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Cochlear implants (CIs) restore hearing in patients with severe-to-profound deafness. Post-CI meningitis is a rare but redoubted complication. We present the case of a five-year-old CI recipient who experienced an episode of chronic meningitis caused by chronic otitis media with cholesteatoma encasing the electrode lead. We hypothesize that the cholesteatoma led to an ascending infection to the cochlea, passing through the labyrinths, resulting in chronic meningitis. Although positive neural responses were initially noted on cochlear electrical stimulation, these responses resolved a few weeks after reimplantation. Our report highlights the importance of careful otoscopic examination and diagnostic work-up in patients presenting with otogenic meningitis to rule out cholesteatoma formation and to ensure prompt surgical exploration if warranted.

The presence of tinnitus does not necessarily imply associated suffering. Prediction models on the impact of tinnitus on daily life could aid medical professionals to direct specific medical resources to those (groups of) tinnitus patients with specific levels of impact. Models of tinnitus presence could possibly identify risk factors for tinnitus. We systematically searched the PubMed and EMBASE databases for articles published up to January 2021. We included all studies that reported on multivariable prediction models for tinnitus presence or the impact of tinnitus on daily life. Twenty-one development studies were included, with a total of 31 prediction models. Seventeen studies made a prediction model for the impact of tinnitus on daily life, three studies made a prediction model for tinnitus presence and one study made models for both. The risk of bias was high and reporting was poor in all studies. The most used predictors in the final impact on daily life models were depression- or anxiety-associated questionnaire scores. Demographic predictors were most common in final presence models. No models were internally or externally validated. All published prediction models were poorly reported and had a high risk of bias. This hinders the usability of the current prediction models. Methodological guidance is available for the development and validation of prediction models. Researchers should consider the importance and clinical relevance of the models they develop and should consider validation of existing models before developing new ones.

Objectives: In this study, we aim to analyze audiometric outcomes of middle ear surgery in patients with congenital middle ear anomalies. Methods: In this single center retrospective cohort study, audiological outcomes were extracted from patient files. Patients with a congenital middle ear anomaly treated surgically in a tertiary referral center between June 2015 and December 2020 were included. Pre- and postoperative short- and long-term audiometric data (at ≥3 and ≥10 months respectively) were compared to analyze hearing outcomes. Results: Eighteen ears (15 patients) were treated surgically with an exploratory tympanotomy. At short term follow up statistically significant improvements in air conduction thresholds and air-bone gaps were found. Hearing improved in 94.4% (17/18) of operated ears. Successful outcome, defined as an air-bone gap closure to within 20 dB after surgery, was reached in 44.4% (8/18). Serviceable hearing (air conduction ≤30 dB) was reached in 55.6% (10/18). Negative outcome (any significant deterioration in hearing) occurred in 1 patient: in this ear otitis media occurred during the postoperative course. At long term follow up, available for 50% of the cohort, hearing remained stable in 5 ears, improved in 1 ear and deteriorated in 3, all of which underwent revision surgery. Sensorineural hearing loss due to surgery, or other complications, were not encountered. Conclusion: middle ear surgery was found to be an effective treatment option to improve hearing in this cohort of patients with congenital middle ear anomalies. Surgical goals of obtained gain in air conduction thresholds and serviceable hearing levels were met by most patients without the occurrence of any iatrogenic sensorineural hearing loss.

Importance: The prevalence of hyperacusis and its relationship with mental and general health is unknown in a nonclinical sample. Therefore, we aimed to determine the prevalence of hyperacusis and its relation with hearing, general and mental health in a population-based study. Study Design: Prospective population-based study. Material and Methods: This study uses data from the Busselton Healthy Ageing Study (BHAS). A sample of 5,107 eligible inhabitants aged 45 to 70 years completed a detailed questionnaire and a clinical assessment. A positive answer to “Do you consider yourself sensitive or intolerant to everyday sounds” was used to indicate hyperacusis. Logistic regression was used to examine the association between hearing, mental and general health factors, and hyperacusis. Results: Of 5,107 participants, 775 (15.2%) reported hyperacusis. The majority of participants with hyperacusis reported an occasional effect on daily life (72.0%). Being female, older in age, having a lower income, physical or mental health difficulties, more severe hearing loss, and tinnitus were all associated with the presence of hyperacusis. Individuals who experience hearing impairment, poorer general or mental health have a higher possibility of hyperacusis having an effect on their daily life. Conclusions: In this community population-based cohort study, we found a prevalence of hyperacusis of 15.2%. Individuals with hearing loss, mental health problems, and lower physical health have a higher possibility of experiencing effects on their daily life associated with their hyperacusis. Unravelling the relationship between hyperacusis hearing, general and mental health can be of major importance for a better understanding of the condition and its consequences. Level of Evidence: 2 Laryngoscope, 131:E2887–E2896, 2021.

Tinnitus is a heterogeneous condition not only in terms of nature of the sound, but also in co-morbidities such as mental health issues. Prevalence number range widely between 5 and 43%. Even though the etiologic pathway between tinnitus and its comorbidities remains unclear, in this study we aim to assess whether people with tinnitus use more primary health care than people without tinnitus. To compare primary healthcare consumption between patients with tinnitus and people without tinnitus. In this cross-sectional study, data on number of consultations with the general practitioner or nurse practitioner mental health services were obtained from Nivel (Netherlands Institute for Health Service Research) Primary Care Database in 2018 (n = 963,880 people). People with an open tinnitus episode (n = 8050) were defined as a patient with tinnitus and compared to all other people. Percentages, means, ranges and mean differences were calculated for the total number of consultations and for organ specific diagnoses registered as ICPC-1 code on the day of consultation. Secondary, the total number of referrals to medical specialists and number of drug prescriptions was collected. Logistic regressions were performed to predict having one or more contacts, referrals, and prescriptions,with having tinnitus, this was corrected for age and gender. Patients with tinnitus had a mean of 9.8 (SD 10.9) primary care consultations in 2018, compared to 5.7 (SD 7.9) for people without tinnitus. More patients with tinnitus had more than one referral to medical specialists (47%) compared to people without tinnitus (25%). Patients with tinnitus have 1.2 (mean difference) more drug prescriptions than people without tinnitus. Compared to people without tinnitus, patients with tinnitus were more likely to have one or more of primary healthcare contact, independent of age group and gender. Patients with tinnitus had more consultations in primary health care than people without tinnitus. They are more often referred to medical specialists and receive more drug prescriptions. The causal relationship between tinnitus and the higher healthcare consumption remains to be researched.

Knowledge on characteristics of people that seek help for tinnitus is scarce. The primary objective of this study was to describe differences in characteristics between people with tinnitus that seek help compared to those who do not seek help. Next, we described differences in characteristics between those with and without tinnitus. In this cross-sectional study, we sent a questionnaire on characteristics in different domains; demographic, tinnitus-specific, general- and psychological health, auditory and noise- and substance behaviour. We assessed if participants had sought help or planned to seek help for tinnitus. Tinnitus distress was defined with the Tinnitus Functional Index. Differences between groups (help seeking: yes/no, tinnitus: yes/no) were described. 932 people took part in our survey. Two hundred and sixteen participants were defined as having tinnitus (23.2%). Seventy-three of those sought or planned to seek help. A constant tinnitus pattern, a varying tinnitus loudness, and hearing loss, were described more frequently in help seekers. Help seekers reported higher TFI scores. Differences between help seekers and people not seeking help were mainly identified in tinnitus- and audiological characteristics. These outcomes might function as a foundation to explore the heterogeneity in tinnitus patients.

Several electrical stimulation patterns of the auditory nerve have been described for tinnitus relief, but there is no consensus on the most effective stimulation pattern. Therefore, we aim to systematically review the literature on the effect of intra- and extracochlear electrical stimulation patterns as a treatment option for patients with tinnitus. Only studies on intra- and extracochlear electrical stimulation for patients with tinnitus were included if the stimulation used did not concern standardized CI stimulation patterns to primarily rehabilitate hearing loss as intervention. A total of 34 studies met the inclusion criteria, with 10 studies (89 patients) on intracochlear electrical stimulation and 25 studies on extracochlear electrical stimulation (1109 patients). There was a high to medium risk of bias in 22 studies, especially due to lack of a non-exposed group and poor selection of the exposed group. All included studies showed subjective tinnitus improvement during or after electrical stimulation, using different stimulation patterns. Due to methodological limitations and low reporting quality of the included studies, the potential of intra- and extracochlear stimulation has not been fully explored. To draw conclusions on which stimulation patterns should be optimized for tinnitus relief, a deeper understanding of the mechanisms involved in tinnitus suppression is needed.

Objectives Cochlear implants (CIs) are implantable hearing devices with a wide variation in clinical outcome between patients. We aim to provide an overview of the literature on prediction models and their performance for clinical outcome after cochlear implantation in bilateral hearing loss or deafness. Study Design and Setting In this systematic review, studies describing the development or external validation of a multivariable model for predicting clinical CI outcome were eligible for selection. Results A total of 4,042 references were screened. We included nine development studies and one external validation study. The outcome measure of all development studies was speech perception performance after cochlear implantation. The most commonly used model predictors were duration of hearing loss or deafness (n = 7), different types of preoperative measurements (n = 5), and etiology (n = 3). In three studies, crucial information to enable the model to be used for individual risk prediction was missing. One study performed internal validation,two models were externally validated. One study reported specific discrimination or calibration performance measures. Conclusion Although many articles describe development studies of prediction models for speech perception performance after cochlear implantation, the value of most of these models for their application in clinical practice remains unclear. Therefore, research should focus on increasing the clinical relevance of existing CI outcome prediction models.

ObjectivesThis systematic review summarises the evidence on the correlation between recurrent acute otitis media (rAOM) or chronic suppurative otitis media (CSOM) and sensorineural hearing loss (SNHL).Research methodsPubMed, Embase and Cochrane Library databases were searched from inception to 15 January 2021. Two authors independently identified articles, extracted data and performed quality assessment for included studies. Studies comparing the sensorineural hearing levels of patients with a history of rAOM/CSOM for >3 months to a control group were included.ResultsScreening of 4168 articles lead to inclusion of two case-control studies (control-group: patients non-OM) and seven cohort-studies (control group: contralateral ear). Quality assessment indicated considerable risk of bias in all studies. Reported populations varied (sample size 13–607, mean age 22–41.5 years, mean duration of disease 6.1–12.4 years). The OR for SNHL in the OM-group was 3.30–7.86 (95% CI 1.16 to 9.40, pConclusionDue to the high risk of bias of included studies, effect estimates heterogeneity and suboptimal research designs, no conclusion on the correlation between OM and SNHL can be made. It emphasises the need for future prognostic studies.

IntroductionTinnitus is the perception of sound without an external stimulus, often experienced as a ringing or buzzing sound. Subjective tinnitus is assumed to origin from changes in neural activity caused by reduced or lack of auditory input, for instance due to hearing loss. Since auditory deprivation is thought to be one of the causes of tinnitus, increasing the auditory input by cochlear implantation might be a possible treatment. In studies assessing cochlear implantation for patients with hearing loss, tinnitus relief was seen as a secondary outcome. Therefore, we will assess the effect of cochlear implantation in patients with primarily tinnitus complaints.Method and analysisIn this randomised controlled trial starting in January 2021 at the ENT department of the UMC Utrecht (the Netherlands), patients with a primary complaint of tinnitus will be included. Fifty patients (Tinnitus Functional Index (TFI) >32, Beck’s Depression Index Ethics and disseminationThis research protocol was approved by the Institutional Review Board of the University Medical Center (UMC) Utrecht (NL70319.041.19, V5.0, January 2021). The trial results will be made accessible to the public in a peer-review journal.Trial registration numberTrial registration number NL8693; Pre-results.

Our objective was to study associations between demographics, tinnitus specific-, audiological-, general- and mental health characteristics, and impact of tinnitus in the general population. In this cross-sectional survey study in the Dutch population, data were prospectively gathered. Tinnitus impact was assessed with the Tinnitus Functional Index (TFI). We included participants who experienced tinnitus and for whom a total TFI score could be calculated (n = 212). We performed univariable and multivariable regression analyses. Due to logarithmical transformation, the B-scores were back-transformed to show the actual difference in points on the TFI. People who considered hyperacusis a small problem had a 12.5-point higher TFI score, those who considered it a mediocre problem had a 17.6-point higher TFI score and those who considered it a large problem had a 24.1-point higher TFI score compared to people who did not consider hyperacusis a problem. People who indicated having minor hearing problems had a 10.5-point higher TFI score, those with mediocre hearing problems had a 20.4-point higher TFI score and those with severe hearing problems had a 41.6-point higher TFI score compared to people who did not have subjective hearing problems. In conclusion, audiological risk factors, such as hearing problems and hyperacusis, have the largest association with the impact of tinnitus on daily life, compared to other assessed variables. The results of this study can be used in future research to find targeted interventions to diminish the impact of tinnitus.

IntroductionTinnitus is the perception of sound without an external stimulus, often experienced as a ringing, buzzing sound. While several studies have shown a reduction in tinnitus distress following cochlear implantation, others showed an increase or no change after implantation. At this stage, clinicians have little certainty when counselling their patients prior to implantation regarding tinnitus post-implantation. To help clinicians to counsel cochlear implant (CI) candidates on the risk of developing or improving tinnitus after implantation, we aim to assess the effect of electrical stimulation with a CI on tinnitus impact for individual adult patients with tinnitus. We will also apply prediction models to individual patient data (IPD) of clinical trials to find predictive factors of the effect of electrical stimulation on tinnitus impact.Method and analysisThe IPD meta-analysis is a follow-up project of the systematic review on cochlear implantation in patients with tinnitus as a primary complaint. First, the systematic searches will be updated to date. Methodological quality of eligible studies will be assessed using the Risk of Bias In Non-randomised Studies of Intervention tool (ROBINS-I). Based on a data-sharing agreement, authors of the eligible studies will be invited to share their deidentified and complete IPD. The primary outcome is the effect of electrical stimulation with a CI on tinnitus impact 1 month or more post-implantation. IPD meta-analysis will be used to assess the primary outcome, while differentiating the tinnitus impact questionnaires. Second, linear regression analyses will be used to model the effect of electrical stimulation on tinnitus impact based on relevant predictors.Ethics and disseminationThe Medical Research Involving Human Subject Act does not apply, and ethical approval is not required. The study results will be made accessible to the public in a peer-review open access journal.PROSPERO registration numberCRD42022319367, review ongoing.

A deeper knowledge of tinnitus is essential in order to better manage and treat tinnitus and its effects. Most studies to date are based on small samples and/or conducted in clinical settings. In this study we assessed the associations between tinnitus, general and mental health, hearing status and demographics in a large population cohort study.The Busselton Healthy Aging Study recruited people born between 1946 and 1964. Data were collected between 2010 and 2015. Logistic regression was used to examine the associations between tinnitus and its effect on daily life, age, gender, hearing, self-reported mental and general health, and doctor diagnosed health conditions.Of 5107 participants, 1154 (22.6%) reported experiencing tinnitus. Of those, 32.4% reported that their tinnitus had an occasional effect on their daily lives, while for a further 8.9% the effect on their daily life was frequent or constant. The odds ratio for having a SF12-PCS was (OR 1.02 (95%CI 1.01-1.03). Furthermore, individuals who experience their tinnitus as having an effect on their daily life, have an increased risk of having a lower general health (OR 1.04 (95%CI 1.02-1.03)) than those without tinnitus. Higher levels of depression, anxiety and stress, as well as doctor diagnosed depression, were all significant risk factors for tinnitus. There were statistically significant worse hearing thresholds related to the presence of tinnitus.The outcomes raise the question for clinicians and researchers whether addressing the mental and general health of individuals will influence the presence or burden of tinnitus.

Background Tinnitus is a phantom sensation of sound, which can have a negative impact on quality of life of those affected. No curative treatments are currently known. Neuromodulation by vagus nerve stimulation has emerged as a new treatment option for tinnitus, though till date the effectiveness remains unclear. Therefore, we aim to review the effect of vagus nerve stimulation on tinnitus distress and tinnitus symptom severity in patients with chronic tinnitus. Methods We searched Pubmed, Embase and the Cochrane Library systematically for RCTs, observational studies and case studies on the effect of VNS treatment for tinnitus on October 29, 2019. Studies including adult patients with subjective tinnitus, comparing transcutaneous or implantable VNS to placebo or no treatment or before and after application of VNS treatment on tinnitus distress and tinnitus symptom severity measured with a validated questionnaire were eligible. The risk of bias was assessed with the appropriate tool for each type of study. Results Our search identified 9 primary studies of which 2 RCTs, 5 cohort studies and 2 case series or reports. 5 studies used transcutaneous VNS treatment and 4 used implanted VNS treatment. 6 studies combined VNS treatment with sound therapy. There was a serious risk of bias in all studies, especially on confounding. Most studies reported a small decrease in tinnitus distress or tinnitus symptom severity. Conclusion Due to methodological limitations and low reporting quality of the included studies, the effect of VNS on tinnitus remains unclear. To draw conclusions for which patient population and to what extent (t)VNS is beneficial in the treatment of tinnitus, a randomised controlled trial should be considered.

Background While research has shown that children with single sided deafness have a lower quality of life and developmental outcomes compared to normal hearing peers, little is known about these domains in children with unilateral congenital conductive hearing loss due to aural atresia. Objectives This study aims to investigate the hearing-related quality of life, developmental outcomes and educational performance in children and young adults with unilateral conductive hearing loss due to aural atresia. Methods Nineteen children and young adults with unilateral aural atresia received a set of five questionnaires. Hearing-related quality of life (SSQ), general quality of life (Kidscreen-27), speech and language development (CCC-2-Nl), educational performance and problems in social-emotional and behavioral domains (CBCL/YSR/ASR) were measured with validated questionnaires. Scores on the questionnaires were compared to their norm scores. Mann-Whitney U tests and independent t-tests were used to identify significant differences between age groups. Results Mean scores on the SSQ subscales were speech 6.78, spatial 5.00 and quality 6.98. Mean scores on the Kidscreen-27, CCC-2-NL, CBCL/YSR/ASR fell within normal or non-clinical range. A high number of cases needed speech therapy (60.7%) or special measures in class (79.3%) or showed grade repetition (>30%) in primary or secondary school. Conclusion Children and young adults with unilateral conductive hearing loss due to congenital aural atresia showed lower scores regarding hearing-related quality of life compared to normal-hearing peers. The result show similarities with children with single sided deafness. Regarding general quality of life, speech and language development and in social-emotional and behavioral domains the studied children and young adults seem to develop according to norm scores. It is important to observe these children closely as they may need guidance during education to allow them to thrive.

Cochlear implantation (CI) is used in patients with severe-to-profound hearing loss when hearing aids provide limited or no benefit for speech perception. Studies on this topic reported tinnitus reduction as a common side effect of the electrical activation after cochlear implantation. So far, it is unclear what the effect is when patients do receive their implant primarily because of tinnitus complaints.To assess the effectiveness of the electrical stimulation with a cochlear implant in patients with tinnitus as a primary complaint, by systematically reviewing the literature.Two independent authors identified studies, extracted data and assessed risk of bias of included studies. Original studies reporting outcomes of electrical stimulation by cochlear implantation for primarily tinnitus (defined as severe or incapacitating distress levels) were included, if they reported a follow-up of at least three months. The pre- and post-implantation tinnitus distress scores on single and/or multi-item questionnaires of the included studies were extracted.In total, 4091 unique articles were retrieved. After screening titles, abstracts and full texts, we included seven prospective cohort studies (105 subjects in total, range: 10-26). All studies had considerable risks of bias. All tinnitus patients in the included studies had asymmetrical hearing loss or single-sided deafness. A statistically significant tinnitus distress improvement based on tinnitus questionnaire scores was found in every study.Our systematic review reveals that electrical stimulation by cochlear implants in patients with a primary complaint of tinnitus has a positive impact on tinnitus distress. Nevertheless, only small sample sizes were found and studies showed considerable risks of bias.

Single-sided deafness (SSD) leads to difficulties with speech perception in noise, sound localisation, and sometimes tinnitus. Current treatments (Contralateral Routing of Sound hearing aids (CROS) and Bone Conduction Devices (BCD)) do not sufficiently overcome these problems. Cochlear implants (CIs) may help. Our aim was to evaluate these treatments in a Randomised Controlled Trial (RCT). Adult SSD patients were randomised using a web-based randomisation tool into one of three groups: CI; trial period of ‘first BCD, then CROS’; trial period of ‘first CROS, then BCD’. After these trial periods, patients opted for BCD, CROS, or No treatment. The primary outcome was speech perception in noise (directed from the front (S0N0)). Secondary outcomes were speech perception in noise with speech directed to the poor ear and noise to the better ear (SpeNbe) and vice versa (SbeNpe), sound localisation, tinnitus burden, and disease-specific quality of life (QoL). We described results at baseline (unaided situation) and 3 and 6 months after device activation. 120 patients were randomised. Seven patients did not receive the allocated intervention. The number of patients per group after allocation was: CI (n = 28), BCD (n = 25), CROS (n = 34), and No treatment (n = 26). In S0N0, the CI group performed significantly better when compared to baseline, and when compared to the other groups. In SpeNbe, there was an advantage for all treatment groups compared to baseline. However, in SbeNpe, BCD and CROS groups performed worse compared to baseline, whereas the CI group improved. Only in the CI group sound localisation improved and tinnitus burden decreased. In general, all treatment groups improved on disease-specific QoL compared to baseline. This RCT demonstrates that cochlear implantation for SSD leads to improved speech perception in noise, sound localisation, tinnitus burden, and QoL after 3 and 6 months of follow-up. For most outcome measures, CI outperformed BCD and CROS. Trial registration: Netherlands Trial Register (www.trialregister.nl): NTR4580, CINGLE-trial.

Objectives: Speech recognition (SR)-tests have been developed for children without considering the linguistic complexity of the sentences used. However, linguistic complexity is hypothesized to influence correct sentence repetition. The aim of this study is to identify lexical and grammatical parameters influencing verbal repetition accuracy of sentences derived from a Dutch SR-test when performed by 6-year-old typically developing children. Design: For this observational, cross-sectional study, 40 typically developing children aged 6 were recruited at four primary schools in the Netherlands. All children performed a sentence repetition task derived from an SR-test for adults. The sentence complexity was described beforehand with one lexical parameter, age of acquisition, and four grammatical parameters, specifically sentence length, prepositions, sentence structure, and verb inflection. A multiple logistic regression analysis was performed. Results: Sentences with a higher age of acquisition (odds ratio [OR] = 1.59) or greater sentence length (OR = 1.28) had a higher risk of repetition inaccuracy. Sentences including a spatial (OR = 1.25) or other preposition (OR = 1.25) were at increased risk for incorrect repetition, as were complex sentences (OR = 1.69) and sentences in the present perfect (OR = 1.44) or future tense (OR = 2.32). Conclusions: The variation in verbal repetition accuracy in 6-year-old children is significantly influenced by both lexical and grammatical parameters. Linguistic complexity is an important factor to take into account when assessing speech intelligibility in children.

IntroductionChronic subjective tinnitus is a condition that affects 5.1% to 42.7% of the population, depending on the definition and studied population. Evidence-based treatment options are limited. Cognitive Behavioural Therapy (CBT) has been proven effective to improve quality of life and to diminish tinnitus distress. Positive short-term effects of mindfulness-based interventions on tinnitus distress have been reported; however, the longer term effects remain to be studied.Methods and analysisWe designed a monocentre randomised controlled, non-inferiority trial to compare the effectiveness of mindfulness-based cognitive therapy (MBCT) and CBT in chronic tinnitus patients. Fifty-four patients (≥32 on the Tinnitus Functional Index (TFI), suffering from tinnitus for at least 6 months) will be included in the trial and randomised into one of two intervention groups. One group will receive MBCT, the other group will receive CBT. Our primary objective is to determine whether MBCT is non-inferior to (as good as) CBT on tinnitus distress (TFI) in chronic tinnitus patients at 12 months follow-up after end of therapy. Non-inferiority will be declared if the mean decrease in TFI score for MBCT is no worse than the mean decrease in TFI score in CBT, with statistical variability, with a margin of 13 points. Most secondary objectives (tinnitus severity of problem, tinnitus intrusiveness, quality of life, anxiety, depression, symptoms of psychopathology, perceived tinnitus complaints, coping style (mostly validated questionnaires)) are expected to show non-inferiority to MBCT compared with CBT. We expect a significant difference between MBCT and CBT for mindfulness awareness.Ethics and disseminationThis research protocol was approved by the Institutional Review Board of the UMC Utrecht (NL67838.041.18, V.4, April 2019). The trial results will be made accessible to the public in a peer-review journal.Trial registration numberNL7745.

Hearing loss is frequently seen in mucopolysaccharidoses (MPS) patients. Although hematopoietic cell transplantation (HCT) increases overall survival, disease progression is observed in certain tissues. This study describes the course of hearing loss (HL) over time in transplanted MPS patients. Transplanted MPS patients between 2003 and 2018 were included and received yearly audiological evaluation, including auditory brainstem response (ABR) or pure tone audiometry (PTA). Twenty‐eight MPS‐1 and four MPS‐6 patients were analyzed with a median follow‐up of 5 years (range 11 months–16 years). Air conduction threshold improved significantly over time (P

Objectives: Postoperative follow-up after cochlear implantation lacks a reliable screening method to detect cochlear implant receiver device migration. This study aims to validate a clinically applicable method to assess the position and migration of the cochlear implant receiver device. Study design: Validation study. Setting: Tertiary university medical center. Participants and method: To assess the cochlear implant receiver device location, round markers representing the external magnet were placed on both sides of the head of volunteers. Four independent clinicians took measurements of the distances between reference points on the head and the center of the marker. The reference points were: the lateral canthus (LC), tragus tip (TT), the mastoid angle (MA), and the mandibular angle (AM). Main outcome measures: The inter-clinician reliability was determined by calculating the intraclass correlation coefficient (ICC) and confidence interval (CI) with a two-way mixed model and both consistency and absolute agreement types for each distance. Results: Eight volunteers were included resulting in 16 individual cases. The consistency type ICC's for each reference point were: LC 0.90 (CI = 0.80, 0.96), TT 0.83 (CI = 0.69, 0.93), MA 0.75 (CI = 0.56, 0.89), and AM 0.29 (CI = 0.05, 0.59). The absolute agreement ICC's were: LC 0.87 (CI = 0.73, 0.95), TT 0.83 (CI = 0.68, 0.93), MA 0.68 (CI = 0.42, 0.86), and AM 0.18 (CI = 0.01, 0.46). The inter-clinician reliability was good to excellent for the lateral canthus and tragus tip reference points. Conclusions: The cochlear receiver device location can be assessed reliably by measuring the distance between the LC, TT, and the external magnet. This method can be used to registrate implant receiver location after implantation and detect implant migration postoperatively.

Background: Cochlear implantation (CI) is used in patients with severe-to-profound hearing loss when hearing aids provide limited or no benefit for speech perception. Studies on this topic reported tinnitus reduction as a common side effect of the electrical activation after cochlear implantation. So far, it is unclear what the effect is when patients do receive their implant primarily because of tinnitus complaints. Objectives: To assess the effectiveness of the electrical stimulation with a cochlear implant in patients with tinnitus as a primary complaint, by systematically reviewing the literature. Methods: Two independent authors identified studies, extracted data and assessed risk of bias of included studies. Original studies reporting outcomes of electrical stimulation by cochlear implantation for primarily tinnitus (defined as severe or incapacitating distress levels) were included, if they reported a follow-up of at least three months. The pre- and post-implantation tinnitus distress scores on single and/or multi-item questionnaires of the included studies were extracted. Results: In total, 4091 unique articles were retrieved. After screening titles, abstracts and full texts, we included seven prospective cohort studies (105 subjects in total, range: 10–26). All studies had considerable risks of bias. All tinnitus patients in the included studies had asymmetrical hearing loss or single-sided deafness. A statistically significant tinnitus distress improvement based on tinnitus questionnaire scores was found in every study. Conclusion: Our systematic review reveals that electrical stimulation by cochlear implants in patients with a primary complaint of tinnitus has a positive impact on tinnitus distress. Nevertheless, only small sample sizes were found and studies showed considerable risks of bias. a

Item does not contain fulltext Importance: To date, no randomized clinical trial on the comparison between simultaneous and sequential bilateral cochlear implants (BiCIs) has been performed. Objective: To investigate the hearing capabilities and the self-reported benefits of simultaneous BiCIs compared with those of sequential BiCIs. Design, Setting, and Participants: A multicenter randomized clinical trial was conducted between January 12, 2010, and September 2, 2012, at 5 tertiary referral centers among 40 participants eligible for BiCIs. Main inclusion criteria were postlingual severe to profound hearing loss, age 18 to 70 years, and a maximum duration of 10 years without hearing aid use in both ears. Data analysis was conducted from May 24 to June 12, 2016. Interventions: The simultaneous BiCI group received 2 cochlear implants during 1 surgical procedure. The sequential BiCI group received 2 cochlear implants with an interval of 2 years between implants. Main Outcomes and Measures: First, the results 1 year after receiving simultaneous BiCIs were compared with the results 1 year after receiving sequential BiCIs. Second, the results of 3 years of follow-up for both groups were compared separately. The primary outcome measure was speech intelligibility in noise from straight ahead. Secondary outcome measures were speech intelligibility in noise from spatially separated sources, speech intelligibility in silence, localization capabilities, and self-reported benefits assessed with various hearing and quality of life questionnaires. Results: Nineteen participants were randomized to receive simultaneous BiCIs (11 women and 8 men; median age, 52 years [interquartile range, 36-63 years]), and another 19 participants were randomized to undergo sequential BiCIs (8 women and 11 men; median age, 54 years [interquartile range, 43-64 years]). Three patients did not receive a second cochlear implant and were unavailable for follow-up. Comparable results were found 1 year after simultaneous or sequential BiCIs for speech intelligibility in noise from straight ahead (difference, 0.9 dB [95% CI, -3.1 to 4.4 dB]) and all secondary outcome measures except for localization with a 30 degrees angle between loudspeakers (difference, -10% [95% CI, -20.1% to 0.0%]). In the sequential BiCI group, all participants performed significantly better after the BiCIs on speech intelligibility in noise from spatially separated sources and on all localization tests, which was consistent with most of the participants' self-reported hearing capabilities. Speech intelligibility-in-noise results improved in the simultaneous BiCI group up to 3 years following the BiCIs. Conclusions and Relevance: This study shows comparable objective and subjective hearing results 1 year after receiving simultaneous BiCIs and sequential BiCIs with an interval of 2 years between implants. It also shows a significant benefit of sequential BiCIs over a unilateral cochlear implant. Until 3 years after receiving simultaneous BiCIs, speech intelligibility in noise significantly improved compared with previous years. Trial Registration: trialregister.nl Identifier: NTR1722.

Objective The objective of this study was to assess the attitude and behaviour of Dutch ENT surgeons and ENT residents towards Evidence Based Medicine. Introduction Evidence Based Medicine is the cornerstone of modern clinical care. It is considered of crucial importance for optimal patient care and health care quality. Practicing it requires positive attitude and behaviour. Little is known about the attitude and behaviour towards Evidence Based Medicine in otorhinolaryngology. Methods We performed a web-based questionnaire among 607 Dutch Ear- Nose & Throat surgeons of whom 106 residents (cross-sectional study). The questionnaire consisted of 3 parts; (1) personal characteristics, (2) questions regarding Evidence Based Medicine attitude (McColl questionnaire, scale 0-100%) and (3) questions regarding Evidence Based Medicine behaviour (barriers and information seeking behaviour). Data were collected between March 26th 2018 and June 1st 2018. Results The median score on the overall McColl questionnaire was 50 (IQR 35). The main barriers respondents experienced were time related. Limited time in the outpatient clinic was considered a more important barrier for residents to practice EBM compared to ENT surgeons. Respondents' gut feeling and their own preference were identified as the main contributing factors in clinical decision making. Conclusion In conclusion Dutch ENT surgeons have a moderate attitude on the McColl questionnaire. The main barriers to practice Evidence Based Medicine they experience are time related.

Background: Fetal exposure to in utero inflammation such as chorioamnionitis is related to central nervous system injury. We hypothesized that chorioamnionitis can provoke inflammatory changes in the perilymph and alter hearing outcome. Methods: Pregnant ewes were randomized into 2 groups: intrauterine injection with lipopolysaccharide (LPS; n = 19) or saline (n = 21). In the first experiment, fetal perilymph samples were taken for cytokine analysis. In the second experiment, consecutive bone-conducted auditory brain stem responses were obtained from 1 to 7 months after birth. Results: Perilymph samples showed a significant elevation in interleukin 8 in the LPS group. Auditory brain stem response analysis demonstrated higher response thresholds and a prolongation of absolute peak V and interpeak intervals I to V and III to V in the LPS group compared to sham treatment. Conclusion: Our study confirms the hypothesis that an intrauterine inflammation by LPS can result in a fetal perilymphatic inflammatory response and functional impaired hearing outcomes after birth in a sheep model.

BackgroundThe Consolidated Standards of Reporting Trials (CONSORT) statement aims to improve clarity and consistency of transparency of reporting in Randomized Controlled Trials (RCTs). The Cochrane Risk of Bias (RoB) tool for RCTs helps authors to judge the RoB. as ''low", "high" or "unclear".ObjectiveIn this study we aimed to assess whether the implementation and updates of the CONSORT statement influenced the trend of "unclear" RoB scores of RCTs included in Cochrane systematic reviews.MethodsAll Cochrane reviews published in December to October 2016 were retrieved. The publication year of RCTS included in the reviews were sorted into time frames (≤1995, 1996-2000, 2001-2009 and ≥2010) based on the release- and updates of the CONSORT statement (1996, 2001 and 2010). The association between "unclear" RoB versus "low or high" RoB and the year of publication in different time frames were calculated using a binary logistic regression.ResultsData was extracted from 64 Cochrane reviews, with 989 RCTS (6471 items). The logistic regression showed that the odds of RCTs published ≥2010, compared to ≤1995 were more likely not to report an "unclear" RoB for the total data (Odds Ratio (OR) 0.69 (95% Confidence interval: 0.59-0.80)), random sequence generation (OR 0.32 (0.22-0.47), allocation concealment (0.64 (0.43-0.95)) and incomplete outcome data (OR 0.60 (0.39-0.91)).ConclusionA slight decrease of "unclear" RoB reporting over time was found. To improve quality of reporting authors are encouraged to adhere to reporting guidelines.

Objectives To investigate whether a squelch effect develops in postlingually deafened adults after sequential bilateral cochlear implantation. Study design Prospective study as part of a randomized controlled trial on the difference between simultaneous versus sequential bilateral cochlear implantation. Setting Tertiary referral center. Participants Sixteen postlingually deafened adults. Intervention Sequential bilateral cochlear implantation with a 2-year interimplant interval. Main outcome measure A squelch effect was defined as a better bilateral score than unilateral score on a speech-intelligibility-in-noise test with spatially separated sources. The squelch effect was evaluated for the participants' best performing cochlear implant (CI) ear, the left CI in the condition with speech from -60 degrees azimuth and noise from +60 degrees azimuth (S-60 N+60), the right CI (N-60 S+60), CI1 and CI2. Evaluations took place 1, 2 and median 4 years after sequential implantation. Results No significant squelch effect was found, except for the right CI (N-60 S+60) after 2 years. No differences in speech perception-in-noise from straight ahead were seen between CI1 and CI2. Comparing performance of participants whose better or worse ear was implanted first did not reveal differences either. For the best performing situation, 7/16, 6/16, and 3/12 participants exhibited a squelch effect after 1, 2, and 4 years of follow-up. Conclusions Participants who underwent sequential bilateral cochlear implantation with a 2-year interimplant interval did not develop an evident squelch effect on group level after a median follow-up of 4 years. Individual participants were able to make use of the squelch effect. The less evident squelch effect is at odds with our group of simultaneously implanted bilateral cochlear implant users. Neither a difference between CI1 and CI2, nor implanting the better or worse ear first could explain the less evident squelch effect in these patients. Trial registration Dutch Trial Register NTR1722.

Background Tinnitus is a symptom defined as the perception of sound in the absence of an external source. In England alone there are an estimated ¾ million general practice consultations every year where the primary complaint is tinnitus, equating to a major burden on healthcare services. Clinical management strategies include education and advice, relaxation therapy, tinnitus retraining therapy, cognitive behavioural therapy, sound enrichment using ear-level sound generators or hearing aids, and drug therapies to manage co-morbid symptoms such as sleep difficulties, anxiety or depression. As yet, no drug has been approved for tinnitus by a regulatory body. Nonetheless, over 100,000 prescriptions for betahistine are being filled every month in England, and nearly 10% of general practitioners prescribe betahistine for tinnitus. Objectives To assess the effects of betahistine in patients with subjective idiopathic tinnitus. Search methods The Cochrane ENT Information Specialist searched the Cochrane ENT Register; Central Register of Controlled Trials (CENTRAL, via the Cochrane Register of Studies); Ovid MEDLINE; Ovid Embase; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The date of the search was 23 July 2018. Selection criteria Randomised controlled trials (RCTs) recruiting patients of any age with acute or chronic subjective idiopathic tinnitus were included. We included studies where the intervention involved betahistine and this was compared to placebo, no intervention or education and information. We included all courses of betahistine, regardless of dose regimens or formulations and for any duration of treatment. Data collection and analysis We used the standard methodological procedures expected by Cochrane. Our primary outcomes included tinnitus loudness and significant adverse effects (upper gastrointestinal discomfort). Our secondary outcomes included tinnitus symptom severity as measured by the global score on a multi-item tinnitus questionnaire, depressive symptoms, symptoms of generalised anxiety, health-related quality of life, other adverse effects (e.g. headache, drowsiness, allergic skin reactions (pruritis, rashes) and exacerbation of tinnitus) and tinnitus intrusiveness. We used GRADE to assess the quality of evidence for each outcome; this is indicated in italics. Main results This review included five studies (with a total of 303 to 305 participants) comparing the effects of betahistine with placebo in adults with subjective idiopathic tinnitus. Four studies were parallel-group RCTs and one had a cross-over design. The risk of bias was unclear in all of the included studies.Due to heterogeneity in the outcomes measured and measurement methods used, very limited data pooling was possible. When we pooled the data from two studies for the primary outcome tinnitus loudness, the mean difference on a 0- to 10-point visual analogue scale at one-month follow-up was not significant between betahistine and placebo (-0.16, 95% confidence interval (CI) -1.01 to 0.70; 81 participants) (very low-quality evidence). There were no reports of upper gastrointestinal discomfort (significant adverse effect) in any study.As a secondary outcome, one study found no difference in the change in the Tinnitus Severity Index between betahistine and placebo (mean difference at 12 weeks 0.02, 95% CI -1.05 to 1.09; 50 participants) (moderate-quality evidence). None of the studies reported the other secondary outcomes of changes in depressive symptoms or depression, anxiety symptoms or generalised anxiety, or health-related quality of life as measured by a validated instrument, nor tinnitus intrusiveness.Other adverse effects that were reported were not treatment-related. Authors' conclusions There is an absence of evidence to suggest that betahistine has an effect on subjective idiopathic tinnitus when compared to placebo. The evidence suggests that betahistine is generally well tolerated with a similar risk of adverse effects to placebo treatments. The quality of evidence for the reported outcomes, using GRADE, ranged from moderate to very low.If future research into the effectiveness of betahistine in patients with tinnitus is felt to be warranted, it should use rigorous methodology. Randomisation and blinding should be of the highest quality, given the subjective nature of tinnitus and the strong likelihood of a placebo response. The CONSORT statement should be used in the design and reporting of future studies. We also recommend the development of validated, patient-centred outcome measures for research in the field of tinnitus.