effective treatment for tinnitus.

Differences Among Patients That Make Their Tinnitus Worse or Better.

PURPOSE: Our objective was to identify activities that influence tinnitus and to determine if conditional probabilities exist among such variables. METHOD: Two hundred fifty-eight patients were asked the following two questions: "When you have your tinnitus, which of the following makes it worse?" and "Which of the following reduces your tinnitus?" RESULTS: Things that made tinnitus better included noise (31%) and relaxation (15%). Things that made tinnitus worse included being in a quiet place (48%), stress (36%), being in a noisy place (32%), and lack of sleep (27%). Almost 6% of patients suggested coffee/tea and 4% said certain foods made their tinnitus worse. Conditional probabilities indicated that for those whose tinnitus is not worse in quiet, it is usually not reduced by noise. For those whose tinnitus is not worse in noise, it is usually not reduced in quiet. CONCLUSION: There are dramatic differences among patients. Such differences need to be considered in planning treatments.

Speech, Spatial and Qualities of Hearing Scale (SSQ) and Spatial Hearing Questionnaire (SHQ) Changes Over Time in Adults With Simultaneous Cochlear Implants.

PURPOSE: The purpose of the present study is to evaluate the changes over time of the Spatial Hearing Questionnaire (SHQ; Tyler, Perreau, & Ji, 2009) and the Speech, Spatial and Qualities of Hearing Scale (SSQ; Gatehouse & Noble, 2004) in adults with simultaneous bilateral cochlear implants by collecting data for 2 years after implantation. METHOD: Nineteen postlingual subjects participated in this study. Average age at implant was 63 years. The subjects' data were included in this retrospective analysis only if they completed both questionnaires. We compared subjects' SSQ and SHQ scores to word recognition and sound localization performances. RESULTS: The study showed SHQ and SSQ scores continuously improved up to 1 year postimplantation, with the most improvement occurring within the first 6 months of implantation. After 1 year, the scores reached a plateau. This is consistent with laboratory tests from the same 19 subjects, and they showed high correlations (p < .05). The present study also showed a high degree of correlation between SHQ and SSQ scores. CONCLUSIONS: Both the SSQ and SHQ scores improved up to 12 months postimplantation, with the most improvement during the first 6 months of implantation. Both were consistent with laboratory tests. There was a high degree of correlation between SHQ and SSQ scores.

Development and validation of the tinnitus primary function questionnaire.

PURPOSE: To create a questionnaire focused on the primary activities impaired by tinnitus and therefore more sensitive to treatments. METHOD: Questions were developed on (a) emotions, (b) hearing, (c) sleep, and (d) concentration. A 20-item questionnaire was administered to 158 patients. First, confirmatory factor analysis was used to select 3 questions per domain. Second, factor analysis was used to evaluate the appropriateness of the 12-item questionnaire. RESULTS: The analysis indicated that the selected questions successfully represented 4 independent domains. Scores were correlated with the Tinnitus Handicap Questionnaire ( r = .77, p < .01) and loudness ( r = .40, p < .01). The Sleep subscale correlated with the Pittsburgh Sleep Index ( r = .68, p < .01); the Emotion subscale correlated with the Beck Inventory ( r = .66, p < .01) and the Trait Anxiety questionnaire ( r = .67, p < .01). The average scores went from 51% to 38% following treatment. CONCLUSION: The Tinnitus Primary Function Questionnaire is valid, reliable, and sensitive and can be used to determine the efficacy of clinical trials.

Zinc to treat tinnitus in the elderly: a randomized placebo controlled crossover trial.

OBJECTIVE: Several reports suggest that zinc, which is involved in several neural transmissions systems throughout the auditory pathway, might help some tinnitus patients. However, previous studies used inadequate experimental designs. Therefore, we tested the effectiveness of zinc to reduce tinnitus. STUDY DESIGN: Randomized, prospective double-blind placebo-controlled design. SETTING: Tertiary referral center. PATIENTS: Tinnitus subjects older than 60 years, who are more likely to have a zinc deficiency. INTERVENTION(S): In Phase 1, 58 subjects were randomized to receive 50 mg of zinc per day for 4 months, and 58 subjects received a placebo. After a 1-month washout period, the 2 groups were crossed over to receive the alternative regime (Phase 2). MAIN OUTCOME MEASURE: Difference scores between before and after measures of the Tinnitus Handicap Questionnaire. Changes on the difference scores 20 or greater were considered as a statistically significant and, therefore, clinically meaningful improvement for THQ. RESULTS: Five percent (5 of 93 patients) had an improvement of 20 points or greater in THQ scores after zinc treatment, whereas 2% (2 of 94 patients) had an improvement of 20 or greater in THQ scores after placebo. The difference between 2 proportions is 5/93 - 2/94 = 0.03, the estimate of relative improvement is (5/93) / (2/94) = 2.53, with 95% confidence interval from 0.5 to 12.7. From chi-square independent test, there was no significant evidence that patients treated by zinc improved better than those treated by placebo (X2 (1) = 1.4, p > 0.05). The observed power in THQ for zinc is 0.16, and that for placebo is 0.06. CONCLUSION: Zinc is not an effective treatment for tinnitus in this subgroup of patients.

Sequential bilateral cochlear implantation: speech perception and localization pre- and post-second cochlear implantation.

PURPOSE: In this study, the authors sought to compare speech perception and localization in subjects who wear 1 cochlear implant (unilateral CI) or 1 cochlear implant and hearing aid (CI+HA) and then receive a second cochlear implant (bilateral CI), and to evaluate the importance of the duration between implant surgeries and duration of deafness. METHOD: Nine subjects were tested on speech perception in quiet, and 13 subjects were tested on speech perception and localization in noise using an array of 8 loudspeakers. All subjects were tested with unilateral CI prior to bilateral implantation and then again with bilateral CI after at least 3 months of bilateral experience. RESULTS: No significant difference was found between bilateral CI and unilateral CI on averaged speech perception in quiet performance. A significant benefit was found for bilateral CI on averaged speech perception in noise and on localization. Nonsignificant correlations were found for duration between surgeries, duration of deafness, and duration of bilateral use. CONCLUSIONS: Improvements for speech perception and localization played in background noise were indicated for most subjects after they received their 2nd implant. The correlations should be reassessed with a larger number of subjects to appropriately evaluate the effects of duration between surgeries, duration of deafness, and duration of bilateral use.

Prediction of cochlear implant performance by genetic mutation: the spiral ganglion hypothesis.

BACKGROUND: Up to 7% of patients with severe-to-profound deafness do not benefit from cochlear implantation. Given the high surgical implantation and clinical management cost of cochlear implantation (>$1 million lifetime cost), prospective identification of the worst performers would reduce unnecessary procedures and healthcare costs. Because cochlear implants bypass the membranous labyrinth but rely on the spiral ganglion for functionality, we hypothesize that cochlear implant (CI) performance is dictated in part by the anatomic location of the cochlear pathology that underlies the hearing loss. As a corollary, we hypothesize that because genetic testing can identify sites of cochlear pathology, it may be useful in predicting CI performance. METHODS: 29 adult CI recipients with idiopathic adult-onset severe-to-profound hearing loss were studied. DNA samples were subjected to solution-based sequence capture and massively parallel sequencing using the OtoSCOPE(®) platform. The cohort was divided into three CI performance groups (good, intermediate, poor) and genetic causes of deafness were correlated with audiometric data to determine whether there was a gene-specific impact on CI performance. RESULTS: The genetic cause of deafness was determined in 3/29 (10%) individuals. The two poor performers segregated mutations in TMPRSS3, a gene expressed in the spiral ganglion, while the good performer segregated mutations in LOXHD1, a gene expressed in the membranous labyrinth. Comprehensive literature review identified other good performers with mutations in membranous labyrinth-expressed genes; poor performance was associated with spiral ganglion-expressed genes. CONCLUSIONS: Our data support the underlying hypothesis that mutations in genes preferentially expressed in the spiral ganglion portend poor CI performance while mutations in genes expressed in the membranous labyrinth portend good CI performance. Although the low mutation rate in known deafness genes in this cohort likely relates to the ascertainment characteristics (postlingual hearing loss in adult CI recipients), these data suggest that genetic testing should be implemented as part of the CI evaluation to test this association prospectively.

Changes in the tinnitus handicap questionnaire after cochlear implantation.

PURPOSE: To determine (a) changes in the Tinnitus Handicap Questionnaire (THQ) for patients using cochlear implants, (b) differences between patients who receive total or partial relief, and (c) identifiable characteristics of those who report tinnitus after implantation. METHOD: Pre- and postoperatively, 244 adults were administered the THQ when they reported tinnitus. RESULTS: Of the 153 patients who had tinnitus preoperatively, 94 (61%) patients reported total suppression and 59 (39%) reported a partial reduction. In 91 patients who did not have tinnitus before implantation, 11 (12%) reported tinnitus postimplantation. The THQ score decreased from 41% preimplant to 30% postimplant. The largest reductions involved social handicap and hearing. Patients with a more severe hearing loss might be more likely to experience an exacerbation of their tinnitus. We were not able to clearly identify differences between patients who received total or partial relief and the characteristics of patients who reported tinnitus after implantation. Those who acquired tinnitus had the shortest duration hearing loss (5.6 years) and were the oldest (63 years). The average THQ score of patients getting tinnitus was 29%. CONCLUSIONS: Most tinnitus patients benefit from receiving a cochlear implant.