Ultrarare heterozygous pathogenic variants of genes causing dominant forms of early-onset deafness underlie severe presbycusis.

Presbycusis, or age-related hearing loss (ARHL), is a major public health issue. About half the phenotypic variance has been attributed to genetic factors. Here, we assessed the contribution to presbycusis of ultrarare pathogenic variants, considered indicative of Mendelian forms. We focused on severe presbycusis without environmental or comorbidity risk factors and studied multiplex family age-related hearing loss (mARHL) and simplex/sporadic age-related hearing loss (sARHL) cases and controls with normal hearing by whole-exome sequencing. Ultrarare variants (allele frequency [AF] < 0.0001) of 35 genes responsible for autosomal dominant early-onset forms of deafness, predicted to be pathogenic, were detected in 25.7% of mARHL and 22.7% of sARHL cases vs. 7.5% of controls (P = 0.001); half were previously unknown (AF < 0.000002). MYO6, MYO7A, PTPRQ, and TECTA variants were present in 8.9% of ARHL cases but less than 1% of controls. Evidence for a causal role of variants in presbycusis was provided by pathogenicity prediction programs, documented haploinsufficiency, three-dimensional structure/function analyses, cell biology experiments, and reported early effects. We also established Tmc1N321I/+ mice, carrying the TMC1:p.(Asn327Ile) variant detected in an mARHL case, as a mouse model for a monogenic form of presbycusis. Deafness gene variants can thus result in a continuum of auditory phenotypes. Our findings demonstrate that the genetics of presbycusis is shaped by not only well-studied polygenic risk factors of small effect size revealed by common variants but also, ultrarare variants likely resulting in monogenic forms, thereby paving the way for treatment with emerging inner ear gene therapy.

Cochlear Implantation and Other Treatments in Single-Sided Deafness and Asymmetric Hearing Loss: Results of a National Multicenter Study Including a Randomized Controlled Trial.

INTRODUCTION: Cochlear implantation is a recent approach proposed to treat single-sided deafness (SSD) and asymmetric hearing loss (AHL). Several cohort studies showed its effectiveness on tinnitus and variable results on binaural hearing. The main objective of this study is to assess the outcomes of cochlear implantation and other treatment options in SSD/AHL on quality of life. METHODS: This prospective multicenter study was conducted in 7 tertiary university hospitals and included an observational cohort study of SSD/AHL adult patients treated using contralateral routing of the signal (CROS) hearing aids or bone-anchored hearing systems (BAHSs) or who declined all treatments, and a randomized controlled trial in subjects treated by cochlear implantation, after failure of CROS and BAHS trials. In total, 155 subjects with SSD or AHL, with or without associated tinnitus, were enrolled. After 2 consecutive trials with CROS hearing aids and BAHSs on headband, all subjects chose any of the 4 treatment options (abstention, CROS, BAHS, or cochlear implant [CI]). The subjects who opted for a CI were randomized between 2 arms (CI vs. initial observation). Six months after the treatment choice, quality of life was assessed using both generic (EuroQoL-5D, EQ-5D) and auditory-specific quality-of-life indices (Nijmegen Cochlear implant Questionnaire [NCIQ] and Visual Analogue Scale [VAS] for tinnitus severity). Performances for speech-in-noise recognition and localization were measured as secondary outcomes. RESULTS: CROS was chosen by 75 subjects, while 51 opted for cochlear implantation, 18 for BAHSs, and 11 for abstention. Six months after treatment, both EQ-5D VAS and auditory-specific quality-of-life indices were significantly better in the "CI" arm versus "observation" arm. The mean effect of the CI was particularly significant in subjects with associated severe tinnitus (mean improvement of 20.7 points ± 19.7 on EQ-5D VAS, 20.4 ± 12.4 on NCIQ, and 51.4 ± 35.4 on tinnitus). No significant effect of the CI was found on binaural hearing results. Before/after comparisons showed that the CROS and BAHS also improved significantly NCIQ scores (for CROS: +7.7, 95% confidence interval [95% CI] = [4.5; 10.8]; for the BAHS: +14.3, 95% CI = [7.9; 20.7]). CONCLUSION: Cochlear implantation leads to significant improvements in quality of life in SSD and AHL patients, particularly in subjects with associated severe tinnitus, who are thereby the best candidates to an extension of CI indications.

Treatment choice in single-sided deafness and asymmetric hearing loss. A prospective, multicentre cohort study on 155 patients.

OBJECTIVES: To describe the treatment choice in a cohort of subjects with single-sided deafness (SSD) and asymmetric hearing loss (AHL). To assess the reliability of the treatment trials. DESIGN: In this national, multicentre, prospective study, the choice of subjects was made after two consecutive trials of Contralateral Routing Of the Signal (CROS) hearing aids and a Bone Conduction Device (BCD) on a headband. Subjects could proceed with one of these two options, opt for cochlear implantation or decline all treatments. SETTING: Seven tertiary university hospitals. PARTICIPANTS: One hundred fifty-five subjects with SSD or AHL fulfilling the candidacy criteria for cochlear implantation, with or without associated tinnitus. MAIN OUTCOME MEASURES: After the two trials, the number of subjects choosing each option was described. Repeated assessments of both generic and auditory-specific quality of life were conducted, as well as hearing assessments (speech recognition in noise and horizontal localization). RESULTS: CROS was chosen by 75 subjects, followed by cochlear implantation (n = 51), BCD (n = 18) and abstention (n = 11). Patients who opted for cochlear implantation had a poorer quality of life (P = .03). The improvement of quality of life indices after each trial was significantly associated with the final treatment choice (P = .008 for generic indices, P = .002 for auditory-specific indices). The follow-up showed that this improvement had been overestimated in the CROS group, with a long-term retention rate of 52.5%. CONCLUSIONS: More than one third of SSD/AHL subjects are unsatisfied after CROS and BCD trials. Repeated quality of life assessments help counselling the patient for his/her treatment choice.

Early Sentence Recognition in Adult Cochlear Implant Users.

OBJECTIVE: Normal-hearing subjects listening to acoustic simulations of cochlear implants (CI) can obtain sentence recognition scores near 100% in quiet and in 10 dB signal-to-noise ratio (SNR) noise with acute exposure. However, average sentence recognition scores for real CI listeners are generally lower, even after months of experience, and there is a high degree of heterogeneity. Our aim was to identify the relative importance and strength of factors that prevent CI listeners from achieving early, 1-mo scores as high as those for normal-hearing-listener acoustic simulations. DESIGN: Sentence recognition scores (100 words/list, 65 dB SPL) using CI alone were collected for all adult unilateral CI listeners implanted in our center over a 5-yr period. Sentence recognition scores in quiet and in 10 dB SNR 8-talker babble, collected from 1 to 12 mo, were reduced to a single dependent variable, the "initial" score, via logarithmic regression. "Initial" scores equated to an improved estimate of 1-mo scores, and integrated the time to rise above zero score for poorer performing subjects. Demographic, device, and medical data were collected for 118 subjects who met standard CI candidacy criteria. Computed tomography of the electrode array allowing determination of the insertion depth as an angle, and the presence or absence of scala dislocation was available for 96 subjects. Predictive factors for initial scores were selected using stepwise multiple linear regression. The relative importance of predictive factors was estimated as partial r with a low bias method, and statistical significance tested with type II analysis of variance. RESULTS: The etiologies chronic otitis and autoimmune disease were associated with lower, widely variable sentence recognition scores in the long-term. More than 60% of CI listeners scored >50/100 in quiet at 1 mo. Congenital hearing loss was associated with significantly lower initial scores in quiet (r 0.23, p < 0.001), as was longer duration of hearing loss (r 0.12, p < 0.001, -0.76 pts per year). Initial scores were negatively correlated with insertion depth (r 0.09, p < 0.001, -0.1 pts per degree), with the highest initial scores being obtained for insertion depths of 300° to 400°. A much greater proportion of scala dislocations was found for perimodiolar arrays compared with straight arrays. Scores were negatively correlated with the proportion of the active electrode array found in scala vestibuli for Nucleus perimodiolar devices (r 0.14, p < 0.01, coefficient -25). Similar overall results were obtained for sentence recognition scores in noise (+10 dB SNR). The intercept value for the obtained regression functions indicated that CI listeners with the least limiting factors generally scored ~95/100 in quiet and ~90/100 in noise. In addition, CI listeners with insertion angles as low as 315° to 360° could obtain sentence recognition scores >80/100 even at 1 day after activation. Insertion depths of 360° were estimated to produce frequency-place mismatches of about one octave upward shift. CONCLUSIONS: Patient-related factors etiology and duration of deafness together explained ~40% of the variance in early sentence recognition scores, and electrode position factors ~20%. CI listeners with insertion depths of about one turn obtained the highest early sentence recognition scores in quiet and in noise, and these were comparable with those reported in the literature for normal-hearing subjects listening to 8 to 12 channel vocoder simulations. Differences between device brands were largely explained by differences in insertion depths. This indicates that physiological frequency-place mismatches of about one octave are rapidly accommodated by CI users for understanding sentences, between 1 day to 1 mo postactivation, and that channel efficiency may be significantly poorer for more deeply positioned electrode contacts.

Cochlear implantation as a treatment for single-sided deafness and asymmetric hearing loss: a randomized controlled evaluation of cost-utility.

BACKGROUND: Single-sided deafness (SSD) and asymmetric hearing loss (AHL) have recently been proposed as a new indication for cochlear implantation. There is still no recommended treatment for these hearing deficits, and most options considered rely on the transfer of sound from the poor ear to the better ear, using Contralateral Routing of the Signal (CROS) hearing aids or bone conduction (BC) devices. In contrast, cochlear implantation allows the poor ear to be stimulated and binaural hearing abilities to be partially restored. Indeed, most recently published studies have reported an improvement in the spatial localisation of an incoming sound and better speech recognition in noisy environments after cochlear implantation in SSD/AHL subjects. It also provides consistent relief of tinnitus when associated. These encouraging hearing outcomes raise the question of the cost-utility of this expensive treatment in an extended indication. METHODS: The final endpoint of this national multicentre study is to determine the incremental cost-utility ratio (ICUR) of cochlear implantation in comparison to the current standard of care in France through simple observation, using a randomised controlled trial. Firstly, the study comprises a prospective and descriptive part, where 150 SSD/AHL subjects try CROS hearing aids and a BC device for three weeks each. Secondly, the choice is made between CROS hearing aids, BC implanted device and cochlear implantation. Hearing outcomes and quality of life measurements are described after 6 months for the subjects who chose CROS, BC or declined any option. The subjects who opt for cochlear implantation are randomised between one group where the cochlear implant is inserted without delay and one group of simple initial observation. Hearing outcomes and quality of life measurements are compared after 6 months. DISCUSSION: The present study was designed to assess the efficiency of cochlear implantation in SSD/AHL. A favourable cost-utility ratio in this extended indication would strengthen the promising clinical results and justify a reimbursement by the health insurance. The efficiency of other options (CROS, BC) will also be described. TRIAL REGISTRATION: This research has been registered in ClinicalTrials.gov (http://www.clinicaltrials.gov/), the 29th July 2014 under the n°NCT02204618.

Quality of life and auditory performance in adults with asymmetric hearing loss.

We evaluated the relationship between binaural hearing deficits and quality of life. The study included 49 adults with asymmetric hearing loss (AHL), and 11 adult normal-hearing listeners (NHL) served as controls. Speech reception thresholds (SRT) were assessed with the French Matrix Test. Quality of life was evaluated with the Speech, Spatial and Qualities of Hearing Scale (SSQ) and the Glasgow Health Status Inventory. Speech recognition in noise was significantly poorer for AHL subjects [-0.12 dB signal-to-noise ratio (SNR) in dichotic (with speech presented to the poorer ear and noise to the better ear), -1.72 dB in diotic and -6.84 dB in reverse-dichotic conditions] compared to NHL (-4.98 dB in diotic and -9.58 dB in dichotic conditions). Scores for quality-of-life questionnaires were significantly below norms. Significant correlations were found between the SRT for the dichotic condition and the SSQ total score (r = -0.38, p = 0.01), and pure-tone average thresholds for both groups.

Identification and evaluation of cochlear implant candidates with asymmetrical hearing loss.

OBJECTIVE: Recommendation for cochlear implant (CI) treatment for individuals with severe to profound single-sided deafness (SSD) and asymmetrical hearing loss (AHL) is on the rise. This raises the need for greater consistency in the definition of CI candidacy for these cases and in the assessment methods of patient-related benefits to permit effective comparison and interpretation of the outcomes with both conventional and implantable options across studies. METHOD: During a dedicated seminar on implant treatment in AHL patients, the panellists of the closing round table reviewed the clinical experience presented with the aim to define clear audiometric characteristics for both AHL and SSD cases, as well as a common data set enabling consistent evaluation of hearing benefits in this population. CONCLUSIONS: The panellists agreed on a clear differentiation between AHL and SSD CI candidates, defining average pure-tone thresholds up to 4 kHz for better and poorer ears. Agreement was reached on a minimum set of assessment procedures, and included the necessity of trials with conventional CROS/BICROS hearing aids and bone conduction devices before considering CI treatment. Objective assessment of sound localisation abilities was identified as the most relevant criterion to quantify performance before and after treatment. In parallel, subjective assessment of overall hearing ability was recommended via the Speech, Spatial and Qualities of hearing questionnaire. Longitudinal follow-up of these parameters and the hours of daily use were considered essential to reflect the potential treatment benefits for this population. The consistency in the data collection and its report will further support health authorities in their decision on acceptable gains from available hearing loss treatment options.

Effect of corticosteroids on facial function after cerebellopontine angle tumor removal: a double-blind study versus placebo.

The aim of this study was to assess the effect of corticosteroids administered intra- and postoperatively on the occurrence of facial palsy after a cerebellopontine angle (CPA) tumor resection, and to investigate pre- and intraoperative prognostic factors. A multicenter, prospective, randomized, double-blind and versus-placebo study was conducted between 2006 and 2010. Three hundred and ten patients operated on for a CPA tumor (96% vestibular schwannomas, 4% miscellaneous) were included by five participating centers. The population was stratified into patients with small (≤15 mm CPA on axial MRI views) and large tumors. In each group, patients were randomized to receive corticosteroid (1 mg/kg/day i.v. methylprednisolone intraoperatively and at postoperative days 1-5) or placebo. Steroids did not affect the facial function at postoperative days 1, 8 and 30 in patients with small or large tumors as evaluated by House and Brackmann grading.

Speech prosody perception in cochlear implant users with and without residual hearing.

OBJECTIVES: The detection of fundamental frequency (F0) variations plays a prominent role in the perception of intonation. Cochlear implant (CI) users with residual hearing might have access to these F0 cues. The objective was to study if and how residual hearing facilitates speech prosody perception in CI users. DESIGN: The authors compared F0 difference limen (F0DL) and question/statement discrimination performance for 15 normal-hearing subjects (NHS) and two distinct groups of CI subjects, according to the presence or absence of acoustic residual hearing: one "combined group" (n = 11) with residual hearing and one CI-only group (n = 10) without any residual hearing. To assess the relative contribution of the different acoustic cues for intonation perception, the sensitivity index d' was calculated for three distinct auditory conditions: one condition with original recordings, one condition with a constant F0, and one with equalized duration and amplitude. RESULTS: In the original condition, combined subjects showed better question/statement discrimination than CI-only subjects, d' 2.44 (SE 0.3) and 0.91 (SE 0.25), respectively. Mean d' score of NHS was 3.3 (SE 0.06). When F0 variations were removed, the scores decreased significantly for combined subjects (d' = 0.66, SE 0.51) and NHS (d' = 0.4, SE 0.09). Duration and amplitude equalization affected the scores of CI-only subjects (mean d' = 0.34, SE 0.28) but did not influence the scores of combined subjects (d' = 2.7, SE 0.02) or NHS (d' = 3.3, SE 0.33). Mean F0DL was poorer in CI-only subjects (34%, SE 15) compared with combined subjects (8.8%, SE 1.4) and NHS (2.4%, SE 0.05). In CI subjects with residual hearing, intonation d' score was correlated with mean residual hearing level (r = -0.86, n = 11, p < 0.001) and mean F0DL (r = 0.84, n = 11, p < 0.001). CONCLUSION: Where CI subjects with residual hearing had thresholds better than 60 dB HL in the low frequencies, they displayed near-normal question/statement discrimination abilities. Normal listeners mainly relied on F0 variations which were the most effective prosodic cue. In comparison, CI subjects without any residual hearing had poorer F0 discrimination and showed a strong deficit in speech prosody perception. However, this CI-only group appeared to be able to make some use of amplitude and duration cues for statement/question discrimination.

A Retrospective Multicenter Study Comparing Speech Perception Outcomes for Bilateral Implantation and Bimodal Rehabilitation.

OBJECTIVES: To compare speech perception outcomes between bilateral implantation (cochlear implants [CIs]) and bimodal rehabilitation (one CI on one side plus one hearing aid [HA] on the other side) and to explore the clinical factors that may cause asymmetric performances in speech intelligibility between the two ears in case of bilateral implantation. DESIGN: Retrospective data from 2247 patients implanted since 2003 in 15 international centers were collected. Intelligibility scores, measured in quiet and in noise, were converted into percentile ranks to remove differences between centers. The influence of the listening mode among three independent groups, one CI alone (n = 1572), bimodal listening (CI/HA, n = 589), and bilateral CIs (CI/CI, n = 86), was compared in an analysis taking into account the influence of other factors such as duration of profound hearing loss, age, etiology, and duration of CI experience. No within-subject comparison (i.e., monitoring outcome modifications in CI/HA subjects becoming CI/CI) was possible from this dataset. Further analyses were conducted on the CI/CI subgroup to investigate a number of factors, such as implantation side, duration of hearing loss, amount of residual hearing, and use of HAs that may explain asymmetric performances of this subgroup. RESULTS: Intelligibility ranked scores in quiet and in noise were significantly greater with both CI/CI and CI/HA than with a CI-alone group, and improvement with CI/CI (+11% and +16% in quiet and in noise, respectively) was significantly better than with CI/HA (+6% and +9% in quiet and in noise, respectively). From the CI/HA group, only subjects with ranked preoperative aided speech scores >60% performed as well as CI/CI participants. Furthermore, CI/CI subjects displayed significantly lower preoperative aided speech scores on average compared with that displayed by CI/HA subjects. Routine clinical data available from the present database did not explain the asymmetrical results of bilateral implantation. CONCLUSIONS: This retrospective study, based on basic speech audiometry (no lateralization cues), indicates that, on average, a second CI is likely to provide slightly better postoperative speech outcome than an additional HA for people with very low preoperative performance. These results may be taken into consideration to refine surgical indications for CIs.

Visual activity predicts auditory recovery from deafness after adult cochlear implantation.

Modern cochlear implantation technologies allow deaf patients to understand auditory speech; however, the implants deliver only a coarse auditory input and patients must use long-term adaptive processes to achieve coherent percepts. In adults with post-lingual deafness, the high progress of speech recovery is observed during the first year after cochlear implantation, but there is a large range of variability in the level of cochlear implant outcomes and the temporal evolution of recovery. It has been proposed that when profoundly deaf subjects receive a cochlear implant, the visual cross-modal reorganization of the brain is deleterious for auditory speech recovery. We tested this hypothesis in post-lingually deaf adults by analysing whether brain activity shortly after implantation correlated with the level of auditory recovery 6 months later. Based on brain activity induced by a speech-processing task, we found strong positive correlations in areas outside the auditory cortex. The highest positive correlations were found in the occipital cortex involved in visual processing, as well as in the posterior-temporal cortex known for audio-visual integration. The other area, which positively correlated with auditory speech recovery, was localized in the left inferior frontal area known for speech processing. Our results demonstrate that the visual modality's functional level is related to the proficiency level of auditory recovery. Based on the positive correlation of visual activity with auditory speech recovery, we suggest that visual modality may facilitate the perception of the word's auditory counterpart in communicative situations. The link demonstrated between visual activity and auditory speech perception indicates that visuoauditory synergy is crucial for cross-modal plasticity and fostering speech-comprehension recovery in adult cochlear-implanted deaf patients.

Predictive factors of cochlear implant outcomes in the elderly.

OBJECTIVE: To analyze predictive factors of cochlear implant outcomes and postoperative complications in the elderly. STUDY DESIGN: Prospective, longitudinal study performed in 10 tertiary referral centers. METHODS: Ninety-four patients aged 65-85 years with a profound, postlingual hearing loss were evaluated before implantation, at time of activation, and 6 and 12 months after cochlear implantation. Speech perception and lipreading were measured using disyllabic word recognition in quiet and noise, and lipreading using disyllabic words and sentences. The influence of preoperative factors on speech perception in quiet and noise at 12 months was tested in a multivariate analysis. Complications, presence of tinnitus and of vestibular symptoms were collected at each evaluation. RESULTS: The effect of age was observed only in difficult noisy conditions at SNR 0 dB. Lipreading ability for words and sentences was negatively correlated with speech perception in quiet and noise. Better speech perception scores were observed in patients with shorter duration of hearing deprivation, persistence of residual hearing for the low frequencies, the use of a hearing aid before implantation, the absence of cardiovascular risk factors, and in those with implantation in the right ear. General and surgical complications were very rare, and the percentage of vestibular symptoms remained stable over time. CONCLUSION: This study demonstrates that cochlear implantation in the elderly is a well-tolerated procedure and an effective method to improve communication ability. Advanced age has a low effect on cochlear implant outcome. Analyses of predictive factors in this population provide a convincing argument to recommend treatment with cochlear implantation as early as possible in elderly patients with confirmed diagnosis of a severe-to-profound hearing loss and with only limited benefit from hearing aid use in one ear.

Reliability of cone beam computed tomography in scalar localization of the electrode array: a radio histological study.

Postoperative imaging plays a growing role in clinical studies concerning prognostic factors in cochlear implantation. Indeed, intracochlear position of the cochlear implant has recently been identified as a contributor in functional outcomes and radiological tools must be accurate enough to determine the final placement of the electrode array. The aim of our study was to validate cone beam computed tomography as a reliable technique for scalar localization of the electrode array. We performed therefore a temporal bone study on ten specimens that were implanted with a perimodiolar implant prototype. Cone beam reconstructions were performed and images were analyzed by two physicians both experienced in cochlear implant imaging, who determined the scalar localization of the implant. Temporal bones then underwent histological control to document this scalar localization and hypothetical intracochlear lesions. In four cases, a dislocation from scala tympani to scala vestibuli was suspected on cone beam reconstructions of the ascending part of the basal turn. In three of these four specimens, dislocation in pars ascendens was confirmed histologically. In the remaining temporal bone, histological analysis revealed an elevation with rupture of the basilar membrane. Histological assessment revealed spiral ligament tearing in another bone. We conclude that cone beam is a reliable tool to assess scalar localization of the selectrode array and may be used in future clinical studies.

The Dubai Definition and Diagnostic Criteria of Laryngopharyngeal Reflux: The IFOS Consensus.

OBJECTIVE: The objective of this work was to gather an international consensus group to propose a global definition and diagnostic approach of laryngopharyngeal reflux (LPR) to guide primary care and specialist physicians in the management of LPR. METHODS: Forty-eight international experts (otolaryngologists, gastroenterologists, surgeons, and physiologists) were included in a modified Delphi process to revise 48 statements about definition, clinical presentation, and diagnostic approaches to LPR. Three voting rounds determined a consensus statement to be acceptable when 80% of experts agreed with a rating of at least 8/10. Votes were anonymous and the analyses of voting rounds were performed by an independent statistician. RESULTS: After the third round, 79.2% of statements (N = 38/48) were approved. LPR was defined as a disease of the upper aerodigestive tract resulting from the direct and/or indirect effects of gastroduodenal content reflux, inducing morphological and/or neurological changes in the upper aerodigestive tract. LPR is associated with recognized non-specific laryngeal and extra-laryngeal symptoms and signs that can be evaluated with validated patient-reported outcome questionnaires and clinical instruments. The hypopharyngeal-esophageal multichannel intraluminal impedance-pH testing can suggest the diagnosis of LPR when there is >1 acid, weakly acid or nonacid hypopharyngeal reflux event in 24 h. CONCLUSION: A global consensus definition for LPR is presented to improve detection and diagnosis of the disease for otolaryngologists, pulmonologists, gastroenterologists, surgeons, and primary care practitioners. The approved statements are offered to improve collaborative research by adopting common and validated diagnostic approaches to LPR. LEVEL OF EVIDENCE: 5 Laryngoscope, 134:1614-1624, 2024.

Factors affecting auditory performance of postlinguistically deaf adults using cochlear implants: an update with 2251 patients.

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.

Evolution of crossmodal reorganization of the voice area in cochlear-implanted deaf patients.

Psychophysical and neuroimaging studies in both animal and human subjects have clearly demonstrated that cortical plasticity following sensory deprivation leads to a brain functional reorganization that favors the spared modalities. In postlingually deaf patients, the use of a cochlear implant (CI) allows a recovery of the auditory function, which will probably counteract the cortical crossmodal reorganization induced by hearing loss. To study the dynamics of such reversed crossmodal plasticity, we designed a longitudinal neuroimaging study involving the follow-up of 10 postlingually deaf adult CI users engaged in a visual speechreading task. While speechreading activates Broca's area in normally hearing subjects (NHS), the activity level elicited in this region in CI patients is abnormally low and increases progressively with post-implantation time. Furthermore, speechreading in CI patients induces abnormal crossmodal activations in right anterior regions of the superior temporal cortex normally devoted to processing human voice stimuli (temporal voice-sensitive areas-TVA). These abnormal activity levels diminish with post-implantation time and tend towards the levels observed in NHS. First, our study revealed that the neuroplasticity after cochlear implantation involves not only auditory but also visual and audiovisual speech processing networks. Second, our results suggest that during deafness, the functional links between cortical regions specialized in face and voice processing are reallocated to support speech-related visual processing through cross-modal reorganization. Such reorganization allows a more efficient audiovisual integration of speech after cochlear implantation. These compensatory sensory strategies are later completed by the progressive restoration of the visuo-audio-motor speech processing loop, including Broca's area.

Comparison of three types of French speech-in-noise tests: a multi-center study.

OBJECTIVE: To compare results on the everyday sentence test 'FIST', the new closed-set sentence test 'FrMatrix', and the digit triplet screening test 'FrDigit3'. DESIGN: First, the FrMatrix was developed and normative values were obtained. Subsequently, speech reception thresholds (SRTs) for the three types of tests were gathered at four study centers representing different geographic regions in Belgium and France. STUDY SAMPLE: Fifty-seven normal-hearing listeners took part in the normative study of the FrMatrix, and 118 subjects, with a wide range of hearing thresholds, participated in the comparative study. RESULTS: Homogenizing the individual words of the FrMatrix with regard to their intelligibility resulted in a reference SRT of -6.0 (±0.6) dB SNR and slope at the SRT of 14.0 %/dB. The within-subject variability was only 0.4 dB. Comparison of the three tests showed high correlations between the SRTs mutually (>0.81). The FrMatrix had the highest discriminative power, both in stationary and in fluctuating noise. For all three tests, differences across the participating study centers were small and not significant. CONCLUSIONS: The FIST, the FrMatrix, and the FrDigit3 provide similar results and reliably evaluate speech recognition performance in noise both in normal-hearing and hearing-impaired listeners.

Stapedotomy Versus Hearing Aids in the Management of Conductive Hearing Loss Caused by Otosclerosis: A Prospective Comparative Study.

OBJECTIVE: To compare the outcomes of patients with unilateral otosclerosis treated consecutively by a hearing aid then stapedotomy. STUDY DESIGN: Monocentric, nonrandomized, prospective, longitudinal, cohort study. SETTING: Tertiary university hospital. PATIENTS: Adult patients with a unilateral conductive hearing loss of at least 30 dB caused by otosclerosis. INTERVENTIONS: Consecutive treatment by an external hearing aid followed by stapedotomy. MAIN OUTCOME MEASURES: Differences between hearing aids and stapedotomy in quality of life, pure-tone audiometry, binaural hearing, tinnitus severity, and patient satisfaction. RESULTS: Twenty-two patients were included, of which 20 (91%) underwent stapedotomy. Stapedotomy demonstrated increased quality of life according to Glasgow Health Status Inventory scores versus hearing aids (+10.4 ± 9.4 [p = 0.0001]). Stapedotomy versus hearing aids showed improved pure-tone averages (-11.1 ± 11.0 dB [p = 0.002]) and air-bone gaps (-11.8 ± 10.7 dB [p = 0.0006]). Stapedotomy was superior to hearing aids for speech-in-noise recognition in the reverse dichotic condition (-8.4 ± 26.9 dB [p = 0.004]) and showed improved sound localization accuracy in root mean square error (-14.5 ± 24.5 degrees [p = 0.02]). Stapedotomy, but not hearing aids, showed improved patient self-evaluated tinnitus after baseline adjustment according to the Tinnitus Handicap Inventory (-8.0 ± 13.4 [p = 0.02]) and visual analog scale for tinnitus intensity (-28.7 ± 34.1 [p = 0.006]). Overall, patients were more satisfied with surgery versus hearing aids. CONCLUSIONS: Stapedotomy remains more effective compared with hearing aids with greater improvements in quality of life, patient satisfaction, hearing outcomes, and self-evaluated tinnitus.

Cardiovascular and thromboembolic risk factors in idiopathic sudden sensorineural hearing loss: a case-control study.

OBJECTIVE: The pathogenesis of idiopathic sudden sensorineural hearing loss (ISSHL) remains unknown, but vascular involvement is one of the main hypotheses. The main objective of this study was to investigate the association between ISSHL and cardiovascular and thromboembolic risk factors. STUDY DESIGN: Multicentric case-control study. METHODS: Ninety-six Caucasian patients with ISSHL and 179 sex- and age-matched controls were included. Patients were evaluated on the day of the inclusion and 1 week, 3 weeks and 3 months later. Clinical information concerning personal and familial cardiovascular and thromboembolic risk factors and concerning the ISSHL was collected. Blood samples were collected for genetic analysis of factor V Leiden and G20210A polymorphism in the factor II gene. The severity of the hearing loss was classified as mild (21-40 dB), moderate (41-70 dB), severe (71-90 dB) and profound or total (>90 dB). Hearing improvement was calculated as a relative improvement of hearing thresholds using the contralateral ear as baseline. RESULTS: Systolic blood pressure was higher in patients (130 ± 1.7 mm Hg) than in controls (124 ± 1.1 mm Hg, p = 0.003). The personal/familial history of cardiovascular events was also more prevalent in patients (p = 0.023 and p = 0.014, respectively), whereas no difference was found in the prevalence of personal cardiovascular risk factors (hypertension, diabetes mellitus, hyperlipidemia, smoking habits). There was no correlation between the audiogram type, the hearing outcome and the presence of cardiovascular risk factors. No significant difference was observed in the personal/familial history or in the presence of thromboembolic risk factors. The prothrombin and factor V mutations were uncommon in both patients and controls. The final hearing threshold was only correlated with the severity of the initial hearing loss (p < 0.001), but not influenced by the presence of vertigo, audiogram type, time elapsed from onset of ISSHL to hospitalization or failure of a previous oral therapy. Hearing stabilization was obtained at 21 days in 92% of patients. CONCLUSION: These results support the theory of vascular involvement as the etiology of some cases of ISSHL. The sole predictive factor of poor final hearing is the severity of the initial hearing loss.

Quality of life of children treated for unilateral hearing loss: a systematic review and meta-analysis.

OBJECTIVE: To evaluate the treatments' consequences for unilateral hearing loss in children. DESIGN: Systematic review and meta-analysis (CRD42018109417). The MEDLINE, CENTRAL, ISRCTN and ClinicalTrials databases were searched between September 2018 and May 2019. Articles were screened and data were collected independently by two authors following the Cochrane and Preferred Reporting Items for Systematic review and Meta-Analysis guidelines. The risk of bias was evaluated using the Cochrane tool, the Newcastle-Ottawa Scale, the National Institute of Health, USA tool and considering the risk of confounding. In the studies with the lowest risk of bias, a meta-analysis was conducted. INTERVENTIONS: Validated hearing rehabilitation devices. PATIENTS: 6-15 years old children with moderate to profound unilateral hearing loss. MAIN OUTCOME MEASURES: The primary study outcome was children's quality of life. Academic performances were studied as an additional outcome. RESULTS: 731 unique articles were identified from the primary search. Of these, 18 articles met the Population, Intervention, Control, Outcomes and Study design selection criteria. In the eight studies with the lowest risk of bias, two meta-analysis were conducted. There was not enough data on academic results to conduct a meta-analysis. In 73 children included in a fixed effect meta-analysis (two studies), no effect of treatment could be shown (g=-0.20, p=0.39). In 61 children included in a random-effect meta-analysis (six studies), a strong positive effect of hearing treatment on quality of life was demonstrated (g=1.32, p<0.05). CONCLUSIONS: The treatment of unilateral hearing loss seems to improve children's quality of life. Further research is needed to identify the most effective treatment and its corresponding indications.