Otology & Neurotology最新文献

{"title":"Speech, Timbre, and Pitch Perception in Cochlear Implant Users With Flat-Panel CT-Based Frequency Reallocations: A Longitudinal Prospective Study.","authors":"Melanie L Gilbert, Rebecca M Lewis, Mickael L D Deroche, Nicole T Jiam, Patpong Jiradejvong, Jonathan Mo, Daniel L Cooke, Charles J Limb","doi":"10.1097/MAO.0000000000004595","DOIUrl":"https://doi.org/10.1097/MAO.0000000000004595","url":null,"abstract":"<p><strong>Hypothesis: </strong>To determine whether chronic use of experimental computed tomography (CT)-based frequency allocations would improve cochlear implant (CI) user performance in the areas of speech and music perception, as compared to the clinical default frequency mapping provided by the CI manufacturer.</p><p><strong>Background: </strong>CIs utilize default frequency maps to distribute the frequency range important for speech perception across their electrode array. Clinical default frequency maps do not address the significant frequency-place mismatch that is inherent after cochlear implantation, nor the variability between individual anatomy or array lengths. Recent research has utilized postoperative high-resolution flat-panel CT imaging to measure the precise location of electrode contacts within an individual's cochlea, in order to generate a custom frequency map and decrease the frequency-place mismatch.</p><p><strong>Methods: </strong>A cohort of 10 experienced CI users (14 CI ears) was recruited to receive CT scans and then use an experimental CT-based frequency map for 1 month. The efficacy of these maps was measured using a battery of speech and music tests.</p><p><strong>Results: </strong>No change in speech or music performance between the Experimental and Clinical Maps was found at the group level, although there was large variability within the cohort. Greater benefit from the Experimental Map on speech in quiet tasks was correlated with better electrode array alignment in the apical (low frequency) region (rho14 = -0.55 to -0.72, p < 0.05).</p><p><strong>Conclusion: </strong>This application of strict CT-based mapping was most beneficial for CI users with the least amount of apical-mid array frequency-place mismatch, and least beneficial for CI users with overly deep or shallow insertions. Results may be limited by long acclimation periods to clinical default frequency maps prior to CT map usage, intervention bias, and small sample size.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675436","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":"Development and Evaluation of a Novel Transcanal Catheter for Delivery of Hypothermia to the Inner Ear.","authors":"Pavan S Krishnan, Maria Fernanda Yepes, Curtis S King, Suhrud M Rajguru","doi":"10.1097/MAO.0000000000004596","DOIUrl":"https://doi.org/10.1097/MAO.0000000000004596","url":null,"abstract":"<p><strong>Abstract: </strong>Mild therapeutic hypothermia (MTH) has demonstrated neuroprotective effects in the cochlea, particularly against noise-induced and electrode insertion trauma, by reducing inflammation and oxidative stress. Prior cadaveric studies have shown that localized cochlear cooling can be achieved using a probe placed on the promontory or a surface cooling device placed on the mastoid. While the effects of MTH on the vestibular system remain unstudied, its proximity and physiological similarity to the cochlea suggest potential benefits. We aimed to develop the first noninvasive, localized MTH device for use in the clinical environment, capable of targeting both cochlear and vestibular structures without obstructing the surgical field. A custom-designed, saline-filled cooling catheter with a balloon tip was designed to be positioned in the ear canal adjacent to the tympanic membrane. Temperature measurements were recorded from the round window, oval window, and all three semicircular canals, and compared to whole-head temperature fluctuations measured via the nasopharynx in human cadaver samples. Thermistors recorded an average temperature reduction of 4-6°C during a 30-minute protocol in cadaver heads. Nasopharyngeal temperature remained stable throughout. Furthermore, a numerical model was used to evaluate the theoretical temperature reduction achieved through ear canal cooling. The computational model further validated the experimental measurements from the cochlea. In conclusion, these findings demonstrate that cochlear and vestibular hypothermia can be effectively induced using an external cooling system positioned in the ear canal, supporting the development of a more accessible and practical clinical approach to protect the inner ear during invasive procedures.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675427","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":"Single-Sided Deafness and Cochlear Implants: Performance in a Novel Combined Speech-in-Noise and Localization Task.","authors":"Nadine I Ibrahim, Obada Abdulrazzak, Chioma Anidi, Gerilyn Jones, Madison V Epperson, Anahita H Mehta, Renee M Banakis Hartl","doi":"10.1097/MAO.0000000000004537","DOIUrl":"https://doi.org/10.1097/MAO.0000000000004537","url":null,"abstract":"<p><strong>Objective: </strong>This study aims to analyze the impact of single-sided deafness (SSD) on listening behavior to evaluate sound localization ability, speech-in-noise performance, and quantifying and comparing compensatory head movements in individuals with normal hearing (NH) and SSD, with and without a cochlear implant (CI).</p><p><strong>Study design: </strong>Nonrandomized, prospective, human-subject study.</p><p><strong>Setting: </strong>Tertiary academic medical center.</p><p><strong>Methods: </strong>NH, SSD, and SSD-CI subjects were presented with Harvard IEEE sentences at varying target azimuths in a darkened, semi-anechoic chamber in background noise while head position was monitored. Head movement (total absolute head displacement, onset delay, and response time), localization accuracy, and speech-in-noise performance were analyzed.</p><p><strong>Results: </strong>SSD subjects demonstrated less accurate speech-in-noise and sound localization performance with a significant effect of hearing status and signal-to-noise ratio (SNR). Sound localization benefit with CI was limited and did not improve, with increasingly optimal SNR and speech-in-noise performance reaching the level of NH controls with more optimal SNR. Head movements varied with and without CI for total response time, whereby CI users had shortest response times. There was no difference between the SSD and SSD-CI conditions for onset delay and head displacement, despite significant differences compared with NH controls.</p><p><strong>Conclusions: </strong>Speech-in-noise performance may be the most significant improvement in CI use for SSD. Although sound localization abilities are present, there may be modest clinical significance. Head movement dynamics may highlight adaptive mechanisms that, if integrated into training or device programming, may further improve speech-in-noise and localization abilities.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675435","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":"Anatomical Relationship of Cochlear Basal Turn and Facial Nerve in Patients With Tip Fold-Over During Cochlear Implantation Using Slim Modiolar Electrode.","authors":"Yeonjoo Choi, Hye Ah Joo, Woo Seok Kang, Joong Ho Ahn, Jong Woo Chung, Hong Ju Park","doi":"10.1097/MAO.0000000000004573","DOIUrl":"https://doi.org/10.1097/MAO.0000000000004573","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the anatomical factors contributing to tip fold-over (TFO) during electrode insertion in cochlear implantation (CI).</p><p><strong>Study design: </strong>Retrospective study.</p><p><strong>Setting: </strong>Tertiary care academic center.</p><p><strong>Patients: </strong>A total of 239 ears underwent CI with Slim Modiolar Electrodes (SME) between August 2018 and December 2023.</p><p><strong>Main outcomes and measures: </strong>Preoperative temporal bone CT scans were analyzed to identify differences between patients with and without TFO. The positional relationship between a horizontal line at the cochlear basal turn and the facial nerve was assessed, and the angle between this horizontal line and the lateral margin of the facial nerve was measured.</p><p><strong>Results: </strong>TFO occurred in 4.2% of cases, requiring an average of 2.7 reinsertion attempts for proper placement. In the TFO group, the horizontal line at the cochlear basal turn was positioned below the facial nerve in 90% of cases, compared with only 11.3% in the non-TFO group (p < 0.001). The mean angle between the cochlear basal turn and the facial nerve was -6.7 degrees (±2.9) in the TFO group and -0.4 degrees (±2.7) in the non-TFO group (p < 0.001).</p><p><strong>Conclusion: </strong>TFO is more likely to occur when the facial nerve is positioned laterally relative to the cochlear basal turn, causing the electrode sheath to prematurely contact the scala tympani floor. These findings highlight a potential anatomical risk factor for TFO, emphasizing the need for tailored surgical strategies in high-risk cases.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675425","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":"Photon-Counting CT Reveals Radiologically Occult Otospongiosis: A Case Report.","authors":"Anna Dordonnat, Neil Grislain, Gabriel Garcia, Jean-Noël Vallée, Michael Eliezer","doi":"10.1097/MAO.0000000000004599","DOIUrl":"https://doi.org/10.1097/MAO.0000000000004599","url":null,"abstract":"","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675434","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":"Genome- and Exome-Wide Identification of Common-to-Rare Variants Associated with Middle Ear Cholesteatoma.","authors":"Ke Qiu, Junhong Li, Ping An, Lin Lou, Tianyi Gu, Xiuli Shao, Min Chen, Minzi Mao, Wendu Pang, Yongbo Zheng, Di Deng, Wei Xu, Jianjun Ren, Yu Zhao","doi":"10.1097/MAO.0000000000004594","DOIUrl":"https://doi.org/10.1097/MAO.0000000000004594","url":null,"abstract":"<p><strong>Hypothesis: </strong>To investigate the genetic susceptibility of middle ear cholesteatoma (MEC) and construct an MEC risk prediction model by integrating genetic risk with clinical factors.</p><p><strong>Background: </strong>MEC represents a relatively rare disorder that is associated with high morbidity, whereas its genetic etiology remains poorly understood.</p><p><strong>Methods: </strong>Using genetic data from the UK Biobank (UKB), we performed both genome-wide association study (GWAS) and exome-wide association study (ExWAS) involving 702 MEC patients and 491,503 controls. Gene-based and gene set-based association studies were then performed to identify risk genes and gene sets of MEC, respectively. In addition, logistic regression models were applied to identify clinically significant MEC-associated diseases, of which the genetic and causal relationships with MEC were further characterized using linkage disequilibrium score regression, genetic analysis incorporating pleiotropy and annotation, and Mendelian randomization. Moreover, logistic regression models were employed to construct MEC risk prediction models by integrating genetic risk with clinical factors.</p><p><strong>Results: </strong>Our study identified 159 common variants across 8 genomic loci and 39 rare variants spanning 17 genomic regions that were significantly associated with MEC, with PLD1 being prioritized as the top-ranked MEC candidate target gene. Additionally, 10 different types of diseases showed significant associations with MEC, but no inconclusive genetic or causal relationship was established between them. Moreover, we successfully constructed a high-performance MEC risk prediction model with an area under the curve of 0.704, showing the potential for clinical application.</p><p><strong>Conclusions: </strong>These findings advance our understanding of the genetic susceptibility of MEC and provide insights into its risk prediction, thus contributing to improved MEC prevention and management.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675431","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":"Impact of the COVID-19 Pandemic and Vaccination on Bell's Palsy: A Retrospective Comprehensive Analysis Using TriNetX Data.","authors":"David Shimunov, Huseyin Isildak","doi":"10.1097/MAO.0000000000004597","DOIUrl":"https://doi.org/10.1097/MAO.0000000000004597","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;Bell's palsy (BP), a sudden onset of facial paralysis, has been reported in patients with COVID-19. The relationship between COVID-19 infection and BP remains unclear, as does the impact of COVID-19 vaccination on its incidence. This study aims to assess the occurrence of BP before and after the COVID-19 pandemic and to explore any potential effects of COVID-19 vaccination on BP cases.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;This study utilized data from the TriNetX global health research network, specifically the US Collaborative Network of 68 healthcare organizations, encompassing 99,852,106 patients with ICD codes from 2016 to 2024. BP cases were identified using ICD-10 code G51.0, while COVID-19 vaccinations and infections were identified using CPT codes and TNX Curated 9088, respectively. We conducted an incidence and prevalence analysis of BP cases annually over the 8-year period to detect trends and compared BP rates between prepandemic (2016-2020) and postpandemic (2020-2024) periods using odds ratios. Additionally, we assessed BP incidence among COVID-19 patients and performed a subgroup analysis on vaccinated individuals to evaluate the impact of vaccination on BP incidence. To reduce potential confounding, we employed propensity score matching using the greedy nearest neighbor method available in the TriNetX platform, matching patients on age, sex, race, type 2 diabetes mellitus, and vascular commodities. Statistical analyses were executed within the TriNetX platform and supplemented by online AI tools using Python.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;The analysis of BP cases from 2016 to 2024 revealed an upward trend in both incidence and prevalence. The incidence proportion rose from 48 cases per 100,000 people in 2016-2017 to 69 cases per 100,000 by 2023-2024, with a significant increase noted from 2021 onwards. Similarly, the incidence rate increased from 0.00000155 to 0.00000333 over the same period. Prevalence also showed a steady rise, from 228 to 434 cases per 100,000 people. Trend analyses confirmed these increases as statistically significant, with p-values indicating upward trends in incidence and prevalence. Comparing prepandemic (2016-2020) to postpandemic (2020-2024) periods, there was a significant rise in incidence proportions (p-value ≈ 0.0074) and prevalence rates (p-value = 0.0180). Among COVID-positive patients, the incidence proportion remained stable, while prevalence increased, indicating a higher burden compared to the general population. After propensity score matching, vaccinated individuals exhibited a significantly lower risk of BP compared to unvaccinated individuals (hazard ratio: 0.723, 95% CI: 0.618-0.84, p &lt; 0.001).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion: &lt;/strong&gt;This highlights a significant increase in the incidence and prevalence of BP during the COVID-19 pandemic years compared to prepandemic levels. While the incidence of BP among vaccinated individuals showed a gradual increase, when mitiga","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675433","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":"Asymmetrical Auditory Dysfunction as a Potential Nonmotor Lateralizing Sign in Parkinson's Disease: A Case-Control Study.","authors":"Reham Mamdouh Lasheen, Mohamed Nasser Elsheikh, Mohamed Osama Tomoum","doi":"10.1097/MAO.0000000000004598","DOIUrl":"https://doi.org/10.1097/MAO.0000000000004598","url":null,"abstract":"<p><strong>Introduction: </strong>Parkinson's disease (PD) is a complex neurodegenerative disorder that often presents with unilateral motor symptoms at the onset. While motor symptoms such as tremor, bradykinesia, rigidity, and postural instability are the signs of PD, nonmotor symptoms also significantly impact the patient's quality of life. One such nonmotor symptom is auditory dysfunction, which has been increasingly recognized as a feature of PD.</p><p><strong>Objectives: </strong>Our purpose is to evaluate auditory impairment in PD patients and its asymmetry as a biomarker to differentiate PD from other neurodegenerative disorders that do not exhibit this lateralized auditory dysfunction.</p><p><strong>Subjects and methodology: </strong>Our study included 60 patients with parkinsonism \"the study group\", and 40 healthy individuals \"the control group.\" All participants underwent three auditory tests: pure tone audiometry (PTA), auditory brainstem response (ABR), and otoacoustic emissions (OAE).</p><p><strong>Results: </strong>PTA thresholds were significantly higher in the PD group, especially on the affected side. ABR absolute latencies and interpeak latencies were significantly delayed in PD, with the delays being more pronounced on the affected side. The distortion product OAEs showed a significant decrease in amplitude on the affected side compared to the nonaffected side. Furthermore, its amplitude was highly significantly reduced when compared to the control group.</p><p><strong>Conclusion: </strong>The asymmetry of the audiological responses in PD patients could be a valuable nonmotor marker of PD, enhancing our understanding of its broader impact on sensory systems and aiding in more precise diagnosis and monitoring of the disease.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675426","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":"Vestibular Dysfunction in Pediatric Patients With Congenital Cytomegalovirus Infection and Hearing Loss: Occurrence and Characteristics.","authors":"Guangwei Zhou, Hannah Peterson, Alice Yun, Jacob Brodsky","doi":"10.1097/MAO.0000000000004600","DOIUrl":"https://doi.org/10.1097/MAO.0000000000004600","url":null,"abstract":"<p><strong>Objective: </strong>To explore the occurrence of vestibular dysfunction in pediatric patients with congenital cytomegalovirus (CMV) infection and define the characteristics of vestibular loss in this population.</p><p><strong>Study design: </strong>Retrospective study with controls.</p><p><strong>Setting: </strong>Tertiary pediatric referral center.</p><p><strong>Patients: </strong>Pediatric patients with hearing loss associated with congenital CMV infection and GJB2 mutation(s).</p><p><strong>Interventions: </strong>Balance and vestibular evaluation.</p><p><strong>Main outcome measures: </strong>Normal versus abnormal results of laboratory vestibular testing.</p><p><strong>Results: </strong>A total of 50 pediatric patients with congenital CMV infection, average age = 3.4 years (ranging from 7 mo to 12 yr, SD = 2.9 yr), underwent vestibular workup, including Videonystagmography, rotary chair test, and cervical vestibular evoked myogenic potential test. Of these children with CMV infection, 35 (70%) had bilateral vestibular loss, 7 (14%) had unilateral loss, and only 8 (16%) had a normal vestibular workup. In contrast, among the 48 pediatric patients without a history of CMV infection and with hearing loss associated with GJB2 mutation(s), only 9 (19%) had bilateral vestibular loss, 2 (4%) had unilateral loss, while 37 (77%) had a normal vestibular workup. Developmental/motor delay was documented in 35 (70%) patients with congenital CMV infection and hearing loss.</p><p><strong>Conclusions: </strong>Children with congenital CMV infection are at high risk for vestibular loss, which is frequently bilateral, impacting the semicircular canals and otolith organs, not just the well-known hearing loss. A comprehensive balance and vestibular workup is warranted for children with congenital CMV infection with the goal of early identification of vestibular dysfunction and earlier management.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675450","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":"A Systematic Review of the Incidence of Cochlear Nerve Deficiency in Pediatric Single-Sided Deafness.","authors":"Christina Zhu, Jasmine Gulati, Daniel Swanson, Anuja Shah, Paul Chisolm, Michael Hoa","doi":"10.1097/MAO.0000000000004474","DOIUrl":"10.1097/MAO.0000000000004474","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the variability in cochlear nerve deficiency (CND) incidence in pediatric single-sided deafness (SSD), assess how study parameters and inclusion criteria affect these rates, and examine the diagnostic tools utilized, including magnetic resonance imaging (MRI) and computed tomography (CT), in identifying CND.</p><p><strong>Data sources: </strong>Databases including MEDLINE, Embase, Cochrane CENTRAL, and Web of Science.</p><p><strong>Review methods: </strong>A systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses reporting guidelines with predefined search criteria using terms related to pediatric unilateral hearing loss and CND from 2010 to January 2024. Studies were selected based on their focus on CND incidence in pediatric SSD, excluding case reports, nonhuman or adult studies, and analyses limited to bilateral deafness.</p><p><strong>Results: </strong>Out of 518 articles screened, 17 underwent full-text review, and 10 were included. Incidence rates of CND ranged from 15.7% to 100%, with all studies using MRI and 60% using CT as an adjunct diagnostic tool. Age criteria varied: five studies included patients 18 years and older, one study included patients up to age 21 years, and four studies targeted children under 5 years, including two studies on neonates. Variability stemmed from differing inclusion criteria; studies on younger cohorts generally excluded noncongenital causes, while older children's studies were less restrictive. Additionally, two studies excluded syndromic SSD, impacting incidence rates. Larger studies show an overall incidence of 35% to 45%, which this systematic review of the literature reports as the approximate incidence of CND in SSD, with higher incidence rates observed in studies excluding syndromic causes and broader criteria linked to decreased incidence.</p><p><strong>Conclusion: </strong>This systematic review highlights how age-specific inclusion criteria and management of syndromic causes significantly influence reported CND incidence rates in pediatric SSD, underscoring the need for standardized reporting to improve clinical understanding and treatment approaches.Level of Evidence: 1.</p>","PeriodicalId":19732,"journal":{"name":"Otology & Neurotology","volume":" ","pages":"668-674"},"PeriodicalIF":1.9,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143586533","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}