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To compare the outcomes between 2 age-matched cohorts of children with cochlear nerve deficiency: those receiving auditory brainstem implants (group A) or cochlear implants (group B).Retrospective cohort study.Tertiary referral center.Subjects were selected from a pool of 537 children fitted with cochlear implants (n = 443) or auditory brainstem implants (n = 94) over the past 14 years. Performance, examined with the Category of Auditory Performance scale, and complications were compared with a mean follow-up of 5 years.All children had bilateral profound sensorineural hearing loss and cochlear nerve deficiency. Magnetic resonance imaging documented an absent cochlear nerve (n = 12) and a small cochlear nerve (n = 8) in group A and an absent cochlear nerve (n = 11) and a small cochlear nerve (n = 9) in group B (P = 1.000). Children with cochlear implants had Category of Auditory Performance scores spanning from 0 to 3 levels of performance, and all required manual communication mode and visual supplementation. Children with auditory brainstem implants had Category of Auditory Performance scores spanning from 2 to 7, and most patients demonstrated behavioral responses irrespective of inner ear malformations and an absent cochlear nerve or small cochlear nerve (P.001).In children with cochlear nerve deficiency, patients fitted with cochlear implants did not develop speech understanding and production. Those fitted with auditory brainstem implants had the opportunity to develop open-set speech perception, acquiring verbal language competence using oral communication exclusively and participating in mainstream education. The overall complication rate of auditory brainstem implants was not greater than that of cochlear implants.

To evaluate retrospectively the long-term safety and efficacy of the first 50 patients, all suffering from severe ossicular chain defects and with moderate to severe mixed hearing loss, who received the Vibrant SoundBridge with the floating mass transducer located on the round window membrane. To evaluate differences in outcome versus etiology and age of the patient population.Case series with planned data collection.Tertiary referral medical center.Patients eligible for implantation of the floating mass transducer on the round window membrane ranged in age from 2 months to 74 years with a moderate to severe conductive or mixed hearing loss from different etiologies. For each adult patient, preoperative versus postoperative bone and air conduction thresholds, air-bone gaps, and speech understanding scores were evaluated at 24-month follow-up. At 60-month follow-up, data were available from 33 patients. Preoperative and postoperative free-field auditory brainstem responses were studied in infants and children. Intraoperative and short- and long-term postoperative complications are presented.There were significant improvements in speech perception and pure-tone audiometry in adults and auditory brainstem response thresholds in infants immediately after surgery and at follow-up examinations (12 to 71 months). No significant complications or device extrusions were observed in the present series.Infants, children, and adults with moderate to severe conductive or mixed hearing loss obtained substantial benefit from implantation of the floating mass transducer on the round window membrane regardless of the etiology of hearing loss and previous surgery.

(1) To determine the long-term outcomes of cochlear implantation in children implanted younger than 6 months and (2) to evaluate auditory-based performance in very young children compared with older children, all with profound sensorineural bilateral hearing loss.Prospective cohort study.Tertiary referral center.Twelve subjects aged 2 to 6 months, 9 aged 7 to 12 months, 11 aged 13 to 18 months, and 13 aged 19 to 24 months, all with profound bilateral hearing loss, were fitted with cochlear implants and followed longitudinally for 4 years. Subjects were developmentally normal with no additional disabilities (visual, motor, or cognitive). Auditory-based communication outcomes included tests for speech perception, receptive language development, receptive vocabulary, and speech production.Age at cochlear implantation was a significant factor in most outcome measures, contributing significantly to speech perception, speech production, and language outcomes. There were no major complications and no significantly higher rates of minor complications in the younger children.This article reports an uncontrolled observational study on a small group of infants fitted with cochlear implants following personal audiological criteria and, up to now, with limited literature support due to the innovative nature of the study. This study shows, for the first time, significantly improved auditory-based outcomes in children implanted younger than 6 months and without an increased rate of complications. The data from the present study must be considered as explorative, and a more extensive study is required.

To determine whether intraoperative electrocochleography during cochlear implant surgery provides online feedback to modify surgical procedure, reduce trauma, and increase preservation of residual hearing.Prospective cohort study.Tertiary referral center, Otolaryngology Department, University of Verona.Twenty-seven adult patients undergoing cochlear implant surgery who had low- to mid-frequency (0.25-2 kHz) auditory thresholds measured preoperatively were enrolled. Fifteen subjects had compound action potentials measured to assess cochlear function during surgery. In those patients, surgery was modified according to electrocochleographic feedback. Twelve control subjects underwent cochlear implant surgery with blinded electrocochleographic monitoring.The average preoperative pure-tone audiometry thresholds (0.25-2 kHz) were 74.3 ± 10.2 and 81.5 ± 12.7 dB hearing level (HL) in the electrocochleographic feedback and control cohorts, respectively (P.05). Compound action potential recordings showed a mean maximum latency shift of 0.63 ± 0.36 ms and normalized amplitude deterioration of 59% ± 19% during surgery. All of these changes reverted to normal after electrode insertion in all but 1 subject in the electrocochleographic feedback group. The average shifts in postoperative pure-tone average threshold (0.25-2 kHz), evaluated before activation, were 8.7 ± 4.3 and 19.2 ± 11.4 dB HL in the electrocochleographic feedback and control cohorts, respectively (P = .0051). Complete hearing preservation (loss of ≤10 dB) at 1 month before activation was achieved in 85% (11/13) of electrocochleographic feedback subjects and in 33% (4/12) of control patients (P = .0154).Monitoring cochlear function with electrocochleography gives real-time feedback during surgery, providing objective data that might help in modifying the surgical technique in ways that can improve the rate of hearing preservation.

Objective The aim of the present study was to verify whether drugs injected into the endolymphatic sac (ES) can reach the cochlea and possibly treat inner ear disorders. Study Design Prospective cohort study. Setting Tertiary referral center, Otolaryngology Department, University of Verona. Subjects and Methods Patients with Meniere's disease (MD) who were candidates for ES decompression were selected. Nineteen subjects received dexamethasone (DEX) via injection into the ES. To objectively define whether substances administered into the ES could reach the cochlea, we added gadolinium (GD) in three patients. All subjects had intraoperative electrocorticogram recordings and an audiologic follow-up. The three subjects who underwent injection of the DEX-GD solution were followed-up with magnetic resonance imaging. The audiological data are presented during a follow-up period of 12 months. Results Intraoperative electrocochleography recordings revealed no changes in two patients and summating potentials and compound action potential latency and wave-form modifications in all the other subjects. GD distribution was observed from 48 hours to one week after ES injection into the cochlea of the three subjects injected with DEX-GD. GD-related enhancement of inner ear structures lasted more than two weeks in all subjects. Pure tone average results showed hearing improvement of at least 20 dB HL in 42 percent of patients (8 of 19) at the 12-month follow-up. Statistically significant differences emerged between the mean pure tone average of the ES procedure subjects at one and 12 months after surgery ( P = 0.0096). Conclusion This novel approach might reveal new prospects for treating viral, metabolic, autoimmune, and genetic disorders of the cochlea.