Troubleshooting Cochlear Implant Malfunction Using Neural Response Telemetry and Normal Saline.

Cochlear implantation has become a standard of care for a child diagnosed with bilateral profound sensorineural hearing loss with a structured surgical standard operating procedure. A 3-year-old boy with bilateral profound prelingual sensorineural deafness underwent a Med-EL Sonata Ti100 implant. We faced a peculiar situation intraoperatively after inserting the electrodes and closing the wound. The impedance recording indicated high ground path impedance with short-circuiting of few electrodes. As a bionic implant, its electronic components may at times malfunction both intraoperatively and/or postoperatively; therefore, neural response telemetry (NRT) was invented to check it. By using NRT and a few milliliters of normal saline, we were able to diagnose as well as rectify the malfunctioning of the implant.

Cochleovestibular Phenotype in a Rare Genetic MED13L Mutation.

The gene MED13 participates in transcription. The MED13L gene is a paralog of MED13 that is involved in developmental gene expression. Mutations in the gene have been shown to result in a heterogenous phenotype affecting several physiological systems. Hearing loss has been reported very rarely, and vestibular weakness has never been reported in the condition. In this report, we present a mutation of MED13L in c.1162A > T (p.Arg388Ter), where we detail and describe a cochleovestibular phenotype with objective vestibulometry for the first time. The child showed bilateral sloping sensorineural hearing loss, a bilateral vestibular weakness, and an inner ear vestibular structural abnormality on imaging. Early intervention with hearing aids and vestibular rehabilitation led to a favorable outcome in terms of speech, communication, and balance. We emphasize the importance of comprehensive audiovestibular assessment in children diagnosed with MED13L mutations for effective management of these children.

[Genetic characteristic analysis of slight-to-moderate sensorineural hearing loss in children].

Objective:To analyze genetic factors and phenotype characteristics in pediatric population with slight-to-moderate sensorineural hearing loss. Methods:Children with slight-to-moderate sensorineural hearing loss of and their parents, enrolled from the Chinese Deafness Genome Project, were studied. Hearing levels were assessed using pure tone audiometry, behavioral audiometry, auditory steady state response（ASSR）, auditory brainstem response（ABR） thresholds, and deformed partial otoacoustic emission（DPOAE）. Classification of hearing loss is according to the 2022 American College of Medical Genetics and Genomics（ACMG） Clinical Practice Guidelines for Hearing Loss. Whole exome sequencing（WES） and deafness gene Panel testing were performed on peripheral venous blood from probands and validations were performed on their parents by Sanger sequencing. Results:All 134 patients had childhood onset, exhibiting bilateral symmetrical slight-to-moderate sensorineural hearing loss, as indicated by audiological examinations. Of the 134 patients, 29（21.6%） had a family history of hearing loss, and the rest were sporadic patients. Genetic causative genes were identified in 66（49.3%） patients. A total of 11 causative genes were detected, of which GJB2 was causative in 34 cases（51.5%）, STRC in 10 cases（15.1%）, MPZL2 gene in six cases（9.1%）, and USH2A in five cases（7.6%）.The most common gene detected in slight-to-moderate hearing loss was GJB2, with c. 109G>A homozygous mutation found in 16 cases（47.1%） and c. 109G>A compound heterozygous mutation in 9 cases（26.5%）. Conclusion:This study provides a crucial genetic theory reference for early screening and detection of mild to moderate hearing loss in children, highlighting the predominance of recessive inheritance and the significance of gene like GJB2, STRC, MPZL2, USH2A.

Externalization of Speech When Listening With Hearing Aids.

Subjective reports indicate that hearing aids can disrupt sound externalization and/or reduce the perceived distance of sounds. Here we conducted an experiment to explore this phenomenon and to quantify how frequently it occurs for different hearing-aid styles. Of particular interest were the effects of microphone position (behind the ear vs. in the ear) and dome type (closed vs. open). Participants were young adults with normal hearing or with bilateral hearing loss, who were fitted with hearing aids that allowed variations in the microphone position and the dome type. They were seated in a large sound-treated booth and presented with monosyllabic words from loudspeakers at a distance of 1.5 m. Their task was to rate the perceived externalization of each word using a rating scale that ranged from 10 (at the loudspeaker in front) to 0 (in the head) to -10 (behind the listener). On average, compared to unaided listening, hearing aids tended to reduce perceived distance and lead to more in-the-head responses. This was especially true for closed domes in combination with behind-the-ear microphones. The behavioral data along with acoustical recordings made in the ear canals of a manikin suggest that increased low-frequency ear-canal levels (with closed domes) and ambiguous spatial cues (with behind-the-ear microphones) may both contribute to breakdowns of externalization.

Adult-onset mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS): a diagnostic challenge.

Rare causes of stroke-like presentations can be difficult to diagnose. We report a case of a man in his 40s who first presented with stroke symptoms, but whose clinical course was not typical for a stroke. A detailed investigation of the patient's medical history revealed bilateral sensorineural hearing loss which prompted a wider diagnostic assessment.Furthermore, lack of vascular risk factors and a normal angiogram strengthened our suspicion of an unusual underlying condition. Raised lactic acid levels and genetic analysis confirmed a diagnosis of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes syndrome.

Association of noise exposure, plasma microRNAs with metabolic syndrome and its components among Chinese adults.

AIMS: We aimed to evaluate the association of occupational noise with metabolic syndrome (MetS) and its components, and to assess the potential role of miRNAs in occupational noise-associated MetS. METHODS: A total of 854 participants were enrolled in our study. Cumulative noise exposure (CNE) was estimated in conjunction with workplace noise test records and research participants' employment histories. Logistic regression models adjusted for potential confounders were used to assess the association of CNE and miRNAs with MetS and its components. RESULTS: We observed linear positive dose-response associations between occupational noise exposure and the prevalence of MetS (OR: 1.031; 95 % CI: 1.008, 1.055). And linear and nonlinear relationship were also found for the association of occupational noise exposure with high blood pressure (OR: 1.024; 95 % CI: 1.007, 1.041) and reduced high-density lipoprotein (OR: 1.051; 95 % CI: 1.031, 1.072), respectively. MiR-200a-3p, miR-92a-3p and miR-21-5p were inversely associated with CNE, or the prevalence of MetS and its components (all P < 0.05). However, we did not find any statistically significant mediation effect of miRNAs in the associations of CNE with MetS. Furthermore, the prevalence of bilateral hearing loss in high-frequency increased (OR: 1.036; 95 % CI: 1.008, 1.067) with CNE level rising, and participants with bilateral hearing loss in high-frequency had a significantly higher risk of MetS (OR: 1.727; 95 % CI: 1.048, 2.819). CONCLUSION: Our study suggests that occupational noise exposure is associated with MetS and its components, and the role of miRNAs in noise-induced increasing MetS risk needs to be confirmed in future studies.

Importance of age at 2nd implantation and interimplant interval to the outcome of bilateral prelingually deafened pediatric cochlear implantation.

BACKGROUND: In Taiwan, the number of cases of sequential bilateral pediatric cochlear implantation (CI) is increasing but data regarding its effectiveness and impact of the reimbursement policy are lacking. We examined the speech perception and quality of life (QOL) of bilateral prelingually deaf children who underwent sequential CI, considering the effects of age at the time of second implantation and interimplant interval. METHODS: We enrolled 124 Mandarin-speaking participants who underwent initial cochlear implant (CI1) in 2001-2019 and a second CI (CI2) in 2015-2020. Patients were followed up for ≥2 years and were categorized into groups based on age at the time of CI2 implantation (<3.5, 3.6-7, 7.1-10, 10.1-13, and 13.1-18 years) and interimplant interval (0.5-3, 3.1-5, 5.1-7, 7.1-10, and >10 years). We evaluated speech perception, device usage rates, and QOL using subjective questionnaires (Speech, Spatial, and Qualities of Hearing and Comprehension Cochlear Implant Questionnaire). RESULTS: Speech perception scores of CI2 were negatively correlated with ages at the time of CI1 and CI2 implantation and interimplant interval. Older age and a longer interimplant interval were associated with higher nonuse rates for CI2 and worse auditory performance and QOL. Among individuals aged >13 years with interimplant intervals >10 years, up to 44% did not use their second ear. Patients aged 7.1 to 10 years had better speech perception and higher questionnaire scores than those aged 10.1 to 13 and 13.1 to 18 years. Furthermore, patients aged 10.1 to 13 years had a lower rate of continuous CI2 usage compared to those aged 7.1 to 10 years. CONCLUSION: Timely implantation of CI2 is essential to achieve optimal outcomes, particularly among sequentially implanted patients with long-term deafness in the second ear and no improvement with hearing aids following CI1 implantation. For CI2 implantation, an upper limit of age of 10 years and interimplant interval of 7 years are essential to prevent suboptimal outcomes. These data can provide useful information to implant recipients, their families, and medical and audiological professionals, enabling a comprehensive understanding of the benefits and potential impacts of the timing of CI2 implantation.

Genetic diagnosis of childhood sensorineural hearing loss.

INTRODUCTION: Congenital/early-onset sensorineural hearing loss (SNHL) is one of the most common hereditary disorders in our environment. There is increasing awareness of the importance of an etiologic diagnosis, and genetic testing with next-generation sequencing (NGS) has the highest diagnostic yield. Our study shows the genetic results obtained in a cohort of patients with bilateral congenital/early-onset SNHL. MATERIALS AND METHODS: We included 105 children with bilateral SNHL that received genetic testing between 2019 and 2022. Genetic tests were performed with whole exome sequencing, analyzing genes related to hearing loss (virtual panel with 244 genes). RESULTS: 48% (50/105) of patients were genetically diagnosed. We identified pathogenic and likely pathogenic variants in 26 different genes, and the most frequently mutated genes were GJB2, USH2A and STRC. 52% (26/50) of variants identified produced non-syndromic hearing loss, 40% (20/50) produced syndromic hearing loss, and the resting 8% (4/50) could produce both non-syndromic and syndromic hearing loss. CONCLUSIONS: Genetic testing plays a vital role in the etiologic diagnosis of bilateral SNHL. Our cohort shows that genetic testing with NGS has a high diagnostic yield and can provide useful information for the clinical workup of patients.

Speech, Sign, or Both? Factors Influencing Caregivers' Communication Method Decision Making for Deaf/Hard of Hearing Children.

PURPOSE: Caregivers of deaf/hard of hearing infants are faced with challenging decisions regarding their child's communication method. The purpose of the current research note is to characterize the advice that caregivers receive and value as well as the factors that influence caregivers' decision making. METHOD: The current study enrolled 105 caregiver-child dyads, including children between 12 and 18 months of age with bilateral, congenital hearing loss. All children were exposed to spoken language, and 63.81% of children were also exposed to sign language. Caregivers completed the "Making Decisions About Sign, Speech, and Multilingualism Survey" (Crowe et al., 2014). RESULTS: Caregivers most frequently received advice to use both speech and sign and highly valued advice from speech-language pathologists. When considering the use of speech, the factors that caregivers most frequently rated as very important were "My child's future academic success" (96.19%), "My child's future literary success" (95.24%), and "My child's future access to higher education" (95.19%). When considering the use of sign, the factors that caregivers most frequently rated as very important were "My child's ability to form friendships and future relationships" (82.52%), "My child's future literary success" (81.37%), and "My child's future academic success" (81.37%). CONCLUSIONS: Results of the current study suggest that throughout the decision-making process, caregivers' highest priority is understanding how their decisions will influence their child's future access to opportunities and relationships. Providers may consider discussing these factors early in the decision-making process to support caregivers' ability to make an informed choice regarding their child's communication method.

Seroprevalence of ToRCH Pathogens among Children Admitted to a Tertiary Care Hospital in Eastern India for Cataract Surgery and Cochlear Transplantation.

AIMS: The study aims to investigate the presence of TORCH infections in a child with bilateral cataracts and deafness and report the ToRCH-serology screening profile (Toxoplasma gondii (TOX), rubella (RV), cytomegalovirus (CMV), and herpes simplex virus (HSV-I/II)) in pediatric cataract and deafness. METHODS: Cases that had a clear clinical history of congenital cataracts and congenital deafness were included in the study. The study population consisted of 18 bilateral cataracts and 12 bilateral deafness child who was admitted to AIIMS Bhubaneswar for cataract surgery and cochlear implantation, respectively. Sera of all children were tested qualitatively and quantitatively for IgG/IgM-antibodies against ToRCH agents in a sequential manner. RESULTS: Anti-IgG antibodies against the torch panel were detected in all cataract and deafness patients. Anti-CMV IgG was detected in 17 of 18 bilateral cataract children and 11 of 12 bilateral deaf children. The rates of anti-CMV IgG antibody positivity were significantly higher. In the cataract group, 94.44% and in the deafness group, 91.66% of the patient was Anti-CMV IgG positive. Besides this, 77.7 % of the patient from the cataract group and 75% from the deafness group was anti- RV IgG antibody positive. In bilateral cataract patients, IgG-alone seropositive cases were mostly attributed to CMV (94.44%; 17/18), followed by RV (77.70%; 14/18), HSV-I (27.70%; 5/18), TOX (27.70%; 5/18), and HSV-II (16.60%; 3/18). In bilateral deafness patients, the spectrum of IgG alone seropositive cases was almost the same except for TOX (0/12). CONCLUSION: The current study recommends interpreting ToRCH-screening in pediatric cataracts and deafness with caution. Interpretation should include both serial qualitative and quantitative assays in tandem with clinical correlation to minimize diagnostic errors. The sero-clinical-positivity needs to be tested in older children who might pose a threat to the spread of infection.

Are children with unilateral sensorineural hearing loss receiving cochlear implants?

INTRODUCTION: The US Food and Drug Administration (FDA) granted its first approval for cochlear implants (CI) in children with bilateral sensorineural hearing loss (SNHL) in 1990. In 2019, the FDA expanded CI indications to include children with unilateral SNHL. OBJECTIVE: The aim of this study was to assess the prevalence of children with unilateral SNHL in the population of new pediatric CI recipients between 2012 and 2021. METHODS: A retrospective analysis using the American College of Surgeons National Surgical Quality Improvement Pediatric database examined patients under 18 years of age with bilateral or unilateral SNHL who underwent CI between 2012 and 2021. Current Procedural Terminology code 69930 identified patients with 'cochlear device implantation, with or without mastoidectomy.' The percentage of children undergoing CI for unilateral versus bilateral SNHL during the study period was calculated and subjected to statistical analysis. RESULTS: 9863 pediatric CI patients were included with a mean age of 5.1 (95 % CI 5.1-5.2) years at the time of implantation. 7.5 % (N = 739) of patients had unilateral SNHL and 92.5 % (N = 9124) had bilateral SNHL. Children with bilateral SNHL undergoing CI were significantly younger (5.0 years versus 6.9 years for those with unilateral SNHL, p < .001). There was a statistically significant difference in the percentage of children receiving CI for unilateral versus bilateral SNHL (3.3 % in 2012 to 14.3 % in 2021, p < .001) before and after the FDA changes. CONCLUSIONS: The proportion of CIs placed for unilateral SNHL has increased annually even before 2019 when the FDA expanded its CI indications to include children with unilateral SNHL for the first time.

Postmeningitic pediatric hearing loss from non-type b Haemophilus influenzae.

BACKGROUND: Postmeningitic hearing loss from Haemophilus influenzae (H. influenzae) is increasingly due to encapsulated serotypes other than type b (Hib) and nontypeable strains (collectively, nHiB H. influenzae). Pediatric hearing loss after nHib H. influenzae meningitis remains poorly described. METHODS: Retrospecive case series of nHiB H. influenzae meningitis cases identified from a microbiologic database at Children's Hospital Colorado from 2000 to 2020. Literature regarding nHiB H. influenzae and H. influenzae postmeningitic hearing loss was also reviewed. RESULTS: Eleven cases of nHib H. influenzae meningitis (median age 15.9 months) were identified due to serotype f (36 %), serotype a (27 %), and nontypable strains (36 %). Seven (64 %) patients were male, 55 % were white and 18 % were Hispanic or Latino. Hearing loss was initially identified in 4 children (40 %), with two patients with moderate conductive hearing loss (CHL) and one child with unilateral moderate sensorineural (SNHL) hearing loss patients recovering normal hearing. One patient with bilateral profound sensorineural hearing loss and associated labyrinthitis ossificans required cochlear implantation. All children (4) with identified hearing loss were noted to have additional intracranial sequelae, which included empyema (2), sinus thrombosis (2), and seizures (2). Of patients receiving steroids, 25 % had hearing loss on initial testing, compared to 66 % of those who did not receive steroids. CONCLUSIONS: nHib H. influenzae can cause both transient and permanent postmeningitic hearing loss. Steroids may offer otoprotection in nHib H. influenzae meningitis similar to Hib meningitis. Given the limited literature, further study is needed to better characterize hearing outcomes after nHib H. influenzae meningitis.

Sudden bilateral deafness in a patient with vertebrobasilar artery occlusion: A case report.

RATIONALE: Sudden bilateral deafness is often associated with serious systematic conditions such as neoplasms, vascular events, autoimmune diseases, infections, and iatrogenic injury, but very rarely to cerebrovascular disease. This is a rare case of sudden bilateral deafness in a patient with the vertebrobasilar artery occlusion. PATIENT CONCERNS: A 46-year-old man was admitted to a local hospital for sudden bilateral deafness, the patient suffered inarticulate speech and walking unsteadily 6 days later. DIAGNOSES: Difusion-weighted magnetic resonance imagin demonstrated acute cerebral infarction in the pons and bilateral cerebellum; Magnetic resonance angiography showed vertebrobasilar artery occlusion. INTERVENTIONS: Aspirin and clopidogrel were given for antiplatelet therapy, revascularization was obtained by endovascular treatment. OUTCOMES: The symptoms of dysarthria, ataxia and weakness gradually improved and were discharged 14 days after admission revascularization. After 3 months telephone followed-up the patient was self-cared. LESSONS: Deafness sometimes can be an early warning sign of impending vertebrobasilar ischemic stroke. Early recognition of deafness with acute ischemic stroke should allow special management, and misdiagnosis may result in significant morbidity, or even mortality.

Cervical vestibular evoked myogenic potential (cVEMP) findings in adults with sensorineural hearing loss (SNHL): comparisons between 500 Hz tone burst and narrowband CE-Chirp stimuli.

There has been a growing interest in studying the usefulness of chirp stimuli in recording cervical vestibular evoked myogenic potential (cVEMP) waveforms. Nevertheless, the study outcomes are debatable and require verification. In view of this, the aim of the present study was to compare cVEMP results when elicited by 500 Hz tone burst and narrowband (NB) CE-Chirp stimuli in adults with sensorineural hearing loss (SNHL). Fifty adults with bilateral SNHL (aged 20-65 years) underwent the cVEMP testing based on the established protocol. The 500 Hz tone burst and NB CE-Chirp (centred at 500 Hz) stimuli were presented to each ear at an intensity level of 120.5 dB peSPL. P1 latency, N1 latency, and P1-N1 amplitude values were analysed accordingly. The NB CE-Chirp stimulus produced significantly shorter P1 and N1 latencies (p < 0.001) with large effect sizes (d > 0.80). In contrast, both stimuli elicited cVEMP responses with P1-N1 amplitude values that were not statistically different from one another (p = 0.157, d = 0.15). Additionally, age and hearing level were found to be significantly correlated (r = 0.56, p < 0.001), as were age and cVEMP amplitude for each stimulus (p < 0.001). To conclude, since both stimuli were presented at an equivalent intensity level (in dB peSPL), the shorter P1 and N1 latencies of cVEMP produced by the NB CE-Chirp stimulus (centred at 500 Hz) were unlikely due to enhanced saccular stimulation. Another more sensible reason is the temporal adjustment of the chirp stimulus.

Case Report: Leptospirosis Complicated by Persistent, Bilateral Sensorineural Hearing Loss.

The clinical manifestations of leptospirosis range from mild to life-threatening and can impact on multiple organ systems. A wide array of neurological manifestations of leptospirosis have been reported, although the pathophysiology of neuroleptospirosis remains incompletely understood. We present a case of leptospirosis complicated by bilateral sensorineural deafness, with nodular meningitis demonstrated in the internal auditory meatus on magnetic resonance imaging. The patient was treated with doxycycline, ceftriaxone, systemic and topical steroids, and hyperbaric oxygen therapy, with modest, but incomplete, improvement.

Effective cochlear implantation for idiopathic hypertrophic pachymeningitis with bilateral profound hearing loss: A case report.

RATIONALE: Hypertrophic pachymeningitis (HP) is a local or diffuse fibrous thickness of the dura mater of the brain or spinal cord, caused by infection or connective tissue disease. Headache is the most common clinical symptom, followed by various cranial nerve disorders such as visual impairment, diplopia, and hearing loss. HP can be classified into secondary and idiopathic. Here, we report a case of bilateral progressive profound sensorineural hearing loss diagnosed in a patient with idiopathic HP, where a cochlear implant was effectively used. PATIENT CONCERNS: The patient was a 77-year-old woman. Hearing loss gradually progressed bilaterally, and magnetic resonance imaging showed a space-occupying lesion with a continuous contrast enhancement in the bilateral internal auditory canals, and diffused dural thickening from the middle to the posterior cranial fossa. DIAGNOSES: A trans-labyrinthine biopsy was conducted, and a definite diagnosis of idiopathic HP was made. Thickening of the dura mater in the bilateral internal auditory canals was thought to cause profound hearing loss. INTERVENTIONS AND OUTCOMES: A cochlear implant was implemented 4 months after biopsy, and a favorable hearing response was obtained postoperatively. LESSONS: This is the first report of a cochlear implant in a patient with idiopathic HP. Cochlear implantation was considered a good treatment for profound hearing loss due to idiopathic HP, which provides a reference for patients to receive timely and correct treatment.

The Association Between Family Resources and Language Among Young Children Who are Deaf and Hard of Hearing.

OBJECTIVE: Our study (1) examined demographic factors in families with children with bilateral hearing loss and how they relate to Family Resource Scale (FRS) questionnaire data and (2) examined correlations between FRS data and measures of language. METHODS: Children aged 6 months to 10 years with bilateral hearing loss were enrolled. Parents completed the FRS questionnaire to assess their access to socioeconomic resources at the first language measurement visit. Assessments measured receptive and expressive language, nonverbal intelligence quotient, and adaptive functioning. RESULTS: Among the 85 children included in the analysis, approximately 40% had hearing loss classified as mild to moderate and 25% had a cochlear implant. Participants' mean FRS score was 130 (SD 16.6) (with the highest possible score of 150 and indicating better access to resources). Significant positive correlations ( p -value ≤ 0.05) were found between maternal education, paternal education, and family income and several FRS subscales (Growth and Support, Necessities in Health, Childcare, Personal Resources). Significant positive correlations were found between the Necessities in Health subscale and all the language measurements. CONCLUSION: Children whose parents reported better access to socioeconomic resources related to health care had higher language performance scores. Although early access to intervention services has improved for deaf or hard-of-hearing children, there are other variables contributing to language development, including access to socioeconomic resources. This study highlights the need for further research addressing more specific and modifiable resources to improve language performance for deaf or hard-of-hearing children.

Longitudinal Auditory Benefit for Elderly Patients After Cochlear Implant for Bilateral Hearing Loss, Including Those Meeting Expanded Centers for Medicare & Medicaid Services Criteria.

OBJECTIVE: To examine the effect of patient age on longitudinal speech understanding outcomes after cochlear implantation (CI) in bilateral hearing loss. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary academic center. PATIENTS: One thousand one hundred five adult patients with bilateral hearing loss receiving a unilateral CI between 1987 and 2022InterventionsNone. MAIN OUTCOME MEASURES: Postoperative speech recognition outcomes, including AzBio sentences, consonant-nucleus-consonant word, and Hearing in Noise Test in quiet were analyzed at short-term (<2 yr), medium-term (2-8 y), and long-term (>8 yr) term postoperative intervals. RESULTS: Eighty-six very elderly (>80 yr), 409 elderly (65-80 yr), and 709 nonelderly (18-65 yr) patients were included. Short-term postoperative AzBio scores demonstrated similar magnitude of improvement relative to preoperative scores in the very elderly (47.6, 95% confidence interval [CI], 28.9-66.4), elderly (49.0; 95% CI, 39.2-58.8), and nonelderly (47.9; 95% CI, 35.4-60.4). Scores for those older than 80 years remained stable after 2 years after implant, but in those 80 years or younger, scores continued to improve for up to 8 years (elderly: 6.2 [95% CI, 1.5-12.4]; nonelderly: 9.9 [95% CI, 2.1-17.7]) after implantation. Similar patterns were observed for consonant-nucleus-consonant word scores. Across all age cohorts, patients with preoperative Hearing in Noise Test scores between 40 and 60% had similar scores to those with preoperative scores of less than 40%, at short-term (82.4, 78.9; 95% CI, -23.1 to 10.0), medium-term (77.2, 83.9; 95% CI, -15.4 to 8.2), or long-term (73.4, 71.2; 95% CI, -18.2 to 12.2) follow-up. CONCLUSIONS: Patients older than 80 years gain significant and sustained auditory benefit after CI, including those meeting expanded Centers for Medicare & Medicaid Service criteria for implantation. Patients younger than 80 years demonstrated continued improvement over longer periods than older patients, suggesting a role of central plasticity in mediating CI outcomes as a function of age.

Cogan Syndrome: A Case Study and Review of the Literature.

Cogan syndrome is an autoimmune disease characterized by vestibular symptoms, bilateral sensorineural hearing loss, and inflammatory ocular manifestations, which may be accompanied by systemic vasculitis. We herein present the case of a patient with bilateral sensorineural hearing loss who presented with pain over her cochlear implantation incision site. She was later found to have evidence of ocular disease and underlying vasculitis leading to a diagnosis of Cogan syndrome.

[Cochlear implantation in Senegal: challenges, limits and perspectives]. / Implantation cochléaire au Sénégal: enjeux, limites et perspectives.

A cochlear implant is an electronic prosthesis used in the rehabilitation of profound bilateral hearing loss. It directly stimulates cochlear nerve fibers by bypassing the hair cells. This high-performance technology, which appeared 60 years ago, has spread widely throughout the world and is regularly used in hearing rehabilitation. In developing countries, the adoption and development of this tool are still lagging behind. The authors analyze factors delaying the penetration of cochlear implants in Senegal.