Objective: To describe a case of unilateral ear clicking caused by isolated tensor veli palatini myoclonus without palatal elevation.

Background: Palatal myoclonus may be secondary to brainstem lesions in the Guillain-Mollaret triangle, or termed “essential myoclonus” without an identifiable structural abnormality. Patients may present with subjective or objective unilateral clicking sounds, and usually have palatal elevation on exam. Treatments include oral medication, surgery, or botulinum toxin injections [1].

Method: A 55-year-old man who was previously healthy presented to Neurology Clinic with ear clicking and pain. After attending a rock concert, he developed right-sided tinnitus, ear fullness, followed by left ear clicking, pain, and hyperacusis. The symptoms interrupted sleep and mood. He was seen by dentists, otolaryngologists, and psychiatrists, and was treated for ear infection with antibiotics, temporomandibular joint pain with dental guard for and anxiety with clonazepam 0.25mg four times daily, all without relief. Two neurologists considered a diagnosis of palatal myoclonus, however did not visualize palatal movement on exam.

Results: Exam was notable for arrhythmic left lateral soft palatal contraction in the region of the tensor veli palatini that did not raise the palate or uvula. This contraction correlated with an audible click by auscultation with stethoscope over the left ear. Otherwise, neurologic exam was normal. MRI Brain and CT Angiogram of the head were normal. He received 2.5 units of Onabotulinum toxin injection into the affected muscle with mild improvement.

Conclusion: Palatal myoclonus generally consists of a visible elevation of the palate and uvula, and may include myoclonus of other oropharyngeal muscles. This is a unique case of a patient with focal, unilateral myoclonus only visualized on close inspection with concurrent auscultation. Middle ear myoclonus due to tensor tympani or stapedius contraction has been reported that cannot be visualized on oral examination [2], however focal palatal myoclonus in itself has rarely been reported [1]. Practitioners should perform careful oral examinations with auscultation to detect focal myoclonic contractions in a patient with ear clicking without notable palatal elevation.

References: [1] Sinclair CF, Gurey LE, Blitzer A. Palatal myoclonus: algorithm for management with botulinum toxin based on clinical disease characteristics. Laryngoscope. 2014;124(5):1164-9. [2] Keidar E, Kwartowitz G. Tensor Tympani Syndrome. StatPearls. 2019.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/isolated-unilateral-tensor-veli-palatini-myoclonus-causing-objective-tinnitus-without-palatal-elevation/