Audiological Patterns in Patients with Autoimmune Hearing Loss

2021 ◽  
pp. 1-10
Author(s):  
George Psillas ◽  
Grigorios G. Dimas ◽  
Michalis Daniilidis ◽  
Paris Binos ◽  
Thomas Tegos ◽  
...  

<b><i>Introduction:</i></b> The aim of this study was to illustrate clinical and audiological patterns of hearing impairment in patients with autoimmune hearing loss (AIHL). <b><i>Methods:</i></b> Fifty-three patients with AIHL were retrospectively recruited, and a tapering schema of steroid treatment was administered in all these patients. The diagnosis of AIHL was essentially based on clinical symptoms, such as recurrent, sudden (sensorineural hearing loss [SSHL]), fluctuating, or quickly progressing (&#x3c;12 months) SSHL (uni-/bilateral), in association with the coexistence of autoimmune diseases, high antinuclear antibodies (ANA) and the presence of human leukocyte antigen (HLA) B27, B35, B51, C04, and C07. Logistic regression analysis was applied to correlate the clinical data and laboratory features of AIHL with final outcomes. <b><i>Results:</i></b> The onset of AIHL was mainly progressive (49%), followed by SSHL (39.6%) or fluctuating (11.3%). The pure-tone audiogram showed more commonly a downsloping pattern (42.6% of ears), but also an upsloping, flat, cookie-bite, or inverse cookie-bite shape. Bilateral progressive AIHL was more frequently simultaneous (23 patients) than heterochronous (4 patients). Nineteen patients (35.8%) showed a favorable response to steroid therapy. The presence of recurrent, bilateral SSHL versus recurrent, unilateral SSHL had statistically negative effect on hearing recovery (OR = 0.042, <i>p</i> &#x3c; 0.05). The heterochronous bilateral SSHL may have better prognosis than simultaneous bilateral SSHL (OR = 10.000, <i>p</i> = 0.099). The gender, age, concomitant autoimmune disease, high ANA, HLA alleles, tinnitus, and vestibular symptoms had no statistical effect on a favorable outcome of AIHL. <b><i>Conclusions:</i></b> A bilateral, simultaneous, and progressive hearing loss combined with downsloping audiogram occurred more often in patients with AIHL. Bilateral simultaneous SSHL with recurrences represents the worse prognostic form of AIHL.

2021 ◽  
Vol 11 (1) ◽  
pp. 31-37
Author(s):  
George Psillas ◽  
Paris Binos ◽  
Grigorios G Dimas ◽  
Michalis Daniilidis ◽  
Jiannis Constantinidis

Background: To evaluate the effect of human leukocyte antigen (HLA) on hearing outcome in patients suffering from autoimmune hearing loss (AIHL). Materials and Methods: The diagnosis of AIHL was essentially based on clinical symptoms, such as recurrent, sudden, fluctuating, or quickly progressing (<12 months) sensorineural hearing loss (uni-/bilateral). The molecular typing of HLA alleles was achieved by using polymerase chain reaction procedures. Patients underwent a tapering schema of steroid treatment and audiometric features were recorded. A logistic regression model was used to identify which HLA typing alleles were statistically significant in patients’ response to treatment. Results: Forty patients with AIHL were found to be carriers of HLA B27, B35, B51, C4, C7, and DRB1*04 alleles. No statistically significant influence of HLA B27, B35, B51, C4, C7, DRB1*04 HLA alleles typing was detected for the prognosis of AIHL. In these patients, the onset of AIHL was mainly progressive (53.8%), 29.2% of them had moderate hearing loss, and most of the cases had both bilateral hearing loss (62.5%) and downsloping audiogram (40%). Conclusion: The presence of HLA B27, B35, B51, C4, C7, and DRB1*04 alleles had no significant effect on a favorable outcome of AIHL. However, larger samples of patients are necessary in order to improve the knowledge about the HLA influence on the clinical course of AIHL.


2021 ◽  
Vol 9 ◽  
Author(s):  
Lung Chang ◽  
Horng-Woei Yang ◽  
Tang-Yu Lin ◽  
Kuender D. Yang

Kawasaki Disease (KD) is an acute inflammatory illness that mostly occurs in children below 5 years of age, with intractable fever, mucocutaneous lesions, lymphadenopathy, and lesions of the coronary artery (CAL). KD is sharing clinical symptoms with systemic inflammatory syndrome in children (MIS-C) which is related to COVID-19. Certain genes are identified to be associated with KD, but the findings usually differ between countries and races. Human Leukocyte Antigen (HLA) allele types and toll-like receptor (TLR) expression are also correlated to KD. The acute hyperinflammation in KD is mediated by an imbalance between augmented T helper 17 (Th17)/Th1 responses with high levels of interleukin (IL)-6, IL-10, IL-17A, IFN-γ, and IP-10, in contrast to reduced Th2/Treg responses with lower IL-4, IL-5, FoxP3, and TGF-β expression. KD has varying phenotypic variations regarding age, gender, intravenous immunoglobulin (IVIG) resistance, macrophage activation and shock syndrome. The signs of macrophage activation syndrome (MAS) can be interpreted as hyperferritinemia and thrombocytopenia contradictory to thrombocytosis in typical KD; the signs of KD with shock syndrome (KDSS) can be interpreted as overproduction of nitric oxide (NO) and coagulopathy. For over five decades, IVIG and aspirin are the standard treatment for KD. However, some KD patients are refractory to IVIG required additional medications against inflammation. Further studies are proposed to delineate the immunopathogenesis of IVIG-resistance and KDSS, to identify high risk patients with genetic susceptibility, and to develop an ideal treatment regimen, such as by providing idiotypic immunoglobulins to curb cytokine storms, NO overproduction, and the epigenetic induction of Treg function.


2007 ◽  
Vol 3;10 (5;3) ◽  
pp. 467-472
Author(s):  
Jeffrey T. Vrabec

Background: The hydrocodone/acetaminophen combination is one of the most commonly used analgesic preparations. Isolated incidences of suspected association between hydrocodone abuse and rapidly progressive hearing loss have been reported. In this study, we describe the clinical characteristics of 5 patients presenting with progressive hearing loss and a history of hydrocodone use. Methods: Patients presenting with rapidly progressive bilateral hearing loss who had a documented history of hydrocodone use were selected for the study. The presentation, audiologic findings, associated comorbidities, and treatment outcomes were reviewed Results: All patients displayed rapidly progressive sensorineural hearing loss without vestibular symptoms. Hearing loss was asymmetric in 3 patients at initial presentation, but progressed to profound loss, usually within months. Steroid treatment has no effect on the progression of the hearing loss. The admitted quantity of hydrocodone consumed ranged from 10 to 300 mg per day. Hepatitis C was the most common comorbidity, present in 60% of the patients. All patients underwent cochlear implantation with satisfactory results. Conclusions: The chronic use of hydrocodone can be associated with progressive sensorineural hearing loss. Successful auditory rehabilitation can be achieved with cochlear implantation. Genetic polymorphisms of drug metabolizing enzymes as well as associated comorbidities such as hepatitis C infection may be significant in the development of hydrocodone ototoxicity, though additional investigations are necessary. Key words: hydrocodone, sensorineural hearing loss, cochlear implant


2021 ◽  
Vol 8 (10) ◽  
pp. 1756
Author(s):  
Santosh Kumar Swain

Coronavirus disease 2019(COVID-19) is a rapidly spreading contagious respiratory disease caused by the severe acute respiratory syndrome coronavirus 2(SARS-CoV-2) which manifests a plethora of health concerns. It may manifest with a wide range of clinical symptoms from no symptoms to respiratory failure and even multi-organ dysfunctions. The COVID-19 infection may present with otological manifestations such as hearing loss, tinnitus, vertigo, and otalgia. The otological manifestations may be due to the involvement of the eustachian tube and cochlea by COVID-19 infections. The damaging effect of the COVID-19 infection on the inner ear is a new finding which is yet to be explored. Pure tone audiometry and impedance audiometry are two important investigations for auditory assessment among pediatric patients with COVID-19 infection. Although hearing loss, tinnitus and vertigo are not common as fever and cough in COVID-19 patients, however, auditory-vestibular symptoms significantly affect the quality of life. Additionally, the anxiety associated with COVID-19 infections may attribute to aggravation of otoneurological symptoms like tinnitus. However, these otoneurological manifestations like hearing loss will affect the speech of the pediatric patients and result in morbid life in the current COVID-19 pandemic. Clinicians or pediatricians should be aware of the otoneurological manifestation in pediatric COVID-19 patients for early identification and management. This review article discusses the details of this epidemiology, etiopathology, assessment, and management of hearing loss, tinnitus, and vertigo among COVID-19 pediatric patients.


1994 ◽  
Vol 114 (sup514) ◽  
pp. 41-44 ◽  
Author(s):  
Minako Yamamoto ◽  
Jin Kanzaki ◽  
Kaoru Ogawa ◽  
Yasuhiro Inoue ◽  
Shunya Ikeda

2021 ◽  
Vol 20 (4) ◽  
pp. 119-125
Author(s):  
Sung Il Nam

Vestibular schwannoma (VS) is commonly encountered in the cerebellopontine angle and benign neoplasms that arise from Schwann cells of the eighth cranial nerve, which can show not only hearing loss but also various vestibular symptoms. Dizziness is the symptom causing significantly negative effect on quality of life in patients with VS. Here, we will review the dizziness in VS.


2016 ◽  
Vol 22 ◽  
pp. 6
Author(s):  
Leena Kinnunen ◽  
Valma Harjutsalo ◽  
Heljä-Marja Surcel ◽  
Christel Lamberg-Allardt ◽  
Jaakko Tuomilehto ◽  
...  

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