Transitory stapedial myoclonus in a patient with benign fasciculation syndrome F BRIGO 1,2 , M STORTI 3 , P LOCHNER 2 , R NARDONE 2,4 1 Department of Neurological, Neuropsychological, Morphological and Movement Sciences, Section of Clinical Neurology, and 3 Department of Medicine, Franz Tappeiner Hospital, Merano, Italy, 2 Department of Neurology, Franz Tappeiner Hospital, Merano, Italy, and 4 Department of Neurology, Christian Doppler Clinic, Paracelsus Medical University, Salzburg, Austria Abstract Objective: We report a previously undescribed association between transitory stapedial myoclonus, objective tinnitus and benign fasciculation syndrome. Method: Case report and review of the world literature regarding stapedial myoclonus. Results: A 30-year-old man with a diagnosis of benign fasciculation syndrome abruptly developed severe, low-pitched tinnitus on the right side. Otoscopic examination revealed rhythmic movement of the tympanic membrane, which was synchronous with the tinnitus. No palatal spasm was noted on nasopharyngeal examination. Brain magnetic resonance imaging and pure tone audiometry were unremarkable. Based on these findings, a diagnosis of objective tinnitus due to stapedial myoclonus was made. The objective tinnitus spontaneously disappeared within 48 hours of its appearance, but in the following days the patient suffered frequent, brief episodes of objective tinnitus lasting only a few seconds. Conclusion: The occurrence of stapedial myoclonus in this patient indicated the presence of an underlying motor unit hyper-excitability. This case suggeststhat, in some patients, stapedial myoclonus may represent the clinical expression of diffuse motor unit hyper-excitability. Key words: Stapedius; Ear, Middle; Myoclonus; Tinnitus; Fasciculation Introduction Tinnitus is defined as the perception of sound in the absence of corresponding external stimulus, and may be classified as subjective or objective. Subjective tinnitus is characterised by an individual’s perception of sound in the absence of any physical source, whereas objective tinnitus refers to a sound that is produced mechanically within the head. Stapedial myoclonus is a rhythmic movement of the tym- panic membrane secondary to repetitive contraction of the tensor tympani and stapedial muscles, and represents a seg- mental myoclonus. It is a rare cause of objective tinnitus, 1 with only a few cases reported in the literature. We describe a previously unreported case of objective tin- nitus caused by transitory stapedial myoclonus in a patient diagnosed with benign fasciculation syndrome. Case report A 30-year-old man presented with severe, low-pitched tinni- tus on the right side which had developed suddenly while he was sitting down watching television. His previous medical history was notable for benign fasciculation syndrome. He did not take any drugs. The tinnitus was described as an unpleasant, buzzing noise resembling that experienced during aircraft landing, but occurring in a rhythmic fashion, dozens of times a minute. There was no pressure in the left ear nor any change in hearing status. The patient denied any precipitating factors, although performing the Valsalva manoeuvre and placing pressure on the tragus were reported to slightly increase the frequency and intensity of tinnitus. On physical examination, the tympanic cavity was clear. Otoscopic examination revealed rhythmic movement of the tympanic membrane, which was synchronous with the tinni- tus. No palatal spasm was noted on nasopharyngeal examination. Brain magnetic resonance imaging and pure tone audio- metry were normal. Based on these findings, a diagnosis of objective tinnitus due to stapedial myoclonus was made. Electromyography showed diffuse fasciculation potentials with normal motor unit recruitment, without features indica- tive of chronic denervation (i.e. neurogenic enlarged motor unit potentials) and/or active denervation (i.e. fibrillation and positive, sharp waves). The patient’s objective tinnitus spontaneously disappeared within 48 hours of its appearance. In the following days, he suffered frequent, brief episodes of objective tinnitus lasting only a few seconds. Discussion Stapedial myoclonus is a rhythmic movement of the tympa- nic membrane caused by repetitive contraction of the tensor Accepted for publication 28 June 2012 The Journal of Laryngology & Otology, 1 of 2. CLINICAL RECORD © JLO (1984) Limited, 2013 doi:10.1017/S0022215113000297