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CASE REPORT
Year : 2019  |  Volume : 25  |  Issue : 3  |  Page : 173-175

A boy with clicking ears


1 Department of Otorhinolaryngology, Head and Neck Surgery, Universiti Sains Malaysia, Health Campus, Kubang Kerian, Kelantan; Department of Otorhinolaryngology, Head and Neck Surgery, Hospital Kuala Lumpur, Wilayah Persekutuan, Malaysia
2 Department of Otorhinolaryngology, Head and Neck Surgery, Hospital Kuala Lumpur, Wilayah Persekutuan, Malaysia
3 Department of Otorhinolaryngology, Head and Neck Surgery, Universiti Sains Malaysia, Health Campus, Kubang Kerian, Kelantan, Malaysia

Date of Submission24-Mar-2019
Date of Acceptance24-Jun-2019
Date of Web Publication18-Oct-2019

Correspondence Address:
Dr. Noor Elyana Ahmad Fawzi
Department of Otorhinolaryngology, Head and Neck Surgery, Universiti Sains Malaysia, Health Campus, Kubang Kerian, Kelantan
Malaysia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.INDIANJOTOL_39_1

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  Abstract 


Middle ear myoclonus is a rare condition which leads to objective tinnitus. We report a 12-year-old boy who presented with bilateral ear tinnitus posttrauma. Endoscopic ear examination reveals rhythmic movement of the tympanic membrane with continuous clicking sound from the bilateral ear; otherwise, other structures were normal. Left endoscopic stapedius and tensor tympani tenotomy were performed and had brought immediate temporary relief for this patient. We discuss the rarity of the disease and the management strategies for this rare condition.

Keywords: Clicking ears, MEM, middle ear myoclonus


How to cite this article:
Fawzi NE, Razuan NA, Hailani I, Ahmad H, Othman NA. A boy with clicking ears. Indian J Otol 2019;25:173-5

How to cite this URL:
Fawzi NE, Razuan NA, Hailani I, Ahmad H, Othman NA. A boy with clicking ears. Indian J Otol [serial online] 2019 [cited 2019 Dec 15];25:173-5. Available from: http://www.indianjotol.org/text.asp?2019/25/3/173/269554




  Introduction Top


Middle ear myoclonus (MEM) is a rare condition with unknown etiology.[1] The usual presentation of this disease is tinnitus which is being described as clicking sound. It can be continuous or intermittent clicking sound. The exact mechanism on how the MEM produces the tinnitus is still not fully understood. However, it has been suggested that the tensor tympani and stapedius muscles undergo repetitive, abnormal contraction, which leads to rhythmical movement of the tympanic membrane.[2] The management can be conservative by utilizing muscle relaxants, anticonvulsants, or just reassurance. The recommended treatment modality is tenotomy (surgical division) of the stapedius and tensor tympani tendon, which has proven to be very effective.[1],[3]


  Case Report Top


A 12-year-old boy presented with fluctuating persistent bilateral objective tinnitus which he describes as clicking sounds for the past 2 years. The tinnitus is progressively becoming louder and worse for the past 1 year. He noted the tinnitus after he had a history of trivial fall from his bicycle 2 years prior; however, he had no other ear symptoms such as ear bleeding, hearing loss, ear pain, or dizziness after the trauma. The tinnitus has been very disturbing and causes him difficulty to concentrate at school, causing psychological withdrawal from his schoolmates. He also had occasionally bilateral ear pain which is worst over the left side. Otherwise, there was no hearing loss, no history of recurrent ear infection, no major trauma to the ear, no aura fullness, no dizziness, no nasal symptoms, and no throat symptoms. He had no other medical illness.

On examination, the patient was generally well. Ear examination showed the presence of rhythmic movement of the tympanic membrane bilaterally, not synchronous with pulse and respiration. The external auditory canals were normal bilaterally. Nasal endoscopy showed that both  Eustachian tube More Details openings were patent and adenoid was not enlarged. Cranial nerve examination was normal, and other systemic examinations were unremarkable.

Tympanogram [Figure 1] showed Type A on the left ear indicating normal middle ear function and Type As on the right ear indicating reduced middle ear compliance. Pure tone audiometry [Figure 2] showed normal hearing (25 dB and better) in both the ears. Magnetic resonance imaging of the brain and internal acoustic meatus were normal. He underwent left endoscopic exploratory tympanotomy. Intraoperatively, the tympanomeatal flap was raised, and middle ear structures were assessed. The stapedius tendon and tensor tympani tendon were identified; however, the muscle contraction cannot be elicited although the muscle relaxant was not given at that point of time. We then proceeded with both stapedius and tensor tympani tenotomy.
Figure 1: Preoperative tympanogram showed Type A on bilateral ears

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Figure 2: Post- and preoperative pure tone audiometry showed normal hearing bilaterally

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Postoperatively, the patient had significant loss of the clicking sound over the left ear. However, it only lasted for 2 weeks. The left ear tinnitus gradually relapsing after 2 weeks; unfortunately, after a month, the tinnitus loudness was as before surgery. Three months postsurgery, the symptoms remains the same. Pure tone audiometry and tympanometry postsurgery for both the ears were the same as before surgery.


  Discussion Top


MEM producing clicking sound can be stressful. In school-age children, this condition may reduce the child concentration in class and thus may severely affect the academic performance. Although the exact etiology of this condition is unknown, some studies have reported that vascular, demyelinating disorder, trauma, and anxiety are the possible causes for MEM.[4] However, most of the cases that were reported have no definite precipitating cause.[5] As in this case, the patient had a history of fall from his bicycle 2 years before the presentation, but no internal or external injury was reported.

The diagnosis for MEM is mainly gained from thorough history and clinical examination. Other differential diagnosis must be excluded such as neurological disorder, infections, and eustachian tube dysfunction. On clinical examination, spontaneous rhythmic movement of tympanic membrane can be elicited, and better view of tympanic membrane can be seen either by endoscopic or microscopic examination. Tympanogram is one of the supportive clinical tests. From tympanogram the “cogwheel effect” can be seen together with repeatable movement of tympanic membrane, below the threshold for acoustic stimulation of the stapedial reflex.[6] Watanabe et al. claimed that tensor tympani muscle contraction produces clicking sounds, whereas stapedius muscle contraction produces buzzing noise.[7] Imaging of the head and neck may be helpful if one cannot exclude a neurological or vascular cause by clinical assessment.[1]

Stapedius muscle is located in the middle ear and its tendon is attached to the neck of the stapes. When the ear is exposed to loud sound, it will stimulate the reflex contraction of the stapedial tendon and this leads to small lift of the stapes footplate away from the oval window. This action dampens the loud sound arriving into the cochlea and thus protects the inner ear from damage. Tensor tympani has the same protective function, but the exact mechanism is not well described.

The management for MEM is still obscure. Few studies and systematic review for the management of this condition have been conducted.[1] The initial treatment should be tailored to patient needs and severity. It is recommended that conservative management is applied first. The examples of conservative managements are zygomatic pressure and medical therapy. The use of anticonvulsant and muscle relaxant has been reported.[4] For the patients who failed medical treatment, surgical intervention is the next option. The suggested surgical treatment is sectioning of the stapedius and tensor tympani tendons and was proven to be effective.[2],[5] Metaanalysis done by Hidaka et al. had shown that out of 23 cases, 21 cases showed full resolution post tenotomy either they underwent selective stapedial tendon, tensor tympani tendon, or both.[3]

This patient had undergone left endoscopic stapedius and tensor tympani tenotomy under general anesthesia. Most of the published studies recommended exploration of the middle ear under local anesthesia as the patient can confirm if the symptom resolves after each individual tenotomy, however since our patient is still young, we opted for general anesthesia.[1]


  Conclusion Top


We describe a case of pediatric age child who presented with clicking sound over bilateral ears secondary to MEM and this has caused psychological disturbance for the patient. The aim of the treatment is to help this patient to overcome the disturbing clicking sound. There are many options of treatment and yet surgical treatment is still the best choice for failure in medical therapy. The diagnosis and treatment must be a holistic approach and based on individual symptoms and preferences. MEM is a rare condition, and the exact management is still obscure. Furthermore, the recurrence of the symptom indicates that the cause of the tinnitus may be multifactorial and not specifically due to the myoclonus.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bhimrao SK, Masterson L, Baguley D. Systematic review of management strategies for middle ear myoclonus. Otolaryngol Head Neck Surg 2012;146:698-706.  Back to cited text no. 1
    
2.
Zipfel TE, Kaza SR, Greene JS. Middle-ear myoclonus. J Laryngol Otol 2000;114:207-9.  Back to cited text no. 2
    
3.
Hidaka H, Honkura Y, Ota J, Gorai S, Kawase T, Kobayashi T. Middle ear myoclonus cured by selective tenotomy of the tensor tympani: Strategies for targeted intervention for middle ear muscles. Otol Neurotol 2013;34:1552-8.  Back to cited text no. 3
    
4.
Golz A, Fradis M, Martzu D, Netzer A, Joachims HZ. Stapedius muscle myoclonus. Ann Otol Rhinol Laryngol 2003;112:522-4.  Back to cited text no. 4
    
5.
Badia L, Parikh A, Brookes GB. Management of middle ear myoclonus. J Laryngol Otol 1994;108:380-2.  Back to cited text no. 5
    
6.
Howsam GD, Sharma A, Lambden SP, Fitzgerald J, Prinsley PR. Bilateral objective tinnitus secondary to congenital middle-ear myoclonus. J Laryngol Otol 2005;119:489-91.  Back to cited text no. 6
    
7.
Watanabe I, Kumagami H, Tsuda Y. Tinnitus due to abnormal contraction of stapedial muscle. An abnormal phenomenon in the course of facial nerve paralysis and its audiological significance. ORL J Otorhinolaryngol Relat Spec 1974;36:217-26.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]



 

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