Hearing Rehabilitation in Patients With Neurofibromatosis Type 2: The Quebec's Experience With Auditory Implants.

OBJECTIVE: To review a single-center experience with hearing rehabilitation in patients with neurofibromatosis type 2 (NF2) and to describe the auditory outcomes of cochlear implants (CIs) and auditory brainstem implants (ABI) in this population. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: Twelve adult patients with NF2 who received a CI (n = 10) and/or an ABI (n = 5) between 2000 and 2021. INTERVENTIONS: Insertion of a CI and/or an ABI in NF2 patients with bilateral vestibular schwannomas (VSs). MAIN OUTCOME MEASURES: Patients' demographic data, treatment history, hearing evolution, hearing rehabilitation methods, implant details, and auditory outcomes after implantation. RESULTS: Among those who received a CI, five patients had a stable untreated VS, one patient underwent a cochlear nerve preserving surgery, and four patients received radiotherapy treatments. Six patients became regular users of their device. The median open-set sentence recognition scores were as follows: 0.5% preoperatively, 60% at 1 year postoperatively, and 80% on the most recent audiological evaluation. All patients with an ABI were implanted concomitantly with VS surgical excision, and three of them also received radiotherapy treatments. The median open-set sentence recognition scores were as follows: 4% preoperatively, 26% at 1 year postoperatively, and 0% on the most recent evaluation. Three patients became regular ABI users. CONCLUSION: Despite major technological advances, auditory outcomes with ABIs remain deceiving. Considering the overall improvement in postoperative auditory performances provided by CIs compared with ABIs, cochlear implantation standouts as a primary mean of hearing rehabilitation in NF2 patients.

The Correlation of Congenital CMV Infection and the Outcome of Cochlear Implantation.

OBJECTIVES: Cytomegalovirus (CMV) infection is the most common intrauterine viral infection, affecting approximately 0.5-2.5% of all live births in the world. The majority of patients are asymptomatic at birth, but several clinical consequences are related to this infection, including neurosensory hearing loss. The cochlear implant is the treatment of choice when the hearing loss is severe to profound. Compare the audiological evolution after cochlear implant surgery in a group of children born with congenital CMV infection compared to a control group of children born with a genetic cause of congenital hearing loss. Determine prognostic factors predicting the outcome of patients with congenital hearing loss secondary to CMV infection following cochlear implantation. METHODS: Our retrospective study aimed at the analysis of 48 patients with cochlear implants, 25 patients with congenital CMV, and 23 patients in the control group with cochlear cause of hearing loss, who were matched for gender, age of onset, and type of hearing loss. Primary outcomes are auditory evolution with the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) and the multimedia audiologic test (MAT). Secondary outcomes are evaluation of prognostic factors for the CMV group, like MRI severity sign, antiviral treatment, and neuropsychological disorder. RESULTS: For the MAT, the results are 73.9% in the CMV group and 81.6% in the control group (P = .03). For the IT-MAIS, the results are 29.3/40 in the CMV group and 29.2/40 in the control group (P = .96). In the CMV group, the children treated with antiviral treatment have IT-MAIS difference of 28.5/40 compared to 23.2/40 for the children without antiviral treatment (P = .03). We found a little trend in the correlation between auditory results and the presence of neuropsychological disorders, but there are no statistically significant results. CONCLUSION: The results of the audiological tests in the CMV group allow an adequate functioning, even if lower than in the control group. There are benefits of implantation in patients with CMV, and audiological results are still satisfactory for proper functioning. CMV patients enjoy superior results in terms of hearing, with antiviral treatment.

Clinical efficiency and safety of the oticon medical neuro cochlear implant system: a multicenter prospective longitudinal study.

OBJECTIVE: This prospective longitudinal cohort study at six tertiary referral centers in Canada and Denmark describes the clinical efficiency and surgical safety of cochlear implantation with the Oticon Medical Neuro cochlear implant system, including the Neuro Zti implant, the EVO electrode array, and the Neuro One sound processor. METHODS: Patients were adult cochlear implant candidates with bilateral sensorineural hearing loss. RESULTS: The mean HINT scores in quiet pre-operatively and at 3, 6, and 12 months post-activation were 13%, 58%, 67%, and 72%, respectively, and in noise (+10 dB SNR) 13%, 46%, 53%, and 59%, respectively. The mean improvement from baseline to 6 months post-activation was 54% in quiet and 40% in noise. The surgical major complication incidence rate was 0% and the post-surgical major complication incidence rate (until 12 months post-activation) was 4%. There was no adverse event that was fatal, that required explantation, or that resulted in sound processor nonuse, and no implant failure. CONCLUSION: Cochlear implantation with the Oticon Medical Neuro system enables speech identification both in quiet and in noise and audiologic outcomes continue to improve in the year following activation. No substantial adverse events occurred during the surgical implantation procedure and during the 12 months post-activation.

A randomized controlled trial to test the efficacy of trans-tympanic injections of a sodium thiosulfate gel to prevent cisplatin-induced ototoxicity in patients with head and neck cancer.

BACKGROUND: Cisplatin-induced hearing loss is frequent and severe. Antioxidants such as sodium thiosulfate (STS) can neutralize the effects of cisplatin. The objective of the trial was to test the efficacy of trans-tympanic injections of a STS gel to prevent cisplatin-induced ototoxicity. METHODS: Eligible participants were newly diagnosed patients with stage III or IV squamous cell carcinoma of the mouth, oropharynx, hypopharynx, or larynx and scheduled to be treated by concurrent chemoradiation (CCR). Patients with asymmetric hearing were not eligible. The planed treatment included cisplatin 100 mg/m2 at days 1, 22 and 43. A baseline pre-treatment complete audiometric evaluation (pure tone at frequencies ranging from 0.5 to 14 kHz, bone conduction at 0.5-4 kHz and DPOAEs) was performed. Adverse effects were noted according to CTCAE. On the day before the beginning of CCR, eligible and consenting patients were randomized to receive a trans-tympanic injection of the gel either in the left ear or in the right ear. A final post-treatment complete audiometric evaluation was scheduled to be performed 1 month after the end of CCR by audiologists kept blind to the ear assignment. For the main outcome, the permanent threshold shift (PTS) in decibel (dB) was calculated as the difference between the final and baseline measures at all pure tone frequencies at 0.5-14 kHz for each patient and for each ear. The main outcome was assessed blindly in a mixed linear model with the PTS as the dependent variable and intervention, frequency, their interaction and radiation dose to the cochlea as independent variables. RESULTS: Between January 2015 and April 2016, 13 patients were randomized. The trial was stopped in June 2016 for poor accrual. The average loss of hearing over all frequencies was 1.3 dB less for treated ears compared to control ears. Although not statistically (p = 0.61) nor clinically significant, the difference was in favor of the treated ears for all frequencies between 3 and 10 kHz. CONCLUSIONS: Our trial suggests that STS deposited on the round window was safe for the middle and inner ears. More work is needed to improve the efficacy of trans-tympanic injections of cisplatin antidotes. TRIAL REGISTRATION: ClinicalTrials.gov, NTC02281006 , Registered 3 November 2014.

Comparative Impacts of Scala Vestibuli Versus Scala Tympani Cochlear Implantation on Auditory Performances and Programming Parameters in Partially Ossified Cochleae.

OBJECTIVE: To compare scala vestibuli versus scala tympani cochlear implantation in terms of postoperative auditory performances and programming parameters in patients with severe scala tympani ossification. STUDY DESIGN: Retrospective case-control study. SETTING: Tertiary referral center. PATIENTS: One hundred three pediatric and adult patients who underwent cochlear implant surgery between 2000 and 2016. Three groups were formed: a scala vestibuli group, a scala tympani with ossification group, and a scala tympani without ossification group. Patients were matched based on their age, sex, duration of deafness, and side of implantation (ratio of 1:2:2). INTERVENTIONS: Postoperative evaluation of auditory performances and programming parameters following intensive functional rehabilitation program completion. MAIN OUTCOME MEASURES: Multimedia adaptive test (MAT), hearing in noise test (HINT SNR +10 dB, HINT SNR +5 dB, and HINT SNR +0 dB), impedances, neural response telemetry thresholds (NRT), neural response imaging thresholds (NRI), comfortable levels (C-levels), and threshold levels (T-levels) were compared between groups. RESULTS: Twenty-one patients underwent scala vestibuli cochlear implantation: 19 adults and two children. Auditory performances were similar between groups, although sentence recognition in a noisy environment was slightly higher in the scala vestibuli group. Impedance values were also higher in the scala vestibuli group, but all other programming parameters were similar between groups. CONCLUSIONS: We present the largest series of patients with scala vestibuli cochlear implantation. This approach provides at least comparable auditory performances without having any deleterious effects on programming parameters. This viable and useful insertion route might be the primary surgical alternative when facing partial cochlear ossification.

Single-sided deaf tactical unit officer treated with cochlear implantation.

OBJECTIVES: To demonstrate the efficacy of cochlear implantation for management of a severe single-sided deaf tactical unit officer. Showing that hearing improvement provided by cochlear implantation was sufficient for him to return to work as a police tactical officer. METHODS: Case report. RESULTS: A 43 years-old man working as a tactical unit officer suffered from a work-related severe single-sided hearing loss. He tried unsuccessfully many types of hearing aids including a contralateral routing of sound (CROS) system and a bone conduction hearing aid with a headband. He was finally treated with cochlear implantation which provided enough localization and hearing abilities for a complete return to work as a tactical unit officer. DISCUSSION: This case report demonstrates that cochlear implantation was very successful for a patient suffering from severe single-sided hearing loss. The current literature demonstrates benefits from cochlear implant over CROS and osseointegrated devices in unilateral hearing loss. Nevertheless, literature does not report any case of patient suffering from single-sided deafness that received a cochlear implant and a complete bilateral hearing rehabilitation that allowed him to return to work as a police tactical officer. CONCLUSION: Cochlear implantation was the only effective treatment to provide our patient enough localization and hearing abilities for a complete return to work as a police tactical officer. As no consensus has been established about selection criteria for cochlear implantation in single-sided deafness, we suggest that the patient's occupation should be considered when reviewing his candidacy for cochlear implantation.

Outcomes review of modern hearing preservation technique in cochlear implant.

INTRODUCTION: Preservation of residual hearing in cochlear implantation is a main concern for patients and otologists. New electrode arrays as well as development of minimally invasive technique have allowed the expansion of indication criteria for cochlear implantation. The loss of residual low-frequency hearing is thought to be the result of many factors. Opinions differ in regards with the electrodes array characteristics, the surgical implantation technique and the pharmacological therapy used. OBJECTIVE: The aim of this research is to analyze the available information pertaining to hearing preservation with cochlear implantation. RESULTS: Both cochleostomy and round window approaches are adequate, but should rely on the anatomic position of the round window membrane. No electrode design had a higher rate of hearing preservation, either a standard or shorter length was used, or a straight or contoured array. The speed of insertion has a significant impact on hearing preservation and vestibular function. A slow insertion should be used for all cochlear implant insertion, hearing preservation or not. However, the optimal speed of insertion is still unclear. Moreover, the use of steroids regardless of the route or the timing, along with intraoperative topical steroids, had a positive impact on hearing preservation. CONCLUSION: Classic atraumatic insertion maneuvers, very slow and delicate insertion and the use of intraoperative corticosteroids improve hearing outcomes. Whichever the surgeon's preferences, all surgical modifications are aimed at the same goal: protection of the delicate intracochlear structures with preservation of residual low-frequency hearing to improve speech perception abilities.

Improving audiologic performance with partial insertion of a compressed array despite intracochlear retention of four electrodes during revision cochlear implant surgery: A case report.

OBJECTIVE AND IMPORTANCE: To report a case of partial extraction of the electrode array during revision cochlear implant surgery and to discuss the surgical management of this rare complication. Clinical presentation The safety of revision cochlear implant surgery has long been demonstrated. Only five cases of partial extraction of the electrode array with intracochlear retention have been reported in the literature. In this report, we describe the case of a 12-year-old boy with post-meningitis deafness who suffered this complication. INTERVENTION: Despite intracochlear retention of four electrodes, the surgical team was able to perform partial insertion of a Med-El compressed array: a total of 8 electrodes out of 12 were implanted in the same scala tympani. Five months after the surgery, the patient had access for the first time to open-set speech recognition. He could recognize 77% of open-set sentences in silence compared to 14% after initial implant activation. Seven months after the surgery, the patient was implanted in his contralateral ear with a Med-El Pulsar split array and now benefits from bilateral auditory stimulation. CONCLUSION: Partial insertion of a compressed array represents a viable option when facing incomplete extraction of a cochlear implant electrode array. Indeed, our patient's audiologic performance improved significantly and the results seem to surpass those obtained with partial insertion of a conventional electrode array.

Hearing outcomes in functional surgery for middle ear malformations.

OBJECTIVES: To standardize the information for families of children having functional surgery for middle ear malformations, we describe the audiometric results of the subgroup of patients with the most favorable anatomic conditions: viable auditory canal, intact tympanic membrane, mobile stapes, and corresponding to a Jahrsdoerfer score of 8 or higher. STUDY DESIGN: Case series, tertiary referral center. METHODS: Charts of patients undergoing functional surgery for congenital middle ear malformations were reviewed for demographic data, preoperative Jahrsdoerfer score, ossicular chain status, type of ossiculoplasty, and audiometric data before and 6 months postsurgery. RESULTS: Eighteen consecutive interventions were performed on 13 patients (average age of 9 years, 8 girls and 5 boys) between 2004 and 2011. The ossiculoplasties performed were as follows: incus repositioning (4), double-layer tragal cartilage (5), intact native chain reconstruction (3), and partial ossicular prosthesis (6). Mean air bone gap (ABG) was 40.8 ± 12.4 dB preoperatively and 20.9 ± 12.9 dB postoperatively (p < 0.0001). Preoperative and postoperatively mean air conduction PTA thresholds were 49.9 ± 9.5 and 30.0 ± 14.1 dB, respectively (p < 0.0001). All ears operated on except one had air conduction improvement. There were no complications. CONCLUSION: Functional surgery for congenital middle ear malformations gives variable hearing outcomes. In this study, with the most favorable anatomic conditions, 12 ears (67%) of 18 had air conduction improvement below 30 dB.

Cochlear implantation in postmeningitic deafness.

OBJECTIVES/HYPOTHESIS: To propose guidelines in the management of a profound bilateral sensorineural hearing loss after bacterial meningitis. STUDY DESIGN: Retrospective chart review. PATIENTS: All 40 postmeningitic cochlear implantation surgeries performed between August 1987 and April 2007. METHODS: Descriptive analysis of data on the cause of meningitis, preoperative imaging evaluation, age at implantation, time elapsed between meningitis and implantation, and relevant surgical findings. RESULTS: Twenty-seven children and 13 adults with postmeningitic deafness were implanted. Mean age was 3 years 8 months for the children and 44 years and 10 months for the adults. The mean time delay between meningitis and surgery was 2 years 1 month for children and 28 years for adults. Eighteen children (67%) were implanted within a year. Labyrinthitis ossificans was evidenced at surgery in 62% of patients. Intraoperative cochlear ossification was classified according to the scale described by Smullen and Balkany (2005). Stage II ossification was seen in 3 patients within 49 days, with 1 of them as soon as 21 days. There was a partial insertion in 9 patients secondary to ossification. Open-set speech discrimination was achieved by 37% of the children (10 of 27) and 23% of the adults (3 of 13). CONCLUSION: We recommend early cochlear implantation for patients with bilateral profound deafness secondary to meningitis.