Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027066 (myoclonus)
4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Palatal myoclonus is an uncommon, rhythmic, "shock-like" involuntary movement of the muscles of the soft palate, throat, and other structures derived from the branchial arcs. Objective tinnitus is frequently neglected in review articles about childhood tinnitus. Our aim was to present the case of a 7-year-old girl with bilateral objective tinnitus due to palatal myoclonus without hearing impairment (normal hearing thresholds between 250 Hz and 8 kHz) but with otherwise normal hearing thresholds (250 Hz-8 kHz) and no evidence of intracerebral or systemic disorders. No treatment was useful.
Int Tinnitus J 2007
PMID:Objective tinnitus associated with essential palatal myoclonus: report in a child. 1822 97

Palatal tremor is a rare neurotological disorder responsible for objective tinnitus in children. Palatal tremor may be symptomatic of an underlying neurological disease or essential when a cause cannot be identified. We report a case of an essential palatal tremor in a 10-year-old girl complaining of clicking tinnitus. No treatment was undergone as she was not obviously bothered by the ear-clicking sound. Different treatment modalities have been used for distressing tinnitus related to palatal myoclonus. Recently several publications reported satisfactory results with botulinum toxin injection, which seems to be the treatment of choice.
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PMID:[Objective tinnitus and essential palatal tremor in children: report of a case]. 1981 74

Objective tinnitus can have many different etiologies, palatal myoclonus being one of the less frequent. This type of tinnitus is generated by involuntary rhythmic contraction of the soft palate, which generates an audible click for the patient and for the explorer. Botulinum toxin achieves temporary muscle paralysis through presynaptic inhibition of the acetylcholine level at the neuromuscular union. We present a patient with long-term objective tinnitus, along with this patient's response to botulinum toxin injection.
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PMID:Objective tinnitus from palatal myoclonus. Use of botulinum toxin: a case report. 2151 7

Middle ear myoclonus is one of the causes of clicking tinnitus and can be psychologically distressing. Current management of intractable clicking includes medication or tenotomy. Two cases with spontaneous intrusive irregular clicking are presented where relief and a sense of control were obtained using non-invasive self - administered zygomatic pressure. This technique may be useful in selected patients before resorting to medication or tenotomy.
Int Tinnitus J 2010
PMID:Middle ear myoclonus: a new technique for suppression of spontaneous clicking tinnitus. 2160 14

Creutzfeldt-Jakob disease (CJD) is characterised by rapidly progressive dementia, myoclonus, ataxia, visual disturbances and motor dysfunction. Most of the cases are sporadic. Only 10% to 15% are familial, and the most frequent point mutation is E200K. A 53-year-old man presented with subacute progressive bilateral hypoacusis, with tinnitus in the left ear. During the following months, his hypoacusis worsened and he progressively developed bilateral stocking-type paresthaesia and gait instability. An audiometric examination showed bilateral neurosensorial hypoacusis and nerve conduction studies showed a mixed axonal polyneuropathy. A CT scan and MRI of the brain were normal and the electroencephalography (EEG) showed non-specific changes. He died of respiratory infection 10 months after onset of symptoms. Neuropathological examination showed neuronal loss, punctate, synaptic-like deposits of protease-resistant prionic protein (PrP(RES)) in the cerebral and cerebellar cortices and auditory nuclei. This is a rare case of sporadic CJD presenting with hearing loss.
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PMID:Familial Creutzfeldt-Jakob disease with E200K mutation presenting with neurosensorial hypoacusis. 2173 19

An 8-year-old boy was seen by his primary care pediatrician with a chief complaint of "intermittent rapid vibrations of the epiglottis" that began several weeks prior. Intraoral examination revealed rapid, symmetrical bilateral contractions of the soft palate muscles (velum), accompanied by clicking sounds audible to physician (objective tinnitus) and patient. The patient was able to volitionally control the initiation and cessation of the palatal movements. The child's mother stated that there had been no clicking noises heard while the boy was sound asleep. Palatal "clonus" was tentatively diagnosed as the cause of the problem. A normal magnetic resonance imaging study with contrast enhancement confirmed that there was no anatomical basis for the localized movement disorder.Palatal myoclonus is an uncommon localized intraoral movement disorder. There are 2 distinct types, and our patient was diagnosed with the essential palatal myoclonus type. This type is characteristically associated with clicking tinnitus, heard by the affected person as well as those in close proximity. The clicking noise is not continuous, ceases during sleep, and is not lifelong.
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PMID:Loud clicking sounds associated with rapid soft palate muscle contractions. 2230 83

Objective tinnitus refers to a tinnitus that can also be heard by the examiner. It is a relatively rare condition, and can be misdiagnosed or neglected. Some causes of objective tinnitus are head and neck vascular malformations, or muscular myoclonus of the tensor tympani, stapedial, or palatal muscles. The case of an 11-year-old girl with an objective tinnitus lasting from 1 year is herein presented, and the diagnostic workup performed in this unusual case is described.
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PMID:A clockwork ear. 2271 Dec 31

Palatal myoclonus is a rare cause of pulsatile tinnitus in patients presenting to the otolaryngology office. Rhythmic involuntary contractions of the palatal muscles produce the pulsatile tinnitus in these patients. Treatment of this benign but distressing condition with anxiolytics, anticonvulsants, and surgery has been largely unsuccessful. A few investigators have obtained promising results with botulinum toxin injection into the palatal muscles. We present a patient with palatal myoclonus who failed conservative treatment with anxiolytics. Unilateral injection of botulinum toxin into her tensor veli palatini muscle under electromyographic guidance resolved pulsatile tinnitus in her ipsilateral ear and unmasked pulsatile tinnitus in the contralateral ear. A novel method of following transient postinjection symptoms using a diary is presented in this study. Botulinum toxin dose must be titrated to achieve optimal results in each individual patient, analogous to titrations done for spasmodic dysphonia. Knowledge of the temporal onset of postinjection side effects and symptomatic relief may aid physicians in dose titration and surveillance. We present suggestions on titrating the botulinum toxin dose to optimal levels. A review of the literature on the use of botulinum toxin for palatal myoclonus and some common complications are discussed.
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PMID:Treatment of palatal myoclonus with botulinum toxin injection. 2422 17

Stapedius and tensor tympani tenotomy is a relatively simple surgical procedure commonly performed to control pulsatile tinnitus due to middle ear myoclonus and for several other indications. We designed a cadaveric study to assess the feasibility of an entirely endoscopic approach to stapedius and tensor tympani tenotomy. We performed this endoscopic ear surgery in 10 cadaveric temporal bones and summarized our experience. Endoscopic stapedius and tensor tympani section is a new, minimally invasive treatment option for middle ear myoclonus that should be considered as the first line surgical approach in patients who fail medical therapy. The use of an endoscopic approach allows for easier access and vastly superior visualization of the relevant anatomy, which in turn allows the surgeon to minimize tissue dissection. The entire operation, including raising the tympanomeatal flap and tendon section, can be safely completed under visualization with a rigid endoscope.
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PMID:Feasibility of endoscopic treatment of middle ear myoclonus: a cadaveric study. 2473 99

Palatal myoclonus is an extremely rare disorder. Tinnitus is secondary to rhythmic involuntary movements of the soft palate. Clinical diagnosis is based on the confirmation of the soft palate movements synchronous with an audible clicking noise outside. In this article, we report a 38-year-old female case with a 10 year-history of continuing ear click, who was diagnosed with essential palatal myoclonus. The disease and its differential diagnosis from symptomatic palatal myoclonus were discussed in the light of literature data.
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PMID:[Palatal myoclonus]. 2479 38


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