Cochlear implantation in children with sickle cell disease.

Sickle cell disease (SCD) is associated with sensorineural hearing loss (SNHL). Although the hearing loss is usually mild, some develop severe-to-profound hearing loss, in whom cochlear implants (CI) may be an option. We present the cases of two children with SCD who developed bilateral severe-to-profound SNHL and underwent cochlear implantation. One patient became profoundly deaf after an acute episode of dizziness. Imaging indicated bilateral cochlear ossification, making subsequent cochlear implant surgery challenging. The second patient developed bilateral severe-to-profound SNHL following acute vaso-occlusive crises. She went on to have uncomplicated cochlear implant surgery. These cases illustrate the variable manner in which children with SCD may develop SNHL, and the difficulties associated with managing such cases. We recommend that children with SCD should undergo regular audiological assessment. Furthermore, clinicians should be aware of the risk of cochlear fibrosis and ossification and ensure prompt assessment following an acute vaso-occlusive crisis or unexplained vestibulocochlear event.

Cochlear implantation through intracochlear fibrosis: A comparison of surgical techniques.

OBJECTIVE: While the implications of ossification on cochlear implantation (CI) have been extensively described, there is a paucity of data regarding the fibrotic stage. We examined the outcomes of different insertion techniques for managing intracochlear fibrosis. STUDY DESIGN: Retrospective review of case series with case-control comparison. SETTING: University-based tertiary-referral otology-neurotology practice. PATIENTS: Between 2009 to 2020, 384 patients underwent CI. Of those, 7 patients (8 ears) demonstrated intracochlear fibrosis. INTERVENTIONS: CI performed 1-4 months following meningitis/labyrinthitis and 12-24 months after idiopathic sudden SNHL. Fibrosis removal (38%) or dilation (63%) permitted implantation. A styleted-electrode was used in 63% due to dense fibrosis. MAIN OUTCOME MEASURES: Postoperative audiometry with CI in place, additional comparisons with audiometric outcomes in age-matched controls. RESULTS: Full insertion achieved in all except one ear with partial ossification. Mean ipsilateral pure tone average (PTA) improved to 29 ± 15 dB and speech discrimination to 72 ± 28%. Fibrosis removal vs. dilation resulted in no PTA differences (p = 0.76). Poorest outcomes occurred with the longest time to surgery. CONCLUSIONS: Good CI audiologic outcomes in the setting of cochlear fibrosis can be achieved and are independent of technique. Instead, they vary with time to implantation. Every attempt should be made to intervene as early as possible.

Bilateral total deafness after preparation of wild boar meat.

INTRODUCTION: Streptococcus suis is responsible for a zoonosis for which Suidae (pigs and wild boars) constitute the reservoir, mainly in Asia, with a much lower prevalence in Europe. The predominant clinical manifestation is meningitis, possibly resulting in deafness. CASE REPORT: We report the case of a woman hospitalised for meningitis complicated by labyrinthitis, occurring several hours after preparing a meal composed of wild boar meat. Despite the presence of intracochlear fibrosis, the patient was managed by sequential bilateral cochlear implants. DISCUSSION: The discussion presents a review of the international literature and describes the mechanisms responsible for hearing loss related to this rare zoonosis.

Functional Loss After Meningitis-Evaluation of Vestibular Function in Patients With Postmeningitic Hearing Loss.

Introduction: The inner ear vestibular system is essential to balance function. Although hearing loss is well-described and quite common following meningitis, the literature evaluating vestibular function following meningitis is very limited. In particular, information on results of contemporary vestibular function tests, e.g., the video head impulse test (VHIT), is scarce. Using contemporary vestibular function tests, this study examines the vestibular function of patients with profound hearing loss (HL) after meningitis. Methods: Review of the literature and retrospective controlled study. Patients: Twenty-one consecutive patients with profound HL after meningitis (cochlear implant candidates) matched with 20 patients with profound HL of unknown etiology and examined during the period 2013-2018. Outcome Measure: Vestibular function loss, as evaluated with VHIT vestibulo-ocular reflex (VOR) gain, eye movement saccades, and cervical vestibular-evoked myogenic potentials (cVEMPs). The results of these tests were correlated to inner ear imaging findings (MRI/CT) and the level of hearing loss. Results: Mean VHIT gain was 0.48 in the meningitis group compared to 0.86 in the control group (p < 0.01). Saccades were present in 21 ears (62%) in the meningitis group compared to six ears (15%) among the controls (p < 0.01). cVEMP responses were present on five ears (18%) in the meningitis group and 25 ears (66%) in the control group (p < 0.01). Discussion: Postmeningitic hearing loss is associated with poor vestibular function, as evaluated by VHIT, saccades, and cVEMP. Loss of vestibular function correlates with the degree of hearing loss and inner ear imaging findings, although not in all cases. Vestibular function should be examined in patients surviving meningitis with hearing loss in order to individualize rehabilitation and improve balance outcome.

Factors Influencing Cochlear Patency after Translabyrinthine Surgery.

Objective To determine predictive factors for cochlear obliteration after translabyrinthine surgery for vestibular schwannoma. Study Design Case series with chart review. Setting Neurotology referral center. Subjects and Methods In total, 345 charts were reviewed, resulting in 103 patients who underwent translabyrinthine surgery between January 2010 and July 2015 and had postoperative magnetic resonance imaging (MRI) for review. Surveillance MRI performed after translabyrinthine resection of vestibular schwannomas was reviewed. Heavily T2-weighted MRI obtained an average of 21.8 months after surgery was reviewed to assess cochlear patency. Tumor size, preoperative audiograms, and MRI cochlear fluid-attenuated inversion recovery (FLAIR) intensity were compared between patients with retained cochlear patency and those without. Results Fifty-four percent of patients retained cochlear patency after translabyrinthine surgery. Tumor size did not differ statistically between the 2 groups. There was no statistically significant difference in speech reception thresholds, pure-tone average, or word recognition scores between patent and nonpatent groups. Preoperative MRI FLAIR intensity did not differ between groups. Conclusions More than half of patients retain cochlear patency after translabyrinthine vestibular schwannoma surgery. Cochlear patency is required for cochlear implant in patients with unilateral deafness. Preoperative tumor size, hearing performance, and intensity on MRI FLAIR do not predict cochlear patency. To prevent loss of opportunity for cochlear implantation, simultaneous implantation and cochlear lumen keeper placement are options.