Otology & Neurotology最新文献

{"title":"A Wait and Scan Protocol for Asymptomatic Postsurgical Cholesteatoma Recurrence: A Novel Management Strategy.","authors":"David Kiderman, Daniel Goldstein, Meital Adi, Elchanan Zloczower, Udi Katzenell","doi":"10.1097/MAO.0000000000005022","DOIUrl":"10.1097/MAO.0000000000005022","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the feasibility and safety of active MRI surveillance as an alternative to immediate surgical re-exploration in patients with small, asymptomatic postoperative cholesteatoma recurrence.</p><p><strong>Study design: </strong>Prospective single-arm pilot study.</p><p><strong>Setting: </strong>Secondary referral otology center.</p><p><strong>Patients: </strong>Patients with prior cholesteatoma surgery demonstrating postoperative recurrence ≤5 mm on non-echo-planar diffusion-weighted MRI without otologic symptoms, suspicious findings on otoscopy for cholesteatoma, or radiologic proximity to critical structures.</p><p><strong>Intervention: </strong>Structured active surveillance with serial non-echo-planar diffusion-weighted MRI scans at 12- to 18-month intervals.</p><p><strong>Main outcome measures: </strong>Surgery-free survival (SFS) is defined as the time from baseline MRI demonstrating recurrence to surgical intervention. The secondary outcome included the annual lesion growth rate.</p><p><strong>Results: </strong>Thirteen patients were included in the longitudinal analysis with a mean follow-up of 43±17 months. Ten patients (77%) remained surgery-free throughout the surveillance. Three patients (23%) required surgical intervention due to lesion enlargement or symptom development. The mean lesion growth rate was 0.32±1.0 mm/year. Three patients (23%) demonstrated spontaneous radiologic regression during follow-up. Kaplan-Meier analysis demonstrated cumulative SFS of 100% at 24 months and 91% (95% CI: 74-100) at 36 months.</p><p><strong>Conclusions: </strong>In selected patients with small, asymptomatic postoperative cholesteatoma recurrence, active MRI surveillance may safely defer surgical re-exploration. These findings support further prospective evaluation of imaging-guided surveillance strategies for the management of MRI-detected recurrence.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"1190-1195"},"PeriodicalIF":1.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148713389","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Otopathology of Pediatric Acute Tuberculous Meningitis-Case Series.","authors":"Natalia Carasek, Lucas Alves Teixeira Oliveira, MengYu Zhu, Anbuselvan Dharmarajan, Fayez Bahmad, Felipe Santos","doi":"10.1097/MAO.0000000000005026","DOIUrl":"10.1097/MAO.0000000000005026","url":null,"abstract":"<p><p>Tuberculous meningitis (TBM) is a severe presentation of the Mycobacterium tuberculosis that can cause sensorineural hearing loss (SNHL) similarly to other etiologies of meningitis. The pathophysiology for this group remains unclear. We report a series with 3 temporal bones of infants who died from TBM, showing acute damage to the inner ear, such as fibrosis of the cochlea, organ of Corti atrophy, neuronal loss and internal auditory canal with abundant pus cells, predominantly mononuclear. The cochlear aqueduct, endolymphatic sac/duct and Rosenthal's canal appear to be routes of dissemination from the subarachnoid space to the inner ear.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"e1122-e1124"},"PeriodicalIF":1.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148631152","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cochlear Implantation and Hearing Rehabilitation in CHARGE Syndrome: Expanding Clinical Knowledge and Outcomes.","authors":"Soraya Abdul-Hadi, Margaret Kettler, Loga Iyer, John S Stein, Anna Guan, Daniel I Choo","doi":"10.1097/MAO.0000000000004973","DOIUrl":"10.1097/MAO.0000000000004973","url":null,"abstract":"<p><strong>Objective: </strong>Characterize patients with CHARGE syndrome (CS) undergoing cochlear implantation (CI) and evaluate factors influencing surgical approaches and outcomes.</p><p><strong>Study design: </strong>Retrospective study.</p><p><strong>Setting: </strong>Quaternary pediatric referral center.</p><p><strong>Patients: </strong>Fifty-five patients with CS aged ≤21 years who underwent CI.</p><p><strong>Intervention: </strong>Demographic, audiologic, radiologic, operative, and postoperative data were reviewed. Outcomes included audiologic performance, device use, communication modality, and the presence of facial nerve stimulation (FNS).</p><p><strong>Main outcome measures: </strong>Surgical feasibility, anatomic characteristics, and auditory outcomes.</p><p><strong>Results: </strong>Fifty-five patients underwent CI, representing 67 ears. The cohort was 62% male, and 91% had neurodevelopmental impairment. Complete electrode insertion was achieved in 81% of ears (54/67), partial insertion in 5% (3/67), and insertion data were unavailable in 15% (10/67). Perimodiolar electrodes were used in 57% of ears. Imaging demonstrated cochlear nerve aplasia or hypoplasia in 54% of implanted ears and cochlear aperture stenosis in 25%. At analysis, 49% of patients were active CI users, while 27% had discontinued use. Audiologic threshold improvement was observed in 47% of patients, and overall clinical benefit was identified in 64% based on combined audiologic and functional measures. Ipsilateral facial nerve stimulation occurred in 15% of implanted ears; among these, 60% remained active users, while 40% discontinued CI use due to FNS unresponsive to reprogramming.</p><p><strong>Conclusion: </strong>CI is feasible and can provide meaningful benefit in patients with CS despite complex anatomic and neurodevelopmental challenges. These findings support an individualized approach to CI candidacy and offer practical guidance for surgical planning and family counseling regarding expected outcomes.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"1108-1117"},"PeriodicalIF":1.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148272545","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"NF2-related Schwannomatosis Diagnosed Before and After 30 Years of Age: Differences in Disease Presentation and Rates of Positive Genetic Testing.","authors":"Brennan G Olson, John P Marinelli, Matthew L Carlson, Brian A Neff, Colin L W Driscoll, Dusica Babovic-Vuksanovic, Lisa A Schimmenti, James R Dornhoffer","doi":"10.1097/MAO.0000000000004975","DOIUrl":"10.1097/MAO.0000000000004975","url":null,"abstract":"<p><strong>Objective: </strong>Characterize pathogenic variants, rates of mosaicism, genetic testing yield, and disease severity among patients with NF2-related schwannomatosis dichotomized by diagnosis before or after the age of 30.</p><p><strong>Study design: </strong>Retrospective analysis.</p><p><strong>Setting: </strong>Tertiary referral center multidisciplinary NF2 clinic from 2021 to 2024.</p><p><strong>Patients: </strong>Patients with NF2-related schwannomatosis.</p><p><strong>Intervention: </strong>Next-generation sequencing.</p><p><strong>Main outcome measure: </strong>Rates of mosaicism, genetic testing rates and yield, and overall disease severity by tumor burden.</p><p><strong>Results: </strong>From 2021 to 2024, there were 32 patients ≥30 years of age and 39 patients diagnosed at younger ages. Patients diagnosed ≥30 years exhibited decreased likelihood of receiving genetic testing (53% vs. 85%; P =0.009) and decreased genetic yield (defined as identification of a pathogenic genetic variant in those undergoing testing; 65% vs. 85%; P =0.20). Both age groups demonstrated similar rates of pathogenic variant type with loss-of-function being the predominant variant detected in 73% vs. 67%, for ≥30 vs. <30 years of age; P =0.90. Those ≥30 years harbored fewer number of average anatomic regions involved by tumor (2.3 vs. 3.4; P <0.001) and decreased total number of tumors (7 vs. 12; P <0.001).</p><p><strong>Conclusions: </strong>Patients diagnosed with NF2-related schwannomatosis at age ≥30 years exhibited reduced disease severity compared with those diagnosed at a younger age despite harboring similar distributions of genetic pathogenic variants inclusive of loss-of-function pathogenic variants. These observations emphasize the importance of considering patient age in addition to genetic testing for diagnostic framing tailored to patients' biology and clinical context to optimize care in the setting of NF2-related schwannomatosis.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"e1090-e1093"},"PeriodicalIF":1.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148265355","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cisplatin-Induced Hearing Loss Prevention With Intratympanic Therapy Systematic Review and Meta-Analysis.","authors":"Zachary A Kons, Calvin J Kersbergen, Deborah Goss, Aaron K Remenschneider","doi":"10.1097/MAO.0000000000005011","DOIUrl":"10.1097/MAO.0000000000005011","url":null,"abstract":"<p><strong>Introduction: </strong>Cisplatin-induced hearing loss (CIHL) is a well-described, long-term consequence of cisplatin treatment for malignancy. Intratympanic (IT) injections have been trialed to prevent CIHL in humans. To provide clarity on which agents have been studied through IT injection and to review their efficacy for hearing loss prevention, we performed a systematic review and meta-analysis.</p><p><strong>Data sources: </strong>OVID Medline, Embase, Web of Science, and Cochrane Library were queried.</p><p><strong>Methods: </strong>Databases were searched in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. Prospective randomized trials were included, and a systematic review was performed for all studies. Demographic, audiometric, and therapeutic data were collected. Random-effects models were used to compare across studies, and subgroup analyses were performed for each IT agent.</p><p><strong>Results: </strong>The initial database search yielded 1017 articles, which were screened according to inclusion and exclusion criteria. Ten studies were identified, involving a total of 284 patients. Studies included data on IT dexamethasone, IT N-acetylcysteine (NAC), and IT sodium thiosulfate (STS). Pooled analysis across all agents and frequencies did not reveal a significant difference in hearing thresholds between treatment and control ears [prediction interval [-3.77, 3.20], negative favors treatment). Subgroup analysis of IT dexamethasone [-1.74, 3.80] and IT NAC [-1.02, 4.64] also did not demonstrate significant differences. STS data were not amenable to pooled analysis; however, one study demonstrated a significant decrease in ASHA-defined ototoxicity (40% vs. 85%, P =0.0027).</p><p><strong>Conclusions: </strong>To date, no IT agent has consistently prevented CIHL, although limited data suggest that IT STS may decrease ototoxicity. More trials are necessary to fully elucidate these effects.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"1051-1060"},"PeriodicalIF":1.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148550160","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Predictive Validity of Hypothesized Risk Factors for Superior Canal Dehiscence Syndrome.","authors":"Ryan A Bartholomew, Brigette L Wang, Obinna I Nwosu, Marta M Williams, Daniel J Lee, Carleton Eduardo Corrales, Neil Bhattacharyya","doi":"10.1097/MAO.0000000000005007","DOIUrl":"10.1097/MAO.0000000000005007","url":null,"abstract":"<p><strong>Objective: </strong>To assess the predictive validity of comorbid conditions hypothesized to be risk factors of superior canal dehiscence syndrome (SCDS).</p><p><strong>Study design: </strong>Retrospective case-control study.</p><p><strong>Setting: </strong>A metropolitan health care system.</p><p><strong>Patients: </strong>Adult patients with superior canal dehiscence syndrome (n=152) and matched controls (n=438) between the years 2016 and 2021.</p><p><strong>Intervention: </strong>Diagnostic.</p><p><strong>Main outcome measures: </strong>Differences in rates of comorbid diagnoses evaluated by multivariate logistic regression: disorders of bone density (osteoporosis, osteopenia, or osteomalacia), vitamin D deficiency, hypocalcemia, Ehlers-Danlos syndrome, obstructive sleep apnea (OSA), idiopathic intracranial hypertension, history of head trauma, and BMI >25.</p><p><strong>Results: </strong>The study patients had a mean age of 57.8 years, with 62.5% females. Two diagnoses achieved significance ( P <0.05) in the multivariate logistic regression: OSA was a positive predictor of SCDS with an OR of 2.29 (95% CI: 1.15-4.54), whereas having a BMI over 25 was a negative predictor with an OR of 0.51 (95% CI: 0.29-0.89). The remaining diagnoses either did not achieve a statistically significant association or were excluded from multivariate analysis due to rarity in the cohort.</p><p><strong>Conclusions: </strong>OSA was a significant positive predictor of SCDS while elevated BMI acted as a negative predictor, suggesting that comorbid OSA may confound previously reported links between elevated BMI and SCDS.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"e1087-e1089"},"PeriodicalIF":1.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148430889","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Increased Adrenergic Receptor Expression in Meniere Disease.","authors":"Adam Y Xiao, Achilles Kanaris, Masanori Ishii, Gregory P Lekovic, Ivan A Lopez, Gail Ishiyama, Akira Ishiyama","doi":"10.1097/MAO.0000000000005010","DOIUrl":"10.1097/MAO.0000000000005010","url":null,"abstract":"<p><strong>Hypothesis: </strong>There is increased expression of adrenergic receptors in the vestibular end organs of Meniere disease.</p><p><strong>Background: </strong>Meniere disease (MD) is an inner ear condition characterized by episodic vertigo, hearing loss, tinnitus, and aural fullness. It is believed to be due to an imbalance in inner ear fluid, often leading to hydrops; however, the exact mechanism is unclear. Pupillometry data have suggested a role for autonomic dysfunction, specifically sympathetic hyperactivity, but few studies have investigated the molecular pathways involved in this.</p><p><strong>Methods: </strong>Vestibular end organs from patients undergoing labyrinthectomy for MD (n=6), as well as control specimens (n=3) from patients with vestibular schwannoma undergoing translabyrinthine resection, were collected and processed for RNA in situ hybridization using the RNAscope Multiplex Fluorescent V2 Assay with probes for β 1 -, β 2 -, and α 1a -adrenergic receptors (AR). Positive and negative control experiments were also performed for quality control. Immunofluorescence was used to characterize protein expression of α 1a -AR and to identify vestibular hair cells (VHC) in the sensory epithelium (Myosin VIIa).</p><p><strong>Results: </strong>There is increased expression of α 1a -AR transcripts in MD utricles compared with control. This appears to be concentrated in the sensory epithelium VHC, confirmed with MyoVIIa positivity, although expression can be seen in the stroma as well. This was further confirmed with immunofluorescence, demonstrating increased expression in MD. There is also transcriptional expression of β 2 -AR with no difference between the 2 groups.</p><p><strong>Conclusions: </strong>There are limited molecular studies investigating the pathogenesis of MD, especially those utilizing human surgical tissue. This is the first study to demonstrate increased expression of α 1a -AR both at the transcriptional and protein levels in MD compared with controls. These results may help explain the mechanism behind sympathetic hyperactivity and guide future therapies.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"e1100-e1105"},"PeriodicalIF":1.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148631162","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cardiovascular and Autonomic Effects of Auricular Vagus Nerve Stimulation in an Anesthetized Rat Model.","authors":"Lohane Moreira Da Costa, Bharadwaj Nandakumar, Allison Oliva, Cameron Herter, Zihan Yan, Fred F Telischi, Xue-Zhong Liu, Jeffrey J Goldberger, Patrick D Ganzer, Vivek V Kanumuri","doi":"10.1097/MAO.0000000000005017","DOIUrl":"10.1097/MAO.0000000000005017","url":null,"abstract":"<p><strong>Hypothesis: </strong>Electrical stimulation of the external ear mediates cardiovascular changes in anesthetized rats.</p><p><strong>Background: </strong>Auricular vagus nerve stimulation (aVNS) has been proposed as a noninvasive treatment for cardiovascular disease. Stimulation is typically achieved via electrodes placed on the external ear, but the exact mechanisms by which aVNS induces cardiovascular changes remains unclear. In particular, the relative contributions of sympathetic and parasympathetic pathways have not been well elucidated. In this study, we use a rodent model to determine the hemodynamic effects of stimulating two auricular regions and assess the autonomic pathways mediating these responses.</p><p><strong>Methods: </strong>Bipolar transcutaneous electrical stimulation was applied to the external ear of adult Sprague-Dawley rats. Cardiovascular parameters including mean arterial pressure (MAP), photoplethysmography (PPG), and cardiac electrical activity were recorded. Cervical vagotomies and hexamethonium sympathetic blockade were performed to assess the parasympathetic and sympathetic contributions to evoked cardiovascular responses.</p><p><strong>Results: </strong>Electrical stimulation at the concha resulted in significant changes in MAP and PPG. MAP changes were larger with stimulation in the concha compared with the anterior pinna. MAP responses increased in a dose-dependent manner with increasing current amplitude. Bilateral vagotomy resulted in persistent but decreased evoked cardiovascular responses. Complete autonomic blockade using intravenous hexamethonium resulted in the absence of any evoked cardiovascular responses with aVNS.</p><p><strong>Conclusion: </strong>Cardiovascular changes from aVNS are mediated by modulation of both parasympathetic and sympathetic activity.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"e1106-e1113"},"PeriodicalIF":1.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148707434","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Round Window Drill-Out in Advanced Otosclerosis.","authors":"Kaitlyn E Aragon, Jeffrey T Vrabec","doi":"10.1097/MAO.0000000000004989","DOIUrl":"10.1097/MAO.0000000000004989","url":null,"abstract":"<p><strong>Objective: </strong>To discuss the effect of round window drill-out for otosclerosis cases with radiographic evidence of round window obliteration.</p><p><strong>Patients: </strong>Four adult patients (5 ears) were included with audiometric findings of a mixed hearing loss and radiographic evidence of otosclerosis causing round window obliteration.</p><p><strong>Interventions: </strong>Round window obliterative disease was addressed with a formal round window drill-out (RWD) by opening the lumen of the basal turn of the cochlea with a micro drill. The opening was then reconstructed with fascia to recreate a 2-window system.</p><p><strong>Main outcome measures: </strong>Feasibility, Δ pure-tone air conduction thresholds, and Δ pure-tone average air-bone gap (PTA-ABG).</p><p><strong>Results: </strong>Three (60%) ears underwent primary or revision stapedectomy with RWD, 1 (20%) ear underwent delayed RWD years after stapedectomy, and 1 (20%) ear underwent RWD without stapedectomy. Median preoperative PTA-ABG was 31.3 dB. One patient (2 ears) had significant benefit with PTA-ABG improvements of 45 dB (left) and 30 dB (right). Two patients (2 ears) had a small improvement in PTA-ABG (2.5 dB, 1.3 dB), whereas 1 patient (1 ear) had worsening in PTA-ABG (2.5 dB).</p><p><strong>Conclusions: </strong>Patients suffering from severe mixed hearing loss due to advanced otosclerosis may benefit from formal RWD to facilitate hearing aid use as an alternative to cochlear implantation.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"e1064-e1068"},"PeriodicalIF":1.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148278314","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cochlear Implantation in Sickle Cell Disease: A Systematic Review of Feasibility and Outcomes.","authors":"Jasmine Stewart, Kenny Nguyen, Ajibola B Bakare, Lindsay Blake, Robert Saadi","doi":"10.1097/MAO.0000000000004997","DOIUrl":"10.1097/MAO.0000000000004997","url":null,"abstract":"<p><strong>Introduction: </strong>Sickle cell disease (SCD) is associated with systemic complications, including sensorineural hearing loss (SNHL) from microvascular occlusion and chronic inflammation. Although reports link SCD to higher rates of SNHL, current evidence is limited by small sample size, varied audiologic methods, and lack of standardized screening. This systematic review summarizes available literature on SNHL and cochlear implantation (CI) in SCD.</p><p><strong>Methods: </strong>A literature search of PubMed MEDLINE, Embase, Scopus, Web of Science, and CINAHL identified 79 citations. After removal of duplicates, 35 records were screened in Rayyan. Studies published between January 1, 2000, and June 30, 2025, were eligible if they reported patients with SCD who developed hearing loss and underwent CI. Nine full texts were reviewed, and 4 met the inclusion criteria. Screening and review were performed independently by 2 authors per PRISMA guidelines.</p><p><strong>Results: </strong>Across 4 case reports, a total of 5 patients with SCD underwent CI, ranging in age from 2 to 42 years. Four presented with bilateral severe-to-profound SNHL and one with unilateral loss. Implantation was technically feasible in all cases, including patients with cochlear fibrosis or ossification requiring modified insertion techniques. Postoperative outcomes were favorable: all patients demonstrated reliable device function and low impedances. Only 60% showed meaningful auditory benefit, characterized by improved functional speech perception in 2 patients (40%) and access to the speech frequency range with hearing testing going from moderate/profound hearing loss to mild hearing loss in 3 patients (60%). Complications occurred in 2 patients (40%): one developed unilateral middle ear infection leading to meningitis, and another experienced a postoperative pulmonary embolism requiring anticoagulation. The remaining 3 patients (60%) had uncomplicated recoveries with reported improved hearing from moderate/profound hearing loss to mild hearing loss post implantation.</p><p><strong>Conclusion: </strong>While CI appears feasible in SCD, our findings suggest that additional data are needed to assess its effectiveness in this patient population. However, evidence is limited to case reports, and complications such as thromboembolism and rapid cochlear fibrosis highlight the need for close perioperative management. More comprehensive studies with larger sample size are required to define surgical risk, optimize management, and establish best practices for timely implantation in this population.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"1103-1107"},"PeriodicalIF":1.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148471992","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}