Middle ear myoclonus: pathophysiology and management.

Abstract

Purpose of review: This review aims to provide a comprehensive analysis of the pathophysiology and treatment of middle ear myoclonus (MEM), a rare and under-recognized cause of objective and subjective tinnitus.

Recent findings: MEM is increasingly recognized as a distinct subset in tinnitus patients, with symptoms arising from involuntary contractions of the stapedius and/or tensor tympani muscles. Pharmacological management currently centres around agents such as clonazepam, carbamazepine, and piracetam. Surgical resection of middle ear tendons has yielded highly encouraging results, albeit with risks such as hyperacusis and symptom recurrence due to tendon reattachment. Endoscopic surgical techniques offer improved visualization and reduced invasiveness. Emerging data support the potential role of botulinum toxin as a less invasive and reversible therapeutic option, particularly with intratympanic delivery methods demonstrating early safety and efficacy in recent studies.

Summary: MEM is a complex otologic condition with limited high-quality data guiding its diagnosis and management. Due to its variable presentations and response to treatment, a multidisciplinary and individualized approach remains essential. While pharmacological treatments and surgical outcomes are generally favourable, interest in botulinum toxin continues to grow. Further large-scale, controlled studies are needed to clarify pathophysiological mechanisms, optimize diagnostic accuracy, and standardize treatment protocols.