Profound sensorineural hearing loss in polyarteritis nodosa

Bomholt, Axel; Jb, Knudsen; Permin, Henrik; Tommerup, B; Gormsen, J

doi:10.1007/bf00464057

Cited by 24 publications

(6 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Although it is still not known how the autoimmune process affects the inner ear, it has been suggested that this type of hearing loss results from cochlear damage and nerve fiber degeneration secondary to thromboembolic occlusion of the end arteries of the inner ear [21]. Autopsy studies support the role of these vascular events of PAN affecting the temporal bone [22][23][24][25].…”

Section: Discussionmentioning

confidence: 99%

A case of MPO-ANCA-positive polyarteritis nodosa complicated by exudative otitis media, mononeuritis multiplex, and acute renal failure

et al. 2011

View full text Add to dashboard Cite

In December 2008, a 69-year-old Japanese woman was admitted to the Department of Otorhinolaryngology because of hearing impairment due to bilateral exudative otitis media, and was discharged without complete recovery despite conventional treatment. Two weeks later, she was readmitted for worsened deafness, numbness, gait disturbance, and general fatigue. She was referred to our department for general investigation. On admission, laboratory examination revealed severe inflammatory signs and active nephritic urinary sediments. Cranial computed tomography (CT) revealed progressive exudative otitis media and sinusitis. Initially, Wegener's granulomatosis was suspected. Nasal cavity biopsy, however, showed no granuloma formation or vasculitis. Serology revealed high titer of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA), suggestive of microscopic polyangitis (MPA). However, contrast CT identified stenosis of a celiac artery, and renal biopsy showed tubulointerstitial changes with minor glomerular abnormalities. Therefore, polyarteritis nodosa (PAN) was suspected and treatment with intravenous methylprednisolone was initiated. However, a lacunar infarct developed followed by cerebral hemorrhage, and the patient died 19 days after readmission. Autopsy revealed fibrinoid necrosis, neutrophilic infiltration, and giant cell reaction in small to medium-sized arteries in multiple organs. These findings led to diagnosis of systemic vasculitis anatomically compatible with PAN. This was a rare case of a patient with MPO-ANCA-positive PAN who may have developed bilateral exudative otitis media and hearing loss as the initial manifestation of PAN.

show abstract

Section: Discussionmentioning

confidence: 99%

A case of MPO-ANCA-positive polyarteritis nodosa complicated by exudative otitis media, mononeuritis multiplex, and acute renal failure

et al. 2011

View full text Add to dashboard Cite

show abstract

“…SNHL is often reported in PAN [64–66] and, in rare instances, may occur as the presenting symptom of the disease [67, 68]. Hearing loss is typically bilateral and symmetrical, with sudden onset [67, 69] or a rapidly progressive course [65, 70].…”

Section: Inner Ear Involvement In Autoimmune Diseasesmentioning

confidence: 99%

Audiovestibular Symptoms in Systemic Autoimmune Diseases

Ralli

D’Aguanno

Stadio

et al. 2018

Journal of Immunology Research

View full text Add to dashboard Cite

Immune-mediated inner ear disease can be primary, when the autoimmune response is against the inner ear, or secondary. The latter is characterized by the involvement of the ear in the presence of systemic autoimmune conditions. Sensorineural hearing loss is the most common audiovestibular symptom associated with systemic autoimmune diseases, although conductive hearing impairment may also be present. Hearing loss may present in a sudden, slowly, rapidly progressive or fluctuating form, and is mostly bilateral and asymmetric. Hearing loss shows a good response to corticosteroid therapy that may lead to near-complete hearing restoration. Vestibular symptoms, tinnitus, and aural fullness can be found in patients with systemic autoimmune diseases; they often mimic primary inner ear disorders such as Menière's disease and mainly affect both ears simultaneously. Awareness of inner ear involvement in systemic autoimmune diseases is essential for the good response shown to appropriate treatment. However, it is often misdiagnosed due to variable clinical presentation, limited knowledge, sparse evidence, and lack of specific diagnostic tests. The aim of this review is to analyse available evidence, often only reported in the form of case reports due to the rarity of some of these conditions, of the different clinical presentations of audiological and vestibular symptoms in systemic autoimmune diseases.

show abstract

“…It has also been suggested that hearing loss in PAN results from cochlear damage and nerve fibre degeneration secondary to thrombo-embolic occlusion of the end arteries of the inner ear. 12 The primary aetiology of the histopathologic alterations in PAN is as yet unclear; however, evidence suggests they are induced by immune mechanisms resulting in immune complex deposition and activation of complement in the inner ear. 5,11 There are also other systemic autoimmune vasculitis syndromes associated with profound hearing loss, for which cochlear implantation may be effective, such as Cogan syndrome 16 -19 and Behcet's disease.…”

Section: Discussionmentioning

confidence: 99%

“…In PAN, treatment based on systemic steroids alone, 4,6 systemic steroids in conjunction with the immunosuppressive drugs chlorambucil, 21 cyclophosphamide 22 and azathioprine, 23 or plasma exchange 24 has been reported to result in significant hearing improvement. Bomholt et al 12 suggested a platelet inhibitory and/or fibrinolysis-increasing treatment, in addition to the conventional medical treatment, when dysfunction of platelet function or fibrinolytic activity were found. Nonetheless, following his diagnosis of PAN, our patient was begun on prednisolone, cyclophosphamide and specific platelet inhibitory treatment, but this was insufficient to prevent total deafness.…”

Section: Discussionmentioning

confidence: 99%

Polyarteritis nodosa and cochlear implantation

2006

View full text Add to dashboard Cite

Polyarteritis nodosa is a systemic disease which affects the small to medium-sized muscular arteries. Sudden or progressive, bilateral hearing loss is a presenting otologic manifestation. To date, no case of cochlear implantation in patients with polyarteritis nodosa has been reported. The authors present a case of polyarteritis nodosa (confirmed by biopsy) in a 71-year-old man with progressive, bilateral sensorineural hearing loss who underwent cochlear implantation. A successful full insertion of the Nucleus 3G electrode array was achieved without surgical or post-operative complications. The patient immediately showed a positive subjective response and, at three month post-operative evaluation, had gained useful open-set speech perception. A review of five temporal bone cases with hearing loss and polyarteritis nodosa revealed the possibility of fibrosis and ossification in the basal turn of the cochlea, of which the surgeon should be aware prior to cochlear implantation.

show abstract

Profound sensorineural hearing loss in polyarteritis nodosa

Cited by 24 publications

References 9 publications

A case of MPO-ANCA-positive polyarteritis nodosa complicated by exudative otitis media, mononeuritis multiplex, and acute renal failure

A case of MPO-ANCA-positive polyarteritis nodosa complicated by exudative otitis media, mononeuritis multiplex, and acute renal failure

Audiovestibular Symptoms in Systemic Autoimmune Diseases

Polyarteritis nodosa and cochlear implantation

Contact Info

Product

Resources

About