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.

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.

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."

[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.

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.

Can Cochlear Implantation in Older Adults Reverse Cognitive Decline Due to Hearing Loss?

INTRODUCTION: Older adults with late-onset hearing loss are at risk for cognitive decline. Our study addresses the question of whether cochlear implantation (CI) can counteract this potential influence. We investigated whether cognitive performance in older adults with severe and profound hearing loss improves 12 months after CI to a level comparable to controls with normal hearing, matched for age, sex, and education level. DESIGN: This cohort study was performed at two tertiary referral centers. The study included 29 patients, of age between 60 and 80 years, with adult-onset, severe to profound bilateral sensorineural hearing loss and indication for CI (study group), as well as 29 volunteers with age-adjusted hearing abilities, according to the norm curves of ISO-702 9:2000-01, (control group). Before CI and 12 months after CI, participants completed a neurocognitive test battery including tests of global cognition, verbal and figural episodic memory, and executive functions (attentional control, inhibition, and cognitive flexibility). RESULTS: Twelve months after CI, the performance of the study group improved significantly in global cognition, compared to the situation before CI. Differences in verbal episodic memory, figural episodic memory, and executive function were not significant. Moreover, the improvement of the study group was significantly larger only in global cognition compared to the control group. Noninferiority tests on the cognitive performances of the study group after CI revealed that comparable levels to normal hearing controls were reached only in global cognition, figural episodic memory (immediate recall), and attentional control. The improvement in global cognition was significantly associated with speech recognition 3 months after CI, but not with speech recognition 12 months after CI. CONCLUSION: One year after CI, cognitive deficits in older individuals with adult-onset hearing loss, compared to normal-hearing peers, could only improve some cognitive skills.

Auditory performance in a group of elderly patients after cochlear implantation.

PURPOSE: The retrospective case review investigated the effect of cochlear implantation in subjects aged 61 years or older with respect to their auditory performance. The study also analysed the effect of age on the performance, and it drew a comparison between the outcomes of older and younger adults. METHODS: The outcome in a group of 446 patients aged 61 to 89 years at the time of unilateral cochlear implantation was compared with the outcome in a group of 110 patients aged 17 to 42 years. Auditory performance was measured with open-set monosyllabic word testing and sentences in quiet and in noise. RESULTS: In the monosyllabic word recognition test, the group of older adults performed significantly better after cochlear implantation compared with their scores prior to implantation (p < 0.001; r = 0.59). Their auditory performance correlated negatively with their age. However, the correlation was of small strength. Significant differences in auditory performance were detected between sexagenarians and octogenarians (p < 0.001; r = 0.27). Additionally, a statistically significant difference was revealed between the groups of older and younger adults in the monosyllabic word test (p = 0.001; r = 0.15). CONCLUSION: Elderly cochlear implant recipients can benefit significantly from cochlear implantation. Although higher age correlates negatively with auditory performance, its influence in the presented sample is small.

First results of electrode reimplantation and its hypothetical dependence from artificial brain maturation.

BACKGROUND: After introducing the first Cochlear Implants also in children theses are grown with electrical intracochlear stimulation and subsequent auditory cortical development. Over the meantime the positioning of the electrode was changed orientated on the development of electrode design, ability to insert atraumatic and on the widening of the indications towards highfrequency deafness. METHODS: In this pilot study we analysed five prelingually deafened patients implanted as child in the late 90's and had a reimplantation 2016 or later. We compared CT and DVT (cone beam CT) scans of the temporal bone and measured the insertion angle, the cochlear coverage, the total length of the electrode in the cochlea and the distance of the first active electrode to the round window. Moreover, we compared their speech understanding before and after reimplantation. RESULTS: The results show a lowering in the insertion angle, the cochlear coverage, the total length of the electrode in the cochlea, in the distance of the first active electrode to the round window and in the speech understanding after reimplantation. CONCLUSION: These results show a difference in the depth of insertion while the speech understanding is not significantly improving in this group-although the technology is advanced. The influence of auditory maturation with CI in these patients will be discussed.

Cognition in older adults with severe to profound sensorineural hearing loss compared to peers with normal hearing for age.

Objective: Cognitive performance of older adults with severe to profound sensorineural hearing loss and indication for cochlear implantation was evaluated compared to peers with age appropriate hearing.Design: Prospective matched case control study.Study sample: Study group consisted of n = 30 patients aged between 60 and 80 years, with adult onset, severe to profound sensorineural hearing loss on both sides and indication for cochlear implantation. Matched control group consisted of n = 30 peers with age-adjusted hearing abilities, based on age- appropriate norms.Results: Differences in Constructional Praxis and Recall, Trail Making Test A and Stroop were not significant between both groups. However, the differences in Clock Drawing Test, Word Lists and Trail Making Test B were significant. The impairment in TMT B (cognitive flexibility) was mediated via the severity of depressive problems. Cognitive performance was not related to word recognition, the percentage and duration of hearing loss and hearing aid use.Conclusion: Severely hearing-impaired older adults show widespread impairments in cognitive performance.

[Diagnosis and Therapy of Hearing Impairment and Cochlear Implant in Migrants in Germany]. / Diagnostik und Therapie von Schwerhörigkeit inkl. Cochlea-Implantat bei Migranten in Deutschland.

In addition to medical issues, the medical care of migrants is an administrative and regulatory challenge. Although it seems to be complicated, comprehensible and legal requirements and professional administrative structures are available 1. Often discussed by physicians and the media is the assumption that the medical care will be decided not by the medical professionals but the administrative authorities 2.In this paper the legal requirements are exemplified and out of the experiences of the authors, the requirements within the clinical routine work with the migrants highlighted.

[Psychosomatic aspects of balance disorders]. / Psychosomatische Aspekte von Gleichgewichtsstörungen.

Diseases in which dizziness symptoms are in the foreground are relatively common in primary care. They can have many causes, both organic and with mental involvement. In most cases, patients primarily undergo only somatic diagnostics and accordingly have delayed contact with psychosomatic medicine. However, the proportion of psychic components or causes of dizziness is relatively high. Accordingly, psychotherapy procedures can show good success rates in many patients. All in all, patients with vertigo suffer from the early addition of psychotherapeutically trained physicians.

Long-Term Outcomes, Education, and Occupational Level in Cochlear Implant Recipients Who Were Implanted in Childhood.

OBJECTIVES: To document the long-term outcomes of auditory performance, educational status, vocational training, and occupational situation in users of cochlear implants (CIs) who were implanted in childhood. DESIGN: This retrospective cross-sectional study of 933 recipients of CIs examined auditory performance, education and vocational training, and occupational outcomes. All participants received their first CI during their childhood between 1986 and 2000. Speech comprehension results were categorized using the categories of auditory performance (CAP) arranged in order of increasing difficulty ranging from 0 to 8. 174 of the 933 pediatric recipients of CIs completed a self-assessment questionnaire regarding their education and occupational outcomes. To measure and compare school education, qualifications were converted into International Standard Classification of Education levels (ISCED-97). Occupations were converted into International Standard Classification of Occupation-88 skill levels. Data from the German General Social Survey (Allgemeine Bevölkerungsumfrage der Sozialwissenschaften/ALLBUS) for 2012 were used as a basis for comparing some of the collected data with the general population in Germany. RESULTS: The results showed that 86.8% of the 174 participants who completed the survey used their devices more than 11 hr per day. Only 2% of the surveyed individuals were nonusers. Median CAP was 4.00 (0 to 8). Age at implantation was significantly correlated with CAP level (r = -0.472; p < 0.001). The mean ISCED level of the 174 surveyed recipients was 2.24 (SD = 0.59; range: 1 to 3). A significant difference (p = 0.001) between users' ISCED levels and those of respondents was found. Participants' ISCED levels and maternal educational levels were significantly correlated (r = 0.271; p = 0.008). The International Standard Classification of Occupation-88 skill levels were as follows: 5% achieved skill level 1; 77% skill level 2; 16% skill level 3; and 5% skill level 4. The average skill level achieved was 2.24 (range 1 to 4; SD = 0.57) which was significantly poorer (t(127) = 4.886; p = 0.001) than the mean skill level of the respondents (mean = 2.54; SD = 0.85). CONCLUSIONS: Data collection up to 17.75 (SD = 3.08; range 13 to 28) years post implant demonstrated that the majority of participants who underwent implantation at an early age achieved discrimination of speech sounds without lipreading (CAP category 4.00). Educational, vocational, and occupational level achieved by this cohort were significantly poorer compared with the German and worldwide population average. Children implanted today who are younger at implantation, and with whom more advanced up-to-date CIs are used, are expected to exhibit better auditory performance and have enhanced educational and occupational opportunities. Compared with the circumstances immediately after World War II in the 20th century, children with hearing impairment who use these implants have improved prospects in this regard.

Cochlear implantation in patients with definite Meniere's disease.

The exact pathomechanism of deafening in Meniere's disease (MD) is still unknown; intoxication of hair cells and neural damage from endolymphatic hydrops is discussed. In the literature, there are only a few reports on hearing outcome of MD patients after treatment with cochlear implantation (CI) whereby especially the comparison of MD vs. non-MD patients with CI differs. In this retrospective study, results in speech understanding [Freiburger Einsilber (FES65) and Hochmair-Schulz-Moser test in quiet (HSM) and in noise (HSM + 10 dB)] of 27 implanted MD patients were collected and compared to a matched standard CI cohort. Alternative diagnoses were excluded as far as possible by re-analyzing neuroradiologic imaging. After first fitting, MD patients showed significantly better results in FES and HSM testing compared to controls. At 1-year refitting, this effect could not be seen anymore. To conclude, cochlear implantation is a safe and effective treatment for deafness in MD patients. Results in speech understanding are at least equal compared to general CI recipients. To the best of our knowledge, this retrospective study examined the largest collective of CI users deafened by MD so far.

Cochlear implantation improves hearing and vertigo in patients after removal of vestibular schwannoma.

OBJECTIVES: With rising quality of speech perception in cochlear implant users, the indication widens. Nowadays, cochlear implantation is reasonable even in vestibular schwannoma patients. Speech perception with a cochlear implant is in these patients as promising as in patients with sensorineural hearing loss. However, the impact of cochlear implantation on vertigo and tinnitus after removal of vestibular schwannoma has not been investigated yet. METHODS: In a retrospective study, we analysed 12 patients treated with a cochlear implant after removal of vestibular schwannoma. RESULTS: In addition to a promising hearing perception - all patients reported improvement of vertigo. This improvement was also demonstrated by postural analysis. Improvement of tinnitus was achieved in 50% of the patients. CONCLUSION: Cochlear implantation seems a promising treatment for hearing loss, vertigo and even tinnitus in patients after removal of vestibular schwannoma. However, for successful cochlear implantation with adequate speech perception and improvement of vertigo and tinnitus, functional hearing nerve and intact inner ear anatomy is necessary.

Non-penetrating round window electrode stimulation for tinnitus therapy followed by cochlear implantation.

One main theory behind the origin of tinnitus is based on the idea that alterations of the spontaneous electrical activity within the auditory system lead to abnormal firing patterns in the affected nervous structures [1]. A possible therapeutic option is the use of electrical stimulation of the auditory nerve for the recovery or at least limitation of the abnormal firing pattern to a level that can be easily tolerated by the patient. The Tinnelec Implant consists of a single non-penetrating stimulation electrode connected to a Neurelec cochlear implant system. As a first feasibility study, before starting implantations in hearing patients, we thought to assess the potential of the Tinnelec stimulation to treat tinnitus in unilateral deaf patients, analysing hereby its effectivity and risks. Three patients suffering from unilateral tinnitus resistant to pharmacological treatment and ipsilateral severe to profound sensorineural hearing loss/deafness were implanted with a Tinnelec system between September 2007 and July 2008, at the ENT Department of Hannover Medical School. The stimulation strategy was chosen to induce alleviation of the tinnitus through suppression, masking and/or habituation and the response of each patient on the treatment was monitored using a visual analogue scale (VAS) on loudness and annoyance of tinnitus, mood of the patient, as well as the tinnitus handicap inventory (THI). All patients had a benefit from the electrical stimulation for their tinnitus (THI-score improvement of 20-70), however, not all participants profited from the Tinnelec system in same way and degree. In one patient, despite good results, the device had to be replaced with a conventional cochlear implant because of Tinnelec-independent increase in hearing loss on the contralateral ear. Additionally, due to the extension of cochlear implant indications, the devices of the other two patients have been meanwhile replaced with a conventional cochlear implant to benefit additionally from hearing improvement. As demonstrated in the present study, sensorineural tinnitus in humans may be suppressed/masked/habituated by electrical stimulation. The main advantage of the Tinnelec implant would be the option to treat patients with normal and usable hearing, stimulating the affected ear with the cochlear non-penetrating stimulation electrode of the device, and extend the treatment in cases of progressive hearing loss by explanation and reimplantation with a penetrating electrode addressing tinnitus as well as the hearing impairment. The present study is the first report on a long-term follow-up on tinnitus patients implanted with Tinnelec. Further clinical studies to implant tinnitus patients with residual or normal hearing on the affected ear are on the way.

Hearing preservation outcomes with different cochlear implant electrodes: Nucleus® Hybrid™-L24 and Nucleus Freedom™ CI422.

OBJECTIVES: In recent years, it has been possible to preserve hearing after cochlear implantation in patients with significant amounts of low-frequency residual hearing. Due to the dimensions and characteristics of the cochlear implants (CIs) Nucleus® Hybrid™-L24 and Nucleus Freedom™ CI422, both can be used to preserve residual hearing. The aim was to investigate the degree and progression of hearing preservation over a longitudinal postoperative period in a large consecutive cohort of implanted patients with preoperative residual hearing who received either the Nucleus Hybrid-L24 or the Nucleus Freedom CI422 implant. The intention was to examine potential characteristics and triggers of resulting postoperative hearing loss which may support a differentiation of CI candidacy criteria for a certain implant type. METHODS: A retrospective data analysis of patient files on consecutively implanted subjects presenting with a severe-to-profound sensorineural hearing loss at frequencies>1,500 Hz and substantial residual hearing at frequencies≤1,500 Hz, implanted with a Nucleus Hybrid-L24 (n=97) or a CI422 implant (n=100), was undertaken. A single-subject repeated-measure design comparing the mean threshold shift for pure-tone thresholds under headphones up to 24 months after implantation was used. RESULTS: Hearing preservation is observed in the majority of subjects with either implant (250-1,500 Hz frequency range). Hybrid-L24 patients exhibited a median hearing loss of 10 dB at initial fitting (n=97) and of 15 dB after 24 months (n=51). A 14.4-dB decrease in median hearing loss at initial fitting (n=100) and a 30-dB decrease after 24 months (n=28) was observed with the CI422 electrode. At initial fitting, 54.6% of the Hybrid-L24 (n=97) and 49.0% of the CI422 (n=100) subjects showed a mean threshold shift<15 dB. After 24 months, 58.8% (Hybrid-L24, n=51) and 28.6% (CI422, n=28) of the patients showed a mean threshold shift<15 dB. CONCLUSIONS: The results indicate that residual hearing was preserved for the majority of implanted patients with the Hybrid-L24 and the CI422 implant. Patients implanted with the Hybrid-L24 implant demonstrate greater stability and less median hearing loss over time than those with the CI422 implant. Assessments of onset and stability of hearing loss prior to implantation are important factors to consider during candidacy evaluation for electrode selection to potentially maximize the performance outcome for each patient.

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.

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.

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.

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.