Paediatric autoimmune inner ear disease: A case series

Objective: Autoimmune inner ear disease (AIED) is an uncommon cause of sensorineural hearing loss which may present differently in paediatric patients. The fluctuating nature of the hearing loss and the inherent challenges of audiological assessment of children can make this disease difficult to diagnose and treat. We present our experience of diagnosis and management of AIED in children in this case series. Study design: This is a retrospective case series review of paediatric AIED patients. Diagnosis was made through clinical presentation, audiometric evaluation, and serological testing. Patients were treated with corticosteroids and/or methotrexate. An improvement in pure tone averages (PTA) or speech reception threshold (SRT) of 10dB or greater was considered a clinically significant improvement. Results: The clinical history of four patients is presented. We observed significant differences in disease presentation and progression in these patients. Patients were largely responsive to steroid therapy in patients with both primary AIED and secondary AIED. Conclusion: Children with AIED fall into primary and secondary disease classifications; however, the disease presentation may be vastly different. These patients seem to benefit from corticosteroid and methotrexate therapy. Close collaboration between otolaryngologists and paediatric rheumatologists can result in timely diagnosis and treatment.