Cochlear Implantation In Patients Research Articles

Octyl-2-cyanoacrylate Tissue Adhesive Closure Versus Subcuticular Suture for Post-Auricular Incisions.

Skin closure techniques in otolaryngology vary based on surgeon preference and wound site. Octyl-2-Cyanoacrylate may be a safe, rapid, and cost-effective option for post-site closure. This randomized controlled trial study aimed to compare Octyl-2-Cyanoacrylate and conventional subcuticular sutures in ear surgeries, assessing wound closure results in both case and control groups. This prospective, randomized, controlled, single-blind study was conducted at Ohud Hospital from May 2021 to May 2022. Ear surgery and cochlear implantation in patients were examined, and each group was randomly assigned to receive Dermabond TM and deep layer subcuticular sutures closure. Incisions were assessed at various time points, including 3 weeks, 6 weeks, 6 months, and 1 year post-surgery. The patient and observer Scar Assessment Scale was used, and two independent ear surgeons used the Stony Brook Scar Evaluation Scale for initial scar assessment. This study involved 126 ear surgery and cochlear implantation patients randomized to use cyanoacrylate tissue adhesive or subcuticular suture for port site closure. The study found that tissue adhesive (OCA) was faster and more efficient than standard sutures, saving an average of 12 min per incision in each ear. Incision cosmesis showed immediate results and significant differences, and patient satisfaction with OCA wound closure was higher than standard sutures. The findings confirmed that cyanoacrylate tissue adhesive significantly reduced the time needed for skin closure during ear surgery and showed immediate cosmetic improvements without any documented instances of bleeding, hematoma, infection, or wound separation. This is a randomized controlled trial, it follows Level 2 of evidence. Randomized trial or observational study with dramatic effects Laryngoscope, 2024.

Perceived social support improves health-related quality of life in cochlear implant patients.

Perceived social support has been shown to positively correlate with health-related quality of life (HR-QoL) in a variety of conditions. This study investigated whether perceived social support is affecting HR-QoL of patients who receive a cochlear implant (CI) for deafness. Eighty eight adults (56 males, 32 females; mean age: 60years) with a uni- or bilateral CI for bilateral high-grade hearing loss were administered two questionnaires: a questionnaire for perceived social support (FSU-14) and the Nijmegen Cochlear Implant Questionnaire (NCIQ) for hearing-specific HR-QoL. Administration of the questionnaires occurred at four points in time: before implantation and three, 12 and 24months after implant activation. The CI patients had quite high levels of perceived social support (mean percentile rank: 71), which remained stable at all four measurement points. Multivariate Analysis showed a significant interaction between perceived social support and HR-QoL indicating that higher perceived social support lead to higher improvement of HR-QoL after cochlear implantation. The CI patients in this study had higher than average levels of perceived social support, which did not change before and after cochlear implantation. Perceived social support and HR-QoL were related such that patients with high levels of perceived social support experienced greater improvement of their HR-QoL after cochlear implantation than patients with low levels of perceived social support. Based on this finding, perceived social support must be considered as an important factor for HR-QoL after cochlear implantation.

[The value of HRCT in predicting cerebrospinal fluid gusher during cochlear implantation].

Objective:To investigate the predictive value of temporal bone high-resolution CT（HRCT） multiplanar reconstruction（MPR） for cerebrospinal fluid（CSF） gusher during cochlear implantation in patients with inner ear malformation. Methods:The clinical data of 33 patients（36 ears） with inner ear malformation who underwent cochlear implantation were retrospectively analyzed. The predictive value of HRCT for cerebrospinal fluid gusher during cochlear implantation was evaluated. Results:The width of the cochlear foramen（P=0.024, OR=1.735） and the diameter of the inner auditory meatus（P=0.022, OR=6.119） were independent risk factors for CSF gusher during cochlear implantation. The area under the curve（AUC） of cochlear foramen width in predicting intraoperative gusher was 0.851, the sensitivity was 93.33%, and the specificity was 61.90%. The AUC of the upper and lower diameter of the internal auditory canal for predicting intraoperative gusher was 0.848, the sensitivity was 80.00%, and the specificity was 80.95%. The AUC of cochlear foramen width combined with the upper and lower diameters of the internal auditory meatus for predicting intraoperative gusher was 0.930, the sensitivity was 80.00%, and the specificity was 95.24%. Conclusion:Based on temporal bone HRCT, the prediction model of cochlear foramen width combined with the upper and lower diameter of the internal auditory canal has crucial predictive value for the "gusher" during cochlear implantation in patients with inner ear malformation.

[Cochlear implantation through retro-facial approach with congenital microtia malformation with facial nerve deformity: a case report].

The difficulty of cochlear implantation in patients with congenital microtia is usually increased due to the vague anatomical marks and facial nerve malformation. The common types of facial nerve malformation include facial nerve bony cover loss, aberrant position, and bifurcation malformation. Bifurcation malformation may obscure the oval window, press against stapes, and bifurcate in the vestibular window while obscuring the round window. It is important to correctly identify the facial nerve and choose a reasonable surgical approach to avoid postoperative complications. This article describes a case of profound sensorineural hearing loss due to facial nerve malformation in our institution. The patient underwent cochlear implantation through the retro-facial approach. There was no facial nerve injury or dysfunction symptoms such as facial paralysis and hemifacial spasm 2 years after the operation, and the cochlear implant works well. The score of the categories of the auditory performance（CAP） questionnaire was 7, and the score of the speech intelligibility rating（SIR） questionnaire was 4. When the round window cannot be exposed through the facial recess approach during surgery, the retro-facial approach is a feasible method. To avoid facial nerve injury, a thin-section CT of the temporal bone should be performed before the middle and inner ear surgery for patients with facial nerve malformation, and the intraoperative facial nerve monitor should be used to clarify the course of the facial nerve to avoid injury.

Autism's Impact on Cochlear Implantation Surgery Outcomes in Deaf Children.

Cochlear implants (CI) provide a hearing sense for severe to profound hearing-impaired patients, both adults and children, and they are a broadly effective and accepted therapeutic method for those patients. Also, Deaf children with comorbidities, including autism spectrum disorders (ASDs), undergo cochlear implantation. ASDs are a group of developing disorders characterized by abnormalities in social interaction and communication with limited repetitive patterns of behavior. This study aimed to assess the effect of Autism on CI surgery outcomes in Deaf Children. We followed 12 autistic patients with cochlear implantation and 12 non-autistic cochlear-implanted patients for two years. The Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores were used to assess 6, 12, and 24 months after cochlear implantation surgery. During the 24-month follow-up, the CAP means scores increased in both groups, and SIR and CAP progresses were considerably greater in non-ASD children (P<0.001). However, in ASD children, the progress of CAP and SIR variables were significant, with 99% and 95% confidence, respectively, at 24 months after surgery. Although the CIs could improve hearing performance in autistic patients, speech development after CIs in autistic children could affected by several factors, including the severity of autism, and this can be effective in providing pre-implant counseling to parents. The application of the alternative communication methods could be taken into account as a potential rehab technique.

Demographic Representation in Clinical Research Relative to a Cochlear Implant Patient Population.

Research samples that are representative of patient populations are needed to ensure the generalizability of study findings. The primary aim was to assess the efficacy of a study design and recruitment strategy in obtaining a participant sample that was representative of the broader cochlear implant (CI) patient population at the CI center. A secondary aim was to review whether the CI recipient population was representative of the state population. Demographic variables were compared for a research participant sample (n = 79) and the CI patient population (n = 338). The participant sample was recruited from the CI patient population. The study design included visits that were at the same location and frequency as the recommended clinical follow-up intervals. The demographics for the combined group (participant sample and patient population) were then compared to the reported demographics for the population in North Carolina. There were no significant differences between the participant sample and patient population for biological sex, age at implantation, racial distribution, socioeconomic position, degree of urbanization, or drive time to the CI center (p ≥ 0.086). The combined CI recipient population was significantly different from the North Carolina population for the distributions of race, ethnicity, and degree of urbanization (p < 0.001). The study design and recruitment strategy allowed for recruitment of a participant sample that was representative of the CI patient population. Disparities in access to cochlear implantation persist, as supported by the significant differences in the combined CI recipient population and the population for our state. 3 Laryngoscope, 2024.

[Cochlear implantation in patients with autoimmune hearing loss].

Autoimmune inner ear disease (AIED) manifests with recurrent fluctuating sensorineural hearing loss and vestibular symptoms. Treatment includes steroids and avariety of immunosuppressants. Despite adequate treatment, sensorineural hearing loss can be progressive to the point of deafness. In these patients, acochlear implant (CI) is indicated. We present the case of a25-year-old male who underwent cochlear implantation in the left ear. After implantation we noticed brisk variations in impedances which were related to application of the previously prescribed tumor necrosis alpha (TNFα) inhibitor adalimumab. Can immunomodulatory therapy in AIED patients after CI fitting affect the quality of hearing rehabilitation? We documented impedances and speech intelligibility (Freiburg monosyllable test) of our patient for 1year in dependence on adalimumab therapy. Directly after implantation, impedances were within the normal range. During the further course, impedances started to rise, and recurrent adjustments of the implant were needed. Adalimumab therapy was reinitiated, which resulted in asubsequent reduction of impedances. Cochlear implantation can be necessary in some AIED patients and poses asufficient method for hearing rehabilitation. Depending on the activity of the underlying disease, arise in impedances may occur. Immunomodulatory treatment may therefore be necessary to maintain adequate hearing results with the CI.

Cochlear implantation in patients with Charcot-Marie-Tooth disease: two cases with a review of the literature.

To report two cases of bilateral cochlear implantation (CI) in Charcot-Marie-Tooth disease (CMT) patients with novel mutations. Furthermore, we conducted a detailed literature review on the profile and outcomes of CI in this uncommon clinical circumstance. Case 1 involved a 25-year-old woman who was referred for sudden hearing loss (HL) in her left ear and had a 7-year history of HL in her right ear. She was diagnosed with CMT type 1 with a thymidine phosphorylase gene mutation. CI was performed on her left side because her hearing gradually worsened to deafness in both ears. At 3months post-operation, her speech discrimination score without lip-reading improved from 0 to 100%. She underwent a second CI on her right ear 6months after her first CI. Two years from her first operation, the speech discrimination score was 100%. Case 2 received her first CI on her right ear at the age of nine for her bilateral HL. She was diagnosed with CMT type 2 with a Twinkle mitochondrial DNA helicase gene mutation. Preoperatively, the speech discrimination score in both ear-aided conditions was 70%. At the 7-year post-operation follow-up, the speech discrimination score was 76%. A second CI was performed due to decreasing hearing ability in her left ear. The speech discrimination score showed 100% at 7months after the second CI. CI is an effective hearing rehabilitation option for CMT patients with severe-to-profound SNHL. Neuro-otologists should consider CI as a treatment option, even though hearing loss in CMT is associated with auditory neuropathy spectrum disease (ANSD).

Cochlear Implantation in Neurofibromatosis Type 2-Related Schwannomatosis: Long-Term Hearing Outcomes.

To evaluate long-term hearing outcomes following cochlear implantation in patients with neurofibromatosis type 2 and ipsilateral vestibular schwannoma. Retrospective study. Tertiary general hospital. Twenty-two patients undergoing cochlear implantation between 2004 and 2018 with at least 1 year of follow-up were included. Patients were categorized as "users" or "nonusers" of their cochlear implant (CI). For users, speech perception (disyllabic words) without lip-reading was assessed in quiet conditions 1-year postimplantation, and annually thereafter. CI users were classified into 2 groups on the basis of speech intelligibility (≥40% or <40%). Demographic data, treatment options, and tumor size were also recorded. One year after implantation, 16 (73%) patients used their CI daily. Twelve of these patients had a speech intelligibility ≥40% (mean: 74 ± 21.9%). Three had a Koos stage IV tumor. At the last visit (mean duration of follow-up: 6 ± 5 years), 12 of these 16 patients were still using their implant daily, and 6 had a speech intelligibility ≥40%. No predictive factors for good performance at 1 year or performance stability were identified. Neurofibromatosis type 2 is a complex disease profoundly affecting patient quality of life, and cochlear implantation should always be considered on a case-by-case basis. In some individuals, cochlear implantation can provide good speech intelligibility for extended periods, even posttreatment or in cases of large tumors.

Renewed Concept of Mastoid Cavity Obliteration with the Use of Temporoparietal Fascial Flap Injected by Injectable Platelet-Rich Fibrin after Subtotal Petrosectomy for Cochlear Implant Patients.

Background: The subtotal petrosectomy procedure may be useful for cochlear implantation in selected patient groups. Although it is highly effective, complications can arise, which may have economic implications for the patient due to the high cost of the device. Therefore, several authors have attempted to identify the most effective concept for obliteration. Methods: We present a pilot descriptive study of application techniques for obliterating cavities after subtotal petrosectomy using a temporoparietal fascial flap (TPFF) modified with injectable platelet-rich fibrin (IPRF+) for three cochlear implant (CI) patients. Results: Our concept preserves important anatomical structures, such as the temporalis muscle, which covers the CI receiver-stimulator. Injection of IPRF+ also increases the available tissue volume for obliteration and enhances its anti-inflammatory and regenerative potential. Conclusions: To the best of our knowledge, the use of TPFF for filling the cavity has not been adopted for CI with SP and for blind sac closure. Our literature review and our experience with this small group of patients suggest that this procedure, when combined with IPRF+ injections, may reduce the risk of potential infection in the obliterated cavity, particularly when used with CI. This technique is applicable only in cases when the surgeons are convinced that the middle ear cavity is purged of cholesteatoma.

Navigating the Usher Syndrome Genetic Landscape: An Evaluation of the Associations between Specific Genes and Quality Categories of Cochlear Implant Outcomes.

Usher syndrome (US) is a clinically and genetically heterogeneous disorder that involves three main features: sensorineural hearing loss, retinitis pigmentosa (RP), and vestibular impairment. With a prevalence of 4-17/100,000, it is the most common cause of deaf-blindness worldwide. Genetic research has provided crucial insights into the complexity of US. Among nine confirmed causative genes, MYO7A and USH2A are major players in US types 1 and 2, respectively, whereas CRLN1 is the sole confirmed gene associated with type 3. Variants in these genes also contribute to isolated forms of hearing loss and RP, indicating intersecting molecular pathways. While hearing loss can be adequately managed with hearing aids or cochlear implants (CIs), approved RP treatment modalities are lacking. Gene replacement and editing, antisense oligonucleotides, and small-molecule drugs hold promise for halting RP progression and restoring vision, enhancing patients' quality of life. Massively parallel sequencing has identified gene variants (e.g., in PCDH15) that influence CI results. Accordingly, preoperative genetic examination appears valuable for predicting CI success. To explore genetic mutations in CI recipients and establish correlations between implant outcomes and involved genes, we comprehensively reviewed the literature to gather data covering a broad spectrum of CI outcomes across all known US-causative genes. Implant outcomes were categorized as excellent or very good, good, poor or fair, and very poor. Our review of 95 cochlear-implant patients with US, along with their CI outcomes, revealed the importance of presurgical genetic testing to elucidate potential challenges and provide tailored counseling to improve auditory outcomes. The multifaceted nature of US demands a comprehensive understanding and innovative interventions. Genetic insights drive therapeutic advancements, offering potential remedies for the retinal component of US. The synergy between genetics and therapeutics holds promise for individuals with US and may enhance their sensory experiences through customized interventions.

Machine learning-based prediction of the outcomes of cochlear implantation in patients with inner ear malformation.

The objectives of this study are twofold: first, to visualize the structure of malformed cochleae through image reconstruction; and second, to develop a predictive model for postoperative outcomes of cochlear implantation (CI) in patients diagnosed with cochlear hypoplasia (CH) and incomplete partition (IP) malformation. The clinical data from patients diagnosed with cochlear hypoplasia (CH) and incomplete partition (IP) malformation who underwent cochlear implantation (CI) at Beijing Tongren Hospital between January 2016 and August 2020 were collected. Radiological features were analyzed through 3D segmentation of the cochlea. Postoperative auditory speech rehabilitation outcomes were evaluated using the Categories of Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR). This study aimed to investigate the relationship between cochlear parameters and postoperative outcomes. Additionally, a predictive model for postoperative outcomes was developed using the K-nearest neighbors (KNN) algorithm. In our study, we conducted feature selection by using patients' imaging and audiological attributes. This process involved methods such as the removal of missing values, correlation analysis, and chi-square tests. The findings indicated that two specific features, cochlear volume (V) and cochlear canal length (CDL), significantly contributed to predicting the outcomes of hearing and speech rehabilitation for patients with inner ear malformations. In terms of hearing rehabilitation, the KNN classification achieved an accuracy of 93.3%. Likewise, for speech rehabilitation, the KNN classification demonstrated an accuracy of 86.7%. The measurements obtained from the 3D reconstruction model hold significant clinical relevance. Despite the considerable variability in cochlear morphology across individuals, radiological features remain effective in predicting cochlear implantation (CI) prognosis for patients with inner ear malformations. The utilization of 3D segmentation techniques and the developed predictive model can assist surgeons in conducting preoperative cochlear structural measurements for patients with inner ear malformations. This, in turn, can offer a more informed perspective on the anticipated outcomes of cochlear implantation.

Audiologists' attitudes and practice toward referring for psychosocial intervention with cochlear implant patients.

Hearing loss is associated with a range of poor psychosocial outcomes. Cochlear implants (CI) are an available treatment option for significant hearing loss and have been linked to improved quality of life in patients. Evidence suggests that audiologists lack the skills to appropriately detect, address, and refer for psychosocial needs among patients with hearing loss. The objective of this study is to examine the attitudes and practice patterns related to psychosocial care among audiologists who work with CI users. A cross-sectional survey was administered to clinical audiologists who work with CI recipients in the United States. The survey evaluated participants' attitudes toward psychosocial services and factors that contribute to their abilities to address the psychosocial needs of their patients. Additionally, participants were surveyed about their practice patterns including the use of psychosocial screeners, clinical protocols regarding psychosocial care, and referral patterns for coordinated psychosocial services. Descriptive statistics were used to summarize survey responses. Sixty-eight audiologists completed the survey. Of these audiologists, a majority (73.6%) held the attitude that most or all CI patients would benefit from psychosocial intervention. Despite clinicians' recognition of psychosocial needs in this population, over 90% of participants reported never screening for psychosocial symptoms. Additionally, a majority of respondents indicated that they seldom refer their patients for psychosocial services, with referrals occurring less than half the time (58%) or never (27%). Additionally, few audiologists reported utilizing protocols or resources for guiding psychosocial practices. Audiologists indicated that the primary factors that influence their psychosocial practices include time available to spend with the patient and their comfort level in counseling. Audiologists working with CI patients recognize the potential benefit of psychosocial intervention in this population. Nevertheless, audiologists encounter barriers in clinical practice which limit their ability to identify and address the psychosocial needs of their patients. Strategies designed to enhance audiologists' capacity to recognize the psychosocial needs of CI users, in addition to improved interprofessional practice on CI teams, implies significant opportunities to improve the provision of patient-centered hearing care.

Caregivers’ Satisfaction with Telemedicine for Aural Rehabilitation of Cochlear Implant Patients during Coronavirus Disease 2019: A Cross-sectional Study

Abstract Objective: During the coronavirus disease 2019 (COVID-19) pandemic, teletherapy was a reasonable alternative to in-person therapy. However, teletherapy has certain limitations, including technological issues, reduced rapport, and challenging difficulties in engaging with patients. Therefore, this study aimed to investigate the effectiveness of virtual rehabilitation sessions during the COVID-19 pandemic in children with cochlear implants (CIs). Materials and Methods: This cross-sectional study was conducted in a specialized tertiary care ear center to assess virtual aural rehabilitation after cochlear implantation during the COVID-19 pandemic. We included prelingually deaf children aged ≤7 years who underwent unilateral or bilateral CIs and were enrolled in aural rehabilitation with a minimum of one virtual rehabilitation session during the COVID-19 pandemic. Children with other disabilities or technical issues related to the implant devices were excluded. Parental experience and satisfaction with the virtual rehabilitation were evaluated using an interview-based questionnaire scored on a five-point Likert scale. Results: We recruited 34 participants, including 19 males (55.9%) and 15 females (44.1%). Only nine participants (26.5%) had siblings with CIs. None of the participants disagreed with the survey questionnaire. When caregivers were stratified based on their level of education, family experience with CIs, and place of residence, no significant differences were found in ease of access to the virtual sessions. Conclusion: High levels of satisfaction were observed between caregivers and professionals regarding usage comfort. However, future studies are required to evaluate the effectiveness of telemedicine rehabilitation compared with conventional methods, particularly after the COVID-19 pandemic, with respect to different age groups.

Digital Health Literacy: Evaluating the Readability and Reliability of Cochlear Implant Patient Information on the Web.

Hearing aids and implants are used to treat hearing loss, with cochlear implants being the most successful option for severe sensorineural hearing loss. Patients frequently use the internet as a trusted source of clinical information before committing to any therapeutic procedure, including receiving a cochlear implant. A health resource's readability and dependability influence its value to patients. Readability refers to how easily language can be understood, whereas reliability refers to the correctness and consistency of the information presented. JAMA standards and the DISCERN tool were used to assess the reliability of the websites listed. For readability analysis, the FRE, FKG and GFI were chosen. The acceptable readability level was set to < 7 for the FKG, GF score over 17 as the equivalent of college-level education and ≥ 80.0 for the FRE. The readability scores vary across the sources, suggesting a range of comprehension levels required for understanding the cochlear implant patient information found on Google. There was a statistical difference detected in Discern score between the groups (p = 0.008). The mean discern score was significantly higher in hospital generated sources when compared to industry (3.13±0.69 vs. 2.11±0.78, p = 0.03).

Genetic Characteristics and Audiological Performance After Cochlear Implantation in Patients With Incomplete Partition Type III.

Genetic Characteristics and Audiological Performance After Cochlear Implantation in Patients With Incomplete Partition Type III.

Calculation of the Faradaic Impedance of the Electrode-Tissue Interface Improves Prediction of Behavioral T/C Levels in Cochlear Implant Patients.

Fitting of cochlear implants is a labor-intensive process, and therefore automated fitting procedures are being sought. The objective of this study was to evaluate if decomposition of the complex impedance of the electrode-tissue interface could provide additional parameters that show improved correlation with the behavioral T/C levels. A new method for decomposing the complex impedance of the electrode-tissue interface was developed and tested in 18 patients in a prospective study in a tertiary otologic referral center. The averaged near-field Faradaic resistance (RF) calculated in study subjects shows a very strong correlation (R2=0.80) with the behavioral C levels and can be used for automated fitting in most patients. The standard deviation for the T levels and the C levels calculated for each of the electrode contacts in all study subjects is in the range of 10-15 CL and 15-20 CL, respectively. These higher values of the standard deviations are caused by a few outliers who require that additional parameters have to be added to the metric equation, allowing for the automated prediction of the T/C levels. A new method for deriving information from the electrode impedance measurements shows excellent correlation of the Faradaic resistance with the behavioral T/C levels in most patients and can be very useful for fitting cochlear implants based on objective measures. Since some patients still show discrepancies between the predicted T/C levels based on the RF calculation, additional parameters have to be added to the metric equation, allowing for automated prediction of the T/C levels.

Lack of Methylation Changes in GJB2 and RB1 Non-coding Regions of Cochlear Implant Patients with Sensorineural Hearing Loss.

Recent advances in epigenetic studies continue to reveal novel mechanisms of gene regulation and control, however little is known on the role of epigenetics in sensorineural hearing loss (SNHL) in humans. We aimed to investigate the methylation patterns of two regions, one in RB1 and another in GJB2 in Filipino patients with SNHL compared to hearing control individuals. We investigated an RB1 promoter region that was previously identified as differentially methylated in children with SNHL and lead exposure. Additionally, we investigated a sequence in an enhancer-like region within GJB2 that contains four CpGs in close proximity. Bisulfite conversion was performed on salivary DNA samples from 15 children with SNHL and 45 unrelated ethnically-matched individuals. We then performed methylation-specific real-time PCR analysis (qMSP) using TaqMan® probes to determine percentage methylation of the two regions. Using qMSP, both our cases and controls had zero methylation at the targeted GJB2 and RB1 regions. Our study showed no changes in methylation at the selected CpG regions in RB1 and GJB2 in the two comparison groups with or without SNHL. This may be due to a lack of environmental exposures to these target regions. Other epigenetic marks may be present around these regions as well as those of other HL-associated genes.

Gene mutations as a non-invasive measure of adult cochlear implant performance: Variable outcomes in patients with select TMPRSS3 mutations.

The cochlear implant (CI) has proven to be a successful treatment for patients with severe-to-profound sensorineural hearing loss, however outcome variance exists. We sought to evaluate particular mutations discovered in previously established sensory and neural partition genes and compare post-operative CI outcomes. Utilizing a prospective cohort study design, blood samples collected from adult patients with non-syndromic hearing loss undergoing CI were tested for 54 genes of interest with high-throughput sequencing. Patients were categorized as having a pathogenic variant in the sensory partition, pathogenic variant in the neural partition, pathogenic variant in both sensory and neural partition, or with no variant identified. Speech perception performance was assessed pre- and 12 months post-operatively. Performance measures were compared to genetic mutation and variant status utilizing a Wilcoxon rank sum test, with P<0.05 considered statistically significant. Thirty-six cochlear implant patients underwent genetic testing and speech understanding measurements. Of the 54 genes that were interrogated, three patients (8.3%) demonstrated a pathogenic mutation in the neural partition (within TMPRSS3 genes), one patient (2.8%) demonstrated a pathogenic mutation in the sensory partition (within the POU4F3 genes). In addition, 3 patients (8.3%) had an isolated neural partition variance of unknown significance (VUS), 5 patients (13.9%) had an isolated sensory partition VUS, 1 patient (2.8%) had a variant in both neural and sensory partition, and 23 patients (63.9%) had no mutation or variant identified. There was no statistically significant difference in speech perception scores between patients with sensory or neural partition pathogenic mutations or VUS. Variable performance was found within patients with TMPRSS3 gene mutations. The impact of genetic mutations on post-operative outcomes in CI patients was heterogenous. Future research and dissemination of mutations and subsequent CI performance is warranted to elucidate exact mutations within target genes providing the best non-invasive prognostic capability.

A Case of Bilateral Sequential Sudden Sensorineural Hearing Loss in Protein S Deficiency and Successful Recovery of Hearing After Cochlear Implantation

Bilateral sequential sudden sensorineural hearing loss (SSHL) is a rare and challenging clinical condition, but its association with protein S deficiency has received limited attention. This paper reports a case of bilateral sequential SSHL in a patient with protein S deficiency who underwent cochlear implantation and achieved significant improvement in hearing. The patient received high-dose systemic corticosteroids and anticoagulation therapy before the implantation, which had no significant effect. Cochlear implantation was then performed in the left ear, and the patient showed significant improvement in speech understanding and overall hearing ability. A year later, the patient underwent surgery for the opposite ear with favorable outcome. This case highlights the potential benefits of cochlear implantation in patients with bilateral sequential SSHL and protein S deficiency and emphasizes the importance of considering cochlear implantation as a treatment option in such patients.