Acceptance and Benefit of Electroacoustic Stimulation in Children.

OBJECTIVE: Children with high-frequency severe-to-profound hearing loss and low-frequency residual hearing who do not derive significant benefit from hearing aids are now being considered for cochlear implantation. Previous research shows that hearing preservation is possible and may be desirable for the use of electroacoustic stimulation (EAS) in adults, but this topic remains underexplored in children. The goal of this study was to explore factors relating to hearing preservation, acceptance, and benefits of EAS for children. STUDY DESIGN: Retrospective review. SETTING: Tertiary academic medical center. PATIENTS: Forty children (48 ears) with preoperative low-frequency pure-tone averages of 75 dB HL or less at 250 and 500 Hz (n = 48). INTERVENTION: All patients underwent cochlear implantation with a standard-length electrode. MAIN OUTCOME MEASURE: Low-frequency audiometric thresholds, speech perception, and EAS usage were measured at initial stimulation, and 3 and 12 months postoperatively. Outcomes were compared between children with and without hearing preservation, and between EAS users and nonusers. RESULTS: Hearing was preserved at similar rates as adults but worse for children with an enlarged vestibular aqueduct. Fewer than half of children who qualified to use EAS chose to do so, citing a variety of audiologic and nonaudiologic reasons. No differences were detected in speech perception scores across the groups for words, sentences, or sentences in noise tests. CONCLUSIONS: Neither hearing preservation nor EAS use resulted in superior speech perception in children with preoperative residual hearing; rather, all children performed well after implantation.

Long-Term Safety and Quality of Life after Vibroplasty in Sensorineural Hearing Loss: Short/Long Incus Process Coupler.

OBJECTIVE: The study shows the long-term effectiveness, safety, and quality of life after Vibrant Soundbridge (VSB) implantation in sensorineural hearing loss (SNHL) using the short process coupler (SP) or the long process coupler (LP). METHODS: This retrospective study evaluated 77 VSB cases. Follow-up (F/U) time-dependent objective measurements (audiological outcomes), subjective data collection (quality-of-life questionnaire), and safety measures are presented. RESULTS: Sixty-two ears were included in the analysis with up to 116 months of postsurgical F/U data (mean 32.15 ± 37.97 months LP and SP coupler). Fifty-three ears (13 bilateral cases) received the LP coupler and 9 subjects the SP coupler. The post-operative bone conduction thresholds remained stable and, in both groups, <10 dB. The benefit in word recognition scores measured at 65 dB SPL and 80 dB SPL showed no significant difference between the couplers (p = 0.559 and p = 0.088, respectively). The functional gain was not significantly different (p > 0.05) with a mean of 20.91 ± 9.77 and 17.19 ± 5.75 for LP and SP coupler, respectively. The utility score deciphered from the Assessment of Quality-of-life Questionnaire-8 dimensions revealed a mean score of 0.75 ± 0.16 which is not significantly different to the age- and sex-matched healthy control group with 0.81 ± 0.02 (p = 0.3547). CONCLUSION: The Incus Vibroplasty utilizing both couplers is a safe and effective method to treat mild-to-severe SNHL. Both fixation methods of the floating mass transducer exhibit good clinical and audiological outcomes with high patient quality of life. The SP coupling method can be a good alternative when the long process is anatomically inaccessible, or the approach is limited due to anatomical reasons.

Cost-Utility Analysis of Bilateral Cochlear Implantation in Adults With Severe to Profound Sensorineural Hearing Loss in Poland.

OBJECTIVE: The aim of the study was to develop a Markov model and apply it for the evaluation of three different treatment scenarios for adult patients with severe to profound bilateral sensorineural hearing loss. STUDY DESIGN: Prospective Observational Study. SETTINGS: Hospital. PATIENTS: A clinical group of 22 adult patients (59.1% men, 40.9% women) aged from 59.13 ±â€Š8.9 years were included in the study. The study comprised two arms: patients in group 1 received the second cochlear implant one to three months after the first implant; while patients in group 2 got the second cochlear implant approximately one year after the first implant. MAIN OUTCOME MEASURES: All participants were first asked to complete an AQoL-8D questionnaire. For the cost-effectiveness analyses, a Markov model analyzed as microsimulation was developed to compare the different treatment options. RESULTS: The analyses show that bilateral cochlear implantation strategies are cost-effective compared to the 'no treatment' alternative when having a 10-year model time horizon. When all three model scenarios are compared, the bilateral simultaneous cochlear implantation strategy (Scenario 3) compared to the 'no treatment' option is even more cost-effective than the Scenarios 1 and 2, compared with the 'no treatment' alternative. CONCLUSIONS: The model results summarize that bilateral (sequential and simultaneous) cochlear implantation that are represented in the model scenarios, are cost-effective strategies for Polish adult patients with bilateral severe to profound sensorineural hearing loss.

Long-term efficacy of triple semicircular canal plugging in the treatment of patients with ipsilateral delayed endolymphatic hydrops.

This study aims to explore the long-term efficacy of triple semicircular canal plugging (TSCP) in the treatment of intractable ipsilateral delayed endolymphatic hydrops (DEH), so as to provide an alternative therapy for this disease. Forty-eight patients diagnosed with ipsilateral DEH referred to vertigo clinic of our hospital between Dec. 2010 and Dec. 2017, were included in this study for retrospective analysis. All patients were followed up for 2 years. Vertigo control and auditory functions were measured and analyzed. Pure tone audiometry, caloric test, and vestibular evoked myogenic potential (VEMP) were performed in two-year follow-up. Forty-five patients who accepted intratympanic gentamicin (26.7 mg/mL) twice given one week apart were selected as a control group. The total control rate of vertigo in TSCP group was 97.9% (47/48) in the two-year follow-up, with complete control rate of 83.3% (40/48) and substantial control rate of 14.6% (7/48). The rate of hearing loss was 22.9% (11/48). The total control rate of vertigo in intratympanic gentamicin group was 80.0% (36/45), with complete control rate of 57.8% (26/45) and substantial control rate of 22.2% (10/45), and the rate of hearing loss was 20.0% (9/45). The vertigo control rate of TSCP was significantly higher than that of intratympanic gentamicin (χ2 = 6.01, p < 0.05). There was no significant difference of hearing loss rate between two groups. (χ2 = 0.12, p > 0.05). TSCP, which can reduce vertiginous symptoms in patients with intractable ipsilateral DEH, represents an effective therapy for this disorder.

[Surgical strategy of minimally invasive cochlear implantation].

Cochlear implantation is currently the most effective treatment for patients with severe-to-profound sensorineural hearing loss. How to achieve minimally invasive treatment, preserve the residual hearing, and further improve curative effect and reduce surgical complications is the goal of cochlear implantation practice. This article introduces the minimally invasive cochlear implantation technique in terms of the idea of minimally invasive operation, the advantages of electric acoustic stimulation, the key points of electrode implantation technique, the design of surgical incision, and the precise processing of perioperative period. This technique not only has the merits of less operative damage and better hearing and speech rehabilitation after surgery, but also reserves favorable structures and function for the future application of gene therapy and hair cell regeneration technique. Therefore, it is strongly recommended for further promotion in clinical practice.

Simultaneous bilateral cochlear implantation under local anaesthesia in a visually impaired adult with profound sensorineural deafness: A case report.

Objective: The National Institute of Clinical Excellence recommends that adult patients with severe to profound deafness are eligible for simultaneous bilateral cochlear implantation if they are blind or have other disabilities increasing their reliance on auditory stimuli. Cochlear implant (CI) surgery is routinely performed under general anaesthesia (GA), precluding patients who have higher risk associated with GA. Recent literature describes the safety and efficacy of performing unilateral CI surgery under local anaesthesia (LA). We report the first simultaneous bilateral CI under LA in the UK in an adult patient with profound sensorineural deafness and visual impairment.Case study: A 46-year-old gentleman, registered blind, presented with a 20-year history of bilateral progressive hearing loss. He was assessed as unfit for surgery under GA due to significant cardiac comorbidities. We performed simultaneous bilateral CI surgery under LA on the patient; he was discharged home the following day. Postoperative free field audiometry showed a significant improvement; speech discrimination using Bamford, Kowal and Bench sentences presented in quiet, increased from 0% pre-operatively to 100% four months post-operatively.Conclusion: Simultaneous bilateral CI surgery under LA is a safe and feasible procedure for eligible patients who may otherwise have been denied surgery due to their GA risk.

Hearing and Quality-of-Life Outcomes After Cochlear Implantation in Adult Hearing Aid Users 65 Years or Older: A Secondary Analysis of a Nonrandomized Clinical Trial.

Importance: Hearing loss, especially moderate to severe forms, has the potential to negatively affect an individual's physical, social, emotional, and cognitive well-being. Moreover, having ineffective binaural hearing increases difficulty understanding speech in noise and leads to a greater degree of social isolation and loneliness and a reduced quality of life (QoL). Objective: To explore the audiometric and holistic effects of cochlear implantation in a group of adults 65 years or older compared with an optimized bilateral hearing aid condition. Design, Setting, and Participants: This ad hoc secondary analysis of a prospective, single-subject, repeated-measures nonrandomized clinical trial included 13 cochlear implantation centers across the United States. Participants 65 years or older with postlingual bilateral moderate-to-profound sensorineural hearing loss with aided Consonant-Vowel Nucleus-Consonant (CNC) word scores in quiet of 40% or less in the ear to undergo implantation and 50% or less in the contralateral ear were included in the analysis. Baseline QoL testing was performed after 1 month of optimized bilateral hearing aid use. Participants were enrolled from February 20, 2017, to May 3, 2018, and follow-up was completed December 21, 2018. Data were analyzed from March 25, 2019, to March 31, 2020. Interventions: Unilateral implantation with a slim, modiolar cochlear implant device. Hearing aid use in the contralateral ear was required through the 6-month primary end-point interval. Main Outcomes and Measures: The primary objective was to evaluate speech perception before and 6 months after activation of a new cochlear implant. Secondary objectives were QoL metrics in the everyday listening condition before and 6 months after implantation. Results: Seventy participants (51 men [73%]) with a median age of 74 (range, 65-91) years were included in the analysis. No major adverse events occurred. Mixed-model analysis with estimated marginal means and 95% CIs compared preimplantation baseline performance with 6-month postimplantation performance. A clinically important improvement in CNC words was shown in the bimodal condition, with a mean difference of 37.2% (95% CI, 32.0%-42.4%), and in the unilateral (cochlear implant only) condition, with a mean difference of 44.1% (95% CI, 39.0%-49.2%). A clinically important improvement in noise (AzBio sentences signal-to-noise ratio of +10 dB) was also shown, with a mean difference of 21.6% (95% CI, 15.7%-27.5%) in the bimodal condition and 24.5% (95% CI, 18.3%-30.7%) in the unilateral condition. The Health Utilities Index Mark 3 multiple-attribute score improved by 0.186 (95% CI, 0.136-0.234); the Speech, Spatial, and Qualities of Hearing Scale total score improved by 2.58 (95% CI, 2.18-2.99); and a novel Device Use Questionnaire reported 94% of participants were satisfied with overall hearing in the everyday listening condition. Conclusions and Relevance: This subgroup analysis of patients 65 years or older enrolled in a within-subject clinical trial of cochlear implantation demonstrated clinically meaningful audiometric and QoL benefit with an acceptable risk profile. These findings suggest that cochlear implantation in older adults may facilitate the concept of healthy aging. Trial Registration: ClinicalTrials.gov Identifier: NCT03007472.

Bartter syndrome: An infrequent tubulopathy of prenatal onset. / Síndrome de Bartter: Una tubulopatía infrecuente de inicio antenatal.

INTRODUCTION: Bartter syndrome (BS) is a rare inherited tubulopathy that has two presentation forms, the first one is a severe form of antenatal onset (neonatal Bartter) and the second one is a later on set form during the first years of life (classic Bartter). In the antenatal form, it manifests with fetal polyuria, polyhydramnios of early and severe onset, premature delivery, and intrauterine growth restriction. In the postnatal stage, it presents recurrent episodes of dehydration and electrolyte im balance that can compromise the survival of the patient. OBJECTIVE: To report a clinical case of neo natal BS and a review of the literature. CLINICAL CASE: Premature newborn of 35 weeks of gestation with history of severe polyhydramnios diagnosed at 27 weeks of gestation, without apparent cause. From birth, the patient presented polyuria and hypokalemic metabolic alkalosis making a diagnosis of Neonatal Bartter Syndrome in the first week of life. Laboratory tests confirmed urinary electrolyte losses. The patient was treated with strict water balance and sodium and potassium supplementa tion, achieving weight and electrolyte imbalance stabilization. The patient remains in control in the nephrology unit, with potassium gluconate and sodium chloride supplementation. At the fourth month, ibuprofen was added as part of treatment. At the seventh month of life, renal ultrasound showed nephrocalcinosis. At one year of life, profound sensorineural hearing loss was observed re quiring a cochlear implant. CONCLUSION: The presence of severe polyhydramnios of early onset with no identified cause should lead to suspicion of neonatal BS which even when infrequent determines severe hydroelectrolytic alterations and should be treated early.

Long-term Outcomes in Down Syndrome Children After Cochlear Implantation: Particular Issues and Considerations.

OBJECTIVE: The aim of the study was to analyze the long-term outcomes after cochlear implantation in deaf children with Down syndrome (DS) regarding age at the first implantation and refer the results to preoperative radiological findings as well as postoperative auditory and speech performance. Additionally, the influence of the age at implantation and duration of CI use on postoperative hearing and language skills were closely analyzed in children with DS. STUDY DESIGN: Retrospective analysis. SETTING: Referral center (Cochlear Implant Center). MATERIALS AND METHODS: Nine children with Down syndrome were compared with 220 pediatric patients without additional mental disorders or genetic mutations. Patients were divided into four categories depending on the age of the first implantation: CAT1 (0-3 yr), CAT2 (4-5 yr), CAT3 (6-7 yr), and CAT4 (8-17 yr). The auditory performance was assessed with the meaningful auditory integration scales (MAIS) and categories of auditory performance (CAP) scales. The speech and language development were further evaluated with meaningful use of speech scale (MUSS) and speech intelligibility rating (SIR). The postoperative speech skills were analyzed and compared between the study group and the reference group by using nonparametric statistical tests. Anatomic abnormalities of the inner ear were examined using magnetic resonance imaging (MRI) and high-resolution computed tomography of the temporal bones (HRCT). RESULTS: The mean follow-up time was 14.9 years (range, 13.1-18.3 yr). Patients with DS received a multichannel implant at a mean age of 75.3 months (SD 27.9; ranging from 21 to 127 mo) and 220 non-syndromic children from reference group at a mean age of 51.4 months (SD 34.2; ranging from 9 to 167 mo). The intraoperative neural response was present in all cases. The auditory and speech performance improved in each DS child. The postoperative mean CAP and SIR scores were 4.4 (SD 0.8) and 3.2 (SD 0.6), respectively. The average of scores in MUSS and MAIS/IT-MAIS scales was 59.8% (SD 0.1) and 76.9% (SD 0.1), respectively. Gathered data indicates that children with DS implanted with CI at a younger age (<6 years of age) benefited from the CI more than children implanted later in life, similarly in a control group. There were additional anomalies of the temporal bone, external, middle, or inner ear observed in 90% of DS children, basing on MRI or HRCT. CONCLUSIONS: The early cochlear implantation in children with DS is a similarly useful method in treating severe to profound sensorineural hearing loss (SNHL) as in non-syndromic patients, although the development of speech skills present differently. Due to a higher prevalence of ear and temporal bone malformations, detailed diagnostic imaging should be taken into account before the CI qualification. Better postoperative outcomes may be achieved through comprehensive care from parents/guardians and speech therapists thanks to intensive and systematic rehabilitation.

Outcomes and Predictive Factors of Electroacoustic Stimulation Rehabilitation in Children With Limited Low-Frequency Hearing.

OBJECTIVE: To evaluate the clinical feasibility and auditory benefits of hearing rehabilitation using electroacoustic stimulation (EAS) after cochlear implantation (CI) and to identify the predictive factors for successful EAS rehabilitation in children with limited low-frequency hearing. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral hospital. PATIENTS: Seventeen children (21 ears) under the age of 15 years with residual low-frequency hearing who underwent CI using hearing preservation techniques. INTERVENTION: Patients underwent CI using hearing preservation techniques, and the postoperative audiograms were obtained to evaluate the hearing preservation rate. EAS rehabilitation was applied in patients with successful low-frequency hearing preservation. OUTCOME MEASURES: Improvements in speech perception in both quiet and noise conditions were compared between the EAS mode and the CI-only mode. The predictive factors for successful EAS rehabilitation in children were analyzed. RESULTS: Functional low-frequency residual hearing less than or equal to 85 dB at 250 and 500 Hz was achieved postoperatively in six of 21 ears, and successful EAS rehabilitation was possible in nine of 21 ears. Better speech perception scores were observed in quiet conditions using the EAS mode compared with the CI-only mode, although the difference did not reach statistical significance. Significantly, better scores were observed in noise conditions with the EAS mode compared with the CI-only mode. Postoperative low-frequency pure-tone average was the only significant predictive factor of successful EAS rehabilitation. CONCLUSION: CI surgery using hearing preservation techniques with EAS rehabilitation should be performed in children, even in patients with limited residual hearing, to improve auditory outcomes.

Síndrome de Bartter: una tubulopatía infrecuente de inicio antenatal / Bartter syndrome: an infrequent tubulopathy of prenatal onset

INTRODUCCIÓN: Síndrome de Bartter (SB) es una tubulopatía hereditaria, poco frecuente que tiene dos formas de presentación, forma grave de inicio antenatal (Bartter neonatal) y forma de aparición más tardía (Bartter clásico). En su forma antenatal se manifiesta con poliuria fetal, polihidroamnios de inicio precoz y severo, parto prematuro secundario y restricción de crecimiento intrauterino. La etapa postnatal presenta episodios recurrentes de deshidratación y desbalance electrolítico que pue den comprometer la sobrevida del paciente. OBJETIVO: Comunicar un caso de SB neonatal y presentar una revisión de la literatura en esta patología. CASO CLÍNICO: Prematuro 35 semanas, con antecedente de severo polihidroamnios diagnosticado a las 27 semanas de gestación, sin causa aparente. Desde su nacimiento evolucionó con poliuria y alcalosis metabólica hipokalémica haciendo plantear, en primera semana de vida, diagnóstico de Síndrome de Bartter neonatal. El laboratorio confirmó per didas urinarias de electrólitos. Fue manejado con balance hídrico estricto y suplementación de sodio y potasio, logrando estabilizar peso y desbalance electrolítico. Se mantiene en control nefrológico, con suplementación de gluconato de potasio y cloruro de sodio. Se agregó ibuprofeno al cuarto mes como parte del tratamiento. Al séptimo mes de vida, ecografía renal demostró nefrocalcinosis. Al año de vida se evidenció hipoacusia sensorioneural profunda requiriendo implante coclear. CONCLUSIÓN: Presencia de polihidroamnios severo de aparición temprana sin causa identificada debe hacer sospechar SB, que aun siendo infrecuente determina graves alteraciones hidroelectrolíticas y debe ser iniciado su tratamiento precozmente.

INTRODUCTION: Bartter syndrome (BS) is a rare inherited tubulopathy that has two presentation forms, the first one is a severe form of antenatal onset (neonatal Bartter) and the second one is a later on set form during the first years of life (classic Bartter). In the antenatal form, it manifests with fetal polyuria, polyhydramnios of early and severe onset, premature delivery, and intrauterine growth restriction. In the postnatal stage, it presents recurrent episodes of dehydration and electrolyte im balance that can compromise the survival of the patient. OBJECTIVE: To report a clinical case of neo natal BS and a review of the literature. CLINICAL CASE: Premature newborn of 35 weeks of gestation with history of severe polyhydramnios diagnosed at 27 weeks of gestation, without apparent cause. From birth, the patient presented polyuria and hypokalemic metabolic alkalosis making a diagnosis of Neonatal Bartter Syndrome in the first week of life. Laboratory tests confirmed urinary electrolyte losses. The patient was treated with strict water balance and sodium and potassium supplementa tion, achieving weight and electrolyte imbalance stabilization. The patient remains in control in the nephrology unit, with potassium gluconate and sodium chloride supplementation. At the fourth month, ibuprofen was added as part of treatment. At the seventh month of life, renal ultrasound showed nephrocalcinosis. At one year of life, profound sensorineural hearing loss was observed re quiring a cochlear implant. CONCLUSION: The presence of severe polyhydramnios of early onset with no identified cause should lead to suspicion of neonatal BS which even when infrequent determines severe hydroelectrolytic alterations and should be treated early.

Cochlear implantation under local anesthesia: a possible alternative for elderly patients.

INTRODUCTION: As average life-expectancy increases, a sufficient hearing rehabilitation for elderly patients with severe-to-profound sensorineural hearing loss becomes more important. Cochlear implantation is a relatively safe surgical procedure also for elderly patients, the higher risk is caused by general anesthesia. We report on four patients who underwent cochlear implantation under local anesthesia. METHODS: After detailed preoperative examinations (audiological tests, imaging, genetic tests, evaluation of motivation and compliance of the patient), four patient with severe-to-profound hearing loss were selected for cochlear implantation under local anesthesia. For the electrode insertion, we used the posterior suprameatal approach technique. Pre- and postoperative pure tone audiometry and speech-perception tests were conducted to prove the success of the procedure. RESULTS: The mentioned technique was applied; the average length of the operation was 52 min. The intraoperative measurements showed normal impedance and normal neuronal response telemetry, all the patients had sound experience during the intraoperative examination of the engineer. No complications were observed. The postoperative audiological tests showed a significant increase in the hearing perception. CONCLUSION: Cochlear implantation under local anesthesia is a safe and fast procedure for elderly patients. The intraoperative sound experience can give an extra motivation in the postoperative rehabilitation. Our results prove that by carefully selected elderly patients cochlear implantation can assure a significant increase in speech perception. We can establish that the new posterior suprameatal approach technique combined with local anesthesia presents a viable future option for those patients who were inoperable beforehand because of high risks of general anesthesia.

Binaural advantages in using a cochlear implant for adults with profound unilateral hearing loss.

BACKGROUND: Recent studies of cochlear implants (CIs) in profound unilateral hearing loss (UHL) patients have demonstrated a restoration of some binaural hearing. AIMS/OBJECTIVES: The objective was to evaluate three possible advantages of binaural hearing in CIs adult users with UHL including single-side deafness (SSD) and asymmetric hearing loss (AHL) subgroups. MATERIAL AND METHODS: A prospective study was conducted that included 70 sequentially implanted patients. Subgroups of these subjects included 64 with a postlingual onset of a profound hearing loss on the implanted side and 6 with a prelingual onset of that loss. Three binaural effects - redundancy, head shadow, and squelch - were evaluated. RESULTS: Significant differences between the 'CI on' and 'CI off' conditions were found for all three binaural effects for the study group as a whole and for the postlingual subgroup. However, results for the subjects in the prelingual subgroup did not demonstrate any of the binaural advantages. CONCLUSION AND SIGNIFICANCE: Patients with a postlingual onset of a profound hearing loss in one ear and normal hearing or only a moderate loss in the other ear are able to make the effective use of a CI in the profound-loss ear in conjunction with acoustic stimulation of the other ear.

Cochlear implant in Thalassemia patient - Case report.

INTRODUCTION: Beta-thalassemias are a group of hereditary blood disorders characterized by anomalies in the synthesis of the beta chains of hemoglobin. Iron overload occurs in thalassemia, with blood transfusion therapy being the major cause. Deferoxamine continues to be the mainstay of therapy to remove excess iron in patients requiring long-term transfusions. One of the most important complications of deferoxamine therapy is neurosensory toxicity, including sensorineural hearing loss (SNHL). Labyrinthine hemmorhage (LH) is thought to result from altered capillary hemodynamics or reperfusion injury. It is theorized that LH incites a reparative response that cascades from fibrosis to sclerosis and ultimately ossification of the inner ear structures. CASE PRESENTATION: We present a case of 3-year-old thalassemic child with bilateral profound sensorineural hearing loss. Patient was on regular blood transfusions with chelation therapy. HRCT temporal bone and MRI brain and temporal bone had features of labyrinthitis ossificans (LO). Child underwent uniateral cochlear implantation and postimplantation speech perception and production outcomes were normal. DISCUSSION: This case illustrates the unique feature of labyrinthitis ossificans in a thalassemia patient which has not yet reported in the English literature. Hearing screening of all thalassemia patients and therefore early diagnosis of SNHL prompts early intervention and improved quality of life.

Comparison of a Traditional and Novel Evoked Compound Action Potentials Recording Approach and Evoked Auditory Brainstem Responses in Pediatric Cochlear Implants Users.

OBJECTIVES: Electrically evoked compound action potentials (eCAP) recordings are widely used in functional evaluation and fitting of cochlear implants (CI) in clinics. We compared the results from two eCAP recording approaches (StandardART and FineGrain, MED-EL, Austria). The FineGrain method is more advanced than the Auditory Nerve Response Telemetry (StandardART) method in terms of the stimulation and algorithm for the eCAP threshold detection. To understand the benefits of these alterations, we compared the two methods on a larger scale in pediatric CI users alongside evoked auditory brainstem responses (eABR). MATERIALS AND METHODS: We collected the eCAP recordings obtained with both methods from a population of pediatric subjects with CI, either intra- or post-operatively. The eABR recordings were only collected post-operatively. For comparability reasons, we used the same stimulation rate and similar amplitude levels for all three approaches. RESULTS: Our results demonstrate that, although the success rates are similar, the FineGrain method outperforms traditional StandardART in terms of robustness and measurement duration. The eCAP recordings in general outperform the eABR in terms of speed. CONCLUSION: We conclude that the eCAP recordings are the method of choice for measuring the auditory neural activity, and FineGrain outperforms StandardART. From the three investigated approaches, we conclude that FineGrain performed best and should be the first-choice method in pediatric patients.

Multicenter US Clinical Trial With an Electric-Acoustic Stimulation (EAS) System in Adults: Final Outcomes.

OBJECTIVE: To demonstrate the safety and effectiveness of the MED-EL Electric-Acoustic Stimulation (EAS) System, for adults with residual low-frequency hearing and severe-to-profound hearing loss in the mid to high frequencies. STUDY DESIGN: Prospective, repeated measures. SETTING: Multicenter, hospital. PATIENTS: Seventy-three subjects implanted with PULSAR or SONATA cochlear implants with FLEX electrode arrays. INTERVENTION: Subjects were fit postoperatively with an audio processor, combining electric stimulation and acoustic amplification. MAIN OUTCOME MEASURES: Unaided thresholds were measured preoperatively and at 3, 6, and 12 months postactivation. Speech perception was assessed at these intervals using City University of New York sentences in noise and consonant-nucleus-consonant words in quiet. Subjective benefit was assessed at these intervals via the Abbreviated Profile of Hearing Aid Benefit and Hearing Device Satisfaction Scale questionnaires. RESULTS: Sixty-seven of 73 subjects (92%) completed outcome measures for all study intervals. Of those 67 subjects, 79% experienced less than a 30 dB HL low-frequency pure-tone average (250-1000 Hz) shift, and 97% were able to use the acoustic unit at 12 months postactivation. In the EAS condition, 94% of subjects performed similarly to or better than their preoperative performance on City University of New York sentences in noise at 12 months postactivation, with 85% demonstrating improvement. Ninety-seven percent of subjects performed similarly or better on consonant-nucleus-consonant words in quiet, with 84% demonstrating improvement. CONCLUSION: The MED-EL EAS System is a safe and effective treatment option for adults with normal hearing to moderate sensorineural hearing loss in the low frequencies and severe-to-profound sensorineural hearing loss in the high frequencies who do not benefit from traditional amplification.

Hearing Preservation During Cochlear Implantation and Electroacoustic Stimulation in Patients With SLC26A4 Mutations.

BACKGROUND AND OBJECTIVES: Patients with SCL26A4 mutations presenting with Mondini deformity and enlarged vestibular aqueduct (EVA) tend to have comparable residual hearing. Although cochlear implantation (CI) produces good results in this group, deterioration of residual hearing can be an adverse event after surgery due to accompanying cochlear malformation and perilymph leakage during cochleostomy. The purpose of this study was to investigate if CI in patients with SCL26A4 mutations via the round window (RW) approach could achieve preservation of residual hearing, and to evaluate their speech reception with electroacoustic stimulation (EAS). SUBJECTS AND METHODS: This is a retrospective chart review of eight patients with bilateral EVA, who were bi-allelic patients with SCL26A4 mutations. CI was performed in all patients by a single surgeon using the RW approach. Audiological results were compared before and after implantation. RESULTS: Additional hearing loss after CI was less than 10 dBHL in five out of eight patients. Average hearing deterioration after CI was 8.75 dB (range, 0-26). Six out of eight patients used EAS mode after CI. The acoustic stimulation frequency ranged from 271 to 438 Hz. Patients showed better speech recognition in quiet and in noise using EAS mode compared with electrical stimulation alone. CONCLUSIONS: Preservation of residual hearing could be achieved after CI in patients with the SLC26A4 mutation via the RW approach. For successful preservation of residual hearing, application of newly-developed soft electrode and meticulous surgical is necessary. Our study showed that patients with the SLC26A4 mutation can be good candidates for EAS surgery.

The Effect of Piston Diameter in Stapedotomy for Otosclerosis: A Temporal Bone Model.

HYPOTHESIS: The use of larger-diameter pistons in stapedotomy leads to better hearing outcomes compared with the use of smaller-diameter pistons. There is an interaction between stapes piston diameter and fenestration diameter. BACKGROUND: Otosclerosis can be treated surgically by removing part of the stapes and bypassing the stapes footplate with a prosthesis. Available piston shaft diameters range between 0.3 and 0.8 mm. There has been a tendency toward the use of smaller-diameter pistons, because of a suspected decreased risk of cochlear trauma and subsequent sensorineural hearing loss (SNHL) with smaller pistons. However, mathematical models, temporal bone studies, and clinical studies suggest that the use of larger-diameter pistons is associated with better hearing outcomes. METHODS: Three fresh-frozen, non-pathologic temporal bones were harvested from human cadaveric donors. Acoustic stimuli in the form of pure tones from 250 to 8000 Hz were generated at 110 dB sound pressure level. A total of 16 frequencies in a 1/3-octave series were used. Stapes and round window velocities in response to the acoustic stimuli were measured at multiple equally spaced points covering the stapes footplate and round window using a scanning laser Doppler interferometry system. Eight sets of measurements were performed in each temporal bone: 1) normal condition (mobile stapes), 2) stapes fixation and stapedotomy followed by insertion of 3) a 0.4-mm-diameter piston in a 0.5-mm-diameter fenestration, 4) a 0.4-mm-diameter piston in a 0.7-mm-diameter fenestration, 5) a 0.4-mm-diameter piston in a 0.9-mm-diameter fenestration, 6) a 0.6-mm-diameter piston in a 0.7-mm-diameter fenestration, 7) a 0.6-mm-diameter piston in a 0.9-mm-diameter fenestration, and 8) a 0.8-mm-diameter piston in a 0.9-mm-diameter fenestration. RESULTS: At midrange frequencies, between 500 and 4000 Hz, round window velocities increased by 2 to 3 dB when using a 0.6-mm-diameter piston compared with a 0.4-mm-diameter piston. Using a 0.8-mm-diameter piston led to a further increase in round window velocities by 2 to 4 dB. CONCLUSION: Our results suggest a modest effect of piston diameter on hearing results following stapedotomy.

Bilateral cochlear implantation under local anaesthesia in a young adult - A case report.

OBJECTIVE: Cochlear implantation is routinely performed under general anaesthesia in most centres in the world. There have been reports of this surgery being performed under local anaesthesia and sedation for unilateral cochlear implantation. Our case study reports the first bilateral cochlear implantation carried out under local anaesthesia with sedation. This paper outlines the protocol used and discusses the challenges and advantages of the procedure being performed under local anaesthesia. CASE STUDY: A 28-year-old female with a history of multidrug-resistant tuberculosis presented with bilateral severe to profound sensorineural hearing loss. On evaluation, she was found to be a suitable candidate for cochlear implantation. However, she was clinically unfit to undergo the surgery under general anaesthesia. After counselling the patient, the decision to undertake the procedure under local anaesthesia was made. The procedure took 105 min and was uneventful. The patient was discharged the next day Conclusion: In the hands of an experienced surgeon and anaesthesiology team, bilateral cochlear implantation is possible under local anaesthesia. Patient motivation and cooperation are extremely important for the procedure to be done under local anaesthesia. This is an option for patients needing cochlear implantation who are medically unfit for general anaesthesia.