Background: Meniere's Disease (MD) may present as a complication in the hearing outcomes of patients with hearing loss who are treated with either hearing aids or cochlear implants (CI). Objective: This case study aims to highlight the course of treatment for MED as well as being inflicted by MD, which was discovered after transferring care from a private practice to an academic medical center for treatment of internal failure of her left device, as indicated by manufacturer recall. Case Presentation: This patient is a long-time user of bilateral cochlear implants who has experienced multiple fluctuations in performance, most notably in her dominant right CI. Pressure changes in patients with middle ear dysfunction (MED) may impact the programming levels. Results: The patient has shown significant improvement following substantial programming changes in her right CI. She has become more aware of Meniere's attacks and is utilizing available self-adjustment of current parameters. In doing so, she is able to improve her hearing status regardless of the presence of Meniere's attacks through programming changes.

Purpose Early cochlear implantation during the critical period of neuroplasticity leads to better auditory and language outcomes but presents unique anesthetic challenges in infants. This study retrospectively examined anesthesia-related events throughout the full diagnostic and surgical process over a 10-year period, focusing on pediatic population undervent cochlear implantation – especially under the age of 18 month. Methods Data from 175 children under age 3 were analized who received cochlear implants between 2014 and 2024. Anesthesia-related events during audiological tests (BERA/ASSR), imaging (CT/MRI), and surgery were recorded. Collected variables were demographics, comorbidities, ASA and Mallampati scores, anesthetic techniques, procedure durations, complications, and ICU admissions. A subgroup analysis was conducted for patients implanted before 18 months. Results Of the 175 patients, 35 (20.2%) received implants before 18 months and 8 (4.6%) before 12 months. Anesthesia-related complications occurred in 14 cases (8.1%) in the study population, with laryngospasm during extubation being the most frequent (n = 8); all resolved without reintubation. Difficult intubation was noted in 3 patients, mainly those with craniofacial anomalies. Four children required postoperative ICU care. No complications were observed during diagnostic procedures. No significant association was found between complication rates and age, ASA status, or Mallampati score. Conclusion Cochlear implantation, even in infants under one year, is safe with appropriate anesthesia and experienced teams. Complications were rare and manageable. Recognizing anesthesia risks helps optimize care and reassure parents, supporting timely intervention within the neuroplastic window.

Objectives This study investigates whether pre-implantation cognitive function, assessed using the Repeatable Battery for the Assessment of Neuropsychological Status for Hearing Impaired Individuals (RBANS-H) correlates with speech perception oneyear post-implantation, and whether it can serve as a factor explaining unexpected outcome variability in adult Cochlear implant (CI) recipients. Methods This prospective longitudinal study involved 68 postlingually deaf CI recipients (ClinicalTrials.gov NCT05525221). RBANS-H assesses five cognitive domains: Immediate Memory (List Learning and Story Memory), Visuospatial/Constructional (Figure Copy, Line Orientation), Language (Picture Naming, Semantic Fluency), Attention (Digit Span, Coding) and Delayed Memory (List Recall, List Recognition, Story Recall, Figure Recall). RBANS-H and vocabulary tests were conducted pre-implantation. Phoneme scores were derived using Dutch consonant–vowel–consonant words-in-quiet, collected oneyear post-implantation in best-aided condition. Results Moderate positive correlations were observed for Coding (r = 0.43, p = 0.007), List Learning (r = 0.39, = 0.015), Story Recall (r = 0.43, p = 0.019), and Story Memory (r = 0.34, p = 0.030). No significant relationships were found for other tasks, domains, total scores or the vocabulary tests. Discussion Correlations of Coding, Immediate Memory and Delayed Memory tasks with speech outcomes highlight the involvement of attention and memory in perceiving speech with CI or acquiring this ability. Conclusion RBANS-H as a battery may not be sensitive enough to predict outcomes, suggesting the need for a tailored test battery that covers relevant cognitive functions and is sensitive to the specific needs of CI users.

Introduction The LittlEARS Early Speech Production Questionnaire (LEESPQ) assesses preverbal and early verbal communication in children from birth to 18 months. This study validates the Farsi LEESPQ for Iranian infants. Methods The LEESPQ was translated into Farsi and back—translated for accuracy. Content validity was reviewed by experts. A sample of 180 monolingual Farsi—speaking infants (birth—18 months; 10 per month) with typical hearing completed the questionnaire via their parents. We evaluated internal consistency, test—retest reliability, and criterion validity (correlation with age). Gender effects were also explored. Results The Farsi LEESPQ showed excellent internal consistency (Cronbach's α = 0.876). Age positively correlated with total scores ( 0.001), reflecting expected developmental progression. No significant gender differences were found. Age-specific normative percentile curves and minimal expected score thresholds were established. Conclusions The Farsi LEESPQ is a reliable and valid tool for monitoring early speech development in Iranian infants up to 18 months, expanding applicability across linguistic and cultural contexts for early detection of delays.

Introduction Labyrinthitis ossificans is a known complication of bacterial meningitis which results in severe to profound hearing loss. Historically, labyrinthitis ossificans was considered a contraindication for cochlear implantation. However, with advances in technology, cochlear implantation is an accepted treatment for hearing loss in labyrinthitis ossificans. Materials and methods 17 cases with varying degrees of ossification in the cochlea were evaluated and treated at our tertiary referral hospital and cochlear implantation center out of a total of 832 profoundly deaf patients evaluated from January 2007 to June 2023. The etiology, radiology, intra-operative findings and treatment protocols followed in these patients are discussed. Results 15 of the 17 patients could successfully be implanted with a cochlear implant, while two were referred to another center for Auditory Brainstem Implantation. The degree/grade of ossification decided the available cochlear lumen for implantation and hence the surgical approach and choice of the electrode. Conclusion A protocol for the order of choice of electrode and site of insertion in various cases of labyrinthitis ossificans depending on the degree of ossification is proposed. In the order of preference, it would be thus: 1. Cochlear implantation with the active electrode in the scala tympani of the basal turn; 2. Electrode in the scala vestibuli of the basal turn; 3. Anterograde insertion of the implant from the second turn; 4. Retrograde insertion of the implant from the second turn; 5. Insertion of a split array electrode and finally 6. Auditory brainstem implantation.

Objective Fixation of the stimulating probe of auditory brainstem implants (ABI) is crucial to maintain beneficial stimulation. Use of Tetrafluorethylen (PTFE, TeflonTM) sheets as a fixation material was compared to fixation without PTFE-sheets. Methods Setting Tertiary referral center. Study Design Retrospective analysis. Patients 46 children with sensorineural hearing loss, not eligible for CI, received an ABI; 27 children without PTFE (Control Group, CG), 18 children with PTFE (TeflonTM Group, TG). Mean age of CG (46.6 months), TG (37.4 months), n.s. Mean follow-up 49.3 months (CG), 33 months (TG), n.s. Main Outcome Measures Number of stimulating probe dislocations, number of active electrodes, electrically evoked auditory-brainstem-response (eABR) morphology performed intraoperatively and postoperatively, maximum comfortable level (MCL), categories of auditory-performance (CAP) scores. Results TG showed no electrode dislocations, CG had one. eABR waves varied from 2 to 5 in both groups, number of active electrodes was similar. Mean MCL was sign. lower in TG (49.2 nC) vs CG (98.6 nC). CAP scores were sign. better in TG (5.0) vs CG (4.2), after 2 years and 5.4 vs 4.6 after 3 years post-activation. Conclusion PTFE sheets proved to be an effective and stable fixation material for ABI stimulating probes, resulting in potentially better auditory outcomes as indicated by significantly lower MCLs and improved CAP scores. Compared to other fixation materials used in the CG, PTFE-sheets appear to be the better choice.

Objectives To perform a systematic literature review (SLR) on hearing and quality-of-life (QoL) benefits of bilateral versus unilateral cochlear implants (CIs) in adults with sensorineural hearing loss (SNHL), and to quantify benefits through a meta-analysis when feasible. Methods Embase, MEDLINE, and Cochrane were searched from January 2005–June 2022. Eligible studies presented effectiveness of CIs and/or QoL of adult CI users. Articles that presented numerical point estimates for, or numerical difference between, bilateral and unilateral CI, and measures of uncertainty were considered for meta-analysis, with data analyzed using fixed- and random-effects models. Results Thirty-five articles were included. There was a trend of significant improvement with bilateral CIs in sound localization and binaural redundancy. A meta-analysis confirmed statistically significant benefits in speech perception (in quiet, 12.6 percentage point [95% confidence interval: 7.1, 18.1] improvement; in noise, improvement in speech reception threshold of 1.5 dB signal-to-noise ratio [–1.5 dB; 95% confidence interval: −2.5, −0.4]). There were beneficial trends of bilateral CIs regarding head-shadow effect and binaural release from masking. Significant improvements in hearing-specific QoL were observed; generic QoL was mostly unchanged. Discussion Bilateral CIs provide audiological benefit over unilateral CI, resulting in hearing-specific QoL improvements. Lack of changes in generic QoL may reflect that instrument domains are insensitive to hearing-related changes when going from unilateral to bilateral CIs. Meta-analyses could not be performed for all hearing and QoL outcomes owing to variability in methodologies across studies. Conclusions These findings should be used to inform clinical decision-making to ensure the best outcomes for adults with bilateral SNHL.

Objective Neurophysiologic measures like the electrically evoked Stapedius Reflex Threshold (eSRT) offer valuable insights into personalized responses to Cochlear Implant (CI) stimuli. This study evaluates the effects of stimulation current amplitude and the number of stimulating electrodes on eSRT and its correlation with the Most Comfortable Loudness level (MCL or C-level) in all-polar, pseudo-monophasic, pulse-width loudness coded stimulations. Approach The study was conducted on seventeen adult patients with Oticon Medical implants. In these implants, current amplitude remains constant while loudness is coded through pulse-width. Stimulation amplitudes were set to low (default clinical) and high values, using single or multi electrode groups of 1, 3, and 5 (G1, G3, and G5) across five cochlear regions, spanning from apical to basal. For each amplitude and group, the eSRT detection rate and correlation with MCL were analyzed regionally and overall. Results Higher eSRT detection rates were observed in the contra-lateral ear, with G3 and G5 significantly enhancing detection over G1 for both low and high amplitudes. The current amplitude facilitated eSRT invocation, particularly for G1 and patients with higher MCLs. The correlation between eSRT and MCL was relatively high (r = 0.64–0.87) across all conditions, indicating accurate MCL estimation from eSRT at both low and high amplitudes. Discussions Among all condition interactions (amplitude, side, electrodes), low-amplitude contra-lateral G5 and high-amplitude contra-lateral G3 showed optimal modes for estimating MCL from eSRT, with linear slopes near 1.0, and detection rates of 54% and 63%, respectively. High-amplitude contra-lateral G5 further increased detection to 68%, though with a slope of 0.8, requiring a correction factor. These findings provide insights into eSRT mechanisms and its application in pseudo-monophasic all-polar CI programming. Conclusion The eSRT detection rate can be improved in pulse-width modulation cochlear implants by using multi-electrode stimulation and/or increasing the stimulation amplitude beyond the default clinical value, without significantly affecting the correlation coefficient between eSRT and MCL.

Objective Early diagnosis, intervention and consistent follow-up of hearing loss is of great importance in children, given the broad impact of untreated childhood hearing loss. Currently, no hearing-specific QOL proxy questionnaire exists for preschool children with hearing loss in the Dutch language. Therefore, the aim of this study was to translate and validate the Preschool HEAR-QL questionnaire into Dutch. Methods Translation was conducted according the forward–backward procedure. Parents or caregivers of 60 normal-hearing children and 50 children with bilateral hearing loss aged 2–6 years old were invited to digitally complete the questionnaire. Results The Preschool HEAR-QL questionnaire showed good discriminant validity between the normal-hearing and the hearing loss group, for its total score and some subscales. We concluded satisfying internal consistency given Cronbach’s alpha values being above 0.70, nearly all alpha if item deleted values approaching the overall values, and item-subscale correlations above 0.30. Test-retest reliability was satisfying with all intraclass correlation coefficients being greater than 0.70. Conclusion This Dutch hearing-specific QOL proxy questionnaire can play an important role in the management and follow-up of early childhood hearing loss in Dutch health care and hearing centers. Future research on clinical data will further verify its utility in clinical practice.

Objectives: Hearing implants often limit the assessment of magnetic resonance examinations due to susceptibility artefacts. Our aim was to evaluate the impact of artefacts attributed to the Osia®2 implant system in terms of utility in visualizing selected cranial structures. Methods: A BI300 implant and an OSI200 actuator were implanted into a human cadaver head in the audiologically most favourable position according to the manufacturer’s guidelines. Scanning was accomplished using the institutional head and inner ear protocol with a General Electric 1.5 Tesla scanner with retained and removed implant magnet, extended with T1 and T2 weighted sequences with metal-artefact reduction (MAVRIC SL). Image quality was evaluated by three radiologists. Results: The Osia®2 produced significant artefacts in most of the series of standard imaging sequences predominantly on the ipsilateral side of the head. The majority of the artefacts were caused by the implant magnet. Even without removing the magnet, MAVRIC SL improved image quality to such an extent that it became comparable with that after magnet removal. Conclusions: The standard sequences suffer considerable quality loss due to the artefacts, attributed predominantly to the magnetic component. Metal-artefact reduction sequences are effective in obtaining sufficient-to-good quality images without surgical magnet removal.