Cochlear implantation in Branchiootorenal syndrome - case report and review of the literature.

INTRODUCTION: Branchiootorenal syndrome (BOR) manifests with branchial fistulae, otological anomalies and renal dysplasia. Management remains largely symptomatic. The most common consequence of BOR is mild-to-profound sensorineural, conductive or mixed hearing loss, where conventional hearing aids (HA) provide limited benefit. Cochlear implantation for BOR-associated hearing loss is an emerging treatment. Existing literature remains limited, centring around paediatric cases. OBJECTIVES: This paper is the first to present a case of an adult/non-paediatric BOR patient receiving bilateral cochlear implants (CI), including pre- and postoperative objective and subjective hearing outcomes. Additionally, an overview of reported cases of cochlear implantation in BOR/BO patients is given. METHODS: A review of the case patient's medical charts from initial visit at our tertiary center to latest follow-up was conducted. A search of the Medline database resulted in four papers on cochlear implantation in BOR/BO patients, forming the basis of a literature review on this topic. RESULTS: Excellent hearing outcomes were achieved after bilateral CI despite the patient's inner ear malformation. DISCUSSION: The case patient's outcomes were comparable to results reported for unselected adult, non-syndromic CI-recipients with normal inner ear anatomy. CONCLUSION: BOR patients experiencing insufficient benefit from conventional HA should be considered for cochlear implantation.

Cochlear Implantation After Radiotherapy of Vestibular Schwannomas.

BACKGROUND AND OBJECTIVES: Vestibular schwannomas (VS) frequently lead to ipsilateral sensorineural hearing loss (HL) as part of its natural history or as a result of treatment. Cochlear implantation represents a well-documented treatment of profound HL that cannot be treated adequately with a conventional hearing aid, thus being offered to selected VS patients. A functional cochlea and cochlear nerve are prerequisites for sound perception with a cochlear implant (CI). The potential impact of radiotherapy on these structures is thus an important issue for subsequent CI hearing outcomes. The objective of this article is to present a case and to review the existing literature on the outcomes of cochlear implantation in irradiated VS patients systematically. METHODS: A systematic literature review using preferred reporting items for systematic reviews and meta-analyses was conducted. Medline was searched systematically. Papers reporting ipsilateral CI outcomes after radiotherapy of VS were included. Additionally, results of CI after stereotactic radiotherapy in a 54-year-old male with neurofibromatosis type 2 are presented. RESULTS: A total of 14 papers (33 patients) fulfilled inclusion criteria. Moderate preoperative HL was found in 11 patients. Six had moderate to severe HL, whereas 16 had severe HL or total deafness. Postoperative hearing outcomes varied from poor in 27% of patients to excellent in 19%, with remaining cases lying in between (mean follow-up of 19 months). Most patients achieved improvement in hearing and quality of life. CONCLUSION: Despite variation in the degree of hearing outcome, CI after radiotherapy of VS appears to be effective in the majority of cases, as more than 70% of patients have good or excellent outcomes within 1-2 years post-implantation. Subjective benefits are considerable, even in cases with relatively poor objective outcome.

Long-Term Vestibular Outcomes in Cochlear Implant Recipients.

Background: Vestibular dysfunction is likely the most common complication to cochlear implantation (CI) and may, in rare cases, result in persistent severe vertigo. Literature on long-term vestibular outcomes is scarce. Objective: This paper aims to evaluate vestibular dysfunction before and after cochlear implantation, the long-term vestibular outcomes, and follows up on previous findings of 35 consecutive adult cochlear implantations evaluated by a battery of vestibular tests. Methods: A prospective observational longitudinal cohort study was conducted on 35 CI recipients implanted between 2018 and 2019; last follow-up was conducted in 2021. At the CI work-up (T0) and two postoperative follow-ups (T1 and T2), 4 and 14 months following implantation, respectively, all patients had their vestibular function evaluated. Evaluation with a vestibular test battery, involving video head impulse test (vHIT), cervical vestibular evoked myogenic potentials (cVEMP), caloric irrigation test, and dizziness handicap inventory (DHI), were performed at all evaluations. Results: vHIT testing showed that 3 of 35 ears had abnormal vHIT gain preoperatively, which increased insignificantly to 4 of 35 at the last follow-up (p = 0.651). The mean gain in implanted ears decreased insignificantly from 0.93 to 0.89 (p = 0.164) from T0 to T2. Preoperatively, 3 CI ears had correction saccades, which increased to 11 at T2 (p = 0.017). Mean unilateral weakness increased from 19 to 40% from T0 to T2 (p < 0.005), and the total number of patients with either hypofunctioning or areflexic semicircular canals increased significantly from 7 to 17 (p < 0.005). Twenty-nine percent of CI ears showed cVEMP responses at T0, which decreased to 14% (p = 0.148) at T2. DHI total mean scores increased slightly from 10.9 to 12.8 from T0 to T1 and remained at 13.0 at T2 (p = 0.368). DHI scores worsened in 6 of 27 patients and improved in 4 of 27 subjects from T0 to T2. Conclusion: This study reports significant deterioration in vestibular function 14 months after cochlear implantation, in a wide range of vestibular tests. vHIT, caloric irrigation, and cVEMP all measured an overall worsening of vestibular function at short-term postoperative follow-up. No significant deterioration or improvement was measured at the last postoperative follow-up; thus, vestibular outcomes reached a plateau. Despite vestibular dysfunction, most of the patients report less or unchanged vestibular symptoms.

Objective Vestibular Test Battery and Patient Reported Outcomes in Cochlear Implant Recipients.

OBJECTIVE: Cochlear implantation (CI) may have undesired effects on the vestibular apparatus. However, the literature holds no consensus on vestibular affection and the testing tools applied to test for vestibular dysfunction after cochlear implantation are inconsistent. We aimed to investigate the impact of CI on vestibular function by an extensive test battery including patient-reported outcomes. STUDY DESIGN: Prospective observational study. SETTING: University hospital. PATIENTS: Forty adult unilateral first-time CI recipients. INTERVENTION: Vestibular function was evaluated pre- and post-implantation with the video head impulse test (VHIT), the caloric test and cervical vestibular evoked myogenic potentials (cVEMPs), and the patient-reported dizziness handicap inventory (DHI). RESULTS: Mean VHIT gain decreased from preoperative 0.92 to 0.84 postoperative (p = 0.018); mean caloric unilateral weakness increased from 20.5% preoperative to 42.9% postoperative (p < 0.0001); cVEMP responses were present on 10 operated ears preoperative and five ears postoperative, and compared with non-implanted ears, cVEMP responses on implanted ears were impaired (p = 0.023). 50% of patients reported early postoperative dizziness, but the mean DHI score remained unchanged (p = 0.94). The DHI scores correlated poorly with the objective outcomes (rs = 0.19 and rs = -0.22). CONCLUSION: Vestibular function is significantly affected after cochlear implantation, but vestibular hypofunction varies with the test used. Although early dizziness after implantation is common, later DHI scores are not significantly higher than before the implantation, indicating that central compensation plays a major role for these patients.