scholarly journals The Successful Use of Infliximab in a Relapsing Case of Susac’s Syndrome

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Suran L. Fernando ◽  
Therese Boyle ◽  
Annika Smith ◽  
John D.E. Parratt
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Early Recognition ◽  
Delayed Diagnosis ◽  
Sensorineural Hearing ◽  
High Dose ◽  
Susac’S Syndrome ◽  
First Case ◽  
Retinal Artery ◽  
Bilateral Sensorineural Hearing Loss

Susac’s syndrome is a rare and debilitating disease characterized by the triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. All manifestations may not be clinically apparent at presentation resulting in delayed diagnosis. Early recognition of the syndrome may prevent disease sequelae such as permanent cognitive, visual, and hearing loss. We present such a case of Susac’s syndrome that was also refractory to conventionally prescribed combination of immunosuppressive treatments including high-dose potent corticosteroids, intravenous cyclophosphamide, methotrexate, plasma exchange, rituximab, and mycophenolate. His disease was stabilized with infliximab in combination with a tapering course of low-dose prednisone. After 2 years of remission with TNF treatment, consideration is being given to ceasing therapy. He has the sequelae of bilateral sensorineural hearing loss but no visual impairment or cognitive deficits on follow-up with neuropsychometric testing. This is the first case report to our knowledge of the successful use of infliximab for a patient with Susac’s syndrome that was necessary following treatment with cyclophosphamide and then rituximab.

Susac's syndrome: a rare cause of fluctuating sensorineural hearing loss

1997 ◽  
Vol 111 (11) ◽  
pp. 1072-1074 ◽  
Author(s):  
Neil D. Bateman ◽  
Ian J. M. Johnson ◽  
Kevin P. Gibbin
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Sensorineural Hearing ◽  
Clinical Severity ◽  
Susac’S Syndrome ◽  
Retinal Artery ◽  
Clinical Triad ◽  
First Time

AbstractSusac's syndrome is extremely rare and is characterized by the clinical triad of encephalopathy, retinal artery occlusion and deafness. A case of this rare syndrome is presented and for the first time the fluctuating nature of the hearing loss is demonstrated. The site and nature of the hearing loss has been investigated. The value of neural hearing loss as a method of monitoring the clinical severity is discussed.

Case of neuromyelitis optica: bilateral sensorineural hearing loss and transverse myelopathy following intrathecal chemotherapy

2020 ◽  
Vol 13 (4) ◽  
pp. e234076 ◽  
Author(s):  
Sergio A Castillo-Torres ◽  
Carlos A Soto-Rincón ◽  
Héctor J Villarreal-Montemayor ◽  
Beatriz Chávez-Luévanos
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Transverse Myelitis ◽  
Intrathecal Chemotherapy ◽  
Sensorineural Hearing ◽  
Intrathecal Methotrexate ◽  
High Dose ◽  
Chemotherapeutic Drugs ◽  
Acute Transverse Myelitis ◽  
Bilateral Sensorineural Hearing Loss

Neurotoxicity from intrathecally administered chemotherapeutic drugs is frequent, particularly with some agents like methotrexate, which are more prone to developing adverse effects. Myelopathy ranks among the most frequently reported neurological entities; with the diagnosis being straightforward, after ruling out infectious, metabolic, autoimmune or paraneoplastic causes. Scarcity of cases precludes evidence-based recommendations for the management of these complications. The most common therapeutic approach consists of the suspension of chemotherapy, exclusion of infectious and neoplastic causes, with prompt administration of high-dose steroids. We report a 21-year-old patient with acute lymphoblastic leukaemia, who developed acute transverse myelitis and bilateral sensorineural hearing loss, after five rounds of intrathecal methotrexate and cytarabine. Although neurotoxicity from both agents has been documented, this combination has not been previously reported.

Sudden Bilateral Sensorineural Hearing Loss following Polysubstance Narcotic Overdose

2011 ◽  
Vol 22 (04) ◽  
pp. 208-214 ◽  
Author(s):  
Vanessa G. Schweitzer ◽  
Ilaaf Darrat ◽  
Brad A. Stach ◽  
Elizabeth Gray
Keyword(s):  
Substance Abuse ◽  
Hearing Loss ◽  
Case Report ◽  
Drug Abuse ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
High Dose ◽  
Recreational Drug ◽  
Brainstem Response ◽  
Bilateral Sensorineural Hearing Loss

Background: Auditory disorders associated with substance abuse are rare. Hearing loss secondary to heroin and hydrocodone abuse has been described variously as not always responsive to steroid management, as not always reversible, and in some cases, as nonresponsive profound sensorineural hearing loss requiring cochlear implantation. We present a case of a teenager with sudden-onset moderate to severe bilateral sensorineural hearing loss after documented polysubstance “binging.” The hearing loss improved substantially after high-dose steroid and vasoactive therapy. Purpose: The purpose of this report is to describe the hearing disorder of a patient who had awakened with a bilateral severe hearing loss following a night of recreational drug abuse. Research Design: Case report and review of the literature. Data Collection and Analysis: The subject of this report is an 18-yr-old patient with a history of substance abuse. Data collected were magnetic resonance /computed tomography brain imaging; metabolic, infectious disease, and autoimmune evaluation; and extensive audiologic evaluation, including pure-tone and speech audiometry, immittance measures, distortion-product otoacoustic emissions, and auditory brainstem response testing. Serial audiograms were collected for 10 mo following the onset of symptoms. Results: Two days of polysubstance abuse (heroin, benzodiazepine, alcohol, and crack [smoked cocaine]) resulted in moderately severe sensorineural hearing loss bilaterally. The loss responded to a 1 mo course of high-dose prednisone and a 10 mo course of pentoxifylline. Hearing sensitivity subsequently improved, leaving only residual high-frequency sensorineural hearing loss. Conclusions: This case report highlights the importance of “recreational” drug abuse in the evaluation of sudden hearing loss. Potential etiologies include altered pharmacokinetics, vascular spasm/ischemia, encephalopathy, acute intralabyrinthine hemorrhage, and genetic polymorphisms of drug-metabolizing enzymes.

Bilateral Sensorineural Hearing Loss in the Setting of Acute Methamphetamine Overdose

2021 ◽  
pp. 014556132110693
Author(s):  
Jeremy S. Reich ◽  
Harleen K. Sethi ◽  
Robert T. Sataloff
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Kidney Injury ◽  
Sensorineural Hearing ◽  
High Dose ◽  
Profound Hearing Loss ◽  
Cochlear Blood Flow ◽  
Methamphetamine Use ◽  
Bilateral Sensorineural Hearing Loss ◽  
The Right

Substance abuse is a rare but known cause of sensorineural hearing loss (SNHL). We report a case of acute SNHL in a 28-year-old man following an overdose of methamphetamine and incidental ingestion of fentanyl. On initial encounter, he had moderate-to-severe hearing loss in the right ear and severe-to-profound hearing loss in the left ear in addition to acute kidney injury, liver failure, and lactic acidosis. The patient was treated with a two-week course of high-dose steroids and expressed a subjective improvement in hearing. This case highlights the importance of auditory testing following a drug overdose and is one of the only documented cases of hearing loss following methamphetamine use in recent years. There is a paucity of literature regarding the mechanism causing acute SNHL secondary to methamphetamines. Proposed etiologies include neurotransmitter depletion or reduced cochlear blood flow as possible causes of ototoxicity.

Biological age and rates of aging of patients with vibration disease and bilateral sensorineural hearing loss

2020 ◽  
pp. 624-624
Author(s):  
S. I. Ereniev ◽  
O. V. Plotnikova
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Biological Age ◽  
Biological Aging ◽  
Vibration Disease ◽  
Bilateral Sensorineural Hearing Loss

Biological age and rates of aging of patients with vibration disease and bilateral sensorineural hearing loss were studied. The biological age of patients exceeded the calendar age by an average of 7.36±0.36 years and the proper biological age by 10.79±0.72 years. The rate of biological aging of the examined patients was 1.14±0.08 times higher than the rate of aging of their healthy peers.

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.

scholarly journals Comprehensive medical evaluation of pediatric bilateral sensorineural hearing loss

2021 ◽  
Author(s):  
Suat Kılıç ◽  
Malek H. Bouzaher ◽  
Michael S. Cohen ◽  
Judith E. C. Lieu ◽  
Margaret Kenna ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Medical Evaluation ◽  
Bilateral Sensorineural Hearing Loss

Is simultaneous bilateral mastoidectomy ever advisable?

2007 ◽  
Vol 121 (11) ◽  
pp. 1041-1047 ◽  
Author(s):  
J J Klemens ◽  
E Mhoon ◽  
M Redleaf
Keyword(s):  
Hearing Loss ◽  
Decision Tree ◽  
Sensorineural Hearing Loss ◽  
Chart Review ◽  
Historical Data ◽  
Sensorineural Hearing ◽  
Opinion Survey ◽  
Potential Patient ◽  
Simultaneous Procedures ◽  
Bilateral Sensorineural Hearing Loss

AbstractIntroduction:We report our experience with bilateral, simultaneous tympanomastoidectomies and the results of an opinion survey of otologists.Methods:A chart review of 116 tympanomastoidectomies revealed 12 patients who underwent bilateral, simultaneous tympanomastoidectomies. An opinion survey generated 121 responses.Results:Of the 12 patients, none suffered any outcome which would have been avoided by staging the procedures. Twenty-three of 24 operated ears had the same or better hearing post-operatively. Of the survey respondents, 74 felt that performing bilateral, simultaneous tympanomastoidectomies was unsafe, largely because of the risk of bilateral sensorineural hearing loss.Discussion:Although bilateral, simultaneous tympanomastoidectomies carry double the risk of unilateral sensorineural hearing loss, compared with the unilateral procedure, the risk of bilateral sensorineural hearing loss is only 0.006–0.2 per cent, as derived mathematically from historical data. Respondents to the survey were mostly opposed to bilateral, simultaneous tympanomastoidectomies, but even those opposed gave indications for simultaneous procedures. This finding probably reflects an ambivalence about the theoretical risks of the operation versus the potential patient benefits. A decision tree for proceeding to the second case is presented.

Cochlear nerve anomalies in paediatric single-sided deafness – prevalence and implications for cochlear implantation strategies

2020 ◽  
pp. 1-4
Author(s):  
K Pollaers ◽  
A Thompson ◽  
J Kuthubutheen
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Sensorineural Hearing Loss ◽  
Cochlear Nerve ◽  
Sensorineural Hearing ◽  
Resonance Imaging ◽  
Bilateral Sensorineural Hearing Loss ◽  
Single Sided Deafness ◽  
Cochlear Nerve Hypoplasia

Abstract Objective To determine the prevalence of cochlear nerve anomalies on magnetic resonance imaging in patients with unilateral or bilateral sensorineural hearing loss. Methods A retrospective case series was conducted at a tertiary referral centre. The inclusion criteria were paediatric patients with bilateral or unilateral sensorineural hearing loss, investigated with magnetic resonance imaging. The primary outcome measure was the rate of cochlear nerve hypoplasia or aplasia. Results Of the 72 patients with unilateral sensorineural hearing loss, 39 per cent (28 cases) had absent or hypoplastic cochlear nerves on the affected side. Fifteen per cent (11 cases) had other abnormal findings on magnetic resonance imaging. Eighty-four patients had bilateral sensorineural hearing loss, of which cochlear nerve hypoplasia or aplasia was identified only in 5 per cent (four cases). Other abnormal findings were identified in 14 per cent (12 cases). Conclusion Paediatric patients with unilateral sensorineural hearing loss are more likely to have cochlear nerve anomalies than those patients with bilateral sensorineural hearing loss. This has important implications regarding cochlear implantation for patients with single-sided deafness.

Bilateral profound sudden sensorineural hearing loss presenting a diagnostic conundrum in a child with sickle cell anaemia

2008 ◽  
Vol 123 (7) ◽  
pp. 811-816 ◽  
Author(s):  
A D Mace ◽  
M S Ferguson ◽  
M Offer ◽  
K Ghufoor ◽  
M J Wareing
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Sensorineural Hearing Loss ◽  
Sickle Cell ◽  
Cochlear Implantation ◽  
World Literature ◽  
Sickle Cell Anaemia ◽  
Early Recognition ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing

AbstractObjective:To present the first published case of a child with bilateral profound sudden sensorineural hearing loss found in association with sickle cell anaemia, and to demonstrate the importance of early recognition, investigation and empirical treatment of sudden sensorineural hearing loss.Method:Case report and review of world literature.Case report:The authors present the case of a seven-year-old child with known sickle cell anaemia, who presented with bilateral profound sensorineural hearing loss developing over a period of five days. There was a history of ophthalmological disease in the preceding weeks, and inflammatory markers were raised. The differential diagnosis included a vaso-occlusive or inflammatory aetiology such as Cogan's syndrome, and treatment for both was instigated. Hearing thresholds did not recover, and the patient underwent cochlear implantation 12 weeks later.Conclusion:Sudden sensorineural hearing loss has a variable aetiology and is rare in children. Immediate treatment for all possible aetiologies is essential, along with targeted investigations and early referral for cochlear implantation if no recovery is demonstrated.

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