Demystifying autoimmune inner ear disease
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Autoimmune inner disease (AIED) is an uncommon cause of sensorineural hearing loss and poses a diagnostic challenge. The present study aims to review the existing knowledge on the clinicopathological aspects, the diagnostic challenges, and therapeutic interventions in AIED.
The incidence of AIED is less than five cases per 100,000 population. There are no definite seromarkers which make diagnosis of AIED difficult. Even though various markers have been studied, their sensitivity and specificity have not been replicated in the clinical scenario. The treatment of the condition is also an enigma. Corticosteroids are the drug of choice and require long-term use to prevent relapse. Various other therapeutic agents have been studied in a small cohort of patients, but the efficacy of these drugs needs to be validated in a large multicentric trial.
Timely intervention can restore hearing loss in AIED patients, but the clinician has to find a delicate balance between the hearing outcome and the potential side effects resulting from long-term use of the drugs. Treatment of steroid resistant AIED is a challenge and there are no universal guidelines for the same. AIED being an uncommon diagnosis, multicentric trials and collaboration are required to formulate diagnostic criteria and therapeutic guidelines.
KeywordsInner ear Autoimmunity Hearing loss Sensorineural Diagnosis Inner ear antigens Circulating antibodies Positron emission tomography Magnetic resonance imaging
Compliance with ethical standards
Conflict of interest
The author declares that they have no competing interests.
Research involving Human Participants and/or Animals
No human participant and/or animals were used in the study.
Not taken as no human participants were used in the study.
- 21.Khalessi A, Borghei P, Khalessi MH (2010) Autoimmune inner ear disease—a clinical viewpoint. Iran J Otorhinolaryngol 22(61):111–116Google Scholar
- 24.Kohlert S, Bromwich M (2017) Mobile tablet audiometry in fluctuating autoimmune ear disease. J Otolaryngol Head Neck Surg 46(18):1–5Google Scholar
- 27.El-Kabarity RH, El-Kabarity RH (2011) Diagnostic value of serum anti—heat shock Protein 70 (Anti-HSP70) in cases of autoimmune inner ear disease. Int Adv Otol 7(1):55–61Google Scholar
- 40.Lasak JM, Sataloff RT, Hawkshaw M, Carey TE, Lyons KM, Spiegel JR (2001) Autoimmune inner ear disease: steroid and cytotoxic drug therapy. Ear Nose Throat J 808–11:815–816 (818 passim) Google Scholar
- 43.Harris JP, Gopen Q, Kiethley E (2009) Autoimmune inner ear disease and other autoimmune diseases with inner ear involvement. In: Snow JB, Wackym PA (eds) Ballenger’s otorhinolaryngology head and neck surgery, 17th edn. BC Decker Inc., Connecticut, pp 305–312Google Scholar