Autoimmune sensorineural hearing loss

Mathews, John; Kumar, B. Nirmal

doi:10.1046/j.0307-7772.2003.00738.x

Cited by 41 publications

(40 citation statements)

References 109 publications

(113 reference statements)

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“…9 The typical course is acute, with severe hearing loss taking place over daysweeks, and the other ear becoming affected months or years later. The etiologic antibodies are unkown, although anticochlear antibodies have been found in 35% of a series of 54 patients, 10 and 32% of 279 patients in another series had antibodies to an antigen similar to heat shock protein 70 (hsp70).…”

Section: Discussionmentioning

confidence: 99%

Malignant Thymoma Presenting as Sensorineural Deafness

Hunter

Ringrose

Kirk

2008

Can. j. neurol. sci.

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Thymoma is well known to have a strong association with autoimmune disorders, most notably myasthenia gravis (MG) which is seen in up to 40% of cases. 1,2 There are also a number of other less common autoimmune disorders associated with thymoma, including hyperexcitability syndromes, 3 myositis, 1 encephalitis, 4 autonomic dysfunction, 5 and lupus. 6 In 2004 Vernino and Lennon 7 reported autoimmune sensorineural hearing loss (SNHL) in two patients from their series of 201 patients with thymoma, a previously unreported association. A third case of paraneoplastic SNHL asociated with thymoma was reported by Evoli et al. 8 We now report a patient presenting with SNHL several years before a diagnosis of malignant thymoma was made. This newly recognized association justifies screening for thymoma as part of the work-up for autoimmune SNHL. CASE REPORTA previously healthy 26-year-old female with no significant medical or family history presented with tinnitus followed by profound sensorineural hearing loss in the right ear over the next two weeks. Audiometry was consistent with sensorineural deafness. The following year she had the same symptoms affecting the left ear leaving her deaf. At age 31 she developed horizontal diplopia with impaired adduction of the left eye. Magnetic resonance imaging scan of the brain and cerebrospinal fluid examination were normal. An edrophonium test did not improve her diplopia. Anti-acetylcholine receptor antibodies were negative, and muscle biopsy of vastus lateralis was unremarkable.Over the next few months she developed proximal weakness, fatigueable muscles of mastication, and dysphagia. Her edrophonium test was now strongly positive. Computed tomogram scan of the chest confirmed the presence of a large thymic mass, and microscopic analysis revealed prominent nucleoli and high mitotic counts consistent with malignant thymoma. The mass was resected and followed by radiotherapy. Her anti-acetylcholine receptor antibodies were now strongly positive at 7.79 pmol/L. Repetitive nerve stimulation was also positive with 14% decrement. She was treated with pyridostigmine and prednisone which was tapered gradually, and she remained stable for several years.At age 38 she presented with shortness of breath and was found to have a large left pleural effusion which was considered parapneumonic in nature. She soon developed bilateral effusions which tended to reaccumulate for the next several years requiring THE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES 265 Malignant Thymoma Presenting as Sensorineural Deafness Gary Hunter, Jennifer Ringrose, Andrew KirkCan. J. Neurol. Sci. 2008; 35: 265-266 PEER REVIEWED LETTER multiple thoracenteses. Follow-up chest x-ray at age 43 revealed a mass involving the left breast and sternal border, which was confirmed on biopsy to be recurrent malignant thymoma. The patient was admitted to hospital with increasing shortness of breath and required intubation and ventilation for respiratory failure. Further testing revealed a strongly positive ANA and anti-DNA antibo...

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Section: Discussionmentioning

confidence: 99%

Malignant Thymoma Presenting as Sensorineural Deafness

Hunter

Ringrose

Kirk

2008

Can. j. neurol. sci.

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“…Pada penelitian ini juga didapatkan bahwa pada penderita LES terjadi peningkatan ambang dengar pada frekuensi 2.000 Hz, 4.000 Hz, 8 kedua telinga pada frekuensi tinggi secara simetris, sedangkan pada nada rendah masih tetap normal. Ambang dengar sesudah terapi prednison selama 4 minggu pada frekuensi 2.000 Hz, 4.000 Hz, dan 8.000 Hz mengalami perbaikan dibanding dengan sebelum terapi (p<0,01).…”

Section: Tabel 1 Derajat Pendengaranunclassified

Pengaruh Prednison terhadap Perbaikan Pendengaran Penderita Lupus Eritematosus

Wijana¹,

Mutiara²,

Agustian³

2016

mkb

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AbstrakLupus eritematosus sistemik (LES) merupakan penyakit kronik mengenai multisistem, merupakan gangguan autoimun yang ditandai oleh produksi autoantibodi dan deposisi kompleks imun di jaringan. Manifestasi klinis dapat meliputi berbagai organ termasuk telinga. Pada telinga dapat menimbulkan gangguan dengar, tinitus, dan vertigo. Gangguan dengar terjadi bilateral pada frekuensi tinggi. Prednison merupakan kortikosteroid oral yang memiliki efek glukokortikoid yang bersifat anti-inflamasi dan imunosupresif. Penelitian ini bertujuan mengetahui pengaruh prednison terhadap perbaikan pendengaran penderita LES. Penelitian dilakukan periode Maret-Mei 2013 di Poliklinik Gangguan Dengar dan Bicara, Telinga Hidung Tenggorok Bedah Kepala dan Leher Rumah Sakit Dr. Hasan Sadikin Bandung menggunakan metode quasi-experimental dengan rancangan one group pretestposttest design dan hasilnya dihitung secara statistik menggunakan uji Rank Spearman. Pada 28 subjek pasien LES dilakukan anamnesis, pemeriksaan fisis, laboratorium, audiometri nada murni, timpanometri, dan distortion production otoacoustic emissions. Subjek diberi prednison 1 mg/kgBB/hari (dosis maksimal 60 mg/hari) selama 4 minggu, kemudian dilakukan evaluasi ulang. Sebelum terapi, 26 subjek mengalami gangguan dengar sedang pada frekuensi tinggi, simetris. Sesudah terapi, 24 subjek ambang dengar menjadi normal dan 4 subjek tetap mengalami gangguan dengar (Rs=0,734; p<0,01 Prednisone Effect to on Hearing Status of Systemic Lupus Erythematosus Patients AbstractSystemic lupus erythematosus (SLE) is a chronic, multi-system, autoimmune disorder characterized by the production of autoantibodies and immune complexes deposition in tissue. Clinical manifestations may include skin, mucosa, joints, blood, heart, lungs, kidneys, central nervous system (CNS), immune system, and ears. SLE Iin the ears can caused sensorineural hearing loss, tinnitus, and vertigo. This hearing disorder is commonly bilateral and has high frequencies. Prednisone is an oral corticosteroid with glucocorticoid and mineralocorticoid effects. Glucocorticoids have anti-inflammatory and immunosuppressive effects. The aim of this study was to evaluate the effect of prednisone in improving the degree of hearing loss in patients with SLE. This study was conducted in from March-May 2013, at tThe Hearing and Speech Disorder Clinic of Ear Nose Throat-Head and Neck Surgery, Dr. Hasan Sadikin Hospital Bandung, using quasi-experimental method with pre-post design in which the results were statistically calculated using Rank Spearman test. This study involved 28 subjects who had been diagnosed for SLE. Anamnesis, physical examination, and laboratory test were performed, followed by pure tone audiometry, tympanometry, and distortion product optoacoustic emissions (DPOAE). Subjects were given prednisone 1 mg/kgBW/day with a maximum dose of 6o mg/day. Re-evaluation was performed after 4 weeksof treatment. Before treatment, 26 subjects had mild hearing loss and 2 subjects had symmetric moderate hearing loss at high freque...

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“…3 Esta premissa faz com que seja uma das poucas formas de hipoacusia neurossensorial potencialmente reversível após tratamento médico. 1,4,5 Só na década de cinquenta é que a sociedade científica admitiu o conceito de autoimunidade. Apesar da etiologia da doença autoimune do ouvido interno (DAIOI) ainda não ser completamente conhecida, foram propostos múltiplos mecanismos para explicar a etiopatogenia da mesma.…”

Section: Introductionunclassified

Doença Autoimune do Ouvido Interno: Revisão da Literatura

Branco¹,

Caroça

Campelo³

et al. 2018

Gaz Med

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A surdez autoimune é uma entidade clínica rara, representando geralmente um desafio diagnóstico, correspondendo muitas vezes à manifestação inicial de um distúrbio autoimune sistémico. Caracteriza-se pela presença de hipoacusia neurossensorial, bilateral e assimétrica de instalação progressiva durante semanas a meses. Em 50% dos casos, associado à surdez, verifica-se a presença de sintomas vestibulares, acufenos ou plenitude auricular. Dada a ausência de testes específicos que permitam a realização de um diagnóstico definitivo, este é baseado na suspeita clínica e na resposta ao tratamento com terapia imunossupressora. O diagnóstico diferencial é realizado através de uma história clínica detalhada com exame objetivo completo, bem como exames audiométricos, laboratoriais e imagiológicos de modo a excluir outras etiologias de surdez neurossensorial. A raridade desta doença, o grande espectro de manifestações clínicas, a ausência de estudos randomizados que demonstrem a eficácia do tratamento, bem como a ausência de resultados a longo prazo, fazem desta doença um desafio. Com este artigo os autores pretendem realizar uma revisão do tema de modo a facilitar a identificação precoce desta patologia e permitir a instituição adequada de tratamento.

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Autoimmune sensorineural hearing loss

Cited by 41 publications

References 109 publications

Malignant Thymoma Presenting as Sensorineural Deafness

Malignant Thymoma Presenting as Sensorineural Deafness

Pengaruh Prednison terhadap Perbaikan Pendengaran Penderita Lupus Eritematosus

Doença Autoimune do Ouvido Interno: Revisão da Literatura

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