Research on PM

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        Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Ludwig-Maximilians-University of Munich, Grosshadern, Munich, Germany.
PURPOSE: The objective of this clinical study was to investigate the history and clinical findings in 10 patients having an essential palatal tremor. Furthermore, a botulinum toxin A (BTA) therapy in 5 cases was carried out, and the outcome was analyzed. MATERIALS AND METHODS: Seven adult and 3 pediatric patients with essential palatal tremor were examined at presentation, before and after start of treatment, and every 3 months or when symptoms recurred. Findings were documented by endoscopic video recordings, electromyography, tympanometry, and ear canal microphone recording. The BTA injections were performed in local or general anesthesia, under elecromyographic guidance. RESULTS: The BTA therapy in all 5 patients was successful. Surprisingly, 2 of these patients, aged 10 and 6 years, remained in remission for several years after a single successful injection. CONCLUSION: Botulinum toxin therapy is a safe and effective treatment of essential palatal tremor and seems to be especially useful in pediatric patients. The long lasting effect in children hints toward a pathophysiologic difference between pediatric and adult essential palatal tremor.
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        Victoria C. Chang, MD Neurological Institute, Columbia University Medical Center, 710 West 168th Street, 3rd Floor, New York, NY 10032, USA. vchang@neuro.columbia.edu.
Myoclonus is a hyperkinetic movement disorder characterized by quick, involuntary jerks. It encompasses a vast range of etiologies and widespread anatomic locations. Treatment frequently requires multiple agents and is often only partially beneficial. These patients pose a considerable challenge for the clinician, further complicated by the fact that many of the treatment choices lack evidence-based support. In the past few years, publications regarding therapy have been largely observational case reports or series. Although the literature on treatment of cortical myoclonus appears to be growing, evidence regarding myoclonus of noncortical origin is less well established. Investigation of more satisfactory treatments is needed, as this condition can be disturbing, debilitating, and sometimes harmful for patients. Continuing investigations are using various animal models (mostly of posthypoxic myoclonus), electrophysiologic studies, new imaging techniques such as diffusion tensor imaging, and genetic studies. Meanwhile, the clinical approach to diagnosing and classifying myoclonus remains largely unchanged. This review updates readers on current investigations and suggests guidelines for diagnosing and treating myoclonus.
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        Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.
We report the outcome of botulinum toxin injection for essential palatal myoclonus, given on two occasions over a period of one year, in an eight-year-old boy, the youngest patient treated with botulinum toxin to date. Though there was significant relief of ear clicks each time after the injection, he developed severe palatal palsy following the second injection, which persisted for a month. We suggest that appropriate caution needs to be exercised when repeating botulinum toxin injections for palatal myoclonus in children.(c) 2007 Movement Disorder Society.

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        Integrated Centre for Research and Treatment of Vertigo, Balance and Ocular Motor Disorders, Ludwig-Maximilians University Munich, Marchioninistr. 15, 81377 Munich, Germany.
Previous studies have used low-frequency tones to modulate distortion product otoacoustic emissions (DPOAEs). The cubic DPOAE (CDPOAE) is mostly chosen because amplitudes sufficient for modulation can be evoked with moderate sound pressure levels. Quadratic DPOAEs (QDPOAEs) however, are more sensitive to minute changes of the cochlear operating point (OP) and are better suited to assess changes of the cochlear OP. Here, we compare the properties of low-frequency (30 Hz, 80-120 dB SPL) modulated CDPOAE and QDPOAEs evoked with f(2) = 2 and 5 kHz in human subjects with normal hearing. The modulation depth was quantified with the modulation index (MI), a measure which considers both amplitude and phase. Modulated CDPOAEs evoked with f(2) = 2 kHz have amplitude maxima at the zero crossings and amplitude minima at the extremes of the biasing tone (BT) which correlate positively with the BT level. CDPOAEs evoked with f(2) = 5 kHz were recorded during biasing in exactly the same way as described before. At the highest BT levels used (120 dB SPL), very little modulation could be detected. Not only the depth, but also the shape of the QDPOAE modulation pattern is correlated with the BT level. At moderate BT levels (about 90-100 dB SPL) QDPOAEs evoked with f(2) = 5 kHz show one amplitude notch around the zero crossing of the positive going flank of the BT (a single modulation pattern). At and above a BT level of about 105 dB SPL, the pattern reverses and shows a double modulation pattern. At the highest BT level used (120 dB SPL), quadratic MIs exceed cubic MIs (2.0 ± 0.5 and 0.97 ± 0.06, respectively). Patterns of low-frequency modulated QDPOAEs in humans are similar to the modulation seen in animal studies and as predicted by mathematical models. Human low-frequency modulated QDPOAEs are ideally suited to estimate cochlear OP shifts because of their high sensitivity to the OP shift.
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        Department of Otorhinolaryngology Head and Neck Surgery, Interdisciplinary Centre for Vertigo and Balance Disorders (IFB-LMU), Grosshadern Medical Centre, University of Munich, Munich, Germany.
OBJECTIVES: The aim of this study was to assess whether gadolinium-based contrast agent influences short-term hearing function in patients with Ménière disease undergoing intratympanically enhanced inner-ear magnetic resonance imaging. DESIGN: This is a prospective cohort study. SETTING: This study was conducted a tertiary referral university hospital, ENT department. PARTICIPANTS: In this study, 21 adult patients with definite, unilateral Ménière disease were included. According to the criteria of the Committee on Hearing and Equilibrium, all patients were in stage 1 or 2 of the disease, with largely preserved hearing function. OUTCOMES: All patients underwent clinical and audiologic testing before and 24 hours after intratympanic application of gadolinium-based contrast agent. The effects of the contrast medium on the hearing function were assessed by analysis of frequency thresholds, pure-tone average from 500 Hz to 3 kHz, and speech audiometry. RESULTS: Pure-tone average and single-frequency thresholds in audiometry showed no statistically significant difference after the application of intratympanic gadolinium-based contrast agent. Furthermore, speech audiometry scores remained stable after the application of the contrast agent. CONCLUSIONS: This study did not demonstrate clinically significant short-term effects of intratympanic application of gadolinium-based contrast agent on hearing function in patients with Ménière disease in initial stages.
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        Department of Otorhinolaryngology Head and Neck Surgery, Grosshadern Medical Centre, University of Munich, Marchioninistr. 15, 81377, Munich, Germany, rguerkov@med.uni-muenchen.de.
Our objective is to determine whether the degree of endolymphatic hydrops as it is detected in vivo in patients with definite Meniere’s disease correlates with audiovestibular function. In this prospective study, 37 patients with definite Meniere’s disease according to AAO-HNS criteria were included. Intratympanic contrast enhanced temporal bone MRI was performed using a 3D FLAIR protocol. The degree of endolymphatic hydrops in the cochlea and the vestibulum was graded on a Likert scale (0-3). The degree of hydrops was then analyzed with respect to its correlation with audiometric hearing levels, electrocochleographic SP/AP ratios, interaural amplitude ratios of vestibular evoked myogenic potentials and degree of horizontal semicircular canal paresis on caloric irrigation. There was a significant correlation between the degree of hydrops on the one hand and the averaged hearing level at 0.25-1 and 0.5-3 kHz and the vestibular evoked myogenic potential interaural amplitude ratio on the other hand. A trend toward a correlation was noticed between the hydrops and the caloric response, no correlation was noticed between the hydrops and the SP/AP ratio. The degree of endolymphatic hydrops correlates with a progressive loss of auditory and sacculus function in patients with Meniere`s disease.
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PURPOSE: Botulinum neurotoxin A (BTA) is a promising therapeutic option in the treatment of idiopathic rhinitis (IR), a disease characterized by nasal obstruction and hydrous rhinorrhea. The conventional localization for the injection of BTA in IR is the nasal turbinates. In our own clinical experience, submucoperichondrial injection of BTA in the nasal septum is an alternative that is easy to perform for the therapist and also well tolerated by the patient. MATERIAL AND METHODS: Five patients received an injection of in total 80 mouse units Dysport (Ipsen Pharma, Ettlingen, Germany) in the nasal septum. The unpleasantness of the nasal injection of BTA was measured on a visual analogue scale. Over the course of 14 days, nasal symptoms (rhinorrhea, nasal obstruction, urge to sneeze, nasal pruritus), the number of facial tissues used daily, and possible complications were evaluated. RESULTS: The unpleasantness of the injection of BTA into the nasal septum after local anesthesia was rated low (visual analogue scale, 0.76 on average). A good subjective symptom control was achieved in 3 patients concerning rhinorrhea and in all patients concerning nasal obstruction. The number of facial tissues used daily as a parameter for rhinorrhea was on average 21.0 before the injection of BTA, decreased in 4 patients over the course of time, and was on average 5.8 after 14 days. No patient reported any adverse effects after the injection of BTA. CONCLUSIONS: This pilot study demonstrates that septal injection of BTA in patients with IR can achieve good symptom control and patient comfort and should be compared in further studies to the conventional turbinal injection technique.
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        Department of Otorhinolaryngology, Head and Neck Surgery, Ludwig Maximilian University, Marchioninistrasse 15, 81377, Munich, Germany. thomas.braun@med.uni-muenchen.de
The objective of the study was to evaluate patient benefit and health-related quality of life after use of botulinum neurotoxin (BoNT) A for various otorhinolaryngological, functional (non-cosmetic) indications. The design consisted of a survey study of a patient cohort (n = 40) treated with BoNT A for functional indications. Patients were asked to answer the Glasgow Benefit Inventory (GBI), a retrospective questionnaire well validated for measuring the effect of otorhinolaryngological interventions on the health-related quality of life. GBI scores can range from -100 (maximal adverse effect), through 0 (no effect), to 100 (maximal positive effect). A total of 29 patients (72.5%) returned a valid questionnaire. Mean total GBI scores for the particular indications were 1.2 (sialorrhea, n = 7), 22.6 (gustatory sweating, n = 8), 20.6 (palatal tremor, n = 5), 15.0 (postlaryngectomy voice disorders due to pharyngoesophageal spasm, n = 5), 38.9 (adductor spasmodic dysphonia, n = 2) and 27.8 (oromandibular dystonia, n = 2), showing a mean overall positive effect of BoNT A treatment on the health-related quality of life, respectively. A varying percentage of patients reported an increase in their health-related quality of life, indicated by positive total GBI scores: sialorrhea 28.6%, gustatory sweating 87.5%, palatal tremor 60%, postlaryngectomy voice disorders 60%, spasmodic dysphonia 100% and oromandibular dystonia 100%. Use of BoNT A can be considered an effective therapeutic option for all the indications investigated. However, the possibility of raising patients’ health-related quality of life with this kind of therapy varies significantly for different indications. Further studies are needed to analyze the patients who will benefit most from a treatment with BoNT A.
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        Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Ludwig-Maximilians-University, Munich, Germany. Eike.Krause@med.uni-muenchen.de
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        Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Ludwig-Maximilians-University of Munich, Grosshadern, Munich, Germany.
PURPOSE: The objective of this clinical study was to investigate the history and clinical findings in 10 patients having an essential palatal tremor. Furthermore, a botulinum toxin A (BTA) therapy in 5 cases was carried out, and the outcome was analyzed. MATERIALS AND METHODS: Seven adult and 3 pediatric patients with essential palatal tremor were examined at presentation, before and after start of treatment, and every 3 months or when symptoms recurred. Findings were documented by endoscopic video recordings, electromyography, tympanometry, and ear canal microphone recording. The BTA injections were performed in local or general anesthesia, under elecromyographic guidance. RESULTS: The BTA therapy in all 5 patients was successful. Surprisingly, 2 of these patients, aged 10 and 6 years, remained in remission for several years after a single successful injection. CONCLUSION: Botulinum toxin therapy is a safe and effective treatment of essential palatal tremor and seems to be especially useful in pediatric patients. The long lasting effect in children hints toward a pathophysiologic difference between pediatric and adult essential palatal tremor.
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Tardive dyskinesia syndromes in the head and neck region may appear as adverse effects of psychopharmacotherapy. They are caused by blockage of central dopamine receptors. Oftentimes, these disorders persist even after discontinuation of the antipsychotic medication, and they are disabling the patients functionally and psychosocially. Medical therapeutic efforts with different psychopharmaceuticals, benzodiazepines or vitamin-E-preparations are frequently unsuccessful. Local application of botulinum neurotoxin A offers a new treatment modality, which can target the overshooting dysfunction directly at the peripheral muscle.
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        Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Ludwig-Maximilians-University Munich, Munich, Germany. eike.krause@med.uni-muenchen.de
PURPOSE: Laryngectomized patients with pharyngoesophageal spasm frequently have poor voice quality and dysphagia. Local botulinum toxin A (BTA) injection can relieve muscular hypertonicity and improve symptoms. This procedure should also prolong the functional life span of the tracheoesophageal voice prosthesis. MATERIALS AND METHODS: This study evaluates 33 BTA treatments in 11 laryngectomees. All patients were having poor voice quality; 6 patients had additional dysphagia. In 10 patients, the BTA injection has been carried out during rigid pharyngoscopy under general anesthesia. One patient was treated in local anesthesia. RESULTS: A subjective improvement of voice quality was reported in 94%. This lasted on average for 20 weeks. The swallowing function improved moderately. For the first time, the functional life span of voice prostheses was examined. After treatment of pharyngoesophageal spasm, their durability was almost tripled. The BTA therapy has a significant effect. CONCLUSIONS: The BTA treatment improves voice quality and prolongs functional durability of voice prostheses in laryngectomees with pharyngoesophageal spasm. The success of treatment is of limited duration but can be repeated in the long-term.
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        Department of Neurology, Ludwig-Maximilians University, Klinikum Grosshadern, Marchioninistrasse 15, D-81366 Munich, Germany. judith.wagner@med.uni-muenchen.de

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        Department of Pediatrics, Section of Pediatric Otolaryngology, Inova Fairfax Hospital for Children, Falls Church, VA, USA. Rhs738@aol.com
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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        Servicio Otorrinolaringología, Hospital Clínico Universitario, Valencia, España.
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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        Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, London, UK.
Myoclonus is a sudden, brief, involuntary muscle jerk. It is caused by abrupt muscle contraction, in the case of positive myoclonus, or by sudden cessation of ongoing muscular activity, in the case of negative myoclonus (NM). Myoclonus may be classified in a number of ways, although classification based on the underlying physiology is the most useful from the therapeutic viewpoint. Given the large number of possible causes of myoclonus, it is essential to take a good history, to clinically characterize myoclonus and to look for additional findings on examination in order to limit the list of possible investigations. With regards to the history, the age of onset, the character of myoclonus, precipitating or alleviating factors, family history and associated symptoms and signs are important. On examination, it is important to see whether the myoclonus appears at rest, on keeping posture or during action, to note the distribution of jerks and to look for the stimulus sensitivity. Electrophysiological tests are very helpful in determining whether myoclonus is cortical, subcortical or spinal. A single pharmacological agent rarely control myoclonus and therefore polytherapy with a combination of drugs, often in large dosages, is usually needed. Generally, antiepileptic drugs such as valproate, levetiracetam and piracetam are effective in cortical myoclonus, but less effective in other forms of myoclonus. Clonazepam may be helpful with all types of myoclonus. Focal and segmental myoclonus, irrespective of its origin, may be treated with botulinum toxin injections, with variable success.
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        Department of Colorectal Surgery, Cleveland Clinic Florida, Weston, Florida.
Paradoxical puborectalis contraction and increased perineal descent are two forms of functional constipation presenting as challenging diagnostic and treatment dilemmas to the clinician. In the evaluation of these disorders, the clinician should take special care to exclude anatomic disorders leading to constipation. Physical examination is supplemented by additional diagnostic modalities such as cinedefecography, electromyography, manometry, and pudendal nerve tefninal motor latency. Generally, these investigations should be used in combination with the two playing the more relied upon techniques. Treatment is typically conservative with biofeedback playing a principal role with favorable results when patient compliance is emphasized. When considering paradoxical puborectalis contraction, failure of biofeedback is usually augmented with botulinum toxin injection. Increased perineal descent is generally treated with biofeedback and perineal support maneuvers. Surgery has little or no role in these conditions. The patient who insists on surgical intervention for either of these two conditions should be offered a stoma.
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        Fondation Adolphe de Rothschild, Unité d’Otologie-Otoneurologie, Paris, France.
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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        Department of Thoracic Surgery, Glenfield Hospital, University Hospitals of Leicester, Leicester, United Kingdom.
Myoclonus as a sequel to thoracotomy has been reported, and its treatment can be challenging to both the patient and the surgeon. We describe a 43-year-old patient with chest wall pain and latissimus dorsi muscle contractions (myoclonus) after video-assisted thoracoscopic lung volume reduction. His symptoms remained refractory to benzodiazepines, nerve blockage, and botulinum toxin injection due to either poor compliance or lack of response to therapy. These symptoms started to resolve spontaneously 18 months after the procedure.
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        Assistant Professor of General Surgery, Department of Surgery, Catholic School of Medicine, University Hospital ‘Agostino Gemelli’, Largo Agostino Gemelli 8, 00168 Rome, Italy. gbrisin@tin.it , Research Fellow of General Surgery, Department of Surgery,’Tor Vergata’ University Hospital, Viale Oxford 81, 00133 Rome, Italy. fede.cadeddu@libero.it , Resident in General Surgery, Department of Surgery, Catholic School of Medicine, University Hospital ‘Agostino Gemelli’, Largo Agostino Gemelli 8, 00168 Rome, Italy. pacomaz81@yahoo.it , Assistant Professor of General Surgery, Department of Surgery, Catholic School of Medicine, University Hospital ‘Agostino Gemelli’, Largo Agostino Gemelli 8, 00168 Rome, Italy. giorgio.maria@rm.unicatt.it.
Since its introduction for the treatment of strabismus, botulinum toxin (BoNT) has been increasingly used in the treatment of several disorders with excessive or inappropriate muscle contractions. The therapeutic effects of BoNT occur through the temporary chemodenervation caused by the injection into the local target muscle or skin. Modulation of muscle relaxation may be achieved by varying the dose of BoNT solution injected; most adverse effects are transient. Indeed, botulinum neurotoxin has been used to selectively weaken the internal anal sphincter as a treatment for chronic anal fissure in several randomized, controlled trials and open-label studies. The use of botulinum neurotoxin seems to be an effective and safe approach for the treatment of chronic anal fissure, particularly in patients at high risk for incontinence.
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        Service d’ORL Pédiatrique, CHU Timone, Marseille, France.
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.
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We report a 30-year-old man with moving ear syndrome caused by focal myoclonic jerks of the right temporal muscle. This focal myoclonus would disappear while the patient was sleeping, swallowing, or speaking. He was treated with botulinum toxin type A with a favorable outcome. Previous reports of this condition and possible therapeutic approaches are discussed.(c) 2007 Movement Disorder Society.
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        Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.

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