Systematic review of management strategies for middle ear myoclonus.

OBJECTIVE: Middle ear myoclonus is a rare condition with distinct characteristics at presentation. Diagnosis is based primarily on history, clinical examination, and long-time-based tympanometry. No consensus exists regarding treatment at present. This review was designed to identify relevant studies on current investigation and management. DATA SOURCE: A systematic electronic literature search of MEDLINE, Embase, Cumulative Index to Nursing and Allied Health Literature (CINAHL), Cochrane Trials register, and Web of Science was conducted for articles describing middle ear myoclonus through to May 2011. English- and non-English-language articles that focused on investigation and treatment were considered for review. REVIEW METHOD: Two authors independently reviewed the articles for study design, treatment, intervention, and outcome. Data from human and experimental studies were considered. RESULTS: A total of 21 articles were identified for this review. Most studies were found to be case reports or small case series. In general, there was no evidence of a conclusive diagnostic test or treatment for middle ear myoclonus. CONCLUSION: There is a need for high-quality prospective controlled trials to determine the most effective management of middle ear myoclonus. The authors describe a treatment algorithm based on the data available and clinical knowledge.