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Purpose: The goal of this study was to evaluate if bilaterally (partially) absent vestibular function during static sound localization testing, would have a negative impact on sound localization skills. Therefore, this study compared horizontal static sound localization skills of normal-hearing patients with bilateral vestibulopathy (BV) and healthy controls., Methods: Thirteen normal-hearing patients with BV and thirteen age-matched healthy controls were included. Sound localization skills were tested using seven loudspeakers in a frontal semicircle, ranging from - 90° to + 90°. Sound location accuracy was analyzed using the root-mean-square error (RMSE) and the mean absolute error (MAE). To evaluate the severity of the BV symptoms, the following questionnaires were used: Dizziness Handicap Inventory (DHI), Oscillopsia severity questionnaire (OSQ), 12-item Spatial, Speech, and Qualities Questionnaire (SSQ12), and Health Utilities Index Mark 3 (HUI3)., Results: The RMSE and MAE were significantly larger (worse) in the BV group than in the healthy control group, with respective median RMSE of 4.6° and 0°, and a median MAE of 0.7° and 0°. The subjective reporting of speech perception, spatial hearing, and quality of life only demonstrated a moderate correlation between DHI (positive correlation) and HUI total score (negative correlation), and localization scores., Conclusion: Static sound localization skills of patients with BV were only mildly worse compared to healthy controls. However, this difference was very small and therefore most likely due to impaired cognitive function. The vestibular system does not seem to have a modulating role in sound localization during static conditions, and its impact is negligible in contrast to the impact of hearing impairment. Furthermore, the subjective reporting of speech perception, spatial hearing, and quality of life was not strongly correlated with localization scores., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To study the evolution of type D personality traits in older adults after cochlear implantation compared with a control group of severely hearing impaired older adults who did not receive a cochlear implant (CI). The influence of COVID-19 on this evolution was also explored. Type D personality combines a high degree of negative affectivity (NA) and social inhibition (SI)., Methods: In this prospective, longitudinal, controlled multicenter exploratory study, 76 older CI users and 21 severely hearing impaired controls without CI were included. The CI group and the control group did not differ significantly regarding age, formal education, residual hearing, Type D Scale-14 (DS14) total score, NA, and SI at baseline. Type D personality traits were assessed with the DS14 at baseline (T0) and 14 months later (T14)., Results: Type D personality traits differed significantly over time between the CI group and the control group (p < 0.001). In the CI group, the DS14 total score (mean delta T = -6.63; p < 0.001), NA (mean delta T = -3.26; p < 0.001), and SI (mean delta T = -3.37; p < 0.001) improved significantly over time (delta T = T14-T0), whereas no significant difference was found in the control group. Significantly fewer subjects were categorized as type D personalities in the CI group (delta T = -12; p = 0.023) at T14, whereas no significant change was found in the control group (delta T = 3; p = 0.250). COVID-19 did not influence the evolution of type D personality traits significantly in the CI group., Conclusion: Cochlear implantation has a positive effect on type D personality traits in older adults with a severe-to-profound hearing impairment., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Background: The aim of this study was to find out how candidacy criteria have evolved differently across the globe., Methods: Candidacy criteria and outcome measurements applied in 19 HEARRING clinics were analyzed., Results: Candidacy criteria vary between clinics. Overall, both bilateral implantation and cochlear implantation in patients with single-sided deafness are becoming more frequent., Conclusion: Standardized outcome measurement instruments need to be applied to provide access to the hearing world to all patients with hearing loss who would benefit from cochlear implantation.

Objective: To compare the cognitive evolution of older adults with severe or profound hearing impairment after cochlear implantation with that of a matched group of older adults with severe hearing impairment who do not receive a cochlear implant (CI)., Design: In this prospective, longitudinal, controlled, and multicenter study, 24 older CI users were included in the intervention group and 24 adults without a CI in the control group. The control group matched the intervention group in terms of gender, age, formal education, cognitive functioning, and residual hearing. Assessments were made at baseline and 14 months later. Primary outcome measurements included the change in the total score on the Repeatable Battery for the Assessment of Neuropsychological Status for Hearing impaired individuals score and on its subdomain score to assess cognitive evolution in both groups. Secondary outcome measurements included self-reported changes in sound quality (Hearing Implant Sound Quality Index), self-perceived hearing disability (Speech, Spatial, and Qualities of Hearing Scale), states of anxiety and depression (Hospital Anxiety and Depression Scale), and level of negative affectivity and social inhibition (Type D questionnaire)., Results: Improvements of the overall cognitive functioning (p = 0.05) and the subdomain "Attention" (p = 0.02) were observed after cochlear implantation in the intervention group; their scores were compared to the corresponding scores in the control group. Significant positive effects of cochlear implantation on sound quality and self-perceived hearing outcomes were found in the intervention group. Notably, 20% fewer traits of Type D personalities were measured in the intervention group after cochlear implantation. In the control group, traits of Type D personalities increased by 13%., Conclusion: Intervention with a CI improved cognitive functioning (domain Attention in particular) in older adults with severe hearing impairment compared to that of the matched controls with hearing impairment without a CI. However, older CI users did not, in terms of cognition, bridge the performance gap with adults with normal hearing after 1 year of CI use. The fact that experienced, older CI users still present subnormal cognitive functioning may highlight the need for additional cognitive rehabilitation in the long term after implantation., (Copyright © 2020 The Authors. Ear & Hearing is published on behalf of the American Auditory Society, by Wolters Kluwer Health, Inc.)

The purpose of this study was to obtain a better understanding of the number and distribution of spiral ganglion cell bodies (SGCB) in the central modiolus trunk of the human cochlea with normal hearing as well as with hearing loss due to various pathological conditions. A literature review was performed using the key words 'human spiral ganglion cell population', 'analysis of spiral ganglion cell population', 'survival of human spiral ganglion cells', 'human Rosenthal's canal', 'human ganglion cell counts', and 'distribution of human spiral ganglion cells' to identify articles published between 1968 and 2018. Articles were included if the number of SGCB in the four segments of the human cochlea and angular depth distribution of the SGCB were stated. Of 236 articles initially identified, 19 articles met the inclusion criteria. SGCB inside the Rosenthal's canal (RC) in the modiolus trunk extended to an angular depth of 630-680° which is near the end of the second turn of the cochlea. SGCBs in Segment IV of the cochlea account for approximately 25-30% of the entire SGCB population irrespective of the cochlear condition (normal vs. pathologic). In normal hearing subjects, the total number of SGCB ranged between 23,910 and 33,702 and in patients with hearing loss between 5733 and 28,220. This literature review elaborates on the current state of knowledge about the number and distribution of SGCB in the human cochlea.

This study aims to obtain a better understanding of the number and distribution of spiral ganglion cell bodies (SGCBs) in the central modiolar trunk of the human cochlea with normal hearing as well as with hearing loss due to various pathological conditions. A detailed PubMed search was performed using the key words "human spiral ganglion cell population," "analysis of spiral ganglion cell population," "survival of human spiral ganglion cells," "human Rosenthal's canal," "human ganglion cell counts," and "distribution of human spiral ganglion cells" to identify articles published between 1931 and 2019. The articles were included if the number of SGCBs in the four segments of the human cochlea and angular depth distribution of the SGCBs were mentioned. Out of the 237 articles that were initially identified, 20 articles met the inclusion criteria. The presence of SGCBs inside the Rosenthal's canal (RC) in the modiolar trunk extended to an angular depth of 630°-680°, which is close to the end of the second turn of the cochlea. SGCBs in Segment-IV of the cochlea account for approximately 25-30% of the entire SGCB population, regardless of the cochlear condition (normal vs. pathologic). In normal-hearing subjects, the total number of SGCB cases ranged between 23,910 and 33,702; in patients with hearing loss, the same was between 5,733 and 28,220. This literature review elaborates on the current state of knowledge regarding the number and distribution of SGCBs in the human cochlea.

There are 466 million people living with a disabling hearing loss and the challenges of managing this public health crisis cannot be underestimated. Yet, adult utilization of cochlear implants is poor with less than 10% of suitable candidates receiving one. The aim of this study was to investigate the awareness levels regarding cochlear implants in older adults after a digital campaign to raise awareness of cochlear implantation in this population. To address the lack of awareness of the cochlear implants in the general population, adverts were placed in online medical magazines and mainstream newspapers. Data were collected in 400 subjects via an online market research questionnaire, in Germany, Austria, Sweden, and the United Kingdom, and compared with baseline data collected in a previous study. Median click rates were in line with expectations for the medical industry and approximately 22 000 individuals clicked through to the cochlear implant Web site. However, there were few significant differences between the 2 sets of data. The Internet was consulted as much as the doctor for medical information in Germany, Austria, and Sweden. The study reinforces the importance of the Internet in accessing information about health, including hearing loss. The click through rates shows that there is interest in learning about cochlear implants. Further work is needed to assess the impact of this type of campaign on individuals who have already been identified as hearing impaired.

Objective: To evaluate the hearing outcomes of cochlear implantation in different age groups by using data collected in the HEARRING registry., Methods: A multicenter study. Data of 146 patients were collected in a HEARRING registry. Patients were divided into three different age groups; ≤ 55 years old (age group 1, n = 66), 56 to 69 years old (age group 2, n = 45), and ≥ 70 years old (age group 3, n = 35). Speech in quiet (SPIQ), speech in noise (SPIN), and hearing implant sound quality index (HISQUI19) scores were evaluated for the different age groups at different test moments (preoperatively, 3, 6, 12, and 24 mo after first fitting)., Results: A statistically significant difference (p < 0.01) was found between preoperative scores and the scores on all the follow-up moments across all age groups. For SPIQ and SPIN, none of the time points showed a statistically significant age effect (p = 0.88 and p = 0.89). For HISQUI19 scores, a statistically significant age effect was found at 12 months after first fitting. The oldest age group scored significantly lower on the HISQUI19 compared with the youngest age group., Conclusion: Hearing outcomes of adult cochlear implant users of different age groups were evaluated. The SPIQ and SPIN tests showed no significant differences between the different age groups. Nevertheless, the youngest group scored significantly better on self -perceived benefit (HISQUI19) with a cochlear implant compared with the oldest age group.Further research is needed to receive more insight into cochlear implantation in the elderly and its implications on rehabilitating and supporting this expanding older population.

Objective: To evaluate cognitive change in severely hearing-impaired older adults after cochlear implantation., Study Design: Prospective, longitudinal cohort study with assessments before, and at 6 and 12 months after implantation., Patients: Twenty older adults (median age: 71.5 yr)., Main Outcome Measures: Change in the Repeatable Battery for the Assessment of Neuropsychological Status for Hearing-impaired individuals (RBANS-H) total score and subdomain scores were used to assess cognitive evolution. In addition, change in best-aided speech audiometry in quiet (monosyllabic words) and in noise (Leuven Intelligibility Sentences Test [LIST]) was examined, as well as patient-reported measures of health-related quality of life (Nijmegen Cochlear Implant Questionnaire [NCIQ]), self-perceived hearing disability (Speech, Spatial, and Qualities of hearing Scale-12 [SSQ12]), sound quality (Hearing Implant Sound Quality Index-19 [HISQUI19]), and states of anxiety and depression (Hospital Anxiety and Depression Scale [HADS])., Results: The RBANS-H total scores improved significantly after 12 months cochlear implant (CI) usage (p < 0.001). At subdomain level, significant improvements were observed in the immediate and delayed memory domain (p = 0.005 and p = 0.002, respectively), and to a lesser extent also in the attention domain (p = 0.047). Furthermore, speech perception in quiet and in noise improved significantly after 6 months and remained stable after 12 months. Similarly, a significant improvement was observed on all patient-reported measures after 6 months of CI usage. These results remained stable after 12 months, except for the HADS., Conclusions: A significant improvement in overall cognition after 12 months of CI usage was established. However, future research is imperative to further disentangle possible practice effects from the effects of the cochlear implantation. The significant, positive effect of cochlear implantation on speech perception and patient-reported measures was confirmed.

Purpose: Postoperative cognitive dysfunction (PCD) is a subtle, prolonged deterioration in cognition after surgery. This complication has been frequently investigated, mainly after major (cardiac) surgery. However, the incidence after cochlear implantation is unknown. Therefore, the aim of the study was to investigate the incidence and possible risk factors of PCD in severely hearing-impaired older adults after cochlear implantation., Methods: In a prospective cohort study, 26 older participants (age: M = 70, SD = 8 years), scheduled for cochlear implantation, were assessed prior to and 1 week after implantation by means of the Montreal Cognitive Assessment (MoCA). The incidence of PCD was calculated. In addition, the following possible risk factors were recorded: age, sex, education, duration of hearing impairment, preoperative signs of depression and anxiety, duration of anesthesia, anesthetic and surgical events and postoperative complications., Results: The incidence of PCD was 11.5%, defined by a Z score of change in MoCA scores ≥ 1.96 (i.e., a decrease of ≥ 4 points). The incidence of PCD was corrected for practice effects by incorporating data from a reference group. Besides an effect of age on the postoperative cognitive performance, no significant risk factors were identified., Conclusions: The incidence of PCD after cochlear implantation is lower than after major surgeries, but higher than after other minor surgeries. Routine cognitive screening before and after cochlear implantation is recommended to identify patients with PCD and to provide additional care for these patients.

Objective: The main goal of this study was to determine the knowledge and beliefs of otorhinolaryngologists in a secondary setting in selected economically advanced European countries concerning severe hearing loss, hearing aids, and cochlear implants (CIs). Secondary goals of the study looked into the information sources of the otorhinolaryngologists and into key topics of importance for these professionals. In Europe, many adults, who could benefit from a CI, do not have one despite their availability via national health care systems. This lack of coverage might be due, in part, to the knowledge and beliefs of otorhinolaryngologists., Methods: Otorhinolaryngologists in a secondary setting in Germany, England, France, Austria, and Sweden were emailed a custom-made questionnaire on their knowledge and beliefs regarding hearing loss and its treatments. Results were presented in relative frequencies (%) according to each nation., Results: Two-hundred and forty otorhinolaryngologists responded (50 from each nation except Sweden). Each nation regarded rehabilitation and hearing preservation as particularly important in CIs. National and international conferences and conversations with colleagues were much more popular methods of keeping abreast of medical issues than other information sources such as online media or company information., Conclusion: The otorhinolaryngologists of the surveyed nations share many common beliefs about hearing loss, hearing aids, and CIs, although some national variation in opinion is present. The otorhinolaryngologists of each nation are knowledgeable, but could still benefit from an increased knowledge and awareness of hearing loss treatment modalities.

Objectives: To provide multidisciplinary cochlear implant teams with a current consensus statement to support hearing preservation cochlear implantation (HPCI) in children, including those children with symptomatic partial deafness (PD) where the intention is to use electric-acoustic stimulation (EAS). The main objectives are to provide guidelines on who is a candidate, how to assess these children and when to implant if Med-El Flex electrode arrays are chosen for implantation., Methods: The HEARRING group reviewed the current evidence and practice regarding the management of children to be considered for HPCI surgery emphasizing the assessment needed prior to implantation in order to demonstrate the benefits in these children over time. The consensus statement addresses following three key questions: (1) Should these children be treated? (2) How to identify these children? (3) How to manage these children?, Summary: The HEARRING group concludes that irrespective of the degree of residual hearing present, the concepts of hearing and structure preservation should be applied in every child undergoing cochlear implantation and that HPCI is a safe and reliable treatment option. Early detection and multidisciplinary assessment are key to the identification of children with symptomatic PD, these children should undergo HPCI as early as possible.

The objectives of this study were to assess the factors which contribute to individuals' health motivation to address hearing loss and to gather baseline data that could then be used to measure the impact of an awareness campaign. An online questionnaire with 13 closed set questions was completed by 100 subjects in each country including Austria, Germany, France, Sweden, and the United Kingdom. The questionnaire was based around the Health Belief Model, which describes how, in order to take action to address a medical problem, the individual must perceive that the condition presents a threat to their well-being that exceeds any barriers to treatment. Good hearing was regarded as being important in all countries. There was agreement that the main sign of hearing loss was turning up the TV or radio. In most countries, hearing aids were thought to be not particularly visible, not require much maintenance, a hindrance while doing sport, and must be removed before bed. Perceptions of hearing implants were that they were permanently fitted, not externally visible, and do not need to be removed before bed. Medical issues were mostly researched through a doctor and then via the Internet. In this sample, there was a good understanding of the consequences and signs of hearing loss, but although hearing implants were viewed as being different to hearing aids, there was little understanding that the external speech processor was similar to a hearing aid in its physical characteristics. When actions were taken, the key professionals consulted about hearing problems were the general practitioner and ear, nose, and throat specialist.

One way to provide pitch information to cochlear implant users is through amplitude-modulation rate. It is currently unknown whether amplitude-modulation rate can provide cochlear implant users with pitch information adequate for perceiving melodic information. In the present study, the notes of a song were encoded via amplitude-modulation rate of pulse trains on single electrodes at the apex or middle of long electrode arrays. The melody of the song was either physically correct or modified by compression or expansion. Nine cochlear implant users rated the extent to which the song was out of tune in the different conditions. Cochlear implant users on average did not show sensitivity to melody compression or expansion regardless of place of stimulation. These results were found despite the fact that three of the cochlear implant users showed the expected sensitivity to melody compression and expansion with the same task using acoustic pure tones in a contralateral acoustic ear. Normal-hearing listeners showed an inconsistent and weak effect of melody compression and expansion when the notes of the song were encoded with acoustic pulse rate. The results suggest that amplitude-modulation rate provides insufficient access to melodic information for cochlear-implant and normal-hearing listeners.

Previous positron emission tomography (PET) studies have shown that various cortical areas are activated to process speech signal in cochlear implant (CI) users. Nonetheless, differences in task dimension among studies and low statistical power preclude from understanding sound processing mechanism in CI users. Hence, we performed activation likelihood estimation meta-analysis of PET studies in CI users and normal hearing (NH) controls to compare the two groups. Eight studies (58 CI subjects/92 peak coordinates; 45 NH subjects/40 peak coordinates) were included and analyzed, retrieving areas significantly activated by lexical and nonlexical stimuli. For lexical and nonlexical stimuli, both groups showed activations in the components of the dual-stream model such as bilateral superior temporal gyrus/sulcus, middle temporal gyrus, left posterior inferior frontal gyrus, and left insula. However, CI users displayed additional unique activation patterns by lexical and nonlexical stimuli. That is, for the lexical stimuli, significant activations were observed in areas comprising salience network (SN), also known as the intrinsic alertness network, such as the left dorsal anterior cingulate cortex (dACC), left insula, and right supplementary motor area in the CI user group. Also, for the nonlexical stimuli, CI users activated areas comprising SN such as the right insula and left dACC. Previous episodic observations on lexical stimuli processing using the dual auditory stream in CI users were reconfirmed in this study. However, this study also suggests that dual-stream auditory processing in CI users may need supports from the SN. In other words, CI users need to pay extra attention to cope with degraded auditory signal provided by the implant., (© 2015 Wiley Periodicals, Inc.)

Conclusion: The results show that the DUET2 offers users speech perception that is equivalent to or better than the DUET. Moreover, the DUET2 offers subjective benefits above those provided by the DUET., Background: The DUET is a combination of hearing aid and CI in one device for electric acoustic stimulation. Since its introduction: a second generation, the DUET2, has been developed. This study aimed to investigate the benefits of the DUET2 compared to the DUET., Methods: Speech reception was determined in quiet and in noise. The sound quality of speech and music was rated using a visual analogue scale. Test intervals were at upgrade and at 3 and 6 months after upgrade., Results: Speech reception in quiet and in noise was significantly better than with the DUET after 6 months. For sentence reception in quiet, the SRT with the DUET2 did not change significantly between test intervals. Sentence reception in noise with the DUET2 improved significantly between 3 and 6 months and upgrade and 6 months. After 6 months, speech reception in quiet and in noise with the DUET2 was significantly better than with the DUET. Subjects rated the sound quality of speech and of music with the DUET2 significantly better than with the DUET.

We performed a genome-wide association study (GWAS) to identify the genes responsible for age-related hearing impairment (ARHI), the most common form of hearing impairment in the elderly. Analysis of common variants, with and without adjustment for stratification and environmental covariates, rare variants and interactions, as well as gene-set enrichment analysis, showed no variants with genome-wide significance. No evidence for replication of any previously reported genes was found. A study of the genetic architecture indicates for the first time that ARHI is highly polygenic in nature, with probably no major genes involved. The phenotype depends on the aggregated effect of a large number of SNPs, of which the individual effects are undetectable in a modestly powered GWAS. We estimated that 22% of the variance in our data set can be explained by the collective effect of all genotyped SNPs. A score analysis showed a modest enrichment in causative SNPs among the SNPs with a P-value below 0.01.

Background: Hearing preservation (HP) surgery was initiated more than 10 years ago for combined electric and acoustic stimulation (EAS). Preserved residual low-frequency hearing has been demonstrated to improve speech perception in noise as well as music appreciation in EAS users up to 2 years. Multiple study groups aimed to evaluate initial loss of residual hearing (RH) as a consequence of HP surgery. However, after 1 year and 2 years of follow-up, further decline was reported. This study aimed to determine RH, speech perception, and the subjective benefits of EAS 10 years after HP surgery., Subjects and Methods: Nine postlingual EAS partially deaf patients who underwent HP surgery at Antwerp University Hospital were included in this study (11 implanted ears). Hearing preservation (0% = loss of hearing; >0%-25% = minimal HP; >25%-75% = partial HP; >75% = complete HP), speech perception and subjective benefits were evaluated preoperatively; at 3, 6, 12, 18, and 24 months postoperatively; and annually thereafter., Results: Complete HP was obtained in three of 11 ears; partial HP in five of 11 ears; and minimal HP in two of 11 ears, measured during their most recent follow-up. One subject lost his RH completely across time. The mean rate of HP was 48% (ranging from 6 months up to 10 years postoperatively). Speech perception analysis up to 10 years showed a continuous statistically significant improvement. The maximum subjective benefit was reached 3 months after implantation and subsequently remained statistically significant unchanged for the next 10 years., Conclusion: Long-term HP in EAS users after HP surgery is feasible, although a small continuous decline of HP rate of 3% per year was observed (measured from first fitting up to 6 years postoperative). Nevertheless, a continuous improvement was found in the speech perception results of the EAS users across 10 years. Moreover, the positive subjective benefit, assessed 3 months postoperative, remained stable up to 10 years.

Background: Patients visiting the department of Otorhinolaryngology-Head and Neck Surgery often recite tinnitus as the primary complaint. Frequently, tinnitus is accompanied by other symptoms such as hyperacusis and hearing loss. The present study is a cross-sectional study analyzing the relationships between tinnitus, hearing loss, and hyperacusis by the use of the audiologic measurements performed in a clinical practice., Methods: All patients visiting the Otorhinolaryngology-Head and Neck Surgery department of the University Hospital Antwerp with complaints of tinnitus (sometimes accompanied by hyperacusis) during the year of 2012, were looked up (n = 588). All patients underwent audiometry and filled out the Tinnitus Questionnaire (TQ) and Hyperacusis Questionnaire (HQ), and tinnitus analysis was performed. The relationships between all measurements were examined by use of correlations, multinomial logistic regression analyses, and descriptive statistics., Results: Scores on the TQ and HQ were significantly positively correlated (r = 0.5, p < 0.001). In general, patients with a combination of tinnitus and hyperacusis showed significantly higher TQ scores (p < 0.001) and higher ratings on the VAS for loudness and distress (p < 0.001) compared with tinnitus patients without hyperacusis. Furthermore, an age-dependent influence on the audiometric configuration and tinnitus type was found., Discussion: Tinnitus and hyperacusis are two frequent symptoms recited at a consultation. The present study found that patients with a high TQ grade more often also perceive hyperacusis compared with patients with a low TQ grade., Conclusions: Tinnitus and hyperacusis are two frequent symptoms recited at a consultation. We have to point out that also other issues, such as additional health problems and stress, may influence tinnitus severity. In addition, tinnitus type seems to be age dependent as younger patients more often experience a pure tone tinnitus and older patients more often experience a noise-like tinnitus.

Introduction: According to current diagnostic criteria, patients exhibiting only cochlear symptoms without vertigo cannot be diagnosed with Ménière's disease (MD). The recently reported Ménière's Disease Index (MDI) combines audiometric and electrocochleographic parameters in a multidimensional measure correlating with the clinical degrees of MD., Materials and Methods: Twenty-one patients with hearing loss, tinnitus and aural fullness, but without vertigo, underwent transtympanic electrocochleography. Based on the previously reported formula, the MDI value for every patient was calculated., Results: Mean MDI value was 5.7 on a scale from 0 (mean value of non-MD patients) to 10 (mean value of definite MD patients)., Conclusions: We can thus hypothesize that, based on audiometry and electrocochleography, 'cochlear MD' patients may represent a separate clinical entity with Ménière-like pathophysiology or underlying endolymphatic hydrops., (Copyright © 2012 S. Karger AG, Basel.)

Objective: To update a 15-year-old study of 800 postlinguistically deaf adult patients showing how duration of severe to profound hearing loss, age at cochlear implantation (CI), age at onset of severe to profound hearing loss, etiology and CI experience affected CI outcome., Study Design: Retrospective multicenter study., Methods: Data from 2251 adult patients implanted since 2003 in 15 international centers were collected and speech scores in quiet were converted to percentile ranks to remove differences between centers., Results: The negative effect of long duration of severe to profound hearing loss was less important in the new data than in 1996; the effects of age at CI and age at onset of severe to profound hearing loss were delayed until older ages; etiology had a smaller effect, and the effect of CI experience was greater with a steeper learning curve. Patients with longer durations of severe to profound hearing loss were less likely to improve with CI experience than patients with shorter duration of severe to profound hearing loss., Conclusions: The factors that were relevant in 1996 were still relevant in 2011, although their relative importance had changed. Relaxed patient selection criteria, improved clinical management of hearing loss, modifications of surgical practice, and improved devices may explain the differences., (Copyright © 2012 S. Karger AG, Basel.)

Objectives: : The goals of the present study were twofold: in the first part, the prevalence and profile of hearing disability in healthy, middle-aged persons were determined by the speech, spatial, and qualities of hearing scale (SSQ). In the second part of this study, the number of SSQ items was reduced to five to make this questionnaire available for routine usage in clinical settings and for screening purposes., Methods: : SSQ responses derived from 103 normal-hearing 18- to 25-year-old persons were compared with the SSQ responses of 24 clinically normal-hearing (all thresholds between 125 and 8000 Hz ≤25 dB HL) and 109 healthy, 55- to 65-year-old persons with age-related hearing impairment to determine the prevalence and profile of hearing disability. The 45 items of the SSQ were reduced to five by cluster analyses and binary logistic regression analyses. The robustness of this five-item version (SSQ5) was determined in three control populations: an adult 25- to 55-year-old population (n = 159), an ENT-patient population (n = 60), and a population of hearing aid candidates (n = 50). The feasibility of the SSQ5 for screening was compared with the feasibility of the simple question "Do you have hearing loss?" by determining, respectively, the sensitivity, specificity, and maximum achievable discriminatory power for predicting hearing status according to speech-in-noise performance., Results: : Prevalence numbers showed data of healthy, middle-aged persons with significant disability, despite minimal impairment (25%) versus data of middle-aged persons with significant impairment and nevertheless, minimal disability (61%). The profile of hearing disability seemed similar in all normal-hearing and hearing-impaired subgroups (i.e., most problems with understanding speech especially in noise conditions, and least problems with sound quality). Compared with the single question: "Do you have hearing loss?" the use of the SSQ5 had 37% more maximum discriminatory power for determining hearing status category based on speech-in-noise performance in 55- to 65-year-old persons. In addition, the SSQ5 seemed robust in adult populations of different ages (89.6% correlation between the answers of the SSQ5 and SSQ45), as well as in ENT-patient populations (93.7% correlation) and hearing aid candidate populations (79.2% correlation)., Conclusions: : The results of this study suggest that disability measures and measures for hearing impairment cannot replace each other, but are complementary. Therefore, it is advised to implement both disability measures and impairment measures in screening and referral policies for hearing loss. To get a first impression of hearing disability, our results suggest that it is useful to ask five disability questions (SSQ5) instead of one general question like "Do you have hearing loss?"

Objective: Otitis media with effusion (OME) is the major reason for failure of neonatal hearing screening. However, little is known about the impact on hearing status of OME in infants during the first months of life., Patients: Infants who failed universal newborn hearing screening., Methods: Prospective evaluation for the presence of OME, the degree of hearing loss and the disease evolution., Results: Between 2007 and 2008, approximately 152 infants were referred because of unilateral or bilateral failure on universal newborn hearing screening with an automated auditory brainstem response device. Eighty-four (55.3%) had OME, 20 of them were lost to follow-up. The remainder 64 infants comprised 37 boys and 27 girls, with a median age of 49 days (range, 40-65 d) at admission. Auditory brainstem response thresholds were 50 dB nHL (range, 40-60) in both ears. A spontaneous resolution of OME was documented in 15 infants. In the remaining, hearing normalized after tympanocentesis or placement of ventilation tubes. Normal hearing could be ascertained in all children at a median age of 4.8 months (range, 3.3-7.8 mo). In the group of infants analyzed, no permanent hearing loss could be detected., Conclusion: OME is an important cause of transient, moderately severe hearing loss during the first months of life--a critical period for development of the auditory system. Active treatment should be considered if spontaneous resolution does not occur to prevent any delay in language acquisition and to exclude an underlying sensorineural hearing loss.

Hearing preservation after cochlear implantation and combined electric acoustic stimulation (EAS) in the same ear has reduced the gap between prosthetic hearing rehabilitation with hearing aids and cochlear implants. This has increased the possibility of successful hearing rehabilitation for patients. This paper describes the history of hearing preservation after cochlear implantation. Indications and criteria for combined electric acoustic stimulation are described, and hearing preservation surgery and outcomes achieved with combined EAS are discussed. EAS has led to a new era in prosthetic hearing rehabilitation providing new understanding of basic physiological, psychoacoustic and medical aspects of the inner ear., (Copyright 2010 S. Karger AG, Basel.)

Aims: This study compared the music perception abilities of 13 electric acoustic stimulation (EAS) users with two control groups: unilateral cochlear implant (CI) users and normal-hearing (NH) listeners., Methods: Groups were matched according to age and musical experience before hearing loss (HL) and tested using the Musical Sounds in Cochlear Implants (Mu.S.I.C.) test., Results: No difference was found on rhythm perception, chord discrimination, dissonance rating, and emotion rating subtest performance between groups. Mean frequency discrimination scores were significantly better in EAS participants than in CI participants and not significantly worse than in NH participants. However, the EAS and CI groups scored similarly (significantly worse than NH participants) on both instrument detection and identification. Results for EAS participants were not significantly worse when the hearing aid component was removed. Frequency of listening to music before HL was negatively correlated with EAS participants' frequency discrimination scores, though singing and playing an instrument appeared to have no effect. EAS participants who indicated many reasons for listening to music and who listen to many genres after implantation scored higher on instrument detection and instrument identification. Better results on these two subtests were correlated with EAS participants' better postoperative auditory thresholds at 250 and 500 Hz., Conclusions: Though EAS participants performed better on music perception testing (though not timbre-based tasks) than CI participants, their scores did not reach the level of NH participants. This indicates that acoustic hearing in the low frequencies is helpful for music perception, though not the only important factor., (Copyright 2010 S. Karger AG, Basel.)

A multicenter study was set up to elucidate the environmental and medical risk factors contributing to age-related hearing impairment (ARHI). Nine subsamples, collected by nine audiological centers across Europe, added up to a total of 4,083 subjects between 53 and 67 years. Audiometric data (pure-tone average [PTA]) were collected and the participants filled out a questionnaire on environmental risk factors and medical history. People with a history of disease that could affect hearing were excluded. PTAs were adjusted for age and sex and tested for association with exposure to risk factors. Noise exposure was associated with a significant loss of hearing at high sound frequencies (>1 kHz). Smoking significantly increased high-frequency hearing loss, and the effect was dose-dependent. The effect of smoking remained significant when accounting for cardiovascular disease events. Taller people had better hearing on average with a more pronounced effect at low sound frequencies (<2 kHz). A high body mass index (BMI) correlated with hearing loss across the frequency range tested. Moderate alcohol consumption was inversely correlated with hearing loss. Significant associations were found in the high as well as in the low frequencies. The results suggest that a healthy lifestyle can protect against age-related hearing impairment.

Hearing loss is the most common sensory disorder, affecting 1 in 650 newborns. Linkage analysis revealed linkage to locus DFNA22 in two Belgian families 1 and 2 with autosomal dominant sensorineural hearing loss. As MYO6 has previously been reported as responsible for the hearing loss at loci DFNA22 and DFNB37, respectively, DNA sequencing of the coding region and the promoter of MYO6 was performed but this analysis did not reveal any mutations. However, only in patients of family 2, an insertion of 108 bp was identified in the mRNA of the gene. The inserted fragment was part of intron 23 and sequencing of this intron revealed a new splice-site mutation c.IVS23+2321T>G, segregating with the hearing loss in the family. The mutation causes a frameshift and a premature termination codon, but real-time PCR revealed that only 15-20% of the mRNA is degraded by nonsense-mediated decay, while the other part may give rise to an aberrant protein. In family 1, a quantitative real-time PCR experiment revealed a 1.5-1.8-fold overexpression of MYO6 in patients compared to controls. The possible presence of a gene duplication could be excluded by real-time PCR on genomic level. Most likely, the overexpression is caused by a mutation in an unidentified regulatory region of the gene. This study indicates that the inner ear hair cells are sensitive to changes in expression levels of MYO6.

Hypothesis: The common GJB2 (Connexin 26) 35delG mutation might contribute to the development of age-related hearing impairment (ARHI) and noise-induced hearing loss (NIHL)., Background: GJB2, a gene encoding a gap junction protein expressed in the inner ear, has been suggested to be involved in the potassium recycling pathway in the cochlea. GJB2 mutations account for a large number of individuals with nonsyndromic recessive hearing loss, with 35delG being the most frequent mutation in populations of European origin. Other genes involved in potassium homeostasis have been suggested to be associated with ARHI and NIHL, and distortion product otoacoustic emission distortions indicative of hearing loss alterations have been found in 35delG carriers., Method: We genotyped 35delG in two distinct sample sets: an ARHI sample set, composed of 2,311 Caucasian samples from nine different centers originating from seven different countries with an age range between 53 and 67 years, and an NIHL sample set consisting of 702 samples from the two extremes of a noise-exposed Polish sample., Results: After statistical analysis, we were unable to detect an association between 35delG and ARHI, nor between 35delG and NIHL., Conclusion: Our findings indicate that there is no increased susceptibility in 35delG carriers for the development of ARHI or NIHL.

Objectives: The place of MRI in the diagnostic work-up of patients with bilateral perceptive hearing loss, tinnitus and vertigo is under discussion. The purpose of this study is to investigate the role of MRI in patients with otovestibular and cranial nerve complaints of unknown aetiology., Methodology: After thorough otologic examination, 430 patients were consecutively referred for an MR examination of the cerebellopontine angle., Results: The detection rate for essential lesions was 4.9%. Two groups of retrocochlear lesions were frequently observed: central WMLs/atrophy and neurovascular conflict affecting a cranial nerve., Conclusions: MR imaging of the cerebellopontine angle, fossa posterior and petrous bones makes it possible to observe abnormalities of the vestibulocochlear nerve and inner ear. Additional T2-weighted FSE images of the whole brain make it possible to evaluate the occurrence of early central lesions. This imaging protocol can diagnose essential lesions relating directly to the complaint in 4.9% of the patients with hearing loss, subjective tinnitus or vertigo. We frequently observed two groups of lesions of uncertain significance in our study population. WMLs are present in 50% of patients with a mean age of 59 years. In the younger subpopulation aged under 51 years the prevalence of WMLs is 24%. It remains unclear whether these lesions can be accounted for by the diversity of symptoms with which the patients presented. In addition, we found a high number of neurovascular conflicts involving different cranial nerves.

Objective: Since 1998 an integrated universal newborn hearing screening programme (UNHSP) based on automated auditory brainstem response (AABR) has been implemented in Flanders. The protocol of the UNHSP is based on guidelines defined by the American Academy of Paediatrics (AAP). The aim of this paper is to report on the screening protocol and to assess its feasibility., Methodology: Descriptive study based upon an analysis of the screening results in the neonatal non-NICU population of Flanders between 1999 and 2004. The UNHSP, organized by Kind en Gezin (K&G), uses a 2-stage protocol: children with a refer at the first screening test are retested, and those with a refer at the retest are referred to a certified centre. Screening and referral centres communicate their data to a central database at K&G., Results: From the beginning of 1999 until the end of 2004 a screening was offered to 97.91% of all eligible babies in Flanders; 91.5% of these babies were screened by K&G using the Algo Portable Newborn Screener. Three-quarters of the referred babies had a confirmed hearing loss. In 57.6% of these babies, hearing loss was bilateral. Some babies had a temporary hearing problem. The false positive rate after two tests was 0.53 per thousand. All ascertained babies started early intervention, most of them before the age of 4 months., Conclusions: K&G has succeeded in organizing a new, well-structured community-based UNHSP according to the guidelines of the AAP on Neonatal Hearing Screening.

Age-related hearing impairment (ARHI) is the most common sensory impairment among the elderly. It is a complex disorder influenced by genetic as well as environmental factors. SNPs in a candidate susceptibility gene, KCNQ4, were examined in two independent Caucasian populations. Two quantitative trait locus (QTL) values were investigated: Zhigh and Zlow, a measure of high and respectively low frequency hearing loss. In the first population, the statistical analysis of 23 genotyped SNPs spread across KCNQ4 resulted in significant p-values for two SNPs for Zhigh-SNP9 (NT&#95;004511:g.11244177A > T) and SNP15 (NT&#95;004511:g.11257005C > T; NP&#95;004691:p.Ala259Ala), and one SNP for Zlow-SNP12 (NT&#95;004511:g.11249550A > T). The linkage disequilibrium (LD) structure of KCNQ4 was subsequently determined in a 34-kb region surrounding the significant SNPs, resulting in three LD-blocks. LD-block 1 contains SNP9 and covers an area of 5 kb, LD-block 2 measures 5 kb and surrounds SNP13 (NT&#95;004511:g.11253513A > G) to SNP18 (NT&#95;004511:g.11257509G > A; NP&#95;004691:p.Thr293Thr), and LD-block 3 spans 7 kb. Five tag-SNPs of block 1 and 2, and 2 extra SNPs were subsequently genotyped in the second population. Again, several SNPs were positively associated with ARHI: one SNP (SNP18) for the high frequencies and three SNPs (SNP9, SNP12, and SNP18) for the low frequencies, although only a single SNP (SNP12) resulted in significant p-values in both populations. Nevertheless, the associated SNPs of both populations were all located in the same 13-kb region in the middle of the KCNQ4 gene.

Hearing impairment (HI) affects 1 in 650 newborns, which makes it the most common congenital sensory impairment. Despite extraordinary genetic heterogeneity, mutations in one gene, GJB2, which encodes the connexin 26 protein and is involved in inner ear homeostasis, are found in up to 50% of patients with autosomal recessive nonsyndromic hearing loss. Because of the high frequency of GJB2 mutations, mutation analysis of this gene is widely available as a diagnostic test. In this study, we assessed the association between genotype and degree of hearing loss in persons with HI and biallelic GJB2 mutations. We performed cross-sectional analyses of GJB2 genotype and audiometric data from 1,531 persons, from 16 different countries, with autosomal recessive, mild-to-profound nonsyndromic HI. The median age of all participants was 8 years; 90% of persons were within the age range of 0-26 years. Of the 83 different mutations identified, 47 were classified as nontruncating, and 36 as truncating. A total of 153 different genotypes were found, of which 56 were homozygous truncating (T/T), 30 were homozygous nontruncating (NT/NT), and 67 were compound heterozygous truncating/nontruncating (T/NT). The degree of HI associated with biallelic truncating mutations was significantly more severe than the HI associated with biallelic nontruncating mutations (P<.0001). The HI of 48 different genotypes was less severe than that of 35delG homozygotes. Several common mutations (M34T, V37I, and L90P) were associated with mild-to-moderate HI (median 25-40 dB). Two genotypes--35delG/R143W (median 105 dB) and 35delG/dela(GJB6-D13S1830) (median 108 dB)--had significantly more-severe HI than that of 35delG homozygotes.

Although hereditary hearing loss is a very heterogeneous disorder, variants in one gene, GJB2 (connexin 26), account for up to 50% of autosomal recessive nonsyndromal sensorineural hearing loss in most populations. This study investigates the contribution of GJB2 to autosomal recessive nonsyndromal hearing loss in the Indonesian population. We performed DNA sequence analysis in 120 patients with profound early childhood nonsyndromal hearing loss and in 100 control individuals and identified three novel variations resulting in amino acid substitutions (p.Gly4Asp, p.Thr5Ala, and p.Gly160Arg). Although we proved that p.Gly4Asp was not disease-causing, the pathological nature of p.Thr5Ala and p.Gly160Arg could not be determined. No recurrent disease-causing mutation could be detected in this Indonesian population. These findings are in contrast with the results obtained in other populations where GJB2 is a major cause of congenital recessive hearing loss., (Copyright (c) 2005 Wiley-Liss, Inc.)

For some patients, genetic testing can reveal the etiology of their hearing impairment, and can provide evidence for a medical diagnosis. However, a gap between fundamental genetic research on hereditary deafness and clinical otology emerges because of the steadily increasing number of discovered genes for hereditary hearing impairment (HHI) and the comparably low clinical differentiation of the HHIs. In an attempt to keep up with the scientific progress, this article enumerates the indications of genetic testing for HHI from a clinical point of view and describes the most frequently encountered HHIs in Belgium. Domains of recent scientific interest, molecular biological aspects, and some pitfalls with HHIs are highlighted. The overview comprises bilateral congenital hearing loss, late-onset progressive high frequency hearing loss, progressive bilateral cochleo-vestibular deficit, and progressive low frequency hearing loss. Also, several syndromal forms of HHI are summarized, and the availability of genetic tests mentioned. Finally, the requirements for successful linkage analysis, an important genetic research tool for localizing the potential genes of a trait on a chromosome, are briefly described.

Introduction: Mutations in GJB2 are the most common cause of non-syndromic autosomal recessive hearing impairment, ranging from mild to profound. Mutation analysis of this gene is widely available as a genetic diagnostic test., Objective: To assess a possible genotype-phenotype correlation for GJB2., Design: Retrospective analysis of audiometric data from people with hearing impairment, segregating two GJB2 mutations., Subjects: Two hundred and seventy seven unrelated patients with hearing impairment who were seen at the ENT departments of local and university hospitals from Italy, Belgium, Spain, and the United States, and who harboured bi-allelic GJB2 mutations., Results: We found that 35delG homozygotes have significantly more hearing impairment, compared with 35delG/non-35delG compound heterozygotes. People with two non-35delG mutations have even less hearing impairment. We observed a similar gradient of hearing impairment when we categorised mutations as inactivating (that is, stop mutations or frame shifts) or non-inactivating (that is, missense mutations). We demonstrated that certain mutation combinations (including the combination of 35delG with the missense mutations L90P, V37I, or the splice-site mutation IVS1+1G>A, and the V37I/V37I genotype) are associated with significantly less hearing impairment compared with 35delG homozygous genotypes., Conclusions: This study is the first large systematic analysis indicating that the GJB2 genotype has a major impact on the degree of hearing impairment, and identifying mild genotypes. Furthermore, this study shows that it will be possible to refine this correlation and extend it to additional genotypes. These data will be useful in evaluating habilitation options for people with GJB2 related deafness.

A retrospective analysis of 190 records of hearing impaired children up to the age of 14, all educated at one of three Flemish Institutes for Deaf and Speech Defective Children in Belgium, was performed. Until 1999, the well-known behavioral test (Ewing test) was used in the Flemish national screening program for hearing losses. Because it presented a lot of disadvantages, it has been replaced by Automated Auditory Brainstem Responses (AABR), enabling the Flemish national neonatal screening program to commence. This study is an extension of the preliminary results of a recently performed retrospective analysis in one Flemish institute of the hearing loss of patients that were diagnosed in the pre-AABR era. The authors analyzed the following data: etiology, risk factors of congenital hearing impairment, the patient's history from the moment of the first suspicion to diagnosis and treatment. The median age of the children was 8.5 years (2-14 year). In 66.5% the parents and/or grandparents were the first to raise suspicion, this was at a median age of approximately 9 months. The diagnosis was often made late, at a median age of 15 months (0-88 months). The etiology was unknown in 32.6% of the cases. The only cast-iron certain diagnoses were pre- and perinatal infections, syndromal and genetic hearing loss, and acquired infections (meningitis and measles). No risk factors of hearing loss, as they are stated by the Joint Committee on Infant Hearing, were found in more than 50% of the cases. Audiometry was performed in all cases, often supplemented with Auditory Brainstem Responses and/or click-evoked otoacoustic emissions, while other diagnostic investigations (imaging, genetics, etc.) were only variably performed. Finally, the authors confirm the need for universal neonatal screening, which only recently started in Belgium, and suggest that a detailed protocol should be established to pursue a coherent diagnostic policy.

The relative occurrence of bell-shaped and various types of W-shaped susceptance-conductance and admittance-phase tympanograms at a probe-tone frequency of 660 Hz was determined from registrations on normal ears. The diagnostic value of the susceptance-conductance versus the admittance-phase representation of tympanograms was studied on pathological middle-ear systems. Using probe-tone frequencies from 510 Hz up to 910 Hz, tympanograms for all four imminent components were recorded on 10 pathological ears and the diagnostic value compared. From the combination of these studies we conclude that the admittance-phase approach at a probe tone frequency between 500 and 700 Hz is a good choice.

Background.Microvascular compressions of the cochlear nerve can lead to hearing loss. Due to the tonotopic organization of the cochlear nerve any focal compression of the cochlear nerve will result in a frequency specific hearing loss. Decompressing the cochlear nerve could result in a frequency specific hearing improvement, without improving overall hearing.Method.Thirty one patients underwent microvascular decompression operations of the vestibulocochlear nerve for vertigo or tinnitus. Preoperative audiograms were substracted from postoperative audiograms obtained 2 years after microvascular decompression. The frequencies of maximal hearing improvement postoperatively were determined.Findings.Of the 31 patients studied, 19 had improvements of 5?dB or more at one or more frequencies postoperatively, and 15 patients had improvements of 10?dB or more. Three patients had improvements of 25?dB or more postoperatively. The postoperative hearing improvement was frequency-specific and related to the anatomical location of the vascular contact on the auditory nerve. The improvement of hearing becomes diluted when the difference between pre- and postoperative hearing thresholds are averaged over all audiometric frequencies. We therefore present results for each frequency that was tested.Conclusions. Microvascular decompression of the cochlear nerve can improve hearing in selected patients. The improvement seems too small to justify decompressive surgery for the sole purpose of hearing improvement, but it could be considered if associated short vertigo spells, ipsilateral tinnitus, otalgia and cryptogenic hemifacial spasm are present. Decompression should be performed early, before BAEP changes become noticeable. 3D-MRI could become a valuable tool for selecting good surgical candidates. [ABSTRACT FROM AUTHOR]