Bilateral sensorineural hearing loss can be a very distressing symptom and can affect the efficiency of a person and one's quality of life. Conditions causing bilateral hearing loss are very few and autoimmune aetiology is one of them. Autoimmune ear disease is characterised by bilateral, mostly fluctuating audiovestibular symptoms and symptoms which respond to steroids. Diagnosis of AIED presents a unique challenge to clinicians due to the lack of standardized diagnostic criteria or reliable pathognomonic tests. The purpose of the study is to evaluate the patients who fit into criteria of autoimmune inner ear disease and understand the clinical features and response to medications for the same. A retrospective chart review of patients presenting with rapidly progressive bilateral hearing loss was done. The clinical presentation including detailed history and examination findings along with the blood investigation reports and audiograms were recorded in a tabular form. The study included 6 patients - 3 male and 3 female patients. Age of the patients at onset of hearing loss varied between 24-35 years. 3 of 6 patients presented with primary autoimmune ear disease and other 3 had hearing loss secondary to systemic autoimmune disease. All patients were treated with systemic steroids, but however showed a varied response. Patients with primary AIED were administered inner ear steroid therapy as well. AIED is thus a diagnosis of exclusion done with high index of suspicion. Patients with bilateral progressive sensorineural hearing loss should be evaluated for autoimmune etiology. Oral steroids with intratympanic steroids are currently the mainstay of treatment for AIED. Guarded prognosis of hearing improvement is noted in these patients. Hence, emphasis should be placed on early hearing rehabilitation for better quality of life.
Keywords: Autoimmune inner ear disease; Bilateral progressive sensorineural hearing loss.
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