Sensorineural Research Articles

The tonotopy of tinnitus: comparison between acoustic masking and intracochlear electrical stimulation through cochlear implant.

To explore the relationship between tinnitus pitch and audiometric profile with the frequency of the most effective stimulus for tinnitus relief using either acoustic or electrical stimulation with cochlear-implant (CI). The pitch and loudness of tinnitus were assessed and tinnitus perception measured with a visual analogue scale (0-10) during acoustic masking or electrical stimulation with CI on different frequencies. A reduction in tinnitus loudness (RT) >50% was considered significant. 52 patients (53 ± 17yrs, 61.5%F-38.5%M) with sensorineural hearing loss (HL) and unilateral tinnitus (noCI group) and 50 patients (52.8 ± 14.6yrs, 56%F-44%M) with unilateral CI and tinnitus on the same side (CI group). In both groups the tinnitus pitch (2715.5 Hz for CI and 4629.8 Hz for noCI) was higher than the audiometric edge (steepest HL) and lower than the maximum HL. In noCI group the most effective frequency overlapped with tinnitus pitch. In CI group the most effective electrode (suppressor electrode-ES) was the most apical one (188-313Hz) with no relationship with the pitch-matched electrode (EPM). The tonotopic differences in the localisation of the effective stimulus between acoustic masking and intracochlear electrical stimulation support different mechanisms involved in the suppression of neural circuits related to tinnitus perception.

Phenotype-genotype correlations in patients with Alport syndrome from the Polish population.

Alport syndrome (AS) is a rare inherited kidney disease associated with progressive renal failure and visual and hearing disorders. The purpose of this study was to find genetic variants in patients with suspected Alport syndrome from Central and Southwestern Poland and their association with the clinical course of the disease, and to evaluate the impact of Alport syndrome on pregnancy. Initially, 90 patients with suspected Alport syndrome were evaluated by molecular-based testing. Clinical analyses, including urinalysis, evaluation of serum parameters, ultrasound, ophthalmologic, cardiovascular and audiology examination, and genetic testing were performedusing next-generation sequencing and the Sanger method. Seventy-seven patients (40.26% male; 59.74% female) with a median age of 6years were included in the study group, after receivinga diagnosis of Alport syndrome. Twenty pathogenic/potentially pathogenic variants within the COL4A3, COL4A4 and COL4A5 genes were identified in these patients. The c.1871G > A variant in the COL4A5 gene was the most common (53.25%). Isolated hematuria was the most common initial sign of Alport syndrome (70.8%). Genetic testing confirmed Alport syndrome in 85% of symptomatic patients and in 15% of asymptomatic patients. Sensorineural hearing loss (17%) and ocular abnormalities (6%) were also detected in patients in the study group. Isolated hematuria showed a significant association with COL4A5 gene variants (p < 0.001). Genetic variants showed an association with initial clinical symptoms and age at Alport syndrome manifestation. Regular urinalysis and genetic testing should be considered in suspected cases of Alport syndrome for rapid diagnosis and effective patient management.

Taurine supplementation improves physical activity level in a hemodialysis patient with mitochondrial disease: a case report.

Mitochondrial diseases (MDs) are inherited metabolic disorders that affect multiple organ systems, including the kidneys. Variability in disease onset and phenotypic expression, combined with the absence of specific kidney pathological findings, pose significant challenges in diagnosing MD. Consequently, many undiagnosed cases of MD may exist among patients undergoing dialysis. No effective treatment for mitochondrial nephropathy has been established. We report the case of a 27-year-old female patient who presented with leg edema, nephrotic range proteinuria attributed to focal segmental glomerulosclerosis, and bilateral sensorineural hearing loss. Immunosuppressive therapy failed to achieve remission, resulting in progressive kidney function decline and eventual end-stage kidney disease. At hemodialysis initiation, worsening atypical cardiac function and hypertrophy prompted genetic testing, which identified an MT-TL1 m.3243 A>G mutation and confirmed the diagnosis of MD. After hemodialysis initiation, the patient experienced persistent fatigue and decreased physical activity levels despite dry weight management. Suspected stroke-like symptoms prompted the initiation of taurine supplementation, which significantly improved headache severity, cardiac function, and physical activity levels. This case highlights the therapeutic potential of taurine supplementation in patients with MD undergoing dialysis and the importance of maintaining clinical vigilance for MD across all stages of chronic kidney disease, even without characteristic renal pathological findings of mitochondrial nephropathy.

Etiology and outcomes of primary renal tubular acidosis.

This study investigates the etiology, outcomes, and genotype-phenotype correlations in patients with renal tubular acidosis (RTA) at a tertiary care center in New Delhi. This cross-sectional study included children and young adults with RTA who underwent clinical, biochemical, radiological and/or genetic evaluations between July 2020 and December 2024. We report clinical phenotype, anthropometry, metabolic control and progression to chronic kidney disease (CKD) in relation to genotype of distal RTA. Of 135 patients enrolled, 69 had distal RTA. The yield of genetic testing was 72% in distal RTA and 88.7% in Fanconi syndrome. Variants in SLC4A1 (42.4%) and ATP6V1B1 (28.8%) were the most common etiologies of distal RTA. Compared to other etiologies, patients with SLC4A1 variants were older at symptom onset (P = 0.008). Hematological abnormalities were more frequent in patients with biallelic compared to heterozygous SLC4A1 variants (50% vs. 12.5%; P = 0.18). Nephrocalcinosis and metabolic control were similarly prevalent across genetic categories of distal RTA. Sensorineural hearing loss was more common with ATP6V1B1 than with ATP6V0A4 variants (61.5% vs. 22.2%, P = 0.099) and did not vary by metabolic control. At median follow-up of 5-years, 74.1% of patients with distal RTA had short stature, 74.6% had poor metabolic control and 2.9% had progressed to CKD G3-5. This study outlines the genetic etiology and phenotype of distal RTA in south Asia. Over short-term follow-up, poor metabolic control and severe stunting were common, while CKD was uncommon.

The P4-phospholipid flippase Atp11a is required for maintenance of eye and ear structure in zebrafish.

The atp11a gene encodes a phospholipid flippase protein required to flip phosphatidylserine (PS) and phosphatidylethanolamine (PE) from the outer leaflet of the cytoplasmic membrane to the inner leaflet. Mutations in ATP11A have been described in patients with sensorineural hearing loss and neurologic deterioration, however little is known regarding the mechanism by which loss of atp11a results in such phenotypes. To this end, we created loss of function atp11a mutant zebrafish to characterize potential disease states. We demonstrate that mutant atp11a zebrafish display a reduced number of stereocilia in the larval ear and a reduced number of hair cells in some sensory neuromasts, indicating that these fish represent an ideal model to study atp11a-attributable hearing loss. In addition, atp11a mutant zebrafish raised in a standard light cycle have reduced photoreceptor outer segments, the severity of which is lessened when mutant larvae are raised in the dark. Photoreceptors that do remain in homozygous atp11a mutants have undergone mitochondrial fission producing an increased number of mitochondria suggesting that defects in energy homeostasis may contribute to or result from outer segment degradation.

Conditional Overexpression of Neuritin in Supporting Cell Protects Cochlear Hair Cell and Delays Age-Related Hearing Loss by Enhancing Autophagy

Age-related hearing loss (ARHL) is a highly prevalent, burdensome sensorineural hearing loss closely associated with impaired autophagic influx. Our previous studies revealed that neuritin, a neurotrophic factor primarily expressed in the central nervous system, could alleviate drug-induced damages in hair cells (HCs) and spiral ganglion neurons. However, its effects on ARHL and whether these effects are closely related to autophagy remain unclear. Using the Nrn1 knock-in mice and cultured cochlear basilar membrane (CBM) of the neonatal mouse, we show that neuritin could restore aging-associated hearing loss and alleviate senescence-associated damage in the cochlea. Overexpression of neuritin in support cells (SCs) alleviates the loss of cochlear HCs and nerve fibers, reducing the damage to spiral ganglion neurons and the shifts in ABR’s high-frequency threshold. Furthermore, conditional overexpression of neuritin in SCs improves autophagic influx by upregulating the expression of microtubule-associated protein 1 light chain 3 type B (LCB3) protein and downregulating the expression of p21 protein. In cultured neonatal mouse CBM, neuritin administration significantly inhibits D-galactose-induced HC loss, cellular apoptosis, and ROS production and promotes autophagic influx. These effects were weakened when the autophagy inhibitor 3-MA was added. In summary, our results confirm the therapeutic potential of neuritin treatment for ARHL.

The State of Spatial Hearing in Grades 1 and 2 Symmetrical Sensorineural Hearing Loss Based on SHQ Questionnaire Data

The State of Spatial Hearing in Grades 1 and 2 Symmetrical Sensorineural Hearing Loss Based on SHQ Questionnaire Data

Fluctuating or progressive hearing loss in the middle-to-high frequencies may suggest the neurotologic endotype of perilymphatic fistula without antecedent traumatic events

BackgroundIdiopathic sudden sensorineural hearing loss (ISSNHL) is a common clinical condition. Recent studies indicate that approximately 20% of ISSNHL cases may involve perilymphatic fistula (PLF). The detection of Cochlin-tomoprotein (CTP) in middle-ear lavage samples confirms the diagnosis of PLF.Aims/ObjectivesTo clarify the clinical characteristics of inner ear–related symptoms in patients with PLF who lacked any antecedent traumatic events prior to symptom onset.Materials and methodsWe retrospectively reviewed clinical records and CTP test results in 769 cases from 70 hospitals in Japan.ResultsAmong these cases, 204 had no history of antecedent events. CTP-positive findings were more frequently observed in patients exhibiting fluctuating and/or progressive hearing loss than in those without these symptoms (p &amp;lt; 0.05, Fisher’s exact test). The odds of a positive CTP test did not differ between patients with and without vestibular symptoms, nystagmus, a fistula sign, a popping sensation, or streaming water–like tinnitus (p &amp;gt; 0.05, Fisher’s exact test). The CTP positivity rate was highest in patients with a high-frequency sloping audiogram.Conclusions and significanceFluctuating or progressive hearing loss in the middle-to-high frequencies may reasonably suggest PLF in the absence of antecedent traumatic events.

Uncovering a Novel Pathogenic Mechanism of BCS1L in Mitochondrial Disorders: Insights from Functional Studies on the c.38A&gt;G Variant

The BCS1L gene encodes a mitochondrial chaperone which inserts the Fe2S2 iron–sulfur Rieske protein into the nascent electron transfer complex III. Variants in the BCS1L gene are associated with a spectrum of mitochondrial disorders, ranging from mild to severe phenotypes. Björnstad syndrome, a milder condition, is characterized by sensorineural hearing loss (SNHL) and pili torti. More severe disorders include Complex III Deficiency, which leads to neuromuscular and metabolic dysfunctions with multi-systemic issues and Growth Retardation, Aminoaciduria, Cholestasis, Iron Overload, and Lactic Acidosis syndrome (GRACILE). The severity of these conditions varies depending on the specific BCS1L mutation and its impact on mitochondrial function. This study describes a 27-month-old child with SNHL, proximal renal tubular acidosis, woolly hypopigmented hair, developmental delay, and metabolic alterations. Genetic analysis revealed a homozygous BCS1L variant (c.38A&gt;G, p.Asn13Ser), previously reported in a patient with a more severe phenotype that, however, was not functionally characterized. In this work, functional studies in a yeast model and patient-derived fibroblasts demonstrated that the variant impairs mitochondrial respiration, complex III activity (CIII), and also alters mitochondrial morphology in affected fibroblasts. Interestingly, we unveil a new possible mechanism of pathogenicity for BCS1L mutant protein. Since the interaction between BCS1L and CIII is increased, this suggests the formation of a BCS1L-containing nonfunctional preCIII unable to load RISP protein and complete CIII assembly. These findings support the pathogenicity of the BCS1L c.38A&gt;G variant, suggesting altered interaction between the mutant BCS1L and CIII.

Hearing loss in Chinese osteogenesis imperfecta patients.

Osteogenesis imperfecta (OI) is a genetic connective tissue disorder primarily characterized by bone fragility. Hearing loss is an extra-skeletal manifestation of OI. This study aims to characterize the audiological findings of a sample of patients with OI in China. A total of 76 patients (aged 4-77 years) were recruited and evaluated using audiometric evaluations, including pure tone audiometry (PTA), acoustic admittance measurements, and distortion-product otoacoustic emissions (DPOAEs). Patients were categorized into 2 age groups: adults (more than 18 years) and children (under 18 years), and classified by hearing type: normal hearing, conductive, sensorineural, or mixed hearing loss. Tympanometry (152 ears) showed 71.7% Type A tympanograms. There was a significant association between tympanogram type and hearing loss (p < 0.01). DPOAEs were predominantly abnormal in ears with sensorineural and mixed hearing loss (p < 0.01). PTA (146 ears) revealed hearing loss in 39.0%: 16.4% conductive, 15.1% sensorineural, and 7.5% mixed. Age correlated significantly with hearing loss type (p < 0.01). Adults' group exhibited higher pure-tone averages (11.3 dB vs. 8.8 dB, p < 0.01), average air-bone gap (2.5 dB vs.2.5 dB, p < 0.05), and bone conduction thresholds than children's group. Hearing loss is common and progressive in Chinese OI patients, with a significant age-related increase in both the prevalence and severity of hearing loss. Establishing a hearing baseline and regular follow-up is essential for early intervention.

Lateral Displacement of Cochlea and Erosion of Internal Auditory Canal by a Congenital Cholesteatoma in a Young Child.

The main objective of this report is to describe a unique clinical case of a congenital cholesteatoma with complete lateral displacement of the cochlea and aggressive erosion of the internal auditory canal and lateral semicircular canals in a very young child. This report involves a 10-month-old female who initially presented with left intermittently bloody otorrhea at 2.5 months of age and audiology testing in the clinic demonstrated sensorineural hearing loss in the left ear. Initial computerized tomography (CT) revealed a destructive left temporal bone lesion displacing the left otic capsule and vestibular aqueduct with erosion and involvement of the inner ear structures including erosion into the cochlea and semicircular canals, and posterior fossa dura. A left transtemporal approach for cholesteatoma removal and ear canal closure was done. 4 months postoperatively, the patient required revision of the left transtemporal approach to the posterior fossa for recurrent cholesteatoma in the hypotympanum adjacent to the carotid artery and eustachian tube. This case is unique in the complete lateral displacement of the cochlea and aggressive erosion of the internal auditory canal and lateral semicircular canals in a very young child. It serves to remind otolaryngologists of the silent yet infiltrative nature of congenital cholesteatoma. The very young age of the patient and the presence of otorrhea at 1 month of age raise the question of whether this lesion could have been developing in utero. It lends support to the theory of developmental epithelial rests in congenital cholesteatoma.

Diagnosis of a patient with severe sensorineural hearing loss as the initial symptom caused by novel compound heterozygous variant in SLA19A2 gene.

Diagnosis of a patient with severe sensorineural hearing loss as the initial symptom caused by novel compound heterozygous variant in SLA19A2 gene.

Acute myocardial infarction after batroxobin treatment of sudden sensorineural hearing loss: Case report.

The drug batroxobin is utilized to enhance microcirculation by reducing fibrinogen levels, making it a treatment option for the acute stage of severe sudden sensorineural hearing loss (SSNHL). However, the efficacy of batroxobin defibrination remains controversial. It is crucial to also consider the potential thrombotic effects of batroxobin. This is the first report of acute myocardial infarction (AMI) in patients after treatment for SSNHL with batroxobin, aiming to enhance its clinical safety profile. A 61-year-old male patient presented with severe SSNHL in the left ear. During hospitalization, the plasma fibrinogen level remained consistently low following batroxobin treatment and subsequently led to AMI. SSNHL, AMI. Percutaneous coronary intervention surgery confirmed thrombus blockage in the middle segment of the right coronary artery, and the symptoms were relieved after thrombus removal. The patient was discharged from the hospital on the 5th day after surgery without any recurrence of AMI up to now. The pharmacology and toxicity of batroxobin are intricate, necessitating careful consideration of potential secondary thrombosis during treatment, particularly regarding fibrinogen levels.

Classification Accuracy of Linear Discriminant Function using Principal Components with Multiple Correlated Variables: A Simulation based Exploration

ABSTRACT Background: Linear Discriminant Analysis (LDA) is a powerful and widely used technique for classification with correlated variables. Principal Components (PCs) group these variables into linear combinations and produce independent variables. The LDA on these PC’s may provide better classification accuracy in clinical diagnostics than on usual measurements. Methodology: Two datasets were utilized for demonstration: one from a Sudden Sensorineural Hearing Loss (SSNHL) case-control study and the other from a Gall Bladder (GB) case-control study. Linear Discriminant Analysis (LDA) was conducted on the actual correlated measured variables for group classification, as well as on the derived principal component variables, to compare their classification accuracies. Performance metrics including Sensitivity, Specificity, Positive Predictive Value (PPV), Negative Predictive Value (NPV), Classification Accuracy, and F1 Score were assessed. For validation, a third simulated dataset was employed. Additionally, LDA was performed on each dataset using eigenvectors of the control group applied to the cases and vice versa, revealing a strong agreement in classification as measured by the kappa statistic. Results: When LDA was applied to the actual lipid measurements in the SSNHL dataset, the classification accuracy was 57.2%, and the F1 score was 39.7%. However, when LDA was performed using principal components (PCs), the classification accuracy markedly improved to 99.2%, with an F1 score of 98.5%. Similarly, for the GB cancer dataset, the classification accuracy and F1 score were initially 77.2% and 77.3%, respectively. Upon applying LDA with the PCs, these metrics were significantly enhanced to 98.4% and 98.3%, respectively. For the simulated dataset, both the classification accuracy and F1 score were 99.1%. The study also demonstrated that the classification accuracy and F1 score remained consistent regardless of whether the eigenvectors from the cases or controls were used to classify new subjects (Kappa Statistic = 0.962, P &lt; 0.001). Conclusion: In group separation, utilizing principal components significantly improves classification accuracy and overall performance metrics, outperforming the use of the original correlated predictors.

Efficacy of an oral narrative language intervention program on children with hearing impairment: a randomized controlled trial

BackgroundChildren with hearing impairment have less opportunities for adequate language exposure which results in delayed narrative and discourse language development. Previous intervention studies have mainly focused on the early developing language forms, with little focus on narrative intervention. The aim of the current study was to develop the Oral Narrative Language Intervention Program (ONLIP) and detect its efficacy on improving narrative and linguistic skills of HI children in comparison to conventional language and auditory training.The randomized controlled study was conducted on 44 children with sensorineural hearing loss. The subjects were randomly divided by block randomization into two groups: group A (cases) which received training with the ONLIP and group B (control) which received conventional intervention for 3 months. The participants were evaluated pre- and post-intervention by the Arabic version of the Test of Narrative Language — Second Edition (TNL-2) and the Comprehensive Arabic Language Test (CALT).ResultsA statistically significant improvement was found in all of the assessed narrative comprehension and production skills, as well as the composite performance in the HI cases group when both groups were compared post-therapy. A statistically significant improvement was found in both groups when the scores for the CALT were compared between pre- and post-intervention.ConclusionsONLIP is an effective tool in improving the narrative skills of HI children in comparison to conventional training. Both forms of intervention are effective in improving linguistic form and structure.

Clinical and audiological profile of patients with sudden sensorineural hearing loss after exposure to recreational noise.

Clinical and audiological profile of patients with sudden sensorineural hearing loss after exposure to recreational noise.

Homozygous variants in EIF3K associated with neurodevelopmental delay, microcephaly, and growth retardation.

Homozygous variants in EIF3K associated with neurodevelopmental delay, microcephaly, and growth retardation.

The efficacy of methylprednisolone acetate nanogel in treating patients with sudden sensorineural hearing loss

Intratympanic injection of methylprednisolone acetate is considered to be one of the effective drugs in the treatment of sudden sensorineural hearing loss. In this study, the efficacy of intratympanic injection of methylprednisolone acetate nanogel in patients with sudden sensorineural hearing loss was investigated. It is a double-blind randomized clinical trial in one of Iran’s hospitals. 96 patients diagnosed with sudden sensorineural hearing loss were examined. The intervention group (40 patients) was treated with intratympanic nanogel form of methylprednisolone acetate (four times, one day in between) along with oral prednisolone, and the control group (56 patients) was treated with the usual ampoule form of methylprednisolone acetate along with oral prednisolone. The patients were followed up two weeks and two months after the treatment by checking the audiometric findings. 22.9% of all patients had complete response to treatment and 58.3% partial response to treatment. Complete response to treatment was more common in patients of the intervention group, and non-response to treatment was more common in the control group. The frequency of tinnitus two months after the start of treatment was significantly lower in the intervention group, but the frequency of dizziness was not significantly different between the two groups. In both groups, a significant improvement in audiometric findings was observed after treatment compared to before treatment (p < 0.0001). In both groups, a significant improvement in audiometric findings was observed after treatment compared to before treatment (p < 0.0001), and this improvement rate was slightly higher in the intervention group than in the control group. Combined treatment of systemic steroid with both nanogel and or conventional intratympanic methylprednisolone acetate can be effective in the treatment of sudden sensorineural hearing loss, but the effectiveness of the nanogel form of the drug is slightly higher. Therefore, drug transfer based on nanogels can be considered to achieve better treatment results.

Gene signatures and genotype-phenotype correlations of sensorineural hearing loss in Noonan syndrome and related RASopathies

Hearing loss frequently occurs in Noonan syndrome and related RASopathies (NS-RAS), with conductive hearing loss being common. However, the genotypic and phenotypic features of sensorineural hearing loss (SNHL) in NS-RAS, as well as genotype-phenotype correlations, remain unknown. Leveraging in-house database for syndromic deafness (N = 1666), we analyzed the genomic landscape and clinical phenotypes of 94 NS-RAS families with genetically confirmed via targeted panel sequencing. In particular, we explored the genetic signature of SNHL in NS-RAS and provided a detailed description of the auditory characteristics of SNHL, including its natural progression and outcomes of audiological rehabilitation. Additionally, molecular modeling and functional assays were conducted to explore how PTPN11 variants with distinct auditory phenotypes affect downstream signaling pathways. Resultantly, eighteen (19.1%) exhibited SNHL, predominantly with PTPN11 (88.9%) and RAF1 (11.1%) variants. Of these patients, the majority exhibited severe-to-profound SNHL with congenital onset, and cochlear implantation yielded favorable auditory outcomes. The remaining patients maintained normal hearing throughout the follow-up periods. The PTPN11 variants linked to SNHL compromise autoinhibition between the N-SH2 and PTP domains or disrupts the interaction between the C-SH2 domain and phosphorylated tyrosine (pTyr), showing a gain-of-function effect on the RAS/ERK cascade. Specifically, those variants associated with high penetrance and severe expressivity significantly enhanced ERK phosphorylation more than the variants associated with low penetrance and milder expressivity. This is the first cohort study on NS-RAS in South Korea, elucidating the gene signatures and phenotypic characteristics of SNHL and suggesting genotype-auditory phenotype correlations that inform clinical practice.

Retrospective Analysis of Hearing Outcomes of Cochlear Implantation in Patients with Deafness Due to Congenital CMV Infection

Cytomegalovirus (CMV) infection in pregnant women is one of the most common causes of congenital infection in children. It is often asymptomatic but can lead to serious complications, including progressive sensorineural hearing loss. Profound hearing loss is an indication for cochlear implantation (CI). Electrode impedance and neural response telemetry (NRT) thresholds can be measured to confirm correct electrode placement and speech processor programming. Background/Objectives: The aim of the study is to evaluate the hearing outcome of children with profound sensorineural hearing loss or deafness due to cCMV infection after CI compared to a control group of children born with other causes of congenital hearing loss and to identify prognostic factors predicting the outcome of patients with hearing loss due to cCMV infection after CI. Methods: A retrospective study was conducted in patients implanted between 2016 and 2023 at the Department of Otolaryngology of the Institute of the Polish Mother’s Memorial Hospital Research Institute in Łódź. Pre- and postoperative hearing levels, electrode impedance and neural response telemetry (NRT) thresholds were compared. The degree of pre-implantation hearing loss was assessed by the level of the recorded V-wave in the ABR test. Post-implantation hearing assessment was based on the last available free-field tonal audiometry measurement. Impedance measurements were included: intraoperative, 1, 6, 12 months after CI, respectively, and NRT thresholds. Results: The final analysis included 84 patients with profound sensorineural hearing loss and complete audiological follow-up data: 13 patients with congenital CMV (cCMV) infection and 71 patients with other causes of deafnes. The analysis included 175 implanted ears: 17 in the CMV group and 158 in the control group. The age at implantation ranged from 1 to 11 years in the CMV and from 1 to 13 years in the control group. Mean preoperative hearing thresholds were 94.54 dB in the CMV group and 97.04 dB in the control group. At the most recent postoperative evaluation, mean thresholds improved to 33.83 dB and 36.42 dB, respectively. No statistically significant differences were observed between the groups. Mean intraoperative NRT values were 79.74 in the CMV group and 86.90 in the non-CMV group. Final NRT values were 129.77 and 130.76, respectively. Mean impedance values measured intraoperatively and at 1, 6 and 12 months postoperatively were 11.09 kOhm, 13.40 kOhm, 8.35 kOhm and 8.25 kOhm in the CMV group; and 12.28 kOhm, 14.06 kOhm, 9.60 kOhm and 8.00 kOhm in the control group, respectively. Conclusions: CI in children with deafness caused by cCMV infection is an effective treatment option. Initial electrical impedance values of the electrodes increase after implant activation and decrease in subsequent months of follow-up, suggesting the absence of active adhesion processes in the cochlea.