Autoimmune inner ear disease: A systematic review of management

Breslin, Nathaniel K.; Varadarajan, Varun V.; Sobel, Eric S.; Haberman, Rex S.

doi:10.1002/lio2.508

Cited by 24 publications

(27 citation statements)

References 55 publications

(137 reference statements)

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“…Corticosteroids remain the primary medical therapy in autoimmune SNHL, with activation of glucocorticoid receptors within the cochlear hair cells causing downregulation of local cytokines and reducing autoantibody production and inflammation [ 20 ]. This attenuates cochleitis and hair cell death, with potential recovery of function.…”

Section: Discussionmentioning

confidence: 99%

Combination Immunosuppressive Therapy in Primary Autoimmune Inner Ear Disease in Pregnancy

Ananthapadmanabhan

Jabbour

Brown

et al. 2022

Case Reports in Otolaryngology

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Objective. Autoimmune inner ear disease (AIED) is a rare disorder characterized by rapidly progressive, sensorineural hearing loss that demonstrates good responsiveness to corticosteroid and immunosuppressive therapy. The pathophysiology is likely driven by chronic trafficking of immune cells into the inner ear, targeting inner ear proteins to coordinate inflammation. Suppression or modulation of the immune response can minimize cochleitis allowing for potential recovery of hearing. It is an otologic emergency requiring a multidisciplinary approach to management to commence immunosuppressive therapy. This can be achieved using steroids, immunomodulators, plasmapheresis, intravenous immunoglobulin, or biologic agents. Treatment decisions are further complicated in pregnancy and require supervision by an obstetrician and maternal-fetal medicine (MFM) specialist. Concerns include safe dosing of steroids and potential for transplacental migration of immune complexes. We provide the first comprehensive literature review on AIED and its implications in pregnancy. We frame our discussion in the context of the second reported case of primary AIED in pregnancy and the first to show excellent response to immunosuppressive therapy. Methods. We reviewed the presented case and literature on AIED. Results. A 27-year-old, pregnant, HSP-70 positive woman was diagnosed with AIED and had excellent recovery of hearing and balance following a combination of steroid treatment, augmented by oral immunomodulators, plasmapheresis, and IVIG. Conclusion. AIED is a diagnostic challenge, and treatment considerations are complex when encountered in pregnancy. Management requires multidisciplinary involvement between otolaryngologists, immunologists, and obstetricians to balance maternal and fetal health outcomes.

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Section: Discussionmentioning

confidence: 99%

Combination Immunosuppressive Therapy in Primary Autoimmune Inner Ear Disease in Pregnancy

Ananthapadmanabhan

Jabbour

Brown

et al. 2022

Case Reports in Otolaryngology

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“…Despite an increase in clinical awareness and in the knowledge of the immune mechanisms involved, AIED is still a challenging diagnosis as it is necessary to conjugate the presence of SNHL with data related to the clinical presentation, audiological testing and response to immunomodulatory drugs [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…There is a common belief that AIED, specifically when associated with a SAID, is a progressive disease that involves both ears and many studies included only cases with bilateral hearing loss [ 8 ]. However, given that the clinical presentation of AIED can be sudden and unilateral [ 8 ], sometimes taking years to become bilateral [ 7 ], the authors chose to include patients with unilateral and bilateral hearing loss.…”

Section: Discussionmentioning

confidence: 99%

“…Despite the limited evidence supporting this approach, it has been used in patients refractory or intolerant to systemic steroids [ 13 ]. Although corticosteroids remain the mainstay of AIED treatment [ 6 , 7 ] and an initial response is reported in up to 70% of patients [ 6 , 9 , 13 ], some authors have stated that only 14% of patients remain steroid-responsive after 34 months of treatment [ 20 ]. This study did not evaluate a specific treatment protocol.…”

Section: Discussionmentioning

confidence: 99%

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Sensorineural Hearing Loss and Systemic Autoimmune Disease: The Experience of a Systemic Immune-Mediated Diseases Unit

Ribeiro¹,

Serôdio²,

Amaral³

et al. 2021

Cureus

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Background Autoimmune inner ear disease (AIED) represents less than 1% of all cases of sensorineural hearing loss (SNHL) but its frequency may be underestimated due to lack of specific clinical and laboratory criteria. AIED can be associated with a systemic autoimmune disease (SAID) in 15%-30% of the cases. The objective of the present study was to characterize the clinical and prognostic factors of a cohort of patients with AIED. Materials and methods The authors conducted a retrospective descriptive analysis of a cohort of patients with AIED referred from the otorhinolaryngology department to a systemic immune-mediated diseases unit between March 2013 and November 2020. A consecutive sample of 39 patients with suspected AIED was referred. SNHL was defined as a fall of the hearing threshold of at least 30 decibels in three consecutive frequencies. Eight patients were excluded for not meeting the audiometric criteria or having confounding factors. The remaining 31 patients were included with a total of 50 affected ears. To classify the intensity of hearing loss, an arithmetic mean of pure tone was calculated. Normal hearing or mild hearing loss at the last pure tone audiometry of the followup were an indicator of good prognosis and were considered the outcome of interest. Results Thirty-two percent of the patients had an associated SAID. There were no differences regarding demographic and clinical characteristics when comparing patients with AIED alone and patients with AIED and a SAID, except for the positivity of antinuclear antibodies (ANA). ANA positivity was superior in patients with the association of AIED and a SAID when compared with patients with AIED alone (90% vs 50%; p=0.037). The SAID was diagnosed after the AIED in 70% of the patients, in which diagnosis of the SAID occurred a median of 4,2 (IQR 0.8-5.1) years after the diagnosis of the AIED. Normal audiometric evaluation or a mild hearing loss was achieved in 31% of the ears at the last audiometric evaluation. A normal audiometry or a mild hearing loss at the time of diagnosis was independently associated with a better outcome (31%, 14%, CI 1.71-273.69; p=0.018). Bilateral hearing loss was independently associated with a worse outcome (54%, 79%, CI 0.01-0.84; p=0.035). The use of systemic corticosteroids (p=0.941), transtympanic corticosteroids (p=0.700) and non-steroid immunomodulator drugs (p=0.986) did not affect prognosis. The presence of a SIAD did not affect the prognosis (p=0.986). Conclusions In this cohort, SAID was present in one-third of the patients with AIED. A good prognosis was achieved in one-third of the patients. A normal audiometry or mild disease at presentation was associated with a good outcome, whilst bilateral involvement was associated with a bad one. Association of a SAID did not seem to influence the hearing-related prognosis. Positivity of ANA antibodies may justify performing a complementary investigation to determine the presence of a SAID.

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“…More recently, various biologics both systemically and intratympanically have been tested. There is little consensus as to the most effective treatment 16 . In some cases, however, the progressive nature of the AIED results in the need for hearing aids and/or cochlear implantation due to failure of medical therapy 12 …”

Section: Introductionmentioning

confidence: 99%

Hearing loss in inner ear and systemic autoimmune disease: A systematic review of post‐cochlear implantation outcomes

Lee

Biggs

Muzaffar

et al. 2021

Laryngoscope Investig Oto

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Objectives To assess outcomes following cochlear implantation (CI) in patients with hearing loss secondary to primary or secondary autoimmune inner ear disease (AIED). Methods A systematic review and narrative synthesis was completed according to PRISMA guidelines. Databases searched included MEDLINE, PubMed, EMBASE, Web of Science, Cochrane Collection, and http://clinicaltrials.gov. No limits were placed on year of publication or language. Results A total of 551 studies were identified, of which 29 were included after removal of duplicates, and screening the title, abstract, and full text. All except one study were OCEBM grade IV. 114 of 115 patients displayed improvement in hearing following cochlear implantation. With implant use, roughly a third of these patients had hearing that improved over time, a third improved and plateaued, and a third remained stable. There was no additional risk of perioperative complications found in AIED patients compared what is generally accepted in general cochlear implantation, although two episodes of device failure after 6 months were noted, and four patients with secondary AIED displayed poor initial audiological outcomes. Conclusion CI in both primary and secondary AIED provides marked improvement in hearing. Early CI may be a valid management option, provide long‐lasting hearing in patients and reduce the side effects of long‐term systemic immunosuppressants. However, patients should be counseled residual hearing may be lost if there is cochlear ossification or fibrosis which may make implant insertion more traumatic. Level of Evidence NA.

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Autoimmune inner ear disease: A systematic review of management

Cited by 24 publications

References 55 publications

Combination Immunosuppressive Therapy in Primary Autoimmune Inner Ear Disease in Pregnancy

Combination Immunosuppressive Therapy in Primary Autoimmune Inner Ear Disease in Pregnancy

Sensorineural Hearing Loss and Systemic Autoimmune Disease: The Experience of a Systemic Immune-Mediated Diseases Unit

Hearing loss in inner ear and systemic autoimmune disease: A systematic review of post‐cochlear implantation outcomes

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