Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis

2021 ◽  
pp. 000348942110059
Author(s):  
Özge Akdoğan ◽  
Smirnov Exilus ◽  
Bryan K. Ward ◽  
Justin C. McArthur ◽  
Charles C. Della Santina ◽  
...  
Keyword(s):  
Cochlear Implantation ◽  
Pure Tone ◽  
Relapsing Polychondritis ◽  
Sensorineural Hearing ◽  
High Dose ◽  
Left Auricle ◽  
Vestibular Loss ◽  
Patient Reported ◽  
Loss Of Hearing ◽  
Hearing Outcomes

Objectives: To report a case of profound bilateral sensorineural hearing and vestibular loss from relapsing polychondritis and hearing outcomes after cochlear implantation. Methods: Case report and literature review. Results: A 43 year-old woman developed sudden loss of hearing and balance that progressed over several weeks to bilateral, profound hearing and vestibular loss. Steroid treatments were ineffective. She underwent vestibular physical therapy and left cochlear implantation. About 10 months after her initial presentation, she developed erythema, warmth, swelling, and pain of the left auricle sparing the lobule, flattening of the bridge of her nose, and right ankle swelling, warmth, and skin erythema. A biopsy of the left auricle revealed histopathologic findings consistent with relapsing polychondritis. She was treated with high dose prednisolone. The ear inflammation resolved, however, despite excellent auditory response to pure tone thresholds, the patient reported no improvement in speech perception after cochlear implantation. Conclusions: Relapsing polychondritis can present with rapidly progressive, profound loss of hearing and vestibular function. Hearing outcomes after cochlear implantation can include poor speech discrimination despite good pure tone detection thresholds.

Sweet's disease and profound, bilateral, sensorineural hearing loss

2009 ◽  
Vol 124 (1) ◽  
pp. 105-107 ◽  
Author(s):  
S Cheng ◽  
M da Cruz
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Definitive Diagnosis ◽  
Sensorineural Hearing ◽  
Immune Mediated ◽  
Inner Ear Disease ◽  
Bilateral Sensorineural Hearing Loss ◽  
Loss Of Hearing ◽  
Hearing Outcomes

AbstractObjective:We report a case of Sweet's disease associated with rapid, profound loss of hearing, against a background of progressive, bilateral, sensorineural hearing loss.Results:The clinical features were indistinguishable from those of immune-mediated inner ear disease. Establishment of a definitive diagnosis was a challenge due to the absence of a reliable diagnostic test. The patient was unresponsive to extensive immunosuppressive therapy and subsequently underwent cochlear implantation, with good hearing outcomes.Conclusions:Profound, bilateral, sensorineural hearing loss in the context of Sweet's disease may be related to the underlying immunological aetiology. Cochlear implantation can successfully restore hearing when immunotherapy fails.

Progressive, reversible sensorineural hearing loss caused by azathioprine

2014 ◽  
Vol 128 (9) ◽  
pp. 838-840 ◽  
Author(s):  
P W Jenkinson ◽  
M I Syed ◽  
L Mcclymont
Keyword(s):  
Adverse Effect ◽  
Hearing Loss ◽  
Case Report ◽  
Sensorineural Hearing Loss ◽  
Pure Tone ◽  
Early Recognition ◽  
Pure Tone Audiometry ◽  
Sensorineural Hearing ◽  
Patient Reported ◽  
Clinical Awareness

AbstractObjective:This paper describes the first reported case of progressive sensorineural hearing loss caused by azathioprine, which was reversed on stoppage of the drug.Case report:A female patient with previously normal hearing presented with progressive sensorineural hearing loss after being started on azathioprine. Otological and neurological examination findings were unremarkable. After stopping the drug, the patient reported an improvement in hearing, which was confirmed on pure tone audiometry.Conclusion:This previously unreported side effect of azathioprine is highlighted in order to increase clinical awareness. Early recognition of this adverse effect is important to minimise the possibility of permanent sensorineural hearing loss.

Assessing ototoxicity and patients’ satisfaction of topical local anaesthesia in myringotomy and ventilation tube insertion

2019 ◽  
Vol 133 (4) ◽  
pp. 285-288
Author(s):  
S Marzouk ◽  
A Al-lami ◽  
R Sykes ◽  
C Neumann ◽  
I J Nixon
Keyword(s):  
Local Anaesthesia ◽  
Pure Tone ◽  
Animal Studies ◽  
Bone Conduction ◽  
Sensorineural Hearing ◽  
Ventilation Tube ◽  
Tube Insertion ◽  
Pure Tone Average ◽  
Patients Satisfaction ◽  
Hearing Outcomes

AbstractObjectivesAnimal studies have suggested that exposure of the middle ear to topical local anaesthesia may be ototoxic. This study aimed to report sensorineural hearing outcomes and patients’ satisfaction in those who underwent myringotomy and ventilation tube insertion using topical local anaesthesia.MethodsTwenty-nine patients (32 ears) were operated on. Pre- and post-operative audiology findings were compared. A Likert-type questionnaire on treatment satisfaction was completed at the end of the procedure.ResultsMedian patient age was 55 years (range, 27–88 years). Pre- and post-operative bone conduction pure tone averages were 26.76 dB and 25.26 dB respectively (mean reduction of −1.22 dB, 95 per cent confidence interval of −5.91 to 8.13 dB; p = 0.7538). One ear (3 per cent) had a reduction in pure tone average of 10 dB.ConclusionThe results suggest that sensorineural hearing loss is not a complication of ear exposure to topical local anaesthesia during myringotomy and ventilation tube insertion. The procedure was well perceived.

scholarly journals Comparison of Hearing Outcomes of Systemic Steroid Monotherapy and Intratympanic Steroid Combined Treatment in Idiopathic Sudden Sensorineural Hearing Loss

2021 ◽  
Vol 64 (3) ◽  
pp. 155-160
Author(s):  
Kyungil Jang ◽  
So Yun Lim ◽  
Eun-Ju Jeon ◽  
Hyun Jin Lee
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Pure Tone ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Systemic Steroid ◽  
Partial Recovery ◽  
Simultaneous Group ◽  
Hearing Outcomes

Background and Objectives Steroid treatment is used as a main treatment modality for sudden sensorineural hearing loss (SSNHL). Intratympanic injection of steroid (ITS) has been used and its therapeutic efficacy reported as being comparable to the systemic steroid administration (SS). This study compares the hearing outcomes of using ITS and SS simultaneously and SS alone.Subjects and Method Retrospective chart review was performed for 146 patients with SSNHL. The patients were divided into 2 groups according to the method of treatment (SS and ITS simultaneously vs. SS alone). The inclusion criteria were starting treatment within 7 days after onset, and follow up pure tone audiometry at least 4 weeks after treatment. Hearing gain for pure tone threshold of each frequency and average of 4 frequencies (500 Hz, 1000 Hz, 2000 Hz, and 4000 Hz) were compared between both groups.Results The improvement in PTA at 1-month follow-up was 27.3±20.0 dB HL in the simultaneous group and 19.1±19.5 dB HL in the SS alone group; this was not statistically significant. Complete or partial recovery at 1-month follow-up was observed in 65.3% of the simultaneous group and 69.6% of the SS alone group; this was also not significant.Conclusion There was no significant difference in hearing outcomes between the simultaneous and SS alone group. The simultaneous therapy does not appear to be superior to the SS alone therapy. Further studies using more population and longer follow-up periods are necessary.

scholarly journals 1136 Cochlear Implant Outcomes in Patients with Otosclerosis: A Systematic Review

2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
C M Lam ◽  
H L Cornwall ◽  
A Chaudhry ◽  
J Muzaffar ◽  
M Bance ◽  
...  
Keyword(s):  
Systematic Review ◽  
Cochlear Implantation ◽  
Case Reports ◽  
Meta Analysis ◽  
Case Series ◽  
Significant Heterogeneity ◽  
Patient Counselling ◽  
Study Objective ◽  
Patient Reported ◽  
Hearing Outcomes

Abstract Introduction In otosclerosis, spongiotic bone replaces normal bone in the ossicular chain. Focal deposits may also be found within the cochlea leading to sensorineural hearing loss refractory to conventional treatment. Cochlear implantation can play an important role in the management of otosclerosis in these patients. Our study objective is to establish hearing outcomes following cochlear implantation in patients with otosclerosis. Method Systematic review and narrative synthesis. Databases searched: Medline, PubMed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Review conducted in accordance with the PRISMA statement. Results Searches identified 68 studies meeting the inclusion criteria and reporting outcomes in a minimum of 481 patients with at least 516 implants. The methodological quality of included studies was modest, predominantly consisting of case reports and non-controlled case series with small numbers of patients. Significant heterogeneity existed in terms of outcomes and methods of reporting, which precluded a meta-analysis. Access to good rehabilitation support is essential to achieving the good hearing outcomes and Patient Reported Outcome Measures that can be expected by 12 months post-implantation in most cases. There was significant association between the radiological severity of otosclerosis and an increase in surgical and post-operative complications. Post-operative facial nerve stimulation can occur and may require deactivation of electrodes and subsequent hearing detriment. Conclusions Hearing outcomes are typically good, but patients should be counselled on associated surgical complications which may compromise hearing. Modern diagnostic techniques may help to identify potentially difficult cases to aid operative planning and patient counselling.

scholarly journals Idiopathic Sudden Sensorineural Hearing Loss: Should Otoacoustic Emissions Be Added to the Monitoring Protocol? A Systematic Review

2020 ◽  
Vol 10 (1) ◽  
pp. 326
Author(s):  
Kaley Babich ◽  
Kathleen T. Dunckley
Keyword(s):  
Systematic Review ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Otoacoustic Emissions ◽  
Sudden Sensorineural Hearing Loss ◽  
Outer Hair Cells ◽  
Sensorineural Hearing ◽  
Initial Onset ◽  
Loss Of Hearing ◽  
Hearing Outcomes

Idiopathic sudden sensorineural hearing loss (ISSNHL) refers to a loss of hearing, most commonly unilateral, that occurs suddenly (≤72 h) with no identifiable cause or etiology. To date, there is no standard protocol to predict prognosis (hearing recovery) for patients with ISSNHL. However, studies have shown that changes in otoacoustic emissions (OAEs) often occur prior to changes in audiometric hearing thresholds. OAEs originate from the electrochemical motility of the outer hair cells (OHC) and reflect the integrity of the inner ear, specifically the cochlear amplifier. Therefore, OAEs may be useful as a prognostic predictive factor in patients with ISSNHL from the initial onset of symptoms through recovery. A systematic review of the literature was undertaken to assess the relationship between pure tone thresholds, OAEs, and subjective hearing improvement and/or recovery. Fourteen studies were identified for inclusion, and they overwhelmingly support the inclusion of OAEs in the protocol to monitor ISSNHL recovery. This finding supports the development of a standard diagnostic protocol that includes OAEs to predict patient hearing outcomes.

scholarly journals Sudden Hearing Loss Treated With Hyperbaric Oxygen (HBO) – Case Study

2015 ◽  
Vol 51 (2) ◽  
pp. 7-9 ◽  
Author(s):  
Michał Fal ◽  
Jacek Piechocki ◽  
Monika Morawska-Kochman ◽  
Maria Michałkiewicz ◽  
Tomasz Kręcicki
Keyword(s):  
Hyperbaric Oxygen ◽  
Pure Tone ◽  
Pure Tone Audiometry ◽  
Sudden Hearing Loss ◽  
Sudden Loss ◽  
Patient Reported ◽  
Noticeable Improvement ◽  
Hyperbaric Therapy ◽  
Loss Of Hearing

Abstract The article presents a case study of a 32-year-old patient admitted to the Mazowia Hyperbaric Therapy Centre due to a sudden loss of hearing. In an interview the patient reported an abrupt hearing deterioration in his right ear and the feeling of congestion in the left ear with bilateral tinnitus present for over 2 months. The patient was qualified to undergo hyperbaric oxygen therapy (HBO). After 15 treatment sessions in a pressure chamber a noticeable improvement of hearing in his right-ear was noted (proved by pure tone audiometry) and subjective hearing upswing in his left ear. The bibliography includes the articles and references concerning the time limits of HBO therapy and its effectiveness in the treatment of sudden deafness.

scholarly journals Cochlear Implantation Following Explorative Tympanotomy in Patients With Sudden Sensorineural Hearing Loss: Surgical Features and Audiological Outcomes

2021 ◽  
pp. 014556132110091
Author(s):  
Robin Rupp ◽  
Joachim Hornung ◽  
Matthias Balk ◽  
Matti Sievert ◽  
Sarina Müller ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Connective Tissue ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Round Window ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Round Window Membrane ◽  
Hearing Results ◽  
Round Window Niche

Objective: To investigate the anatomical status of the round window niche and hearing outcome of cochlear implantation (CI) after explorative tympanotomy (ExT) with sealing of the round window membrane in patients with sudden sensorineural hearing loss at a tertiary referral medical center. Methods: Between January 1, 2007, and July 30, 2020, 1602 patients underwent CI at our department. Out of these, all patients previously treated by ExT with sealing of the round window membrane because of unilateral sudden hearing loss were included in the study. A retrospective chart review was conducted concerning method of round window membrane sealing, intraoperative findings during CI, postoperative imaging, and hearing results. Results: Twenty one patients (9 females; 8 right ears; 54.3 years [± 12.9 years]) underwent ExT with sealing of the round window membrane with subsequent CI after 26.6 months (± 32.9 mo) on average. During CI, in 76% of cases (n = 16), the round window niche was blocked by connective tissue due to the previous intervention but could be removed completely in all cases. The connective tissue itself and its removal had no detrimental effects on the round window membrane. Postoperative computed tomography scan showed no electrode dislocation. Mean postoperative word recognition score after 3 months was 57.4% (± 17.2%) and improved significantly to 73.1% (± 16.4%, P = .005) after 2 years. Conclusion: Performing CI after preceding ExT, connective tissue has to be expected blocking the round window niche. Remaining tissue can be removed safely and does not alter the round window membrane allowing for a proper electrode insertion. Short- and long-term hearing results are satisfactory. Consequently, ExT with sealing of the round window membrane in patients with sudden sensorineural hearing loss does not impede subsequent CI that can still be performed safely.

Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

2013 ◽  
Vol 127 (7) ◽  
pp. 708-711 ◽  
Author(s):  
A C Hall ◽  
A C Leong ◽  
D Jiang ◽  
A Fitzgerald-O'Connor
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Skin Lesions ◽  
Sudden Hearing Loss ◽  
Sudden Onset ◽  
Sensorineural Hearing ◽  
Urticarial Vasculitis ◽  
Wells Syndrome ◽  
Bilateral Sensorineural Hearing Loss

AbstractBackground:Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle–Wells syndrome. Previous reports have described the hearing loss to be progressive in nature.Method:To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle–Wells syndrome.Results:The patient underwent a cochlear implantation with a modest outcome.Conclusion:Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

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