V Sreenivas, Sen Rosemary, B Pratibha C, G Chaithra B, M Balasubramanya A, M Nagapoornima
Department of Otorhinolaryngology, Head and Neck Surgery, St John's Medical College Hospital, Bangalore, India.
Indian J Otolaryngol Head Neck Surg. 2024 Feb;76(1):633-638. doi: 10.1007/s12070-023-04231-0. Epub 2023 Oct 13.
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.
双侧感音神经性听力损失可能是一种非常令人痛苦的症状，会影响人的工作效率和生活质量。导致双侧听力损失的病症非常少，自身免疫病因是其中之一。自身免疫性耳病的特征是双侧、大多为波动性的听前庭症状以及对类固醇有反应的症状。由于缺乏标准化的诊断标准或可靠的特异性诊断测试，自身免疫性内耳病（AIED）的诊断对临床医生提出了独特的挑战。本研究的目的是评估符合自身免疫性内耳病标准的患者，并了解其临床特征及对药物的反应。对表现为快速进行性双侧听力损失的患者进行了回顾性病历审查。以表格形式记录了包括详细病史和检查结果在内的临床表现以及血液检查报告和听力图。该研究包括6名患者，3名男性和3名女性。听力损失开始时患者的年龄在24至35岁之间。6名患者中有3名患有原发性自身免疫性耳病，另外3名患有继发于全身性自身免疫性疾病的听力损失。所有患者均接受了全身类固醇治疗，但反应各不相同。原发性AIED患者还接受了内耳类固醇治疗。因此，AIED是一种在高度怀疑下进行的排除性诊断。双侧进行性感音神经性听力损失的患者应评估自身免疫病因。口服类固醇联合鼓室内类固醇目前是AIED的主要治疗方法。这些患者的听力改善预后不佳。因此，应强调早期听力康复以提高生活质量。
