The natural history and genotype-phenotype correlations of TMPRSS3 hearing loss: an international, multi-center, cohort analysis.

TMPRSS3-related hearing loss presents challenges in correlating genotypic variants with clinical phenotypes due to the small sample sizes of previous studies. We conducted a cross-sectional genomics study coupled with retrospective clinical phenotype analysis on 127 individuals. These individuals were from 16 academic medical centers across 6 countries. Key findings revealed 47 unique TMPRSS3 variants with significant differences in hearing thresholds between those with missense variants versus those with loss-of-function genotypes. The hearing loss progression rate for the DFNB8 subtype was 0.3 dB/year. Post-cochlear implantation, an average word recognition score of 76% was observed. Of the 51 individuals with two missense variants, 10 had DFNB10 with profound hearing loss. These 10 all had at least one of 4 TMPRSS3 variants predicted by computational modeling to be damaging to TMPRSS3 structure and function. To our knowledge, this is the largest study of TMPRSS3 genotype-phenotype correlations. We find significant differences in hearing thresholds, hearing loss progression, and age of presentation, by TMPRSS3 genotype and protein domain affected. Most individuals with TMPRSS3 variants perform well on speech recognition tests after cochlear implant, however increased age at implant is associated with worse outcomes. These findings provide insight for genetic counseling and the on-going design of novel therapeutic approaches.

Age-related High-frequency Hearing Loss Is Not Associated With Horizontal Semicircular Canal Function.

OBJECTIVE: Investigate the association between age-related hearing loss and reduced peripheral vestibular function using paired assessments of high-frequency hearing and horizontal semicircular canal (HSC) function. We hypothesized that age-related high-frequency hearing loss would be correlated with reduced HSC function and, therefore, useful to predict age-related vestibular hypofunction. DESIGN: We conducted a single center, retrospective cross-sectional study in a tertiary/academic referral hospital. This study included 185 patients who were diagnosed with a cerebellopontine angle (CPA) tumor and referred to the academic hospital to evaluate treatment options. Data collected included pure-tone audiometry, caloric reflex test, video head-impulse test (vHIT), and medical history. High-frequency hearing loss was quantified by the high Fletcher index (hFI), and horizontal semicircular canal (HSC) function were quantified by the caloric reflex test and vHIT. RESULTS: We observed a significant association between age and high-frequency hearing loss that was significantly worse in men compared with women. In contrast, we observed no significant association between age and HSC function assessed by either the caloric reflex test or vHIT. We observed associations between HSC function and sex, with male sex predicting reduced HSC function by caloric reflex testing but enhanced HSC function by vHIT. High-frequency hearing loss did not predict HSC hypofunction. CONCLUSIONS: We found no evidence indicating age-related decline in HSC function or an association between age-related high-frequency hearing loss and age-related decline in HSC function. We did observe sex-specific differences in HSC function. Our study highlights the need for sex-specific normative values for identifying age-related reduced peripheral vestibular function and for future work linking comprehensive assessments of inner ear function with tests of balance and stability to understand the complex interactions underlying hearing loss and imbalance, especially in the elderly.

Changes in Tinnitus by Cochlear Implantation: A Parametric Study of the Effect of Single-Electrode Stimulation.

INTRODUCTION: While cochlear implantation may have a positive effect on tinnitus, it is not effective in reducing tinnitus in all patients. This may be due to different patients requiring different strategies of electrical stimulation in order to obtain a positive effect on tinnitus. It is, therefore, important to identify the most effective stimulation strategies to reduce tinnitus. The simplest possible strategy is stimulation by only one electrode. In this study, we investigated tinnitus suppression by electrical stimulation via a single electrode of the cochlear implant. METHODS: We performed a listening experiment in 19 adult participants, who had received a unilateral cochlear implant (CI) because of severe bilateral hearing loss. All of these patients had indicated that they suffered from tinnitus. During a 300-s interval, patients listened to blocks of single-electrode stimulation and rated the loudness of the stimulus and any effects on their tinnitus. The 300-s interval included a block of single-electrode stimulation (duration 120 s). In consecutive intervals, the stimulus differed in its cochlear location (basal or apical), its pulse rate (720 or 725 Hz, 1,200 Hz, and 2,400 or 2,320 Hz), and amplitude (just above threshold or equivalent to moderate loudness). Thus, 2 × 3 × 2 = 12 stimulus conditions were tested in each participant, and each condition was presented only once. During the experiment, the participants promptly rated the loudness of the stimuli and the loudness of their tinnitus on a Visual Analogue Scale (10-point VAS). RESULTS: Significantly more tinnitus reduction was observed with stimuli at a moderate intensity level (30%) compared to stimuli at near-threshold level (18%) (χ2 [1, N = 222] = 14.115, p < 0.01). No significant differences in tinnitus levels resulted from the different pulse rates and stimulation sites. Eight participants reported an increase of tinnitus loudness under at least one stimulus condition. Changes in tinnitus loudness were generally minor, and never exceeded 3 points on the VAS. The overall effect of cochlear implantation on tinnitus, that is, the effect with full-array stimulation, was not correlated with the effectiveness of the single-electrode stimulation on tinnitus. CONCLUSION: In conclusion, the effect of single-electrode stimulation on tinnitus is relatively insignificant in comparison to the effect of full-array stimulation. However, in some individual cases, sustained single-electrode stimulation may be beneficial for tinnitus management.

De novo and inherited loss-of-function variants of ATP2B2 are associated with rapidly progressive hearing impairment.

ATP2B2 encodes the PMCA2 Ca2+ pump that plays an important role in maintaining ion homeostasis in hair cells among others by extrusion of Ca2+ from the stereocilia to the endolymph. Several mouse models have been described for this gene; mice heterozygous for loss-of-function defects display a rapidly progressive high-frequency hearing impairment. Up to now ATP2B2 has only been reported as a modifier, or in a digenic mechanism with CDH23 for hearing impairment in humans. Whole exome sequencing in hearing impaired index cases of Dutch and Polish origins revealed five novel heterozygous (predicted to be) loss-of-function variants of ATP2B2. Two variants, c.1963G>T (p.Glu655*) and c.955delG (p.Ala319fs), occurred de novo. Three variants c.397+1G>A (p.?), c.1998C>A (p.Cys666*), and c.2329C>T (p.Arg777*), were identified in families with an autosomal dominant inheritance pattern of hearing impairment. After normal newborn hearing screening, a rapidly progressive high-frequency hearing impairment was diagnosed at the age of about 3-6 years. Subjects had no balance complaints and vestibular testing did not yield abnormalities. There was no evidence for retrocochlear pathology or structural inner ear abnormalities. Although a digenic inheritance pattern of hearing impairment has been reported for heterozygous missense variants of ATP2B2 and CDH23, our findings indicate a monogenic cause of hearing impairment in cases with loss-of-function variants of ATP2B2.

A Prospective Study of the Effect of Cochlear Implantation on Tinnitus.

Previous studies have shown diverse and sometimes even contrary results concerning the effect of cochlear implantation on tinnitus and the factors that can influence this effect. The aim of this prospective questionnaire study was to determine the effects of cochlear implantation on tinnitus and explore which factors can influence the effect of cochlear implantation on tinnitus. Forty-four of the patients implanted in our hospital returned 2 questionnaire packages, i.e., one before the cochlear implantation and one 6 months after implantation. Before implantation, 66% of the patients experienced tinnitus. This study shows that cochlear implantation could help to reduce tinnitus and the tinnitus handicap in at least 28% of the patients with preoperative tinnitus. In 72% of the patients the tinnitus remained after implantation. None of the patients developed tinnitus after implantation. A shorter duration of tinnitus prior to implantation, a more fluctuating type of tinnitus, a higher tinnitus handicap prior to implantation, and a round-window surgical approach might have a positive influence on the effect of cochlear implantation on tinnitus, but further research is necessary to confirm these findings.

Musician effect on perception of spectro-temporally degraded speech, vocal emotion, and music in young adolescents.

In adult normal-hearing musicians, perception of music, vocal emotion, and speech in noise has been previously shown to be better than non-musicians, sometimes even with spectro-temporally degraded stimuli. In this study, melodic contour identification, vocal emotion identification, and speech understanding in noise were measured in young adolescent normal-hearing musicians and non-musicians listening to unprocessed or degraded signals. Different from adults, there was no musician effect for vocal emotion identification or speech in noise. Melodic contour identification with degraded signals was significantly better in musicians, suggesting potential benefits from music training for young cochlear-implant users, who experience similar spectro-temporal signal degradations.

Guidelines in CHARGE syndrome and the missing link: Cranial imaging.

"CHARGE syndrome" is a complex syndrome with high and extremely variable comorbidity. As a result, clinicians may struggle to provide accurate and comprehensive care, and this has led to the publication of several clinical surveillance guidelines and recommendations for CHARGE syndrome, based on both single case observations and cohort studies. Here we perform a structured literature review to examine all the existing advice. Our findings provide additional support for the validity of the recently published Trider checklist. We also identified a gap in literature when reviewing all guidelines and recommendations, and we propose a guideline for neuroradiological evaluation of patients with CHARGE syndrome. This is of importance, as patients with CHARGE are at risk for peri-anesthetic complications, making recurrent imaging procedures under anesthesia a particular risk in clinical practice. However, comprehensive cranial imaging is also of tremendous value for timely diagnosis, proper treatment of symptoms and for further research into CHARGE syndrome. We hope the guideline for neuroradiological evaluation will help clinicians provide efficient and comprehensive care for individuals with CHARGE syndrome.

Influence of hearing loss and cognitive abilities on language development in CHARGE Syndrome.

Hearing loss and cognitive delay are frequently occurring features in CHARGE syndrome that may contribute to impaired language development. However, not much is known about language development in patients with CHARGE syndrome. In this retrospective study, hearing loss, cognitive abilities, and language development are described in 50 patients with CHARGE syndrome. After informed consent was given, data were collected from local medical files. Most patients (38.3%; 18/47 patients) had moderate hearing loss (41-70 dB) and 58.5% (24/41 patients) had an IQ below 70. The mean language quotients of the receptive and expressive language were more than one standard deviation below the norm. Both hearing loss and cognitive delay had an influence on language development. Language and cognitive data were not available for all patients, which may have resulted in a pre-selection of patients with a delay. In conclusion, while hearing thresholds, cognitive abilities and language development vary widely in CHARGE syndrome, they are mostly below average. Hearing loss and cognitive delay have a significant influence on language development in children with CHARGE syndrome. To improve our knowledge about and the quality of care we can provide to CHARGE patients, hearing and developmental tests should be performed regularly in order to differentiate between the contributions of hearing loss and cognitive delay to delays in language development, and to provide adequate hearing amplification in the case of hearing loss. © 2016 Wiley Periodicals, Inc.

Musician effect in cochlear implant simulated gender categorization.

Musicians have been shown to better perceive pitch and timbre cues in speech and music, compared to non-musicians. It is unclear whether this "musician advantage" persists under conditions of spectro-temporal degradation, as experienced by cochlear-implant (CI) users. In this study, gender categorization was measured in normal-hearing musicians and non-musicians listening to acoustic CI simulations. Recordings of Dutch words were synthesized to systematically vary fundamental frequency, vocal-tract length, or both to create voices from the female source talker to a synthesized male talker. Results showed an overall musician effect, mainly due to musicians weighting fundamental frequency more than non-musicians in CI simulations.

Perception of spectrally degraded reflexives and pronouns by children.

Speech perception skills in cochlear-implant users are often measured with simple speech materials. In children, it is crucial to fully characterize linguistic development, and this requires linguistically more meaningful materials. The authors propose using the comprehension of reflexives and pronouns, as these specific skills are acquired at different ages. According to the literature, normal-hearing children show adult-like comprehension of reflexives at age 5, while their comprehension of pronouns only reaches adult-like levels around age 10. To provide normative data, a group of younger children (5 to 8 yrs old), older children (10 and 11 yrs old), and adults were tested under conditions without or with spectral degradation, which simulated cochlear-implant speech transmission with four and eight channels. The results without degradation confirmed the different ages of acquisition of reflexives and pronouns. Adding spectral degradation reduced overall performance; however, it did not change the general pattern observed with non-degraded speech. This finding confirms that these linguistic milestones can also be measured with cochlear-implanted children, despite the reduced quality of sound transmission. Thus, the results of the study have implications for clinical practice, as they could contribute to setting realistic expectations and therapeutic goals for children who receive a cochlear implant.

Musical Emotion Categorization with Vocoders of Varying Temporal and Spectral Content.

While previous research investigating music emotion perception of cochlear implant (CI) users observed that temporal cues informing tempo largely convey emotional arousal (relaxing/stimulating), it remains unclear how other properties of the temporal content may contribute to the transmission of arousal features. Moreover, while detailed spectral information related to pitch and harmony in music - often not well perceived by CI users- reportedly conveys emotional valence (positive, negative), it remains unclear how the quality of spectral content contributes to valence perception. Therefore, the current study used vocoders to vary temporal and spectral content of music and tested music emotion categorization (joy, fear, serenity, sadness) in 23 normal-hearing participants. Vocoders were varied with two carriers (sinewave or noise; primarily modulating temporal information), and two filter orders (low or high; primarily modulating spectral information). Results indicated that emotion categorization was above-chance in vocoded excerpts but poorer than in a non-vocoded control condition. Among vocoded conditions, better temporal content (sinewave carriers) improved emotion categorization with a large effect while better spectral content (high filter order) improved it with a small effect. Arousal features were comparably transmitted in non-vocoded and vocoded conditions, indicating that lower temporal content successfully conveyed emotional arousal. Valence feature transmission steeply declined in vocoded conditions, revealing that valence perception was difficult for both lower and higher spectral content. The reliance on arousal information for emotion categorization of vocoded music suggests that efforts to refine temporal cues in the CI user signal may immediately benefit their music emotion perception.

Musical background not associated with self-perceived hearing performance or speech perception in postlingual cochlear-implant users.

In normal-hearing listeners, musical background has been observed to change the sound representation in the auditory system and produce enhanced performance in some speech perception tests. Based on these observations, it has been hypothesized that musical background can influence sound and speech perception, and as an extension also the quality of life, by cochlear-implant users. To test this hypothesis, this study explored musical background [using the Dutch Musical Background Questionnaire (DMBQ)], and self-perceived sound and speech perception and quality of life [using the Nijmegen Cochlear Implant Questionnaire (NCIQ) and the Speech Spatial and Qualities of Hearing Scale (SSQ)] in 98 postlingually deafened adult cochlear-implant recipients. In addition to self-perceived measures, speech perception scores (percentage of phonemes recognized in words presented in quiet) were obtained from patient records. The self-perceived hearing performance was associated with the objective speech perception. Forty-one respondents (44% of 94 respondents) indicated some form of formal musical training. Fifteen respondents (18% of 83 respondents) judged themselves as having musical training, experience, and knowledge. No association was observed between musical background (quantified by DMBQ), and self-perceived hearing-related performance or quality of life (quantified by NCIQ and SSQ), or speech perception in quiet.

Self-reported music perception is related to quality of life and self-reported hearing abilities in cochlear implant users.

OBJECTIVES: To investigate the relationship between self-reported music perception and appreciation and (1) quality of life (QoL), and (2) self-assessed hearing ability in 98 post-lingually deafened cochlear implant (CI) users with a wide age range. METHODS: Participants filled three questionnaires: (1) the Dutch Musical Background Questionnaire (DMBQ), which measures the music listening habits, the quality of the sound of music and the self-assessed perception of elements of music; (2) the Nijmegen Cochlear Implant Questionnaire (NCIQ), which measures health-related QoL; (3) the Speech, Spatial and Qualities (SSQ) of hearing scale, which measures self-assessed hearing ability. Additionally, speech perception was behaviorally measured with a phoneme-in-word identification. RESULTS: A decline in music listening habits and a low rating of the quality of music after implantation are reported in DMBQ. A significant relationship is found between the music measures and the NCIQ and SSQ; no significant relationships are observed between the DMBQ and speech perception scores. CONCLUSIONS: The findings suggest some relationship between CI users' self-reported music perception ability and QoL and self-reported hearing ability. While the causal relationship is not currently evaluated, the findings may imply that music training programs and/or device improvements that improve music perception may improve QoL and hearing ability.

The Relation Between Tinnitus and a Neurovascular Conflict of the Cochleovestibular Nerve on Magnetic Resonance Imaging.

INTRODUCTION: Magnetic resonance (MR) imaging is often used in diagnostic evaluation of tinnitus patients. Incidental findings like a neurovascular conflict (NVC) in the cerebellopontine angle are often found; however, the diagnostic value of this finding remains unclear. The aim of this study is to investigate whether the type or degree of compression of the vestibulocochlear nerve is of diagnostic value in patients with a NVC. METHODS: A retrospective study was performed in 111 tinnitus patients with available MR imaging between 2013 and 2015. Clinical and audiometric variables were gathered and MR imaging was reevaluated by two neuroradiologists. NVCs were analyzed using a grading system based on previous research by Sirikci et al. RESULTS:: In total, 220 ears were available for assessment. In patients with unilateral tinnitus a loop compression and an indentation of the cochleovestibular nerve were more frequent than in patients with bilateral tinnitus. However, there was no significant difference in distribution of the type of compression between tinnitus and nontinnitus ears. Patient with unilateral tinnitus had a significantly higher degree of hearing loss in the symptomatic ear, compared with the asymptomatic ear and with the bilateral tinnitus group. Also, it was found that the degree of hearing loss did not differ between the various types of compression. CONCLUSION: This study did not find a diagnostic value of specific types of compression in patients with a NVC. Although the distribution of NVC classification was different in patients with unilateral and bilateral tinnitus, there was no definite relation between the type of NVC and the presence of ipsilateral tinnitus. Also, the degree of hearing loss was not related to specific types of NVC.

Early Deafened, Late Implanted Cochlear Implant Users Appreciate Music More Than and Identify Music as Well as Postlingual Users.

Introduction: Typical cochlear implant (CI) users, namely postlingually deafened and implanted, report to not enjoy listening to music, and find it difficult to perceive music. Another group of CI users, the early-deafened (during language acquisition) and late-implanted (after a long period of auditory deprivation; EDLI), report a higher music appreciation, but is this related to a better music perception? Materials and Methods: Sixteen EDLI and fifteen postlingually deafened (control group) CI users participated in the study. The inclusion criteria for EDLI were: severe or profound hearing loss onset before the age of 6 years, implantation after the age of 16 years, and CI experience more than 1 year. Subjectively, music perception and appreciation was evaluated using the Dutch Musical Background Questionnaire. Behaviorally, music perception was measured with melodic contour identification (MCI), using two instruments (piano and organ), each tested with and without a masking contour. Semitone distance between successive tones of the target varied from 1 to 3 semitones. Results: Subjectively, the EDLI group reported to appreciate music more than postlingually deafened CI users. Behaviorally, while clinical phoneme recognition test score on average was lower in the EDLI group, melodic contour identification did not significantly differ between the two groups. There was, however, an effect of instrument and masker for both groups; the piano was the best-recognized instrument, and for both instruments, the masker with non-overlapping pitch was best recognized. Discussion: EDLI group reported higher appreciation of music than postlingual control group, even though behaviorally measured music perception did not differ significantly between the two groups. Both surprising findings since EDLI CI users would be expected to have lower outcomes based on the early deafness onset, long duration of auditory deprivation, and on average lower clinical speech scores. Perhaps, the music perception difficulty comes from similar electric hearing limitations in both groups. The higher subjective appreciation in EDLI might be due to the lack of a musical memory, with no ability to compare music heard via the CI to acoustic music perception. Overall, our findings support a benefit from implantation for a positive music experience in EDLI CI users.

No Difference in Behavioral and Self-Reported Outcomes for Simultaneous and Sequential Bilateral Cochlear Implantation: Evidence From a Multicenter Randomized Controlled Trial.

Objective: The primary aim of this study was to longitudinally compare the behavioral and self-reported outcomes of simultaneous bilateral cochlear implantation (simBiCI) and sequential BiCI (seqBiCI) in adults with severe-to-profound postlingual sensorineural hearing loss. Design: This study is a multicenter randomized controlled trial with a 4-year follow-up period after the first moment of implantation. Participants were allocated by randomization to receive bilateral cochlear implants (CIs) either, simultaneously (simBiCI group) or sequentially with an inter-implant interval of 2 years (UCI/seqBiCI group). All sequential patients where encouraged to use their hearing aid on the non-implanted ear over of the first 2 years. Patients were followed-up on an annual basis. The primary outcome was speech perception in noise coming from a source directly in front of the patient. Other behavioral outcome measures were speech intelligibility-in-noise from spatially separated sources, localization and speech perception in quiet. Self-reported outcome measures encompassed questionnaires on quality of life, quality of hearing and tinnitus. All outcome measures were analyzed longitudinally using a linear or logistic regression analysis with an autoregressive residual covariance matrix (generalized estimating equations type). Results: Nineteen participants were randomly allocated to the simBiCI group and 19 participants to the UCI/seqBiCI group. Three participants in the UCI/seqBiCI group did not proceed with their second implantation and were therefore unavailable for follow-up. Both study groups performed equally well on speech perception in noise from a source directly in front of the patient longitudinally. During all 4 years of follow-up the UCI/seqBiCI group performed significantly worse compared to the simBiCI group on spatial speech perception in noise in the best performance situation (8.70 dB [3.96 - 13.44], p < 0.001) and localization abilities (largest difference 60 degrees configuration: -44.45% [-52.15 - -36.74], p < 0.0001). Furthermore, during all years of follow-up, the UCI/seqBiCI group performed significantly worse on quality of hearing and quality of life questionnaires. The years of unilateral CI use were the reason for the inferior results in the UCI/SeqBiCI group. One year after receiving CI2, the UCI/seqBiCI group performance did not statistically differ from the performance of the simBiCI group on all these outcomes. Furthermore, no longitudinal differences were seen in tinnitus burden prevalence between groups. Finally, the complications that occurred during this trial were infection, dysfunction of CI, facial nerve palsy, tinnitus and vertigo. Conclusion: This randomized controlled trial on bilaterally severely hearing impaired participants found a significantly worse longitudinal performance of UCI/seqBiCI compared to simBiCI on multiple behavioral and self-reported outcomes regarding speech perception in noise and localization abilities. This difference is associated with the inferior performance of the UCI/seqBiCI participants during the years of unilateral CI use. After receiving the second CI however, the performance of the UCI/seqBiCI group did not significantly differ from the simBiCI group. Trial Registration: Dutch Trial Register NTR1722.

An auditory brainstem implant for treatment of unilateral tinnitus: protocol for an interventional pilot study.

INTRODUCTION: Tinnitus may have a very severe impact on the quality of life. Unfortunately, for many patients, a satisfactory treatment modality is lacking. The auditory brainstem implant (ABI) was originally indicated for hearing restoration in patients with non-functional cochlear nerves, for example, in neurofibromatosis type II. In analogy to a cochlear implant (CI), it has been demonstrated that an ABI may reduce tinnitus as a beneficial side effect. For tinnitus treatment, an ABI may have an advantage over a CI, as cochlear implantation can harm inner ear structures due to its invasiveness, while an ABI is presumed to not damage anatomical structures. This is the first study to implant an ABI to investigate its effect on intractable tinnitus. METHODS AND ANALYSIS: In this pilot study, 10 adults having incapacitating unilateral intractable tinnitus and ipsilateral severe hearing loss will have an ABI implanted. The ABI is switched on 6 weeks after implantation, followed by several fitting sessions aimed at finding an optimal stimulation strategy. The primary outcome will be the change in Tinnitus Functioning Index. Secondary outcomes will be tinnitus burden and quality of life (using Tinnitus Handicap Inventory and Hospital Anxiety and Depression Scale questionnaires), tinnitus characteristics (using Visual Analogue Scale, a tinnitus analysis), safety, audiometric and vestibular function. The end point is set at 1 year after implantation. Follow-up will continue until 5 years after implantation. ETHICS AND DISSEMINATION: The protocol was reviewed and approved by the Institutional Review Board of the University Medical Centre Groningen, The Netherlands (METc 2015/479). The trial is registered at www.clinicialtrials.gov and will be updated if amendments are made. Results of this study will be disseminated in peer-reviewed journals and at scientific conferences. TRIAL REGISTRATION NUMBER: NCT02630589. TRIAL STATUS: Inclusion of first patient in November 2017. Data collection is in progress. Trial is open for further inclusion. The trial ends at 5 years after inclusion of the last patient.

Comparison of Two Music Training Approaches on Music and Speech Perception in Cochlear Implant Users.

In normal-hearing (NH) adults, long-term music training may benefit music and speech perception, even when listening to spectro-temporally degraded signals as experienced by cochlear implant (CI) users. In this study, we compared two different music training approaches in CI users and their effects on speech and music perception, as it remains unclear which approach to music training might be best. The approaches differed in terms of music exercises and social interaction. For the pitch/timbre group, melodic contour identification (MCI) training was performed using computer software. For the music therapy group, training involved face-to-face group exercises (rhythm perception, musical speech perception, music perception, singing, vocal emotion identification, and music improvisation). For the control group, training involved group nonmusic activities (e.g., writing, cooking, and woodworking). Training consisted of weekly 2-hr sessions over a 6-week period. Speech intelligibility in quiet and noise, vocal emotion identification, MCI, and quality of life (QoL) were measured before and after training. The different training approaches appeared to offer different benefits for music and speech perception. Training effects were observed within-domain (better MCI performance for the pitch/timbre group), with little cross-domain transfer of music training (emotion identification significantly improved for the music therapy group). While training had no significant effect on QoL, the music therapy group reported better perceptual skills across training sessions. These results suggest that more extensive and intensive training approaches that combine pitch training with the social aspects of music therapy may further benefit CI users.

Hearing Restoration in Cochlear Nerve Deficiency: the Choice Between Cochlear Implant or Auditory Brainstem Implant, a Meta-analysis.

OBJECTIVE: To answer the dilemma clinician's face when deciding between cochlear implant (CI) and auditory brainstem implant (ABI) treatment options in patients with cochlear nerve deficiency (CND). STUDY DESIGN: Case study supplemented with literature review and meta-analysis. SETTING: Tertiary referral center. PATIENT(S): Child with CHARGE syndrome and congenital deafness. INTERVENTION(S): ABI as there was no benefit after bilateral cochlear implantation. MAIN OUTCOME MEASURES: Speech and language development, quality of life. RESULTS: In one ear the cochleovestibular nerve was present on magnetic resonance imaging (MRI) without preoperative ABR responses. In the contra lateral ear the nerve could not be identified, despite present ABR responses. Nevertheless, there was no positive outcome with CI. The patient had improved speech and language and quality of life with ABI. Of the 108 patients with CND and CI identified in the literature review, 25% attained open-set speech perception, 34% attained closed-set speech perception, and 41% detected sounds or less. The appearance of the cochlear nerve on MRI was a useful predictor of success, with cochlear nerve aplasia on MRI associated with a smaller chance of a positive outcome post cochlear implantation compared with patients with cochlear nerve hypoplasia. CONCLUSION: Although patients with (apparent) cochlear nerve aplasia are less likely to benefit from CI, CI before ABI is supported as some patients attain closed or open-set levels of speech perception after cochlear implantation.

Effect of Direct Stimulation of the Cochleovestibular Nerve on Tinnitus: A Long-Term Follow-Up Study.

OBJECTIVE: Tinnitus is a common entity that may lead to severe impairment in quality of life. An adequate treatment modality for severe tinnitus is currently lacking. Neurostimulation of the auditory tract may serve as a promising adjunct in tinnitus treatment. The aim is to investigate the effect of direct stimulation on the cochleovestibular nerve for intractable tinnitus. METHODS: This study was conducted at the University Medical Center Groningen, The Netherlands. We studied 10 patients with severe, unilateral, intractable tinnitus, who were implanted with a cuff electrode around the cochleovestibular nerve between 2001 and 2013. All patients had preoperative ipsilateral hearing loss. Tinnitus Handicap Inventory (THI) scores and audiometric values were collected. Treatment success was determined based on the self-assessment of satisfactory usage by each patient. RESULTS: The mean preoperative tinnitus duration was 8.0 ± 5.9 years. The preoperative THI score was 71 ± 18 points. During mean follow-up of 49 months, the mean THI reduction was 24 ± 26 points (P = 0.02). Treatment was regarded successful in 6 patients (60%). In these patients, tinnitus did not disappear, but transformed into a more bearable sound. In 4 patients, transient complications occurred, and 1 patient experienced permanent vertigo postoperatively. Furthermore, hearing deterioration was a result of implantation in 86% of the patients. CONCLUSIONS: Direct neurostimulation resulted in treatment success in a small majority of the patients, with a significant decrease in THI score. However, because of a high risk of additional hearing damage, this technique seems not viable for patients with moderate hearing loss.