Combining sound with tongue stimulation for the treatment of tinnitus: a multi-site single-arm controlled pivotal trial.

Bimodal neuromodulation is emerging as a nonsurgical treatment for tinnitus. Bimodal treatment combining sound therapy with electrical tongue stimulation using the Lenire device is evaluated in a controlled pivotal trial (TENT-A3, NCT05227365) consisting of 6-weeks of sound-only stimulation (Stage 1) followed by 6-weeks of bimodal treatment (Stage 2) with 112 participants serving as their own control. The primary endpoint compares the responder rate observed in Stage 2 versus Stage 1, where a responder exceeds 7 points in the Tinnitus Handicap Inventory. In participants with moderate or more severe tinnitus, there is a clinically superior performance of bimodal treatment (58.6%; 95% CI: 43.5%, 73.6%; p = 0.022) compared to sound therapy alone (43.2%; 95% CI: 29.7%, 57.8%), which is not observed in the full cohort across all severity groups. Consistent results are observed for the secondary endpoint based on the Tinnitus Functional Index (bimodal treatment: 45.5%; 95% CI: 31.7%, 59.9%; sound-only stimulation: 29.6%; 95% CI: 18.2%, 44.2%; p = 0.010), where a responder exceeds 13 points. There are no device related serious adverse events. These positive outcomes led to FDA De Novo approval of the Lenire device for tinnitus treatment.

Long-term experience with biohybrid cochlear implants in human neurosensory restoration.

OBJECTIVE: The implantation of biohybrid electrodes was introduced a few years ago in our clinic. These electrodes coated with autologous mononuclear cells releasing anti-inflammatory and neuroprotective factors are thought to reduce insertion trauma and maintain the vitality of surviving spiral ganglion neurons. The clinical feasibility of this approach has already been demonstrated. In the present retrospective study, the four-year results of the two sides (classical electrode and biohybrid electrode) in the bilaterally implanted patients were compared in order to investigate possible adverse long-term effects. METHODS: All patients received a complete audiological diagnosis which also included a speech audiogram and impedance measurement. The measurements were carried out 1 month, 3 months, 6 months, 1 year, 2 years, 3 years and 4 years after implantation. The hearing results were assessed by pure tone audiometry. RESULTS: All patients showed satisfactory speech understanding and similar impedances on both sides although they had a long-term deafness before implantation of the side provided with a biohybrid electrode array. The results of speech understanding and impedance measurements were stable for years. Cone beam computed tomography was performed in 4 patients three years after implantation and could rule out cochlear ossification. Other complications were also not registered in any of the patients. CONCLUSION: Due to satisfactory outcomes and lack of complications, the biohybrid electrode is considered to be a safe option in cochlear implantation. The simplicity of application of autologous cells as a source of anti-inflammatory and neuroprotective factors via a biohybrid electrode array is a key step for cell-based, regenerative therapies for deafness.

Hamartomas of the Tuber Cinereum Associated with X-Linked Deafness Show Signs of Pubertas Tarda Instead of Pubertas Praecox and No Gelastic Seizures-Long-Term Follow-Up of 12 Years.

PURPOSE: Hamartomas of tuber cinereum present as ectopic tissue in the hypothalamic region. Clinically, the usual hypothalamic hamartomas manifest themself by gelastic seizures and pubertas praecox. We observed an increased coincidence of the presence of X-linked recessive deafness DFNX2 (DFN3) and a hamartoma of the tuber cinereum. Initially five patients presented with hearing loss in childhood, two additional were already adults, not showing any characteristic symptoms for a hamartoma but signs of delayed puberty. METHODS: Seven patients who underwent computed tomography imaging due to a sensorineural hearing loss and had a hamartoma of the tuber cinereum in addition to X-linked deafness DFNX2 (DFN3) were included in a retrospective study. Patients underwent initial neurologic, endocrinologic, and genetic evaluation. Long-term follow-up was performed after 10 to 12 years. RESULTS: The average age at the initial exam was 12.9 years (range 4-29). All patients genetically proven nonsyndromic, X-linked deafness associated with the POU3F4 gene. Three out of six patients presented signs of delayed puberty. None of all seven showed any evidence of pubertas praecox or gelastic seizures at mean age of 17 years (range 17-29 years) at any time. CONCLUSION: Hamartomas of tuber cinereum are often coincident with DFNX2. Clinically, half of the cases are-in contrary to the usual pubertas praecox-associated with growth hormone deficiency and delayed puberty, in the sense of pubertas tarda, when coincident. Clinicians' and radiologists' knowledge and awareness of this rare combination are crucial to identify children early enough for hormone-sensitive treatment.

A New Methodology for Evaluation of Large Vestibular Aqueduct in CT and MRI Images.

HYPOTHESIS: Development of a new method for large vestibular aqueduct (LVA)/large endolymphatic sac anomaly (LESA) assessment using magnetic resonance imaging (MRI) and computed tomography (CT)/cone beam CT (CBCT) images. The secondary objective was to compare both modalities. BACKGROUND: The gold standard for LVA diagnosis is the analysis of CT images using Valvassori and Clemis or Cincinnati criteria. The previous studies showed inconclusive results regarding the correlation between audiological and radiological data. METHODS: Retrospective analysis of radiological images from 173 patients (315 ears), who were diagnosed with LVA/LESA based on CT/CBCT and/or MRI images of the temporal bone. The images obtained using both techniques were used to measure the following dimensions of vestibular aqueduct (VA)/endolymphatic duct (ED)/intraosseous endolymphatic sac (ES): width of the opening, length, and width at external aperture. In MRI images, the maximal contact diameters of the extraosseous or intraosseous ES and dura mater were measured as well. RESULTS: LVA has been reported to be bilateral in 82% (142 patients) and unilateral in 18% (31 patients) of cases. Comparison of MRI and CT/CBCT measurements showed a moderate correlation (0.64) in external aperture, a moderate correlation (0.57) in the width of the VA opening, and a weak correlation (0.34) in length measurements (p < 0.05). CONCLUSION: We developed a new method to identify the heterogeneous pathology of LVA/LESA using reconstruction along the VA/ED/intraosseous ES axis, three measurements on two planes, and focus on the maximal contact diameter between the extraosseous or intraosseous ES and dura mater.

Variability in Receptive Language Development Following Bilateral Cochlear Implantation.

OBJECTIVES: The primary aim was to investigate the variability in language development in children aged 5-7.5 years after bilateral cochlear implantation (CI) up to the age of 2 years, and any impact of the age at implantation and additional noncognitive or anatomical disorders at implantation. DESIGN: Data of 84 congenitally deaf children that had received simultaneous bilateral CI at the age of ≤ 24 months were included in this retrospective study. The results of language comprehension acquisition were evaluated using a standardized German language acquisition test for normal hearing preschoolers and first graders. Data on speech perception of monosyllables and sentences in quiet and noise were added. RESULTS: In a monosyllabic test, the children achieved a median performance of 75.0 ± 12.88%. In the sentence test in quiet, the median performance was 89 ± 12.69%, but dropped to 54 ± 18.92% in noise. A simple analysis showed a significant main effect of age at implantation on monosyllabic word comprehension (p < .001), but no significant effect of comorbidities that lacked cognitive effects (p = .24). Language acquisition values correspond to the normal range of children with normal hearing. Approximately 25% of the variability in the language acquisition tests is due to the outcome of the monosyllabic speech perception test. CONCLUSIONS: Congenitally deaf children who were fitted bilaterally in the 1st year of life can develop age-appropriate language skills by the time they start school. The high variability in the data is partly due to the age of implantation, but additional factors such as cognitive factors (e.g., working memory) are likely to influence the variability.

Long-Term Hearing Outcome of Cochlear Implantation in Cases with Simultaneous Intracochlear Schwannoma Resection.

OBJECTIVES: The aim was to analyze the long-term hearing results after simultaneous microsurgical extirpation via enlarged cochleostomy and cochlear implantation in intracochlear schwannoma as compared with non-tumor single-side deafness patients. METHODS: Microsurgical extirpation via enlarged cochleostomy with simultaneous cochlear implantation was performed in 15 cases of intracochlear schwannoma between 2014 and 2021. Speech recognition tests in German language and impedance performances were collected over 36 months of observation and compared with an internal cohort of 52 age matched non-tumor single-side deafness patients. Retrospective cohort study in a tertiary referral center. RESULTS: The surgery proved feasible and uneventful in all cases. In the case of intracochlear schwannoma, the hearing rehabilitation results were highly satisfactory and comparable to those of the non-tumor single-side deafness cohort. The speech recognition performance improved steadily in the first 12 months; afterward, it remained stable, providing indirect evidence against tumor recurrence during the follow-up. One patient required implant revision surgery related to device failure, but no recurrence was registered in the 36 months of observation. CONCLUSIONS: Cochlear implantation is the strategy of choice for hearing rehabilitation in case of intracochlear schwannomas in the long term. In particular, the combination of tumor extirpation via cochleostomy with a cochlear implantation in the same surgical time offers a viable therapy for intracochlear schwannoma, granting a sufficient degree of radicality without compromising the cochlear integrity. This technique allows for revision surgery if required. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:1854-1860, 2024.

Auditory capacity of the better-hearing ear in asymmetric hearing loss.

PURPOSE: Our aim was to investigate the course of the hearing capacity of the better-hearing ear in single-sided deafness (SSD) and asymmetric hearing loss (AHL) over time, in a multicenter study. METHODS: We included 2086 pure-tone audiograms from 323 patients with SSD and AHL from four hospitals and 156 private practice otorhinolaryngologists. We collected: age, gender, etiology, duration of deafness, treatment with CI, number and monosyllabic speech recognition, numerical rating scale (NRS) of tinnitus intensity, and the tinnitus questionnaire according to Goebel and Hiller. We compared the pure tone audiogram of the better-hearing ear in patients with SSD with age- and gender-controlled hearing thresholds from ISO 7029:2017. RESULTS: First, individuals with SSD showed a significantly higher hearing threshold from 0.125 to 8 kHz in the better-hearing ear compared to the ISO 7029:2017. The duration of deafness of the poorer-hearing ear showed no relationship with the hearing threshold of the better-hearing ear. The hearing threshold was significantly higher in typically bilaterally presenting etiologies (chronic otitis media, otosclerosis, and congenital hearing loss), except for Menière's disease. Second, subjects that developed AHL did so in 5.19 ± 5.91 years and showed significant reduction in monosyllabic word and number recognition. CONCLUSIONS: Individuals with SSD show significantly poorer hearing in the better-hearing ear than individuals with NH from the ISO 7029:2017. In clinical practice, we should, therefore, inform our SSD patients that their disease is accompanied by a reduced hearing capacity on the contralateral side, especially in certain etiologies.

Deep intracochlear injection of triamcinolone-acetonide with an inner ear catheter in patients with residual hearing.

Introduction: In a previous study, an inner ear catheter was used to deliver low- and high-dose steroids into the cochlea prior to cochlear implant electrode insertion. With this approach, more apical regions of the cochlea could be reached and a reduction of electrode impedances in the short term was achieved in cochlear implant recipients. Whether intracochlear application of drugs via the catheter is a safe method also for patients with residual hearing has not been investigated hitherto. The aim of the present study was therefore to investigate the effect of intracochlear triamcinolone application in cochlear implant recipients with residual hearing. Patients and methods: Patients with residual hearing were administered triamcinolone-acetonide (4 mg/ml; n = 10) via an inner ear catheter just prior to insertion of a MED-EL FLEX28 electrode. Impedances were measured at defined time points (intra-operatively, post-operatively and at first fitting) and retrospectively compared with a control group (no steroid application) and low- and high-dose group. Hearing thresholds were measured preoperatively, 3 days after surgery and at first fitting by pure tone audiometry. Pre- to postoperative hearing loss was determined at first fitting and compared to results from a previous study. Results: The median hearing loss after implantation (125-1,500 Hz) was 20.6 dB. Four patients (40%) showed a median hearing loss of less than 15 dB, three patients (30%) between 15 and 30 dB and three patients (30%) more than 30 dB. The median hearing loss was similar to the results obtained from our previous study showing a median hearing loss of 24 dB when using FLEX28 electrode arrays. Conclusion: No difference in residual hearing loss was found when comparing application of triamcinolone-acetonide using an inner ear catheter prior to the insertion of a FLEX28 electrode array to the use of the FLEX28 electrode array without the catheter. Thus, we conclude that application of drugs to the cochlea with an inner ear catheter could be a feasible approach in patients with residual hearing.

Variability in Cochlear Implantation Outcomes in a Large German Cohort With a Genetic Etiology of Hearing Loss.

OBJECTIVES: The variability in outcomes of cochlear implantation is largely unexplained, and clinical factors are not sufficient for predicting performance. Genetic factors have been suggested to impact outcomes, but the clinical and genetic heterogeneity of hereditary hearing loss makes it difficult to determine and interpret postoperative performance. It is hypothesized that genetic mutations that affect the neuronal components of the cochlea and auditory pathway, targeted by the cochlear implant (CI), may lead to poor performance. A large cohort of CI recipients was studied to verify this hypothesis. DESIGN: This study included a large German cohort of CI recipients (n = 123 implanted ears; n = 76 probands) with a definitive genetic etiology of hearing loss according to the American College of Medical Genetics (ACMG)/Association for Molecular Pathology (AMP) guidelines and documented postoperative audiological outcomes. All patients underwent preoperative clinical and audiological examinations. Postoperative CI outcome measures were based on at least 1 year of postoperative audiological follow-up for patients with postlingual hearing loss onset (>6 years) and 5 years for children with congenital or pre/perilingual hearing loss onset (≤6 years). Genetic analysis was performed based on three different methods that included single-gene screening, custom-designed hearing loss gene panel sequencing, targeting known syndromic and nonsyndromic hearing loss genes, and whole-genome sequencing. RESULTS: The genetic diagnosis of the 76 probands in the genetic cohort involved 35 genes and 61 different clinically relevant (pathogenic, likely pathogenic) variants. With regard to implanted ears (n = 123), the six most frequently affected genes affecting nearly one-half of implanted ears were GJB2 (21%; n = 26), TMPRSS3 (7%; n = 9), MYO15A (7%; n = 8), SLC26A4 (5%; n = 6), and LOXHD1 and USH2A (each 4%; n = 5). CI recipients with pathogenic variants that influence the sensory nonneural structures performed at or above the median level of speech performance of all ears at 70% [monosyllable word recognition score in quiet at 65 decibels sound pressure level (SPL)]. When gene expression categories were compared to demographic and clinical categories (total number of compared categories: n = 30), mutations in genes expressed in the spiral ganglion emerged as a significant factor more negatively affecting cochlear implantation outcomes than all clinical parameters. An ANOVA of a reduced set of genetic and clinical categories (n = 10) identified five detrimental factors leading to poorer performance with highly significant effects ( p < 0.001), accounting for a total of 11.8% of the observed variance. The single strongest category was neural gene expression accounting for 3.1% of the variance. CONCLUSIONS: The analysis of the relationship between the molecular genetic diagnoses of a hereditary etiology of hearing loss and cochlear implantation outcomes in a large German cohort of CI recipients revealed significant variabilities. Poor performance was observed with genetic mutations that affected the neural components of the cochlea, supporting the "spiral ganglion hypothesis."

Genetic Evaluation of Prelingual Hearing Impairment: Recommendations of an European Network for Genetic Hearing Impairment.

The cause of childhood hearing impairment (excluding infectious pathology of the middle ear) can be extrinsic (embryofoetopathy, meningitis, trauma, drug ototoxicity, noise trauma, etc [...].

[Comparison of speech understanding taking into account the exact electrode position (SRA/MRA/CA)]. / Vergleich des Sprachverstehens unter Berücksichtigung der genauen Elektrodenposition (SRA/MRA/CA).

Cochlear implantation has been a routine hearing rehabilitation procedure for years. Nevertheless, not all parameters that influence speech understanding after implantation are known. We test the hypothesis whether there is a connection between speech understanding and the position of different electrode types in relation to the modiolus in the cochlea with identical speech processors. For this purpose, in this retrospective study, we compare the hearing results with different electrode types ("Straight Research Array" [SRA], "Modiolar Research Array" [MRA] and "Contour Advance" [CA]) from the manufacturer Cochlear in matched pair groups.After creating three groups using "matched pairs" (n=52 patients per group), the cochlear parameters (length of the outer wall, angle of insertion, insertion depth, cochlear coverage and total length of the electrode in the cochlea, wrapping factor) were measured in the routinely performed manner pre- and post-operative high-resolution CT or DVT. The Freiburg monosyllabic understanding was used as a target variable one year after implantation.In the Freiburg monosyllabic test one year postoperatively, patients with MRA had a monosyllabic understanding of 51.2%, patients with SRA 49.5% and patients with CA 58.0%. It could be shown that with increasing cochlear coverage with MRA and CA, the speech understanding of the patients decreases and with SRA it increases. In addition, it could be shown that the monosyllabic understanding increases with increasing "wrapping factor".The results show that the position of the electrode to the modiolus is not the only factor explaining differences in outcome after cochlear implantation.

Auditory rehabilitation after temporal bone fracture with cochlear implants - a case control study.

OBJECTIVES: Temporal bone fracture can cause posttraumatic deafness. Sequelae like ossification or obliteration of the cochlea can impact the outcome of cochlear implantation. This study highlights the effect of localisation of the fracture to morphologic, electric and functional criteria. METHODS: The study group consists of patients suffering from hearing loss caused by temporal bone fracture (n = 61 ears). Patients were divided into otic capsule sparing (OCS) and otic capsule involving (OCI) fractures. The OCI group was additionally divided into subgroups with or without signs of ossification inside the cochlea. Postoperative imaging, hearing tests and electrode impedances were analysed. RESULTS: The results of postoperative hearing rehabilitation showed lower speech understanding scores for the OCI group, especially for the ossification group. OCI fractures with signs of ossification showed increased impedances. Patients in the OCI group suffered more frequently from facial nerve stimulation (FNS). FNS was most frequently observed within the ossification group. CONCLUSION: Cochlear implantation in patients with temporal bone fracture is adequate therapy for the treatment of fracture-induced deafness. In long-term observation, these patients show comparable results with regular cochlear implant (CI) patients. Implantation should be performed as soon as possible after hearing loss, before obstructing obliteration or ossification of the cochlea start.

Influence of the Electrode Array Design on Incidence of Vertigo Symptoms and Vestibular Function After Cochlear Implantation.

PURPOSE: To evaluate if a specific type of cochlear implant (CI) electrode array (EA) reveals higher rates/prevalence of vestibular symptoms and to characterize their respective relationship to intracochlear position and objective vestibular function. METHODS: This retrospective study included 71 cochlear implantations in patients older than 18 years. The electrode position within the cochlea, electrode insertion angle, and cochlear coverage were determined from postoperative multiplanar reconstructed cone-beam computed tomography scans. All device manufacturers were represented. Data related to preoperative and postoperative PTA as well as vestibular symptoms in the preoperative and postoperative stages were collected from the patient's records. RESULTS: Twelve of the 71 (16.9%) CI patients experienced vertigo symptoms in the early postoperative period. In 5 (7.0%) patients, the vertigo complaints lasted until the time of the first activation (5-6 weeks postoperative). Postoperative onset of vestibular symptoms was more often seen in patients receiving lateral wall (LW)/straight EAs (19%) compared to perimodiolar/precurved EAs (7%), but this was only a trend and no statistical significance was observed. Moreover, preoperative pathologic caloric responses (CRs) better predicted the postoperative onset of vestibular symptoms. CONCLUSION: The preoperative consideration of a complicated CI-induced vertigo is important in the counseling particularly of elderly patients. We identified some risk factors for post-CI vertigo that should be considered in the patient's counseling: preoperative pathologic CRs, the extent of surgical trauma, and possibly the use of an LW EA, regardless of the length.

Do Not Go Gentle into That Deaf Night: A Holistic Perspective on Cochlear Implant Use as Part of Healthy Aging.

Research suggests that cochlear implant (CI) use in elderly people improves speech perception and health-related quality of life (HRQOL). CI provision could also prevent dementia and other comorbidities and support healthy aging. The aim of this study was (1) to prospectively investigate potential changes in HRQOL and speech perception and (2) to identify clinical action points to improve CI treatment. Participants (n = 45) were CI recipients aged 60-90 with postlingual deafness. They were divided into groups, according to age: Group 1 (n = 20) received a CI between the age of 60-70 years; group 2 (n = 25) between the age of 71-90 years. HRQOL and speech perception were assessed preoperatively, and three and twelve months postoperatively. HRQOL and speech perception increased significantly within one year postoperatively in both groups. No difference between groups was found. We conclude that CI treatment improves speech perception and HRQOL in elderly users. Improvement of the referral process for CI treatment and a holistic approach when discussing CI treatment in the elderly population could prevent auditory deprivation and the deterioration of cognitive abilities.

A potential serological biomarker for inner ear pathologies: OTOLIN-1.

Objective: The goal of the study was to determine whether the level of OTOLIN-1, a protein whose expression is highly restricted to the inner ear,is increased in the body fluids of patients with inner ear disorders in comparison to healthy subjects. Methods: In the preliminary part of the study, OTOLIN-1 levels were measured in the serum, urine, and saliva of patients with an acute onset of Ménière´s disease and in healthy individuals. Subsequently, only serum OTOLIN-1 levels were taken into account and were compared between patients with acute onset of Ménière´s disease, sudden hearing loss, vestibular neuritis and healthy subjects. Results: The most reliable diagnostic parameter was OTOLIN-1 levels in serum. Serum samples of patients with Ménière's disease and sudden hearing loss showed significantly higher OTOLIN-1 levels than those from healthy individuals. In addition, there was no significant difference between the serum concentration of OTOLIN-1 in patients with vestibular neuritis and the control group. Conclusions: Serum levels of OTOLIN-1 can potentially be used as a biomarker for acute onset of inner ear disorders due to its significant increase in patients with acute Meniere´s disease and sudden hearing loss in comparison to healthy individuals.

GenOtoScope: Towards automating ACMG classification of variants associated with congenital hearing loss.

Since next-generation sequencing (NGS) has become widely available, large gene panels containing up to several hundred genes can be sequenced cost-efficiently. However, the interpretation of the often large numbers of sequence variants detected when using NGS is laborious, prone to errors and is often difficult to compare across laboratories. To overcome this challenge, the American College of Medical Genetics and Genomics and the Association for Molecular Pathology (ACMG/AMP) have introduced standards and guidelines for the interpretation of sequencing variants. Additionally, disease-specific refinements have been developed that include accurate thresholds for many criteria, enabling highly automated processing. This is of particular interest for common but heterogeneous disorders such as hearing impairment. With more than 200 genes associated with hearing disorders, the manual inspection of possible causative variants is particularly difficult and time-consuming. To this end, we developed the open-source bioinformatics tool GenOtoScope, which automates the analysis of all ACMG/AMP criteria that can be assessed without further individual patient information or human curator investigation, including the refined loss of function criterion ("PVS1"). Two types of interfaces are provided: (i) a command line application to classify sequence variants in batches for a set of patients and (ii) a user-friendly website to classify single variants. We compared the performance of our tool with two other variant classification tools using two hearing loss data sets, which were manually annotated either by the ClinGen Hearing Loss Gene Curation Expert Panel or the diagnostics unit of our human genetics department. GenOtoScope achieved the best average accuracy and precision for both data sets. Compared to the second-best tool, GenOtoScope improved the accuracy metric by 25.75% and 4.57% and precision metric by 52.11% and 12.13% on the two data sets, respectively. The web interface is accessible via: http://genotoscope.mh-hannover.de:5000 and the command line interface via: https://github.com/damianosmel/GenOtoScope.

Serum N-glycomics of a novel CDG-IIb patient reveals aberrant IgG glycosylation.

Rare genetic mutations of the mannosyl-oligosaccharide glucosidase (MOGS) gene affecting the function of the mannosyl-oligosaccharide glucosidase (glucosidase I) are the cause of the congenital disorder of glycosylation IIb (CDG-IIb). Glucosidase I specifically removes the distal α1,2-linked glucose from the protein bound precursor N-glycan Glc3Man9GlcNAc2, which is the initial step of N-glycan maturation. Here, we comparatively analyzed N-glycosylation of the whole serum proteome, serum-derived immunoglobulin G (IgG), transferrin (TF), and α-1-antitrypsin (AAT) of a female patient who is compound heterozygous for 2 novel missense mutations in the MOGS gene, her heterozygous parents, and a sibling with wildtype genotype by multiplexed capillary gel electrophoresis coupled to laser induced fluorescence detection (xCGE-LIF) at unprecedented depth. Thereby, we detected the CDG-IIb-characteristic non-de-glucosylated N-glycans Glc3Man7-9GlcNAc2 as well as the free tetrasaccharide Glc3-Man in whole serum of the patient but not in the other family members. The N-glycan analysis of the serum proteome further revealed that relative intensities of IgG-specific complex type di-antennary N-glycans with core-fucosylation were considerably reduced in the patient's serum whereas TF- and AAT-characteristic sialylated di- and tri-antennary N-glycans were increased. This finding reflected the hypogammaglobulinemia diagnosed in the patient. We further detected aberrant oligo-mannose (Glc3Man7GlcNAc2) and hybrid type N-glycans on patient-derived IgGs and we attributed this defective glycosylation to be the reason for an increased IgG clearance. This mechanism can explain the hypogammaglobulinemia that is associated with CDG-IIb.

CI decision making and expectations by older adults.

OBJECTIVE: To document and analyze the cochlear implant (CI) decision-making process of hearing-impaired older adults. The aim of this study is to assess what support could be helpful during this process in order to improve care delivery. METHODS: 32 older adult CI recipients (≥ 60 years) with severe to profound sensorineural hearing loss were interviewed about their CI decision-making process 3-12 months after obtaining their first CI. RESULTS: Minimal information was provided to CI candidates by hearing aid acousticians or patient associations. High to very high expectations were reported by patients concerning issues beyond hearing improvement per se. Even though not all expectations were fulfilled by the CI, nearly all recipients who used an implant for at least six months would recommend a CI to others. DISCUSSION: We identified an opportunity for those professionals to play a greater role in supporting older CI candidates during the decision-making process. It is desirable to establish a comprehensive network of hearing care professionals to collaborate with CI clinics. CONCLUSION: In order to support older patients adequately in deciding about CI, intensive training should be offered to hearing care professionals in order to provide realistic expectations and reduce fear and uncertainty about the implantation process. These topics need to be communicated in a professional manner and adapted to the candidate's age and personality.

Cochlear Implantation in Elderly Patients with Residual Hearing.

This retrospective study aimed to investigate the range of hearing levels in a cochlear implant (CI) elderly population receiving electric-acoustic-stimulation (EAS) or electric-stimulation (ES) alone. The investigation evaluates the degree of hearing preservation (HP) and the speech comprehension resulting from EAS or ES-only to identify audiometric factors that predict adequate EAS and ES use. We analyzed the pure tone audiometry and speech perception in quiet and noise preoperatively and 12-months after activation of 89 elderly adults (age of 65 years old or older), yielding in total 97 CIs. Thirty-two (33.1%) patients were potential EAS candidates preoperatively, of which 18 patients used EAS at the time of first fitting and the other 14 patients continued to use their residual hearing for EAS at 12-months. Post-treatment, patients with EAS system and ES-only users' with longer electrodes showed better results in monosyllable word scores in quiet than ES-only users with shorter electrodes. A similar trend was revealed for the speech recognition in noise. Patients with an EAS system benefit from maintaining their natural residual hearing. Nevertheless, strict preoperative patient selection is warranted particularly in elderly patients, in whom the hearing thresholds for EAS indication differ slightly from that in younger adults.