scholarly journals Granulomatosis with Polyangiitis as a Cause of Sudden-Onset Bilateral Sensorineural Hearing Loss: Case Report and Recommendations for Initial Assessment

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Paul R. Ratmeyer ◽  
Benjamin R. Johnson ◽  
Luis P. Roldan ◽  
Tania L. Kraai
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Granulomatosis With Polyangiitis ◽  
Early Recognition ◽  
Sensorineural Hearing ◽  
Initial Assessment ◽  
Laboratory Evaluation ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

Granulomatosis with polyangiitis (GPA) is a severe systemic vasculitis that commonly affects the paranasal sinuses, upper and lower respiratory tracts, and kidneys. GPA has also been associated with sensorineural hearing loss (SNHL), through inflammation of the cochlear apparatus. Early recognition, diagnostic laboratory evaluation, and appropriate treatment are essential to improve outcomes and achieve remission for patients with GPA. Here, we present a case of bilateral sudden sensorineural hearing loss (SSNHL) and distal symmetric polyneuropathy as the first presenting signs of GPA. A specific diagnostic work-up to rule out autoimmune inner-ear disease in patients with bilateral SSNHL is not clearly stated in the clinical practice guidelines from the American Academy of Otolaryngology-Head and Neck Surgery. The aim of this paper is to delineate an appropriate diagnostic work-up for patients with bilateral SSNHL when there is concern for autoimmune disease.

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.

scholarly journals Audit of the Aetiological Investigations Performed in Children with Sensorineural Hearing Loss At Salford

2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Saud K AlHajeri ◽  
Dr Mohammed Iqbal
Keyword(s):  
Hearing Loss ◽  
Genetic Testing ◽  
Sensorineural Hearing Loss ◽  
Genetic Counselling ◽  
Gold Standard ◽  
Sensorineural Hearing ◽  
British Association ◽  
Hearing Tests ◽  
Work Up

Objective: This project aims to look at the Audiovestibular Physician’s practice at Salford and how closely it aligns with the gold standard guidelines set in the protocol lately published by the British Association of Audiological Physicians. Method: An audit was done retrospectively on 20 patients suffering from sensorineural hearing loss. As such, patient notes were utilised to ascertain which aetiological investigations have been completed and which were not. Any inadequacy in the aetiological work up has been dissected to help know the underlying reasons. Results: All patients had a thorough history taken and were comprehensively physically examined. 95% of patients underwent imaging in the form of MRI/CT. 80% received CMV testing. 75% underwent ECG testing. 60% received family hearing tests. Only 35% had ophthalmology examinations and 25% underwent urine and genetic testing. Conclusion: In some cases, the low compliance rates were due to the Audiovestibular Physician not ordering the investigation as part of the aetiological work up. This could be improved with the use of a dedicated checklist to act as an aid to the physician. Moreover, genetic counselling has been proposed to attempt to boost the compliance rates with genetic testing and similarly, leaflets briefing patients’ families about the importance of undergoing hearing tests themselves is another promising proposition to help improve the adherence

scholarly journals A Structured Assessment to Decrease the Amount of Inconclusive Endometrial Biopsies in Women with Postmenopausal Bleeding

2016 ◽  
Vol 2016 ◽  
pp. 1-5
Author(s):  
M. C. Breijer ◽  
N. C. M. Visser ◽  
N. van Hanegem ◽  
A. A. van der Wurff ◽  
B. C. Opmeer ◽  
...  
Keyword(s):  
Retrospective Cohort ◽  
Endometrial Hyperplasia ◽  
Outcome Measure ◽  
Endometrial Biopsy ◽  
Initial Assessment ◽  
Postmenopausal Bleeding ◽  
Pathology Laboratory ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

Objective.To determine whether structured assessment of outpatient endometrial biopsies decreases the number of inconclusive samples.Design.Retrospective cohort study.Setting.Single hospital pathology laboratory.Population.Endometrial biopsy samples of 66 women with postmenopausal bleeding, collected during the usual diagnostic work-up and assessed as insufficient for a reliable histological diagnosis.Methods.Endometrial biopsy samples were requested from the pathology laboratories. The retrieved samples were systematically reassessed by a single pathologist specialized in gynecology.Main Outcome Measure.Disagreement between initial assessment and conclusion after structured reassessment.Results.We retrieved 36 of 66 endometrial biopsy samples from six different pathology laboratories. Structured reassessment of the retrieved samples by a single pathologist specialized in gynecology did not change the conclusion in 35 of the 36 samples. The remaining sample contained a large amount of endometrial tissue and the diagnosis at reassessment was endometrial hyperplasia without atypia. All other samples contained insufficient material for a reliable diagnosis.Conclusion.A structured reassessment of endometrial biopsies samples, which were classified as inconclusive due to insufficient material, did not change the conclusion. Although it might be helpful for pathologists to have diagnostic criteria for adequacy and/or inadequacy of an endometrial biopsy sample, the gain in efficiency is likely to be small.

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.

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.

scholarly journals Sensorineural hearing loss and status epilepticus associated with ulcerative colitis: Is there enough evidence to support immune-related mechanisms?

2015 ◽  
Vol 6 (01) ◽  
pp. 077-079 ◽  
Author(s):  
Sinem Yazici ◽  
Gulcin Benbir ◽  
Birsen Ince
Keyword(s):  
Ulcerative Colitis ◽  
Hearing Loss ◽  
Status Epilepticus ◽  
Sensorineural Hearing Loss ◽  
Early Recognition ◽  
Systemic Disease ◽  
Sensorineural Hearing ◽  
Inflammatory Disorder ◽  
Major Morbidity ◽  
Immune Mediated

ABSTRACTUlcerative colitis (UC) is characterized by an inflammatory disorder of the gastrointestinal tract. Immune-mediated extraintestinal manifestations of UC have increasingly attracted attention in the literature recently, for which UC is now considered as a systemic disease. Neurologic involvement associated with UC is probably under-reported because of the unawareness of many physicians, although early recognition and treatment are crucial in preventing major morbidity and sequel. In this case report is presented a patient newly diagnosed as UC, who developed both sensorineural hearing loss and intractable status epilepticus that we suggest to have resulted from immune-mediated mechanisms.

Sign in / Sign up

Export Citation Format

Share Document