Search Results (143)

Objectives: Incomplete partition type III is a rare malformation of the inner ear characterized by the absence of the modiolus, fixation of the stapes, and significant communication between the cochlea and the internal auditory canal (IAC). During cochlear implant (CI) surgery, there is a risk that the electrode array may enter the IAC, resulting in non-specific stimulation of auditory neurons or other nerve fibers inside the IAC. In this study, we present our experience with CI surgery in patients with incomplete partition type III and report the types of devices utilized. Methods: A retrospective analysis was conducted on all our patients with cochlear incomplete partition type III who underwent surgical cochlear implantation in a tertiary referral unit over the past 16 years. Results: Of the perimodiolar electrodes used initially, four were correctly positioned within the cochlea from initial insertion, and no reinsertion was necessary. A lateral wall electrode array CI was selected to replace the last failed perimodiolar insertion, but it also required two insertion attempts due to its initial misplacement into the internal auditory canal. Conclusions: In this study, electrode array selection was influenced by surgeon preference and by intraoperative factors such as the severity of the gusher. Our observations suggest that perimodiolar CIs can be used in selected cases of incomplete partition type III and did not appear to increase the risk of IAC misplacement compared with the lateral wall array used in one patient. However, these findings should be interpreted cautiously as observations from the authors’ experience rather than as generalized conclusions regarding the relative safety of perimodiolar versus lateral wall electrode arrays. Lateral wall arrays may also be misplaced into the IAC, and their use may be considered in cases with severe and difficult-to-control gusher. Full article

Background/Objectives: For patients with profound deafness resulting from auditory nerve pathology, as in Neurofibromatosis type 2, auditory brainstem implantation (ABI) can restore meaningful acoustic input. The literature reporting real-world results for ABI users is limited, especially regarding patients with bilateral implants. Here, we provide an updated report on the audiologic outcomes among all ABI patients treated at a tertiary institution, including high-performing bilateral ABI users. Methods: In this updated and expanded retrospective case series, audiologic outcomes were reviewed in sixteen consecutive patients who underwent ABI placement by a single neurosurgeon-neurotologist team at our center since 2018. Implantation in four of these patients was on their second side after having undergone first side implantation prior to receiving care at our hospital. Main outcome measures were sound awareness (sound-field threshold testing) and speech understanding (pattern perception, spondee, open-set speech testing). Results: Sound awareness was achieved in 100% of patients (16/16) using an average of 12 electrodes (range 7–20). Persistent non-auditory sensations were reported by 12.5% (2/16). Postoperative speech differentiation (with or without lip-reading) was experienced in 87.5% (14/16). Two second-sided ABI recipients experienced exceptional outcomes as high-performing outliers: one achieved 57% audio only and 86% audio + visual hearing in noise test (HINT) sentence scores; the second bilateral user scored 92% with auditory-only input. Conclusions: ABI represents a viable option for patients who are at risk of developing bilateral profound deafness resulting from auditory nerve disruption. Second sided device implantation is safe and has the potential to significantly improve auditory outcomes. Full article

Background/Objectives: Auditory neuropathy spectrum disorder (ANSD) is a well-established hearing disorder in the pediatric population and is estimated to account for at least 10% of children with sensorineural hearing loss. Compared to auditory function, vestibular function in children with ANSD has not been well described in the past. The purpose of this study is to examine vestibular testing results in children with ANSD and to better characterize vestibular dysfunction in these children. Methods: A retrospective review of vestibular laboratory testing results was conducted in pediatric patients diagnosed with ANSD. Vestibular evaluation included vestibular evoked myogenic potential (VEMP), rotary chair test, video head impulse test (vHIT), and videonystagmography (VNG). Results: A total of 30 pediatric patients with ANSD were identified, including 18 boys and 12 girls, with a mean age of 4.5 years. Bilateral ANSD was found in 24 cases, while 6 cases were unilateral. Etiologies of ANSD included a history of hyperbilirubinemia in infancy, cochlear nerve dysplasia, genetic-related conditions, etc. Vestibular dysfunction was found in 12 cases, as indicated by at least one abnormal outcome in VEMP, vHIT, or rotary chair testing. Nineteen children were cochlear implant candidates and eventually underwent unilateral or bilateral implantation. Conclusions: Vestibular dysfunction is significant in pediatric patients with ANSD, and vestibular outcomes appear to be related to underlying etiologies. Formal vestibular evaluation is necessary to identify such vestibular losses, and these findings will be helpful to guide clinical management and rehabilitation strategies for these children. Full article

Auditory Brain–Computer Interfaces (BCIs) constitute the vital intervention for profound sensorineural hearing loss where the auditory nerve is compromised, yet their clinical efficacy remains restricted by substantial biological bottlenecks and limited spectral resolution. This review critically examines the evolutionary paradigm of auditory restoration, tracing the transition from static physical replacement to dynamic biological symbiosis. We systematically analyze physiological barriers across cochlear, brainstem, and cortical levels, elucidating how rigid interfaces provoke chronic tissue responses and why linear encoding protocols fail in distorted central tonotopy. The article synthesizes emerging methodologies in material science, demonstrating how soft, bio-integrated electronics and biomimetic topologies effectively address mechanical impedance mismatches. Furthermore, the trajectory of neural encoding is evaluated, highlighting the paradigm shift from traditional envelope extraction to deep learning-driven non-linear mapping and adaptive closed-loop neuromodulation. Finally, the potential of high-resolution modulation techniques, including optogenetics and sonogenetics, alongside AI-facilitated intent perception for active listening, is assessed. It is concluded that future neuroprostheses must evolve into symbiotic systems capable of seamlessly integrating with neural plasticity to enable high-fidelity cognitive reconstruction. Full article

Objective: The aim was to evaluate cochlear implantation (CI) outcomes in children with Down syndrome (DS) with severe-to-profound sensorineural hearing loss (SNHL), addressing a literature gap and discussing challenges including anatomical abnormalities, cognitive deficits, and Eustachian tube dysfunction. Data Sources: Systematic searches were conducted in PubMed, Web of Science, Scopus, and Embase from inception through to June 2025. Review Methods: A systematic review adhering to PRISMA guidelines was performed. Included studies reported CI outcomes in DS patients receiving otolaryngologic care for SNHL. Extracted data included findings on ear anatomy, auditory performance, speech/language development, intelligibility, and duration of CI use. Results: A total of 149 abstracts were screened, yielding six studies with 26 patients that met the inclusion criteria. The review included pediatric DS patients with documented ages at implantation spanning from 11 months to 17.9 years. CI provided significant benefits for DS patients, including improved audiometric results, enhanced environmental awareness, and psychosocial gains. Optimal outcomes were associated with early implantation, thorough preoperative imaging (CT/MRI), and management of middle ear disease. Variability in outcomes often reflected cognitive limitations and anatomical challenges such as cochlear nerve hypoplasia and Eustachian tube dysfunction. Conclusions: CI can significantly improve quality of life and communication in children with DS when tailored to their unique needs. Preoperative imaging is essential to assess candidacy, and middle ear disease should be addressed prior to surgery. Clinicians should counsel families with individualized goals that emphasize functional hearing gains over normative speech benchmarks. Broader adoption of CI in this population may be supported by standardized, population-sensitive outcome measures and future prospective studies. Full article

Background/Objectives: A Cochlear implantation (CI) is well-established auditory rehabilitation for severe to profound sensorineural hearing loss (SNHL), yet outcomes vary widely among implantees. Even with advancements in surgical methods and device technology, CI is still commonly applied as a generally uniform procedure, with limited attention to the underlying cause of SNHL. This review aims to summarize current evidence supporting etiology-based personalization of CI and to examine how etiology influences electrode selection, implantation timing, and clinical outcomes. Methods: We reviewed clinical and translational studies focusing on congenital cytomegalovirus infection, genetic hearing loss, cochlear nerve deficiency, and inner-ear malformations, emphasizing how etiology influences cochlear anatomy, neural integrity, and CI outcomes. Results: Etiology significantly affects neural survival, cochlear anatomy, and auditory plasticity, all of which influence optimal electrode design, insertion strategy, and timing of CI. Tailoring CI approaches to specific etiologies may help explain the substantial variability in outcomes observed in both children and adults. Conclusions: CI should be viewed as a precision-based intervention rather than a uniform treatment. Integrating etiology into clinical decision-making is essential for advancing truly personalized CI. Full article

Background: Occipital nerve stimulation (ONS) is an effective therapy for patients with refractory chronic cluster headache (rCCH); however, it is not without complications, and to date, there are no conclusive findings regarding factors that would allow the prediction of treatment response. The primary objective of this study is to identify such factors to improve patient selection. Methods: This single-center prospective observational study will be conducted at the Department of Neurology, Hospital Universitario La Paz (Madrid, Spain). Given the low prevalence of rCCH, a convenience sampling approach will be adopted, with an expected enrollment of a minimum of 15 patients over 24 months of the study. The study is structured into three periods: Pre-ONS (pre-implantation), ONS (implantation), and Post-ONS (follow-up at 12 months). During the pre-implantation phase, patients will undergo a multidimensional assessment encompassing structural 3T brain magnetic resonance imaging (MRI), blood analysis (calcitonin gene-related peptide (CGRP), pituitary adenylate cyclase-activating peptide 38 (PACAP38), and vasoactive intestinal peptide (VIP)), neuropsychological evaluation, auditory evoked potentials, algometry (pressure pain threshold, temporal summation, conditioned pain modulation), and transcutaneous electrical nerve stimulation (TENS). Follow-up visits will be conducted at 3, 6, and 12 months post-implantation. Results: This study aims to identify biomarkers or their combinations capable of reliably predicting patients who would benefit from ONS. Conclusions: Through this multidimensional assessment, this study seeks to identify predictive factors of response to ONS, thereby improving patient selection, optimizing healthcare resources, and advancing the understanding of treatment response mechanisms. Full article

Background/Objectives: Cochlear implants (CIs) constitute a viable method for auditory rehabilitation in patients with profound sensorineural hearing loss after temporal bone fractures (TBFs). These patients comprise a challenging population due to the anatomical deformity and neural injury. Methods: By performing this systematic review, we attempted to evaluate the viability of CIs in the context of TBF. The literature search, across Pubmed/MEDLINE, Scopus and Google Scholar, was performed under the PRISMA guidelines. The selected time period was from December 1995 to September 2025. The final analysis included 11 manuscripts. The majority of the studies were retrospective case series with a moderate risk of bias. Results: The primary outcome was postoperative auditory function, evaluated with speech perception tasks and aided sound-field pure-tone audiometry. The secondary outcomes were the report of radiological and electrophysiologic prognosticators of implants’ viability, timing of surgery, procedural feasibility and complications. Across the studies, CIs conferred meaningful auditory benefit when the cochlear nerve was intact. High-Resolution Computed Tomography (CT) was utilized for TBF classification and cochlear patency, whereas Magnetic Resonance Imaging (MRI) and a promontory test were crucial for the assessment of neural integrity. Prompt placement, optimally within 12 months after trauma, was related to improved outcomes by limiting cochlear fibrosis and ossification. Despite patients’ impedance fluctuation, restricted speech perception in noise and frequent abnormal facial nerve excitation, the overall audiologic and speech discrimination results are comparable to non-trauma recipients. Conclusions: A CI appears to be the choice of treatment over auditory brainstem implants, as long as the cochlear nerve remains intact. Rapid implantation in well-selected patients coupled with ordinal mapping and follow-up can restore dysfunctional hearing and improve patients’ quality of life. Full article

Long COVID-19 syndrome (or so-called post-COVID-19) is indicated by miscellaneous symptoms, usually starting 3 months from the COVID-19 infection and lasting for at least 2 months, which cannot be explained by an alternative diagnosis. There has been more and more reports addressing the audiovestibular dysfunction related to long COVID-19 syndrome. Emerging evidence suggests that the linkage between audiovestibular dysfunction and long COVID-19 syndrome might rely on (a) direct inner ear system damage related to viral invasion and consequent inflammation, (b) micro thromboembolic events, which might result from the COVID-19-induced autoimmune reaction against endothelial cells, and consequent transient-ischemia and hypoxia of the auditory pathways, (c) the disturbed nerve conduction in vestibulocochlear nerves due to viral invasion, and finally (d) altered auditory cortex function, either imbalanced central gain or neurotransmitter disturbance. However, most of the aforementioned mechanism remained hypothetic and still needed further studies to approve or refute. This systematic review synthesizes current evidence on the characteristics, pathophysiology, diagnostic approaches, and management of audiovestibular dysfunction related to long COVID-19 syndrome. Literature searches across PubMed, Embase, ClinicalKey, Web of Science, and ScienceDirect (up to 15 December 2025) were conducted in accordance with PRISMA guidelines. Through this systematic review, we provided a schematic diagram of the physiopathology of long COVID-19 syndrome-related audiovestibular dysfunction. Further, we summarized the currently available diagnostic tools to explore the audiovestibular function in such patients. The currently available treatment, either pharmacotherapy or nonpharmacotherapy, mainly tackles idiopathic audiovestibular dysfunction but not specifically long COVID-19 syndrome-related audiovestibular dysfunction. Timely recognition and intervention may prevent progression to permanent hearing loss or vestibular disability, improving quality of life. Trial registration: PROSPERO CRD420251265741. Full article

(1) Background: Hemifacial spasm (HFS) is most commonly caused by neurovascular compression at the root exit zone (REZ) of the facial nerve; however, isolated compression along the distal cisternal segment is uncommon and remains poorly characterized. This study aimed to analyze the clinical features, intraoperative neurophysiological patterns, and surgical outcomes of patients with HFS caused by cisternal segment arterial compression. (2) Methods: Among 874 patients who underwent microvascular decompression (MVD) for HFS, 18 (2.1%) were identified as having isolated neurovascular conflict at the cisternal segment, all involving the anterior inferior cerebellar artery (AICA). Clinical characteristics, offender location, intraoperative monitoring results including lateral spread response (LSR), brainstem auditory evoked potentials, and postoperative outcomes were retrospectively evaluated. A standardized Teflon interposition technique was used in all cases. (3) Results: Postoperatively, 83.3% of patients experienced immediate spasm relief, and at the latest available follow-up, 94.4% achieved significant improvement without severe complications. (4) Conclusions: Although rare, cisternal segment arterial compression can produce typical HFS and should be considered when REZ compression is unclear or when intraoperative neuromonitoring does not respond as expected. Microvascular decompression using Teflon interposition is a safe and effective treatment option for this anatomically challenging offender location. Full article

NF2-related Schwannomatosis (NF2-SWN) remains a disorder with few effective treatment options. Patients develop vestibular schwannomas (VSs) on both auditory nerves, which gradually impair hearing and often result in significant communication difficulties, social withdrawal, and higher rates of depression. Progress in understanding NF2-SWN biology and translating discoveries into therapies has been slowed by the absence of robust animal models that faithfully reproduce both tumor behavior and the associated neurological deficits. In this review, we summarized the development of animal models that not only reproduce tumor growth in the peripheral nerve microenvironment but also reproduce tumor-induced neurological symptoms, such as hearing loss and ataxia. We further highlight the currently available organotypic models for NF2-SWN. Together, these systems provide an essential foundation for advancing mechanistic studies and accelerating the development of effective therapies for this devastating disorder. Full article

Background: Sudden, non-traumatic hearing loss has been associated with vascular or inflammatory disorders. Hearing loss in Neuromyelitis optica spectrum disorder (NMOSD) is a very rare presentation. Methods: In this paper, we describe the case of a 58-year-old female patient with aquaporin-4-positive NMOSD exhibiting bilateral tinnitus and right-sided deafness in the context of a relapse. The auditory brainstem responses pointed to a lesion of the right peripheral auditory pathway (cochlea and/or auditory nerve). The patient’s hearing failed to improve after high-dose intravenous steroids; however, it showed slight improvement after plasmapheresis. We also conducted a systematic literature review in databases MEDLINE and Scopus in English, searching for all reported cases of hearing loss in NMOSD. Results: We included 10 studies reporting 15 cases of NMOSD with hearing loss. The vast majority of patients were female (11 out of 15, 73.3%), with an age range of 26 to 70 years. Hearing loss, ranging from mild to severe, seems more frequent in AQP4-positive cases, and it can even be the presenting symptom. It can present isolated or in combination with tinnitus, ataxia, and/or intractable vomiting. The auditory pathway impairment in NMOSD seems to be localized either centrally, i.e., cochlear nuclei or higher brainstem levels, or peripherally, i.e., in the cochlea or cochlear nerve itself. Intravenous methylprednisolone in high doses, followed by oral tapering, was the most common treatment option, resulting in a gradual improvement. Conclusions: This paper describes a rare case of peripheral auditory pathway affection in NMOSD, which is an inflammatory astrocytopathy mainly affecting the central nervous system. Early recognition of hearing loss in the context of an NMOSD relapse and subsequent treatment have a crucial impact on the hearing outcome of NMOSD patients. This expands our knowledge of NMOSD as an autoimmune aquaporin-4 channelopathy. Full article

Background: Narrow duplicated internal auditory canal (IAC) is a rare congenital malformation frequently associated with severe-to-profound sensorineural hearing loss. Case Presentation: We present a one-year-old girl with bilateral narrow duplicated IAC and profound hearing loss evaluated through CT/MRI and electrically evoked auditory brainstem response (EABR). Methods: We conducted a systematic review (1990–2023), identifying 59 published cases of which 24 were bilateral. The mean age at diagnosis was 10.34 years, and 25 cases presented additional inner ear malformations. Only seven patients underwent cochlear implantation, and EABR was performed in four cases. Outcomes of cochlear implantation were heterogeneous. Discussion: In our case, EABR showed a reproducible wave V on the right side, supporting candidacy for cochlear implantation which led to positive early auditory responses. Conclusions: This case and review highlight the role of EABR in identifying residual cochlear nerve functionality and guiding candidacy for cochlear implantation in narrow duplicated IAC. Full article

Background and Objectives: To evaluate the surgical management and outcomes of lateral semicircular canal fistulas (LSCFs) in patients with middle ear cholesteatoma, focusing on hearing preservation and vestibular function. Materials and Methods: A retrospective study was conducted on nine adult patients diagnosed with LSCFs secondary to cholesteatoma who underwent surgery at a tertiary referral center between 2018 and 2024. The preoperative evaluation included otoscopy, audiometry, vestibular testing (HINTS), and high-resolution CT (HRCT) of the temporal bone. Surgical techniques included canal wall up (CWU) or canal wall down (CWD) mastoidectomy, depending on the disease extent. Cholesteatoma matrix removal from the fistula was performed carefully. Fistula closure involved layered grafts of temporalis fascia, temporalis muscle, and/or gelfoam. The postoperative follow-up included audiometry and vestibular assessments. Results: Nine patients with LSCFs were identified (one Type III, three Type IIb, and four Type I/IIa). Five patients were found to have additional disease complications intraoperatively, including facial nerve involvement and middle fossa dehiscence. Postoperatively, hearing outcomes varied, with some patients experiencing improvement, others demonstrating stable hearing, and some exhibiting further decline, particularly in cases with extensive disease. Vestibular symptoms, including vertigo, generally resolved postoperatively, although some patients required prolonged vestibular rehabilitation. Conclusions: LSCF management in cholesteatoma surgery requires a careful preoperative assessment, meticulous surgical technique, and individualized fistula closure based on the size and type. While hearing preservation remains a challenge, particularly in extensive cases, the “underwater technique” and layered grafting may contribute to minimizing further damage and promoting fistula closure. Vestibular rehabilitation plays a crucial role in managing postoperative balance issues. A long-term follow-up is essential to monitor for recurrence and assess both auditory and vestibular function. Full article

Background/Objectives: Tinnitus lateralization, a perceptual characteristic often neglected in clinical assessment, may reflect underlying auditory plasticity. This study aimed to investigate whether changes in tinnitus lateralization following a multimodal neuromodulation protocol are associated with improved clinical outcomes, particularly regarding tinnitus severity and discomfort. Methods: A retrospective interventional study was conducted with 104 adults diagnosed with chronic tinnitus. All participants underwent a combined protocol involving transcutaneous auricular vagus nerve stimulation (taVNS), cervical transcutaneous electrical nerve stimulation (TENS), and photobiomodulation (PBM) targeting auditory pathways. Clinical assessments included the Tinnitus Handicap Inventory (THI), Visual Analog Scales (VAS) for loudness and discomfort, and lateralization reports before and after treatment. Lateralization patterns were categorized and compared using ANOVA and Kruskal–Wallis tests. Linear models explored demographic and clinical predictors of symptom change. Results: Substantial changes in lateralization were observed post-treatment. Participants who shifted from bilateral to unilateral tinnitus or from unilateral to non-perception showed the greatest symptom reductions (p < 0.001). The Bilateral → Unilateral group presented the most marked THI reduction (−20.82 ± 7.12), while minimal changes were observed in the Bilateral → Bilateral group. Loudness and discomfort improvements followed similar trends. No significant influence of age or sex on clinical response was identified, whereas longer tinnitus duration showed a modest positive association with symptom improvement. Conclusions: Perceptual reorganization of tinnitus lateralization may serve as a clinical marker of response to neuromodulation. Tracking lateralization could provide a simple, cost-effective adjunct to outcome monitoring in tinnitus management. Full article