BACKGROUND: To develop a nomogram model for predicting the risk of adhesive otitis media based on temporal bone computed tomography (CT). METHODS: A retrospective cross-sectional study was performed to analyze high-resolution CT images of the temporal bone in patients diagnosed with adhesive otitis media (AdOM). The comparison of the incidence of anatomical variants of the temporal bone between groups was performed. A nomogram for predicting the risk of AdOM was developed and validated. RESULTS: A total of 153 ears from 110 patients were analyzed. In the AdOM group, 86% of the cases had poor mastoid pneumatization (MC0/MC1). The incidence rates of high jugular bulb (HJB), jugular bulb dehiscence, and agenesis of the mastoid antrum were 45.4%, 6.4%, and 2.7%. The incidence of HJB was significantly higher (P=.047), the sigmoid sinus−external auditory canal (SS−EAC) distance was significantly shorter (P < .001), and the depth of the dural plate was significantly deeper (P=.019) than in the control. The SS−EAC distance shortening was identified as an independent risk factor for the incidence of AdOM (odds ratio=1.34, P < .001). A nomogram for predicting the risk of AdOM was developed, with an area under the receiver operating characteristic curve of 0.751. The optimal cutoff value was −0.753 with sensitivity of 64.3% and specificity of 80.0%. CONCLUSION: The presence of HJB, a shorter SS−EAC distance, and a deeper dural plate depth may be associated with a higher incidence of AdOM. A novel nomogram based on anatomical variations of the temporal bone for predicting the risk of AdOM was developed to assist clinical decision-making in the treatment of otitis media.
BACKGROUND: Perform a statistical analysis of the long-term hearing results for 2 groups after stapedotomy using different heat-memory stapes prostheses. METHODS: A retrospective study of 42 patients who underwent stapedotomy using either NiTiBOND (n=25) or SMart Nitinol (n=17) thermal shape memory prostheses. Statistical differences were assessed with univariate and multivariate analysis. Success was defined as a postoperative air-bone gap (ABG) ≤ 10 dB. A Pvalue < .05 was considered statistically significant. RESULTS: The audiological outcomes were measured at an average of 7.3 and 13.4 years postoperatively for NiTiBOND and Nitinol, respectively. The postoperative ABG was a mean of 5.18 ± 5.59 dB and 4.71 ± 5.02 dB; no statistically significant difference was observed between the groups. Three patients achieving >10 dB postoperative ABG underwent high-resolution computed tomography, but none showed noticeable pathological changes. CONCLUSION: The study demonstrated excellent audiological outcomes with both prostheses and appears to be stable in the long term.
BACKGROUND: Pendred syndrome (PS) is one of the main causes of congenital hearing loss and is estimated to be the cause of 4-7.5% of hereditary deafness cases worldwide. Pendred syndrome is an autosomal recessive disorder associated with alterations in the SLC26A4 gene characterized by sensorineural hearing loss and goiter. The aim of the study is to compile a review providing an accurate and updated description of the audiological features of PS, offering clinicians a practical tool for a feasible and early diagnosis. METHODS: A detailed review of the English literature to date on hearing loss and PS has been performed using Pubmed, Scopus, Google Scholar and Medline databases. The literature review was performed using the guidelines proposed by the study “Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA)” for scoping review. RESULTS: A total of 13 full text articles were included in this review, collecting 75 patients with PS. The audiological outcomes, clinical variants, presence of enlarged vestibular aqueduct and Mondini dysplasia, and thyroid status were described for each patient. CONCLUSIONS: Pendred syndrome is a condition in which hearing loss is among the main features and can manifest at early stages, eventually impacting children’s language development. Pendred syndrome may occur in different clinical variants, which can make diagnosis challenging. It is therefore crucial for clinicians to have a detailed knowledge of the condition. Further studies on large, multicenter case series will therefore be essential to expand knowledge of the disease and enable multidisciplinary development of care.
BACKGROUND: Patients in whom the facial nerve has been resected or divided during surgical treatment may have it repaired by direct anastomosis, interposition graft, or may either primarily or up to 2 years later undergo reinnervation of the facial musculature, in most cases using the masseteric or hypoglossal nerve. The aim of this study is to compare outcomes of facial reanimation procedures for those who have undergone either direct anastomosis, nerve grafting, or masseteric nerve transfer. METHODS: Retrospective case note review of patients attending the Facial Palsy Clinics. Patients in whom surgery was not part of the treatment provided, revision cases, patients with recurrence of pathology, or incomplete datasets were excluded. Patient factors, etiology, operative findings, and pre- and post-operative Sunnybrook Facial Grading were recorded. In order to allow for differences in recovery time, the post-operative score was considered as the score recorded when improvement had plateaued. RESULTS: Thirty (16 female, 14 male) patients underwent primary surgery for facial palsy. Median age was 44 years. Sixteen patients underwent “Nerve Transfer,” 10 “Nerve Grafting,” and 4 “End-to-End.” Pre-operative Sunnybrook Facial Grading Scale did not differ between the three subgroups. Nerve transfer patients had a mean post-operative score of 58.1, nerve graft 51.4, and end-to-end 68.8 (Chi2=2.196, P=.33) CONCLUSION: End-to-end anastomosis, where viable, appears to give the greatest improvement in facial nerve function. Where this is not viable, both grafting and nerve transfer should be given equal weighting when considered in a patient specific context.
Cisplatin is a widely used chemotherapeutic agent in pediatric oncology; however, it is well known for its marked ototoxicity. While hearing impairment typically emerges during or soon after therapy, progressive or previously undetected auditory dysfunction may occasionally present years later. The reported case involves an 11-year-old boy who previously received cisplatin-based chemotherapy for hepatoblastoma and was first identified as having bilateral sensorineural hearing loss 9 years after completion of treatment. Pure-tone audiometry revealed bilateral elevation of high-frequency thresholds without an air–bone gap, and distortion product otoacoustic emissions (DPOAE) were absent at high frequencies. Auditory brainstem responses (ABRs) demonstrated elevated thresholds at 4 kHz, and neuroimaging showed no structural abnormalities of the cochlea or auditory nerve. Expanded genetic testing revealed no pathogenic variants associated with hereditary hearing loss. Although the exact etiology could not be definitively established, the audiologic pattern and treatment history suggested that cisplatin may have contributed to the hearing loss. This case highlights the need for long-term hearing surveillance in childhood cancer survivors, as progressive or previously undetected deficits may not become clinically apparent until many years after treatment.
Arnold–Chiari malformation (ACM) is a rare congenital hindbrain anomaly, with types 1-4 depending of degree of herniation of posterior fossa contents. The incidence of type 1 ACM is highest, with most cases being asymptomatic. Cases are incidentally picked up; however, some may present with headache, cough, paresthesia, and very rarely, symptoms of compression of lower cranial nerves such as sensorineural hearing loss. Surgical challenges are anticipated due to associated skeletal malformations and raised intracranial pressure. The youngest reported case—a 3-year-old girl with delayed speech and language milestones—is presented, in whom ACM type 1 was incidentally diagnosed. Audiological workup revealed profound sensorineural hearing loss in both ears. After multidisciplinary clearance, she underwent right-sided cochlear implantation. Intraoperative challenges faced were dilated emissary veins, an anteriorly placed sigmoid sinus, and a low-lying dura. However, a full electrode insertion was achieved and confirmed on neural response telemetry. The postoperative course was uneventful. Literature review suggests this to be the youngest reported case of cochlear implantation in ACM type 1. While hearing loss in this case may have been unrelated to ACM, nonetheless, successful cochlear implantation is feasible with meticulous preoperative planning and intraoperative vigilance. After a thorough anatomical assessment, multidisciplinary preoperative workup, intraoperative vigilance, and neuro-anesthetist backup, cochlear implantation can be safely performed in children with ACM type 1.
OBJECTIVE: Hearing loss is an underrecognized comorbidity in patients with chronic kidney disease (CKD) and chronic liver disease (CLD), particularly among those awaiting solid organ transplantation. Early identification of auditory dysfunction is essential to optimize communication, treatment adherence, and post-transplant care. To describe the prevalence, severity, and audiometric patterns of hearing loss in adult candidates for kidney or liver transplantation. METHODS: A cross-sectional study was conducted in 36 adult patients with advanced CKD or CLD evaluated for kidney or liver transplantation at a tertiary academic center. All participants underwent otomicroscopy, tympanometry, pure-tone audiometry, distortion product otoacoustic emissions (DPOAEs), and tinnitus assessment using the Tinnitus Handicap Inventory. Hearing loss was defined as a pure-tone average >20 dB HL across 0.5-4 kHz in either ear. RESULTS: Hearing loss was identified in 30.6% of patients (CLD: 31.6%; CKD: 29.4%), predominantly mild to moderate, with symmetrical high-frequency sensorineural patterns. Distortion product otoacoustic emissions abnormalities were observed in 56.9% of ears, suggesting subclinical cochlear dysfunction. Tinnitus was reported in 38.2% of participants, with no significant differences between groups. All cases of tinnitus were classified as slight or mild handicap. Use of acetylsalicylic acid was significantly associated with hearing loss among CKD patients (P=.003). CONCLUSIONS: Hearing loss and tinnitus are common in patients with advanced CKD or CLD awaiting transplantation. Comprehensive audiological assessment, including DPOAEs, may improve pre-transplant evaluation by identifying patients at risk for auditory impairment and informing ototoxic medication decisions.
BACKGROUND: Meniere’s disease remains a controversial condition not only in terms of its etiology and diagnosis but also its treatment. Approximately 2%-5% of patients do not improve or reach the control of their vestibular symptoms with any pharmacological therapy. Surgery of lateral semicircular canal (LSCC) occlusion could be an alternative to control the symptoms. METHODS: Twenty consecutive patients with clinical and oto-neurological diagnosis of Meniere’s disease were included. Twelve women and 8 men with a mean age of 51 years (range, 29-72) were studied. All affected with unilateral or at least marked unilateral asymmetry in audio-gram. All patients who underwent surgery received general anesthesia. Canal wall up mastoidectomy was performed. After the LSCC was identified, a bony surface of the semicircular canal was drilled using a 1 mm diamond burr until the membranous labyrinth was discovered (blue line). The canal was then blocked with a mixture of bone wax and bone dust in an area of 2 or 3 mm, at the most prominent part of the semicircular canal. RESULTS: All patients in the study had an average hospital stay of 36 hours, of which 10 required additional medication (ondansetron). All were given 75 mg of cinnarizine orally once daily for 10 days. After that, only 4 patients continued taking betahistine for 1 month. One month later, no patients were taking medication for vertigo. CONCLUSION: Lateral semicircular canal occlusion seems to be an effective surgical option for patients with refractory Meniere’s disease.
BACKGROUND: Atrial fibrillation (AF) is associated with various end-organ complications through mechanisms including thromboembolism and microvascular dysfunction. The cochlea, with its high metabolic demands and limited collateral circulation, is particularly vulnerable to circulatory disturbances. However, the association between AF and hearing loss remains poorly understood, with limited and conflicting evidence from prior studies. Therefore, this study aimed to investigate the association between AF and hearing thresholds. METHODS: A single-center, cross-sectional study was conducted on 129 adults who underwent pure-tone audiometry, 12-lead electrocardiography, and 24-hour Holter monitoring. Subjects were categorized into an AF group (n=42) and a control group (n=87). The primary outcome was the better-ear average hearing threshold across 0.5, 1, 2, and 4 kHz. Generalized additive models were used to assess the association between AF and hearing thresholds, adjusting for age, sex, body mass index, and other cardiometabolic risk factors. Frequency-specific analyses were also conducted. RESULTS: After adjusting for covariates, AF was independently associated with a 4.28 dB HL elevation in hearing thresholds (95% CI: 3.47-5.10, P=.017). Frequency-specific analyses revealed that the association was most pronounced at 4 kHz, with a 7.40 dB HL elevation at this frequency (95% CI: 2.27-10.05, P=.004). CONCLUSION: This study provides preliminary evidence of an association between AF and hearing loss, suggesting that AF could be considered in investigations aimed at identifying modifiable risk factors for hearing loss.
BACKGROUND: Ménière’s disease (MD) is an inner ear disorder characterized by unpredictable vertigo attacks, tinnitus, and aural fullness, ultimately leading to a decline in audiovestibular function. Linked to endolymphatic hydrops (EH), MD often progresses to bilateral involvement (BMD). When conservative therapies fail, MD requires destructive treatments. However, this is a high-risk choice in bilateral cases for hearing preservation. Pressure therapy (PT), a non-invasive treatment using low-pressure pulses to the middle ear, has shown potential in reducing EH and alleviating MD symptoms. This study aims to evaluate the efficacy of PT in treating BMD. METHODS: This prospective study enrolled patients with BMD refractory to standard therapy. Electrocochleography (ECochG) using click stimuli was used to identify the active MD ear. Patients underwent PT for 24 months on the affected side. Outcomes were re-assessed using the ECochG Summating Potential-to-Action Potential (SP/AP) ratio and the Dizziness Handicap Inventory (DHI) after 1, 3, 6, and 24 months. The contralateral ear was used as a control. RESULTS: Pressure therapy significantly reduced the EH (ECochG SP/AP ratio) in active MD ears (P < .001) and improved DHI scores (P < .001). The contralateral non-treated ear showed no significant changes (P=.9687). CONCLUSION: Pressure therapy alleviates symptoms and improves quality of life in refractory BMD, offering a non-destructive alternative before invasive procedures in case of standard conservative therapy failure.
Congenital aural stenosis (CAS) sometimes can be a risk for external auditory canal (EAC) cholesteatoma and may require surgical treatment. We report a child of Down syndrome with bilateral CAS who underwent bilateral canalplasty with underwater transcanal endoscopic ear surgery (TEES). Case Report: A 12-year-old boy with bilateral EAC stenosis, in whom tympanostomy tube insertion for secretory otitis media (SOM) had failed at 5 and 8 years of age, was referred to our department for surgical treatment. The bilateral EACs were so narrow and curved that the tym-panic membranes could not be entirely observed. The osseous EAC (OEAC) diameters were measured on sagittal computed tomography (CT) images with ImageJ. The minimum Feret diameter was 3.05 mm in the right ear and 2.98 mm in the left ear. Bilateral canalplasty with underwater TEES and tympanostomy tube insertion was performed for CAS complicated by SOM. One month after surgery, bilateral EACs were well-epithe-lialized and the tympanic membranes were easily observed. Canalplasty with underwater TEES is minimally invasive, secure, and provides a clear surgical field, and is therefore a useful option for children with EAC stenosis.
BACKGROUND: The aim of this study was to evaluate the histopathological effects of using amniotic membranes to heal traumatic perforations of the tympanic membrane (TM). METHODS: Thirty Wistar albino rats were randomly divided into 3 groups. Under general anesthesia, acute TM perforation was performed in the posterior quadrant of each ear. Group A received amniotic membrane (AM) treatment, while Group B received vector paper treatment. Group C was left to heal spontaneously. All the rats were killed on day 21 for a histopathological evaluation. RESULTS: In total, 3 rats were excluded from the study for various reasons. Tympanic membrane thickness, the presence of residual perforation, fibroblastic activity, and neovascularization were evaluated histologically. There was persistent perforation in one of the 18 ears in Group A, 9 of the 20 ears in Group B, and 7 of the 16 ears in Group C. Significant differences in perforation closure rates were observed between Groups A and B and Groups A and C (P=.0089 and .0121, respectively). Tympanic membrane thickness also differed significantly between Groups A and B and Groups A and C (P=.0291 and .0106, respectively). However, no significant differences were found among the groups regarding neovascularization (P=.1094) or fibroblastic activity (P=.7275). CONCLUSION: This study demonstrates that applying an AM significantly improves the healing of the TM in terms of closure rate, and membrane thickness. These results suggest that AM could play a therapeutic role in enhancing the healing of traumatic TM perforations.
BACKGROUND: Pediatric patulous Eustachian tube dysfunction (PETD) is uncommon in daily practice and is often overshadowed by more prevalent middle ear disorders. Because symptoms may be difficult for children to describe and objective confirmation is inconsistently used, the true frequency and clinical burden of PETD in childhood remain uncertain. This systematic review synthesized the literature on pediatric PETD to clarify reported prevalence, clinical presentation, diagnostic approaches, associated factors, and treatment strategies. METHODS: Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses principles, cohort studies, clinical trials, and case series published from 2000 to July 2025 that reported pediatric PETD were reviewed. Data were extracted on study design, patient population, prevalence, presenting symptoms, diagnostic methods, associated conditions, management, and outcomes. Methodological quality was interpreted using study-design-appropriate appraisal approaches. RESULTS: Forty-five studies met the inclusion criteria. Reported prevalence in pediatric populations ranged from 0.3% to 1.2%, likely underestimating the true burden. The evidence base was heterogeneous and largely composed of small observational series. Recurrent themes included misclassification as obstructive Eustachian tube dysfunction or otitis media with effusion, inconsistent use of objective testing, limited pediatric-specific diagnostic criteria, and sparse longitudinal outcome data. Conservative strategies were most frequently described, whereas evidence for procedural intervention in children was limited. CONCLUSION: Pediatric PETD is likely substantially underdiagnosed. Greater awareness of characteristic symptoms, structured age-appropriate diagnostic pathways, and standardized outcome reporting are needed to improve recognition and management. Prospective pediatric studies should validate diagnostic criteria and compare conservative and interventional strategies.
BACKGROUND:The study aims to demonstrate the possible protective effects of carbon monoxide-releasing molecule-3 (CORM-3) on the auditory system after noise exposure through electrophysiological measurements and histopathological methods. METHODS:Twenty-four albino Wistar male rats were included in the study. They were equally divided into 3 groups. The control group was administered intraperitoneal physiological saline for 7 days. Eight rats in the second group were exposed to 4 kHz octave band noise at 120 dB SPL intensity for 4 hours, and then intraperitoneal saline was administered for 7 days. Group 3, after exposure to noise with the same characteristics, was administered 10 mg/kg of CORM-3 intraperitoneally for 7 days. Hearing levels were measured at baseline, on the first, and seventh days with distortion product otoacoustic emissions (DPOAEs) and auditory evoked brainstem potentials at 4, 6, and 8 kHz. After the measurements, the cochlear structures of all rats were dissected, and the cochlear structures were evaluated by light microscopy. RESULTS:Auditory evoked brainstem potential test results showed that the CORM-3 group had better hearing thresholds at all frequencies than the other group exposed to noise. These findings were statistically significant (P < .05). The DPOAE responses disappeared after acoustic trauma. Histopathological evaluation showed cellular damage caused by noise in the cochlea. CONCLUSION:The findings suggest that CORM-3 may be an effective protective and therapeutic agent against noise-induced hearing loss. However, additional research is needed to prove this protective effect.
BACKGROUND:Congenital deafness disrupts the early critical phases of auditory development. Animal models mimicking congenital deafness are essential for studying auditory system maturation, necessitating neonatal deafening protocols achieving complete deprivation before hearing onset. This study investigated whether kanamycin alone or in combination with furosemide induces complete hearing loss in neonatal rats during the prehearing period, with the expectation that the combination would produce faster and more profound deprivation. METHODS:Sprague-Dawley rat pups received kanamycin (n=9) or kanamycin-furosemide (n=9) from postnatal day (P) 5 to 11. Controls (n=3)received saline. Auditory brainstem responses (ABRs) to tone pips (4-32 kHz) and clicks were recorded daily from P12 to P21, and at 8 weeks. RESULTS:Both ototoxic protocols elevated ABR thresholds across all tested frequencies. However, neither regimen induced complete deafness by P12, as all animals retained low-frequency hearing (4-8 kHz). The combined treatment group showed significantly higher thresholds at low frequencies on P14 compared to the kanamycin-only group, but the effect diminished by P17. Auditory brainstem response thresholds remained stable between P21 and 8 weeks, indicating no further progression of hearing loss. Click-evoked responses did not reliably reflect low-frequency hearing during early development, leading to high false-negative rates when used alone as the deafness criterion. CONCLUSION:Although both protocols elevated ABR thresholds, neither fully eliminated auditory input during the prehearing period. The combined treatment transiently enhanced ototoxicity but did not achieve complete deafness. These findings suggest that current protocols may allow residual high-threshold hearing, which should be considered when interpreting developmental outcomes.
UNLABELLED:Cornelia de Lange syndrome (CdLS) is a rare congenital multisystem syndrome characterized by a distinctive craniofacial appearance, developmental delay, intellectual disability, limb abnormalities, and hypertrichosis. Hearing loss is one of the common manifestations in CdLS. This study presents a 6-year-old child with CdLS, who exhibited developmental delay, intellectual disability, and displayed autistic features, and experienced challenges during right cochlear implant (CI) surgery at the age of 5 due to inner ear abnormality. After the CI, the sound field hearing test showed minimal response levels of 60-70 dB HL at post-CI 12 months. He obtained scores of 2%, 14%, 16%, and 27% on the PEACH rating scale at pre-CI, post-CI 3 months, post-CI 6 months, and post-CI 12 months, respectively. Nevertheless, for CdLS patients with severe-to-profound hearing loss, CI can offer potential improvements in auditory skills and social connections, even though the benefits may be limited. It remains a viable solution.
BACKGROUND:To investigate the clinical features of patients with and without endolymphatic hydrops (EH) under idiopathic sudden sensorineural hearing loss (ISSNHL) and to evaluate the predictive value of gadolinium (Gd)-enhanced inner ear magnetic resonance imaging (MRI) in the prognosis of ISSNHL. METHODS:Seventy ISSNHL patients were enrolled, of whom 15 (21.4%) were identified with EH via Gd-enhanced MRI. Using 1 : 1 nearestneighbor propensity score matching (PSM) based on age, sex, and body mass index (BMI), 13 matched pairs (n=26) were generated for adjusted analysis. Factors influencing hearing recovery were analyzed by univariate and multivariate analysis. RESULTS:After adjusting for age, sex, and BMI using PSM, the EH group demonstrated significantly higher bilirubin levels (P=.044) and a markedly lower hearing recovery rate (P=.030) compared to the non-EH group. Univariate analysis identified that older age (P=.013), certain audiogram curve types (P=.024), a higher (poorer) initial hearing threshold (P=.002), and a positive finding on Gd-enhanced inner ear MRI (presence of EH) (P =.015) were negatively associated with hearing recovery. Multivariate logistic regression indicated that the presence of EH on MRI (odds ratio [OR]=9.80, P=.048) and increasing age (OR=0.95 per year, P=.041) were independently associated with a lower likelihood of hearing recovery. CONCLUSION:The significantly poorer hearing recovery in EH patients, even after adjusting for demographics, strengthens the evidence for EH as a negative prognostic factor. Consequently, Gd-enhanced inner ear MRI, which reliably detects EH, could serve as a novel prognostic biomarker. This tool aids in early risk stratification, identifying patients who may benefit from more aggressive or alternative treatment strategies, thereby potentially guiding a more personalized management approach for ISSNHL.
BACKGROUND:Brain-derived neurotrophic factor (BDNF) plays a crucial role in the initial formation of the central auditory system and the sensory epithelium within the inner ear. Mounting evidence indicates that BDNF administration promotes microRNA (miRNA) production in neurons, despite the typical suppressive effect of miRNAs on BDNF expression. Therefore, miRNAs regulating BDNF expression may impact the auditory system and serve as potential gene polymorphisms affecting human hearing ability. This study aimed to investigate the contribution of miRNA polymorphisms affecting BDNF to tinnitus pathophysiology. METHODS:A total of 70 tinnitus patients aged 18-55 years and 70 age-matched healthy controls without tinnitus or systemic illnesses were recruited from an Ear, Nose & Throat clinic. Tinnitus assessment included tympanometric, audiological, and psychoacoustic evaluations. Seven miRNA single-nucleotide polymorphisms (miR-30e rs112439044, rs10489167, miR-206 rs16882131, miR-30a rs1491500379, miR-26b rs565919718, rs188612260, and miR-124 rs5315564) regulating the BDNF gene were analyzed using the Fluidigm platform. RESULTS:Significant differences in genotype distributions were observed for miR-30e rs112439044, miR-124 rs5315564, and miR-206 rs16882131 between the tinnitus patients and controls (P < .05). Genetic inheritance-model analysis revealed that miR-30e rs112439044 was associated with 0.20- and 0.83-fold risks under dominant and additive models, respectively. The miR-124 rs5315564 showed a 1.65-fold risk in the dominant model, while miR-206 rs16882131 exhibited an 11.1-fold protective effect under the dominant model and an 8.57-fold risk under the additive model. CONCLUSION:The findings suggest that polymorphisms in miR-206, miR-30e, and miR-124 may influence the auditory pathway by modulating BDNF gene expression, potentially contributing to the pathophysiology of chronic tinnitus.
