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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 28  |  Issue : 2  |  Page : 156-159

Palatal tic disorder causing objective clicking tinnitus in an 8 years old


Department of ENT, Raja Rajeswari Medical College, Bengaluru, Karnataka, India

Date of Submission07-Jul-2021
Date of Acceptance19-Oct-2021
Date of Web Publication21-Sep-2022

Correspondence Address:
Dr. Rajiv Ranganath Sanji
85, 6th Cross, AG's Layout, New BEL Road, Bengaluru - 560 054, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.indianjotol_98_21

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  Abstract 


Palatal tremors including myoclonus are well-known causes of objective tinnitus. Essential palatal tremors and tic disorders are recognized but rarer causes of objective pulsatile tinnitus. An 8-year-old boy presented with a chief complaint of clicking sounds in both the ears for 1 month, intermittent, occurs frequently in episodes, wherein intraoral examination revealed bilateral rhythmic, low frequency, symmetrical contractions of the soft palate muscles accompanied by clicking sounds audible to physician as well (objective tinnitus). The child's mother stated that the clicking sounds were not present when he was asleep. When the child was distracted by tuning fork sound, the palatal movements stopped. CNS examination, developmental history, birth, and growth history were all normal. Magnetic resonance imaging study with contrast enhancement was normal. The child was started on clonazepam and clonidine and follow-up was done after 1st and 2nd months. The tinnitus reduced but did not resolve completely. Clinical features of a patient with palatal tremor should be carefully assessed with prior knowledge of possible etiopathologies to guide the investigations and management. We highlight a case of palatal tic disorder to bring the possibilities quickly to the reader's mind.

Keywords: Essential tremor, palate, tic disorders, tinnitus, tremor


How to cite this article:
Kumar N M, Sanji RR, Krishnaswamy S. Palatal tic disorder causing objective clicking tinnitus in an 8 years old. Indian J Otol 2022;28:156-9

How to cite this URL:
Kumar N M, Sanji RR, Krishnaswamy S. Palatal tic disorder causing objective clicking tinnitus in an 8 years old. Indian J Otol [serial online] 2022 [cited 2022 Nov 27];28:156-9. Available from: https://www.indianjotol.org/text.asp?2022/28/2/156/356459




  Introduction Top


Objective tinnitus can occur from perception of an abnormal perception of a normal somatosound or from perception of an abnormal somatosound. Objective tinnitus is less common than subjective tinnitus but can be easily distract the physician with many differential diagnoses.

Causes of objective tinnitus are divided into vascular (pulsatile) causes such as arteriovenous malformations, vascular tumors, venous hum (cardiac murmurs, anemia, benign intracranial hypertension, thyrotoxicosis, pregnancy, dehiscent jugular bulb), atherosclerosis, ectopic carotid artery, persistent stapedial artery, vascular loops; and neuromuscular causes such as palatomyoclonus, stapedial muscle spasm, and patulous  Eustachian tube More Details.

Palatal myoclonus is one of the prominent causes of objective tinnitus. Palatal tic disorder is an unusual palatal cause of objective pulsatile tinnitus, a rare neurological disorder of the soft palate and other oropharyngeal muscles characterized by rhythmical, involuntary contractions, and oscillatory movements of the soft palate at fixed amplitude and frequency.[1] It may be easily confused with palatal myoclonus by the clinician. We present a rare case of palatal tic disorder to highlight this rare condition. Treatment differs from case to case, different modalities have been used, and some patients have responded to clonazepam, carbamazepine, trihexyphenidyl, anticonvulsants, and anxiolytics. At present, no specific treatment has been established; although successful treatment by botulinum has recently been reported, it has been reported to decrease the episodes of tremors – not resolving completely.


  Case Report Top


A previously healthy 8-year-old boy was presented in the otorhinolaryngology outpatient department with clicking sound in both ears for 1 month. When he told his mother about the sound, she could also hear it. The mother was not able to hear the sound when her son was asleep. Birth history, growth history, and developmental history were normal; he was of nonconsanguineous union. He had no history of head trauma, chronic ear disease, or other neurologic symptoms. There was no significant family history; mother's pregnancy and delivery had been normal and without complications, immunization history was up to date; there was no history of any drug intake.

Clinical examination revealed involuntary, bilateral rhythmic, low frequency, symmetrical contractions of the soft palate muscles accompanied by clicking sounds synchronous with an audible click – at a rate of about 150/min. The clicking sound could easily be heard by the examiner when observed closely within 15–20 cm with the patient [Figure 1]. The sound did not decrease with change in head position, Valsalva maneuver. However, when the child was distracted with a tuning fork sound to the ear, the palatal tic stopped immediately [Figure 2] and resumed afterward. No ataxia or nystagmus was present. The tinnitus was present with the mouth both open and closed. No other facial or oropharyngeal movements were evident. A detailed cranial nerve examination was done, and it was normal. Pediatric neurologist was consulted, and detailed neurological examination was done, and it was reported normal. A psychodiagnostic assessment was done after consulting psychiatrist. Yale-Brown Obsessive Compulsive Scale was used to rule out obsessive compulsive disorder. Conners' scale was used to rule out ADHD.
Figure 1: A series of photographs showing symmetrical bilateral palate contraction

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Figure 2: Sudden disappearance of palatal tremors when child was distracted and tuning fork was placed along the ear

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Tympanogram showed reduced middle ear pressure (-6daPa) in the right ear compared to left (33daPa); compliance was also reduced. A magnetic resonance imaging (MRI) of the brain was done, with special attention to the brainstem, including axial fluid-attenuated inversion-recovery, diffusion-weighted imaging and focus on posterior fossa and cerebellopontine angle. It was reported normal [Figure 3]. Electroencephalogram was also done reported normal.
Figure 3: Magnetic resonance imaging study

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Pediatric neurologist was consulted and after evaluation of all the parameters, he was started on clonidine 100 mcg and clonazepam 0.25 mg for a month. A provisional diagnosis of essential palatal tremor (EPT)/palatal tic disorder was established. The child was evaluated every 2 weeks, and by the end of 2 months, the child's palatal tremor was significantly reduced. The mother also reported reduction in the objective tinnitus.


  Discussion Top


Palatal tic disorder is one of the missed causes of palatal tremor. It presents as a visible periodic elevation of the palate and uvula and may include tremors of other oropharyngeal muscles. In this case, the palatal tremor stopped immediately once the patient was distracted by using a tuning fork which was highly suggestive of the diagnosis. It is crucial to rule out any structural lesions (e.g., inferior olivary hypertrophy) before diagnosing palatal tic disorder. Etiology of objective tinnitus can be muscular or vascular. Tinnitus of muscular origin has no relation with the pulse, is higher pitched, and has a clicking quality in comparison to sounds of vascular origin. These features may be used to clinically differentiate the diagnoses.

In this case, the palatal movements were bilateral, symmetrical, rhythmic, unrelated to the pulse, and involving the whole palate. In contrast, the tremors of palatal myoclonus are nonrhythmic, asymmetrical and involve bundles of muscle fibers. These clinical features enabled us to differentiate between the two.

The distraction test – where in the tinnitus as well as palatal tremor were both stopped once the tuning fork was struck and placed before his ear [Figure 2] – was useful to develop a clinical suspicion of EPT in this case.

When the soft palate is involved, abrupt, rhythmic, anteroposterior, and vertical movements are present. This rhythmic activity can open and close the eustachian tubes, thereby producing a clicking sound transmitted to the middle ear. The clicking sound is the most disturbing feature of this illness and can result in a severe inability to initiate sleep.[2] It is postulated that tremors of the soft palate produce secondary movements of the eustachian tube which results in the clicking sound. Vascular tinnitus typically coincides with the pulse, may increase with exercise, and subside with ipsilateral carotid artery compression. The origin of palatal tremor may be a disturbance in the upper brainstem (Mollaret's triangle).[3] Hence, the patient should be evaluated clinically and radiologically to rule out all other central causes including brainstem lesions, vascular causes, and neuromuscular causes.[4] As cases of palatal tic disorder have been found to be associated with central functional disorders like ADHD, these need to be ruled out before planning treatment.

Three types of palatal tremor are described in the literature, EPT, symptomatic palatal tremor (SPT), progressive ataxia, and palatal tremor (PAPT) [[Table 1] summarizes the differences].[5] EPT reveals no underlying structural pathology; SPT happens due to any lesion secondary to brainstem or cerebellar disease.[6] PAPT is a special subtype of SPT where the ataxia worsens irrespective of the tremor.[7]
Table 1: Summary of differences of essential palatal tremor, symptomatic palatal tremor, and psychogenic palatal tremor

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Clinical evaluation of such a case includes eliciting a detailed history, recording clinical signs and symptoms; audiological assessment, videorecording of the palatal myoclonus, MRI of the brain, with contrast, and consultation with a pediatric otolaryngologist, and pediatric neurologist or neurosurgeon if a brainstem lesion is suspected or confirmed.

When palatal tic disorder is proven to be under volitional control, simple firm suggestion therapy placebo therapy, or medical hypnosis may be useful. Treatment of essential nonvolitional palatal tic has included anticonvulsants such as sodium valproate, carbamazepine, or clonazepam.[8] Although many other pharmaceutical therapies, including cannabis, have been reported, results with these drugs have been disappointing, with a paucity of reproducible success. Botulinum toxin therapy has shown significant improvement as well as superior results, however, the cost and its side effects remain its Achilles heel in clinical practice.


  Conclusion Top


Palatal tremors are unusual causes of objective pulsatile tinnitus. Clinical features of a patient with palatal tremor should be carefully assessed with prior knowledge of possible etiopathologies to guide the investigations and management. This case of palatal tic disorder highlights important clinical differences of palatal tics and reminds us to keep in mind such unusual causes while managing relatively uncommon conditions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Blitzer A, Sadoughi B, Guardiani E. Neurologic disorders of the larynx. Cummings Otolaryngology Head and Neck Surgery. 6th ed. Philadelphia: Saunders; 2009. p. 860-67.  Back to cited text no. 1
    
2.
Ludlow, Christy, Bielamowicz, Steven. Neurogenic Disorders of the Larynx. Ballengers Otorhinolaryngology Head and Neck Surgery. 17th ed. USA: BC Decker Inc.; 2009. p. 913-21.  Back to cited text no. 2
    
3.
MacDonald JT. Objective tinnitus due to essential palatal tremor in a 5-year-old. Pediatr Neurol 2007;36:175-6.  Back to cited text no. 3
    
4.
Selvadurai C, Schaefer SM. Focal unilateral palatal myoclonus causing objective clicking tinnitus without uvula elevation diagnosed by concurrent auscultation. J Mov Disord 2020;13:223-4.  Back to cited text no. 4
    
5.
Margari F, Giannella G, Lecce PA, Fanizzi P, Toto M, Margari L. A childhood case of symptomatic essential and psychogenic palatal tremor. Neuropsychiatr Dis Treat 2011;7:223-7.  Back to cited text no. 5
    
6.
Schwartz RH, Bahadori RS, Myseros JS. Loud clicking sounds associated with rapid soft palate muscle contractions. Pediatr Emerg Care 2012;28:158-9.  Back to cited text no. 6
    
7.
Bhattacharjee S. Palatal tremor – Pathophysiology, clinical features, investigations, management and future challenges. Tremor Other Hyperkinet Mov (N Y) 2020;10:40.  Back to cited text no. 7
    
8.
Jero J, Salmi T. Palatal myoclonus and clicking tinnitus in a 12-year-old girl-case report. Acta Otolaryngol Suppl 2000;543:61-2.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

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