China
Africa
Explore chapters and articles related to this topic
Tinnitus and Hyperacusis
Published in R James A England, Eamon Shamil, Rajeev Mathew, Manohar Bance, Pavol Surda, Jemy Jose, Omar Hilmi, Adam J Donne, Scott-Brown's Essential Otorhinolaryngology, 2022
Investigation of pulse-asynchronous pulsatile tinnitus often draws a blank. If a particular pathology is proven or strongly suspected, specific treatments are available including division of the tensor tympani and stapedius tendons for intratympanic myoclonus or Botox injection of the relevant muscles for palatal myoclonus. These treatments have significant risks attached and in the case of intratympanic tendon division are irreversible. Surgery should therefore probably be best regarded as an option only after careful consideration.
Krabbe disease/galactosylceramide lipidosis/globoid cell leukodystrophy
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
The electroencephalogram (EEG) is disorganized and slow [14, 19, 20], and there are paroxysmal discharges. There may be asymmetry. The electromyogram (EMG) may be abnormal, and there may be fibrillations [19, 23, 24]. Motor nerve conduction velocity is regularly decreased [19, 23, 25]. In 82 percent of 27 patients, one day to eight years old, there was uniform slowing of sensory and motor nerve condition [50]. The patient may have hyperactive deep tendon reflexes, while electrophysiologic studies indicate a prominent peripheral neuropathy [24]. Among adult patients nerve conduction may be normal [34], or there may be EMG evidence of demyelinating neuropathy [35]. Visual or auditory evoked responses may be abnormal [35]. The former are abnormal early, while the latter abnormalities occur later. In 20 early-onset patients, most had abnormal brain stem auditory evoked potentials (BAEP) and visual evoked potentials (VEP); and 65 percent had abnormal EEGs [51] while less than half of later patients had these abnormalities. Palatal myoclonus has been described in this disease [52].
Tinnitus and Hyperacusis
Published in John C Watkinson, Raymond W Clarke, Christopher P Aldren, Doris-Eva Bamiou, Raymond W Clarke, Richard M Irving, Haytham Kubba, Shakeel R Saeed, Paediatrics, The Ear, Skull Base, 2018
The role of specialist testing for the assessment of middle ear or palatal myoclonus is unclear. While middle ear myoclonus can be diagnosed based on history and impedance changes on long-time-based tympanometry, an accurate differentiation between myoclonus of the tensor tympani and the stapedius muscle is not possible.203 It is, however, important to differentiate a middle ear myoclonus from a palatal myoclonus. Palatal myoclonus usually produces an objective rhythmic sound that is associated with an involuntary movement of the soft palate and/or suprahyoid muscles. As palatal myoclonus has been associated with lesions in the central nervous system, MRI is recommended to exclude a pathology with the triangle of Guillain–Mollaret.204
Francis Forster, the last Horseman: A career in academic neurology
Published in Journal of the History of the Neurosciences, 2018
Although Daroff had later concluded Forster was in error and characterized Forster’s response as “What do you do when you’re stuck and you don’t know what the hell the patient has?” (Lanska & Sommer, 2014), Forster was in fact correct in his proclamation (Lanska & Sommer, 2014).6Objective tinnitus can occur with either palatal myoclonus or middle-ear myoclonus (including stapedial muscle myoclonus; Lanska, 2018). Palatal myoclonus may be associated with objective tinnitus because of repetitive Eustachian tube opening and closing and resultant fluctuating middle-ear pressures (Deuschl, Mishke, Schenck, Schulte-Monting, & Lucking, 1990; Slack, Soucek, & Wong, 1986; Toland, Porubsky, Coker, & Adams, 1984; Virtanen, 1983). This may sometimes be audible to an examiner even without a stethoscope (Fabiani et al., 2000). Objective tinnitus is more common in patients with essential palatal myoclonus compared with those with symptomatic myoclonus, but it occurs in both (Deuschl et al., 1990). Furthermore, given that symptomatic cases are approximately three times more frequent than essential cases, symptomatic cases with objective tinnitus are not, in fact, so rare. In an extensive literature review by Deuschl and colleagues (1990), 16 of 192 (8%) cases with symptomatic rhythmic palatal myoclonus had objective tinnitus, compared with 64 of 71 (90%) cases with essential rhythmic palatal myoclonus; of those with rhythmic palatal myoclonus and objective tinnitus, 16 of 80 (20%) had the symptomatic form of rhythmic palatal myoclonus (Deuschl et al., 1990).
Management of palatal myoclonic tinnitus based on clinical characteristics: a large case series study
Published in Acta Oto-Laryngologica, 2020
Jung Mee Park, Woo Jin Kim, Jae Sang Han, So Young Park, Shi Nae Park
Botulinum toxin injection is used in several diseases related to muscle spasms or myoclonic movements in the otolaryngology field, such as blepharospasm, hemifacial spasm, and vocal tremor [8]. Botulinum toxin A, the serotype most widely used for therapeutic purposes, prevents the release of acetylcholine at the neuromuscular junction by cleaving the synatosomal protein of SNAP-25. This stops nerve signaling, eventually leading to paralysis of the affected muscles [9]. Although the clinical effect of botulinum toxin is known to lasts for 3 to 4 months [9,10], 87.5% of the injected patients showed complete remission of symptoms with a single injection in our study. Palatal myoclonus belongs to the spinal segmental myoclonus group, which involves a loss of spinal inhibitory interneuron activity that results in spontaneous firing of anterior horn cells [11]. Botulinum toxin not only paralyzes the targeted muscle through the inhibition of acetylcholine exocytosis at the neuromuscular junction but also reduces Ia and II afferent signals from the muscle spindle organs, inhibiting the abnormal firing of spinal reflex that causes muscle spasm [9]. In addition to the spinal reflex, muscle afferent input is also controlled by the cortical area at the supra-spinal level, which can excite the neurons responsible for muscle contraction [12]. Botulinum toxin may affect this cortical excitation through reduced motor spindle signal output, normalizing altered intracortical inhibition [13]. This cortical plasticity may explain why most patients with PMT did not require more than one-time injection of botulinum toxin. Relatively small volume of the palatal muscles compared to other myoclonic diseases may be the presumed reason for rapid re-regulation of intracortical feedback.
Adjustment disorder after botulinum toxin injection in an adolescent palatal myoclonus case: The importance of informed consent in the treatment of neuropsychiatric disorders in children and adolescents
Published in Alexandria Journal of Medicine, 2018
Yusuf Öztürk, Zehra Topal, Nuran Demir, Ali Evren Tufan
A 14-year-old female high school junior, applied to our outpatient department with a complaint of “clicking sounds” in her ears. It was learned from the history that those sounds began one year ago, involved a tinnitus barely noticeable by the patient at first, gradually became more intense and culminating in being audible by her peers and family 4–5 months ago. The sounds even prevented her falling asleep. There were no other complaints. Baseline mental status examination was normal and there was no family history of psychopathology. A preliminary diagnosis of palatal myoclonus was made and she was directed to the otolaryngology and neurology outpatient clinic where the final diagnosis of essential palatal myoclonus was corroborated. The patient and her parents were informed about the diagnosis but as learned later, were not informed about treatment options, effects and side effects. After a two-week trial of carbamazepine 200 mg/day without benefit, the patient was injected with botulinum toxin. The botulinum toxin injection helped control palatal myoclonus and clicking sounds. However, she applied again to our department a week after the injection with complaints of “continuous crying, not going to school, lack of sleep, social isolation”. It was learned from the history that those complaints started after the injection and that she was unaware of potential side effects of botulinum toxin injection, especially dysphonia. She reported social avoidance due to dysphonia and blamed herself for accepting the treatment. She was hopeless about remission of this side effect and prepared to stay at home for its duration. Evaluation with CGI-S and BDI revealed scores of 4 (moderately impaired) and 12 (mild depressive symptoms); respectively. Mental status examination revealed depressed mood and affect, anhedonia, reduced appetite and sleep. History, mental status examination and psychometric tests supported the diagnosis of adjustment disorder (with depressive mood) according to DSM-IV-TR criteria. Neurology and otolaryngology departments were contacted and detailed information about palatal myoclonus, its treatments and side effects was given. She was reassured that the dysphonia after injectionwill remit within one to two months and sertraline started 50 mg/day. The patient was followed with visits every two weeks. Depressive symptoms remitted dramatically at the second week while dysphonia was still continuing. In the meantime she started attending school after a week of rest at home. At the fourth week dysphonia started to reduce and ceased at the 6th week. Sertraline was continued until the 12th week and was gradually tapered thereafter. At the 16th week the patient was free of symptoms. CGI-S and BDI scores were 1 (Normal) and 1 (no depressive symptoms).