Profound Sensorineural Hearing Loss Research Articles

[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.

Novel likely pathogenic variant in the EYA1 gene causing Branchio oto renal syndrome and the exploration of pathogenic mechanisms

ObjectiveBranchio-oto-renal syndrome (BOR, OMIM#113,650) is a rare autosomal dominant disorder that presents with a variety of symptoms, including hearing loss (sensorineural, conductive, or mixed), structural abnormalities affecting the outer, middle, and inner ear, branchial fistulas or cysts, as well as renal abnormalities.This study aims to identify the pathogenic variants by performing genetic testing on a family with Branchio-oto-renal /Branchio-otic (BO, OMIM#602,588) syndrome using whole-exome sequencing, and to explore possible pathogenic mechanisms.MethodsThe family spans 4 generations and consists of 9 individuals, including 4 affected by the BOR/BO syndrome. Phenotypic information, including ear malformation and branchial cleft, was collected from family members. Audiological, temporal bone imaging, and renal ultrasound examinations were also performed. Whole-exome sequencing was conducted to identify candidate pathogenic variants and explore the underlying molecular etiology of BOR/BO syndrome by minigene experiments.ResultsIntra-familial variability was observed in the clinical phenotypes of BOR/BO syndrome in this family. The severity and nature of hearing loss varied in family members, with mixed or sensorineural hearing loss. The proband, in particular, had profound sensorineural hearing loss on the left and moderate conductive hearing loss on the right. Additionally, the proband exhibited developmental delay, and her mother experienced renal failure during pregnancy and terminated the pregnancy prematurely. Genetic testing revealed a novel heterozygous variant NM_000503.6: c.639 + 3 A > C in the EYA1 gene in affected family members. In vitro minigene experiments demonstrated its effect on splicing. According to the American College of Medical Genetics (ACMG) guidelines, this variant was classified as likely pathogenic.ConclusionThis study highlights the phenotypic heterogeneity within the same family, reports the occurrence of renal failure and adverse pregnancy outcomes in a female patient at reproductive age with BOR syndrome, and enriches the mutational spectrum of pathogenic variants in the EYA1 gene.

Early-stage use of hearing aids preserves auditory cortical structure in children with sensorineural hearing loss.

Hearing is critical to spoken language, cognitive, and social development. Little is known about how early auditory experiences impact the brain structure of children with bilateral sensorineural hearing loss. This study examined the influence of hearing aid use and residual hearing on the auditory cortex of children with severe to profound congenital sensorineural hearing loss. We evaluated cortical preservation in 103 young pediatric cochlear implant candidates (55 females and 48 males) by comparing their multivoxel pattern similarity of auditory cortical structure with that of 78 age-matched children with typical hearing. The results demonstrated that early-stage hearing aid use preserved the auditory cortex of children with bilateral congenital sensorineural hearing loss. Children with less residual hearing experienced a more pronounced advantage from hearing aid use. However, this beneficial effect gradually diminished after 17months of hearing aid use. These findings support timely fitting of hearing aids in conjunction with early implantation to take advantage of neural preservation to maximize auditory and spoken language development.

The geometry of photopolymerized topography influences neurite pathfinding by directing growth cone morphology and migration

Objective. Cochlear implants provide auditory perception to those with severe to profound sensorineural hearing loss: however, the quality of sound perceived by users does not approximate natural hearing. This limitation is due in part to the large physical gap between the stimulating electrodes and their target neurons. Therefore, directing the controlled outgrowth of processes from spiral ganglion neurons (SGNs) into close proximity to the electrode array could provide significantly increased hearing function. Approach. For this objective to be properly designed and implemented, the ability and limits of SGN neurites to be guided must first be determined. In this work, we engineer precise topographical microfeatures with angle turn challenges of various geometries to study SGN pathfinding and use live imaging to better understand how neurite growth is guided by these cues. Main Results. We find that the geometry of the angled microfeatures determines the ability of neurites to navigate the angled microfeature turns. SGN neurite pathfinding fidelity is increased by 20%–70% through minor increases in microfeature amplitude (depth) and by 25% if the angle of the patterned turn is made obtuse. Further, we see that dorsal root ganglion neuron growth cones change their morphology and migration to become more elongated within microfeatures. Our observations also indicate complexities in studying neurite turning. First, as the growth cone pathfinds in response to the various cues, the associated neurite often reorients across the angle topographical microfeatures. Additionally, neurite branching is observed in response to topographical guidance cues, most frequently when turning decisions are most uncertain. Significance. Overall, the multi-angle channel micropatterned substrate is a versatile and efficient system to assess neurite turning and pathfinding in response to topographical cues. These findings represent fundamental principles of neurite pathfinding that will be essential to consider for the design of 3D systems aiming to guide neurite growth in vivo.

Outcomes and Measures of Children with Bilateral Cochlear Implants: A Single-center Experience in Bangladesh

Children dealing with hearing impairment encounter challenges associated with speech and language delays, significantly impacting their quality of life. Recent data from the World Health Organization indicate that 466 million people worldwide experience disabling hearing loss, with 34 million being children. Cochlear implantation (CI) is a novel approach for severe to profound sensorineural hearing loss. In pediatric CI, the main objective is to enable hearing-impaired children to perceive sound promptly, facilitating speech and language development. This study investigated the outcomes of bilateral pediatric cochlear implant (CI) users in Bangladesh, applying the Speech, Spatial, and Qualities of Hearing questionnaire and the Classification of Auditory Performance-II (CAP-II). Conducted at the Combined Military Hospital in Bangladesh, the study involved 10 children with bilateral cochlear implants. The results revealed commendable scores in speech understanding, spatial hearing, and qualities of hearing, underscoring the effectiveness of cochlear implant interventions. Deeply examining the implications is the topic of discussion of these findings, emphasizing the positive impact of early implantation and the adaptability of assessment tools in diverse cultural contexts.

Experiencia clínica con el implante coclear Nurotron™ en una población postlingual hispanohablante: seguridad clínica y resultados audiólogicos

Objectiveto assess clinical safety and postoperative audiological outcomes in postlingual deafness Spanish speaking patients, who underwent surgery with Nurotron™ cochlear implant. Material and MethodsRetrospective descriptive case series study. We performed follow-up of complications and audiological measurements before and after cochlear implantation. Patients with bilateral severe to profound sensorineural hearing loss or patients with unilateral deafness with/without tinnitus were included. Repeated-measures within-subjects for assess pure tone thresholds and speech performance (bilingual test) with a detailed monitoring to establish security or adverse effects were performed. Analysis of variance tests, repetitive measures, were used for statistical analysis. Results31 patients were included, 17 (54.8%) men and 14 (45.2%) women. Mean age at the time of surgery was 49.82 ± 18.8 years. The mean follow-up of the group was 31.56 ± 9.57 months (minimum = 19.6 months and maximum = 52.50months). As major complication one patient (3.23%) had a hard failure that required removal and re-implantation. 25.8% of the patients presented minor complications, the most frequent being vertigo/unsteadiness in 22.6%.The mean of language discrimination (free field at 65 dB SPL) was 62.19% ± 16.66; being 69.82% ± 7.35 in the group of severe to profound bilateral sensorineural hearing loss. A statistically significant reduction was observed in patients with tinnitus, assessed using the visual analog scale, preoperative = 7.2 ± 1,6 vs postoperative (18 months postoperative) = 1.7 ± 1.3 (p < 0.001). ConclusionsThe Nurotron™ cochlear implant shows satisfactory audiological results, in accordance with what has been reported in the literature. Minor complications were similar to previous studies, but the percentage of hard failure should continue to be observed, which was higher than other reports with comparable follow-up.

Bone Conduction Implants: Comparative of Audiometric Results and Quality of Life Bonebridge® vs Osia®.

Bone conduction implants have been indicated for patients with conductive hearing loss, mixed hearing loss, and even profound unilateral sensorineural hearing loss. With the introduction of Bonebridge®, new transcutaneous implant options emerged. The latest is Osia®, a direct-drive variant or active systems where the implant directly generates and applies vibration to the bone. Retrospective study of two cohorts of patients treated with active bone conduction implants at a single center, one with the Bonebridge® device and the other with Osia®. Fourteen patients were included, seven in each group (n=14). The Bonebridge® group showed an average hearing gain in tonal intelligibility thresholds of 32.43±21.39 dB and a gain in the average intelligibility threshold (with 50% discrimination) of 26.29±19.10 dB. In the Osia® group, there was a gain in average tonal thresholds of 41.49±14.16 dB and 23.72±6.98 dB in average intelligibility thresholds. Both devices contributed to improvements in patients' quality of life, as assessed with APHAB in all the variables studied in the test. Both devices offer rehabilitation for hearing loss as an alternative to hearing aids. The Osia® system shows statistically significant(p<0.05) improvements in mid and high frequencies, but Bonebridge® slightly outperforms in speech understanding at 50%. Differences in average tonal thresholds and quality of life are not statistically significant. While auditory improvement is observed post-implantation, other aspects, such as intelligibility thresholds and quality of life, lack statistical significance. Given the limited experience with Osia® and the small sample size, the choice of the device should be personalized. Although the literature is inconsistent due to small sample sizes and variable approaches, some studies suggest potential advantages of the Osia® system, especially in speech comprehension in different environments and greater hearing gain compared to Bonebridge®.

The therapeutic effect of hyperbaric oxygen therapy in patients with severe to profound idiopathic sudden sensorineural hearing loss

The optimal treatment for sudden sensorineural hearing loss (SSNHL) is unclear. Hyperbaric oxygen therapy (HBOT) has been suggested as a viable option for treatment of SSNHL as it improves vascular dysfunction. In this study, we evaluated the therapeutic effects of HBOT by retrospectively reviewing the records of 2206 patients with SSNHL. 54 who had received HBOT were selected for the HBOT groups, while 59 age-matched controls who had not were selected for the control groups. The HBOT and control groups were divided into subgroups according to intratympanic steroid (ITS) use. Groups A–D had received oral steroids + HBOT, oral steroids only, oral steroids + ITS + HBOT, and oral steroids + ITS, respectively. Of the 113 SSNHL patients, 21 had diabetes mellitus (DM) (2, 0, 9, and 10 patients in Groups A–D, respectively). There was no notable difference in hearing improvement between patients receiving HBOT and those in the control group. However, among diabetic patients, those who underwent HBOT demonstrated a significant improvement in hearing when compared to the control group. The combination of HBOT and steroids could potentially be beneficial for treating severe to profound SSNHL patients with DM.

[The application of endoscope and microscope in the stapes surgeries].

Objective:To compare the application of endoscope and microscope in all kinds of stapes surgeries. Methods:Fifty-nine stapes surgeries have been collected from April 2020 to May 2023 in Senior Department of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Chinese PLA Medical School. Hearing level, hospital stay post-operation, times of hospital visit post-operation, etc. have been compared between the endoscopic group and microscopic group. Patients who were failed to place the stapes prosthesis because of the poor exposure of the oval window have been analyzed. Results:Otosclerosis was the most common diagnosis in both groups. There was 1（1/23） middle ear malformation in the endoscopic group and 5（5/36） middle ear malformations in the microscopic group. There were 2 Van Der Hover syndromes and 4 Treacher Collins syndromes in the microscopic group. In the endoscopic group ABG of 10 ears（43.5%） ≤ 10 dB, and ABG of 21 ears（91.3%） ≤20 dB.In the microscopic group ABG of 13 ears（41.9%） ≤ 10 dB, and ABG of 28 ears（90.3%） ≤ 20 dB. There was no statistic difference between 2 groups. Times of hospital visit post-operation in the endoscopic group was less than in the microscopic group（P<0.01）. There was no facial palsy, tympanic perforation or profound sensorineural hearing loss in both groups. Conclusion:Endoscope is more suitable for patients who are evaluated with no severe stapes malformation, or less manipulation of drilling the bone. It could also reduce the hospital visit post-operation. Patients with narrow ear canal or severe middle ear malformation are recommended to perform the surgery with microscope, because it provides the chance of manipulation with 2-hands of surgeons.

Genetic and phenotypic presentation of Usher syndrome - a case report

Usher syndrome is a genetic, clinically heterogeneous condition characterized by sensorineural hearing loss, progressive retinal degeneration, and vestibular dysfunction. There are three phenotypically recognizable types of Usher syndrome. Individuals with Usher syndrome type 1 have no vestibular function and profound sensorineural hearing loss. Individuals with Usher syndrome type 2 have normal vestibular function and mild-to-severe hearing loss with visual impairment that is presented later in life. People with Usher syndrome type III experience hearing and vision loss beginning later in life. In this case report, we are reporting a 7-year-old boy consulted for progressive hearing loss and bilateral vision impairment, and fundus exam revealed mild bilateral retinal vessel attenuation and bone spicule deposits in both eyes. A molecular genetic test done by next-generation sequencing identified a homozygous pathogenic variant in the CDH23 gene (NM_022124.5:c.2255del variant coordinate with amino acid change of p.(Gly752Valfs*13)), confirming the diagnosis of autosomal recessive Usher syndrome type ID (USH1D). The patient had a remarkable improvement with visual and optical aids. Genetic counseling, including reproductive counseling, was provided to the parents. Clinical evaluation, visual hearing tests, and genetic workup confirmed Usher syndrome, which is a rare but dangerous cause of hearing loss and visual impairment that needs to be thoroughly evaluated by a multi-disciplinary team approach.

Efficacy of balance exercises intervention on postural control-related impairment in children with sensorineural hearing loss.

Sensorineural hearing loss is the most common type of permanent hearing impairment and results in postural control and motor deficits in children that may affect or delay all developmental indicators. The purpose of the study was to investigate the efficacy of balance exercises intervention on postural control-related impairment in children with sensorineural hearing loss. Forty students of both genders, ages ranging from 10 to 16 years, diagnosed with severe to profound sensorineural hearing loss, were selected from the Public School for the Deaf and Hard of Hearing in El-Minia district, Minia governorate, Egypt. They divided randomly into two groups, 20 (study group), received balance exercises in addition to their ordinary daily living activities. Meanwhile, the control group of 20 children practiced only the ordinary daily living activities. The outcome was assessed pre-treatment and post-treatment by Humac Balance System and Bruininks-Oseretsky Test (BOT-2) subtest (5) for balance. Regarding the Humac balance system and subtest (5) of BOT-2, there was a statistically significant difference between pre-treatment data and post-treatment data of the study group with a p value equal to 0.036 or less. However, no statistically significant difference was observed in the control group with a p value equal to 0.096 or more. Finally, there was a statistically significant difference between the groups with respect to the post-treatment data, where the p value was 0.014 or less. Postural control of children with sensorineural hearing loss has been improved by balance exercises.

Evaluation of Insertion Speed and Hand Kinematics During Cochlear Implantation in a Fixed Cadaveric Temporal Bone Model

Background:Cochlear implant is the treatment of choice for replacement of sensory deprivation from severe to profound sensorineural hearing loss. Slower insertion of the electrode into the cochlea leads to less intracochlear trauma. Accurate measures of hand motion and insertion speed during live cochlear implant electrode insertions on human subjects are limited.&#x0D; Methods:Data from a single surgeon was collected during insertion of a cochlear electrode into a cadaveric temporal bone using inertial measurement units (IMUs), devices that measure linear acceleration in 3 dimensions (x,y,z). The sensors were affixed as follows: dorsal second digit, dorsal hand, and dorsal wrist with three trials at each position. The extremity inserting the electrode into the inner ear was labeled “inserting limb,” while the other extremity was labeled “non-inserting limb.” Root mean square (RMS) calculations were utilized to record the net acceleration, from which average speed per trial was calculated using the midpoint method.&#x0D; Results:Mean speeds for first digit, hand, and wrist for the inserting limb were 0.23 ± 0.11, 0.15 ± 0.06, 0.16 ± 0.04 mm/s respectively. Mean speeds for first digit, hand, and wrist for the non-inserting limb were 0.26 ± 0.10, 0.13 ± 0.03, 0.16 ± 0.09 mm/s, respectively. The difference in speed between “inserting” and “non-inserting” limbs for all three IMU positions, as well as the relative speeds between all three positions, were statistically significant (p&lt;0.001).&#x0D; Conclusion and Potential Impact:Available literature suggests the mean insertion speed during live cochlear implant surgery is 96.5 mm/min (1.60 mm/s), which is significantly faster than the mean speeds collected during this fixed cadaveric temporal bone study regarding all three positions (p&lt;0.001). Though preliminary, this data of insertions speeds highlights the need for further investigation during live cochlear implant surgery on human subjects using IMUs.

The challenge of a mature final diagnosis of hearing loss severity and early cochlear implantation.

Despite the recent trend of cochlear implantation (CI) at the age of six or even four months is prevalent in many centers around the world, clinicians should be cautious because perinatal risk factors of auditory neuropathy and/or delayed maturation carry the possibility of reversible hearing loss, yielding better auditory performance at the age of one year. The purpose of this study is to raise awareness that early CI may not be universal for all patients. In addition, we specify the factors to be considered in the pre-operative evaluation of CI in infants younger than one year. This study describes four cases provisionally diagnosed with severe to profound sensorineural hearing loss that were presented to the CI clinic to determine candidacy for implantation. Two cases had histories of prematurity, one hadDown syndrome, and one had a family history of hearing loss. None of the study cases were candidates for CI, as they had varying degrees of hearing improvement. Although early CI may yield better auditory performance, the final diagnosis should be made only after repeated subjective and objective measurements as well as family feedback on the child's auditory performance, especially in preterm children. Early auditory brainstem response (ABR) prior to the age of one year in children with cognitive, neurologic, or developmental comorbidities should be interpreted with caution, as ABR "alone" could not accurately represent the child's true hearing ability in this patient population.

Clinical experience with the Nurotron™ cochlear implant in a Spanish-speaking postlingual population: Clinical safety and audiological results

Objectiveto assess clinical safety and postoperative audiological outcomes in postlingual deafness Spanish speaking patients, who underwent surgery with Nurotron™ cochlear implant. Material and methodsRetrospective descriptive case series study. We performed follow-up of complications and audiological measurements before and after cochlear implantation. Patients with bilateral severe to profound sensorineural hearing loss or patients with unilateral deafness with/without tinnitus were included. Repeated-measures within-subjects for assess pure tone thresholds and speech performance (bilingual test) with a detailed monitoring to establish security or adverse effects were performed. Analysis of variance tests, repetitive measures, were used for statistical analysis. Results31 patients were included, 17 (54.8%) men and 14 (45.2%) women. Mean age at the time of surgery was 49.82 ± 18.8 years. The mean follow-up of the group was 31.56 ± 9.57 months (minimum = 19.6 months and maximum = 52.50 months). As major complication one patient (3.23%) had a hard failure that required removal and re-implantation. 25.8% of the patients presented minor complications, the most frequent being vertigo/unsteadiness in 22.6%.The mean of language discrimination (free field at 65 dB SPL) was 62.19% ± 16.66; being 69.82% ± 7.35 in the group of severe to profound bilateral sensorineural hearing loss. A statistically significant reduction was observed in patients with tinnitus, assessed using the visual analogue scale, preoperative = 7.2 ± 1,6 vs postoperative (18months postoperative) = 1.7 ± 1.3 (p < .001). ConclusionsThe Nurotron™ cochlear implant shows satisfactory audiological results, in accordance with what has been reported in the literature. Minor complications were similar to previous studies, but the percentage of hard failure should continue to be observed, which was higher than other reports with comparable follow-up.

Management of a Case of Bilateral Severe External Auditory Canal Stenosis, Bilateral Cholesteatoma Causing Right-sided Inner Ear Erosion, and Bilateral Profound Sensorineural Hearing Loss

Bilateral simultaneous external auditory canal (EAC) stenosis with bilateral cholesteatoma is a rare presentation. We present a case report of surgical and audiological management of an elderly male who presented with bilateral EAC stenosis, bilateral cholesteatoma, right-sided inner ear erosion, and bilateral profound sensorineural hearing loss.

Sensorineural Hearing Loss and Cochleovestibular Malformations in Children in Côte d’Ivoire

Objective: to find the distribution of various malformations and to report the clinical features of commonly encountered IEMs in our practice. Methods: This was a retrospective cross-sectional study, over a period of 14 months (January 2019 to March 2020), in the ENT departments of University Hospitals and the Mother and Child Hospital in Bingerville. All patients, aged up 15 years with computed tomography and/or MRI available, who had a cochleovestibular malformation and sensorineural or predominantly sensorineural mixed hearing loss confirmed were included. Results: The prevalence of sensorineural hearing loss related to cochleovestibular malformations was 3.75% (6 cases out of 160 patients). Patients ranged in age from 2 to 14 years. The diagnosis was made before the age of 3.5 years in 5/6 cases. The sex ratio was 1. Language delay was noted in 5/6 cases. No contributing risk factors were identified. All patients had profound and bilateral hearing loss. Associated malformations were present in 2/6 cases. The most common abnormalities were semicircular canal hypoplasia, internal auditory canal abnormalities and cochlear malformations. There was an association between sensorineural hearing loss linked to malformation and the profound degree of hearing loss. Conclusion: Sensorineural deafness linked to cochleovestibular malformations in children appears to be uncommon. However, they are associated with a risk of profound sensorineural hearing loss. It is therefore important, in the face of any sensorineural hearing loss in the child, to systematically carry out an imaging assessment, even in our low income’s countries.

COMPLICATIONS OF COCHLEAR IMPLANT SURGERY: A DETAILED STUDY

INTRODUCTION Cochlear implantation is nowadays the most important means to restore hearing in patients with severe to profound sensorineural hearing loss who are not managed by conventional hearing amplification methods. The cochlear implant helps patients in better understanding and improvement in auditory abilities, speech, and linguistic development. The best results are obtained by doing the implantation at the earliest possible age. As with any other surgical procedure, cochlear implantation also has risks and complications associated with it. It can be minor or major complications. In our study, we have discussed in detail the challenges and complications of cochlear implant surgery. MATERIAL AND METHODS This is a retrospective study including 500 patients who were evaluated and operated for cochlear implants at MEHROTRA ENT HOSPITAL, Kanpur, India. All patients were investigated by audiological tests such as aided audiometry, ABR, and otoacoustic emission and found to have severe to profound bilateral SNHL. All patients were radiologically evaluated with CT and MRI. Aretrospective review of the patient's data was done including the patient's characteristics, surgery, outcome, and complications. RESULTS A total of 500 patients were included in our retrospective study out of which 337 were males and 163 were females. All the surgeries were carried out by the same group of surgeons using the standard technique of post-auricular incision and posterior tympanotomy. Patients have been followed up regularly and a close watch was kept on the vital signs, lightreflex, pupil size, consciousness, and wound healing. After surgery, patients were kept in the hospital for 2–3 days. Parenteral antibiotics and the compressive mastoid dressing were given, and the patient was discharged with oral antibiotics. We followed up with the patients regularly and managed the complications experienced by them. CONCLUSION Cochlear implantation remains a safe and effective surgical procedure for profound sensorineural hearing loss. We have a moderate rate of minor complications and a minimal rate of major complications in relation to other studies. The majority of minor and major complications can be avoided with adequate surgical techniques and can be managed conservatively. Keywords- Cochlear implants(CI), Minor complications, Major complications, Reimplantation, Cochlear implant surgery.

Treatment of Meniere's disease with simultaneous triple semicircular canal occlusion and cochlear implantation.

Report three cases of simultaneous triple semicircular canal occlusion (TSCO) and cochlear implantation (CI) as the treatment of intractable Meniere's disease (MD). Patients with MD can present occasionally with intractable vertigo and profound sensorineural hearing loss (SNHL). TSCO and CI have been proposed to control vertigo and restore profound deafness in patients with MD separately. However, a few studies have reported simultaneous TSCO and CI in the same surgical procedure for the treatment of MD. In the present study, we described three patients with MD showing incapacitating vertigo and severe SNHL who underwent simultaneous TSCO and CI after examinations of auditory system, vestibular system, and imaging. Their symptoms were significantly alleviated during the follow-up period. The combined TSCO and CI remains a viable treatment option which is effective for the control of vertigo as well as the restoring of hearing in patients with MD.

Strategies for the implementation of the living guidelines for cochlear implantation in adults.

Clinical guidelines for cochlear implants (CI) exist in several countries, however, they lack consistency and often do not encompass the full user journey. This study aims to explore the barriers and facilitators for implementing global Living Guidelines for cochlear implantation in adults with severe, profound or moderate sloping to profound sensorineural hearing loss (SPSNHL) as well as identify guideline implementation (GI) tools that may support uptake. A convenience sample of Task Force members were recruited for semi-structured interviews. Interview transcripts were thematically analysed to group country-specific barriers, facilitators and GI tools into three levels: health care provider (HCP), consumer and structural. Once identified, barriers and facilitators were classified into four themes related to awareness, economic, guideline or other. Interviews were conducted with 38 Task Force members, representing 20 countries. Lack of CI and hearing loss awareness was a major barrier at the HCP (85% of countries), consumer (80%) and structural (20%) levels. Economic and guideline barriers followed at the HCP (35%; 25%), consumer (45%; 0%) and structural (55%; 30%) levels, respectively. Facilitators focused on raising awareness of hearing loss and CIs as well as guideline related initiates at the HCP (80%; 70%), consumer (70%; 10%) and structural (25%; 70%) levels. GI tools including education, economic evaluations, quick reference resources and social media can help improve awareness and uptake. Awareness is the primary barrier to implementing Living Guidelines globally for adults with SPSNHL. Endorsement from key professional bodies and using the best available evidence can enhance uptake.

Children then, adults now: long-term outcomes-performance at 15, 20, and 25 years of cochlear implant use.

Severe to profound sensorineural hearing loss interferes with a child's development at the cognitive, linguistic, academic, and social levels. Since the beginning of the pediatric auditory rehabilitation program through cochlear implantation in the Ear, Nose, and Throat (ENT) Service of the Coimbra Hospital and University Center (CHUC), Portugal, its mentors defended the early diagnosis of hearing loss followed by timely intervention, and this was considered the starting point to optimize (re)habilitation through this method. Three decades or so later, recently we conducted this study to evaluate the performance of patients implanted in the initial phase of the cochlear implantation program. The study aimed to evaluate the performance of individuals with severe to profound congenital hearing loss who underwent pediatric cochlear implantation and have used the cochlear implant for at least 25 years, to analyze the beneficial effect of early intervention in improving performance results. The study sample is composed of 31 individuals with severe to profound congenital hearing loss and no other comorbidities, divided into two groups (Group 1: age at implantation was under 3 years; Group 2: age at implantation was over 3 years). All 31 subjects were evaluated at 15, 20, and 25 years of cochlear implant (CI) use with a comprehensive set of tests. In addition, data were collected regarding the academic level of each participant. The results of both groups were compared to find out if there is an effect of age at implantation on auditory performance, and if there is an improvement in the performance with CI over time (15, 20, and 25 years of use). The results show that there is a positive effect, with statistical significance, of early implantation on auditory performance, and telephone use. In both groups, there is an increase in performance over time, but it tends to stabilize after 20 years of CI use. The results obtained in this work support the importance of early intervention in patients with severe to profound hearing loss who are cochlear implant users and show that CI is an effective and reliable method in the treatment of these patients, contributing to their improved socio-educational integration, and that the benefits last over time.