CPC - bilateral ear discharge and unilateral intermittent facial deviation.pptx

Education ear

1. CPC March 2021 Department of ENT Clinical case discussion: Dr Drashty Bavarva 2 nd year PG resident Dr Suktara Sharma Associate Professor

2. H istory 44 year old female presented with chief complains of:- - b/l earache - 8 years - b/L intermittent ear discharge - 4 years - b/L decreased hearing -3 years -mild giddiness - 3 months - Ringing sensation in the right year -1 month

3. History of presenting illness Patient was apparently asymptomatic 8 years back b/l Earache - mild, dull aching, about once a month, relived without any medication b/l Ear discharge - intermittent, scanty, purulent, foul smelling b/l Hearing loss -gradually progressing [Right>left] Giddness - mild unsteadiness while walking -3 month Rt Tinnitus - continuous ringing sensation - 1month

4. Negative history No history of: -headache -nausea and vomiting -fever - neck pain/neck stiffness In 8 last years, patient consulted 3-4 ENT surgeons for the same, was temporarily relieved with medications and was advised surgery at every visit. Records not available!

5. Family history - not significant Past history - not significant Personal history – known case of hypertension, diagnosed 3 months back, on treatment

6. General physical examination Patient was oriented to time place and person, well built and nourished Temperature- afebrile BP- 130/84 mmHg WNL Pulse –82/min Respiratory rate –14/min No cyanosis, clubbing, anemia, jaundice, lymphadenopathy or neck swelling.

7. Systemic examination Respiratory system – Air entry B/l equal no abnormal or added sounds on auscultation Cardiovascular system - S1, S2 normal, no murmurs Gastrointestinal system : Per abdomen- non tender and soft Umblicus - centrally placed No dilated veins or discharge present No palable lump present

8. Investigations Hb 12.6 HIV/ HBsAg Non reactive RBC 4.84 SGPT 24.50 WBC 6400 Blood grp A + ve Platelet 281000 Serum eletrolytes : S bilirubin 0.41 Na 136.60 S . creatinine 0.98 K 4.14 RBS 94 Cl 102.20

9. Local examination External ear- Pinna - Pre-auricular region - Post-auricular region B/L normal - Post-auricular sulcus - External auditory canal Fistula test –B/L negative Tympanic membrane

10. Pars flaccida Cone of light Handle of malleus Pars tensa

11. Otoendoscopic examination Right ear – Grade 4 retraction pocket involving attic and PSQ filled with cholesteatoma Left ear -Grade 4 retraction involving attic and PSQ with cholesteatoma Retraction pocket Cholesteatoma Retraction pocket and Cholesteatoma in PSQ Cholesteoma flakes

12. Pure tone Audiogram Frequency

13. Pure tone Audiogram Right ear- profound sensorineural hearing loss Left ear- moderate conductive hearing loss

14. Diagnosis Bilateral Chronic Otitis Media Unsafe type/ Squamous type Surgical plan Modified Radical Mastoidectomy of Rt ear followed by Lt ear

15. Consent for Surgery Possibility of facial N palsy Recurrence of disease Unlikely improvement in hearing Patient has been suffering from intermittent facial deviations for last one month

16. Review of history Painless Intermittent spontaneous deviation of the right side of face mainly involving the angle of mouth and the eyes. Intensity of the deviations varied from episode to episode Each episode lasts about 20-30 minutes, occurring once every 2-3 days Each episode subsides by itself without any medications Patient consulted a physician and ENT surgeon for the same.

17. Frequency of facial nerve dysfunction in COM ranges from 0.16 to 5.1% Transient facial nerve dysfunction due to COM/ Cholesteatoma is rare Reversal of facial paralysis due to chlosteatoma without surgical intervention is unlikely Ref: Facial Nerve Paralysis due to Chronic Otitis Media: Prognosis in Restoration of Facial Function after Surgical Intervention Yonsei Med J. 2012 May 1; 53(3): 642–648. So, why did this history surprise us?

18. Are we missing something? The video is been shown only after an explicit written consent from the patient

19. Explicit written consent has been taken from the patient for all photographs shown for medical teaching purpose only

20. Most likely bilateral, only involves muscles around eye Involves lower part of face Involves lower part of face typically can be suppressed occures in context of voluntary movement Did not progress to other parts / no secondary generalisation Hemifacial spasm Blepharospasm Hemimasticatory spasm Oromandibular dystonia Facial nerve tics Synkinesias post facial palsy Focal seizures Possibilities Ref : Hemifacial spasm: conservative and surgical treatment options . Dtsch Arztebl Int.  Review Article 2012 Oct; 109(41): 667–673.

21. Hemifacial spasm Hemifacial spasm is a movement disorder affecting the muscles innervated by the facial nerve resulting in involuntary tonic or clonic contractions of the facial muscles, which are almost always unilateral Most common cause: compression of facial nerve at the root exit zone of the brainstem by an aberrant blood vessel Rare causes : SOL like meningiomas , arachnoid cysts and schwannomas Hemifacial spasm: conservative and surgical treatment options . Dtsch Arztebl Int.  Review Article 2012 Oct; 109(41): 667–673.

22. CNS Examination Higher Mental Functions- Normal Cranial Nerves I IX II X III Normal XI Normal IV XII VI VII nerve examination was normal

23. Trigeminal nerve Motor : normal Sensory: Reduced touch, pain and temperature sensation along Right side of face involving V1,V2&V3   Corneal reflex: Ey e tested Direct Consensual Right Absent Absent Left Present Present

24. Corneal reflex arc Peripherally lesions causes ipsilateral reflex deficits while supranuclear lesions have bilateral deficits

25. Vestibulocochlear nerve

26. Vestibular component of VIIIth nerve Stance and gait examinatin : normal Nystagmus : absent Caloric testing- contraindicated in CSOM Head thrust test: positive RT suggest hypofunction of the RT labyrinth Romberg – negative VNG/ Rotatory chair testing/ computerised dynamic posturography : not available

27. CNS examination contd … Motor Examination: bulk, tone and power – Normal Rest of sensory examination normal except Rt Trigeminal component Deep Tendon Refexes : Normal B/L Planter – Normal Cerebellar sign absent Romberg / Sharpened Romberg: negative Gait/Stance / coordination: normal

28. How is trigeminal N related to ear ? Petrous apex Temporal bone Petrous apex Internal acoustic meatus Internal acoustic meatus Anatomical jewel box

29. Relationship of V, VII &VIII nerves to petrous apex Trigeminal ganglion Internal acoustic meatus

30. Differentials of diseases affecting the petrous apex CSOM/ tubercular otitis media Cholesteatoma Cholesterol granuloma Petrous apicitis Benign tumors/malignant tumors Vascular lesions- carotid aneurysms, AV fistula Fibrous dysplasias

31. HRCT Temporal bone Loss of cellularity Soft tissue lesion in the middle ear attic and mastoid cortex Petrous apex Internal auditory canal Right

32. Soft tissue lesion in the middle ear attic and mastoid cortex Petrous apex Internal auditory canal left

33. Soft tissue lesion with collection involving B/L middle ear cavity and extending into mastoid air cells with erosion of ossicular chain and erosion of temporal bone suggestive of chronic otomastoiditis probably cholesteatoma . The petrous pyramids appear normal and symmetrical on both sides Rt internal acoustic meatus appears dilated 7mm Right V/S 4mm Left HRCT temporal bone report

34. Contrast MRI Brain Cerebellopontine angle tumor

35. MRI report Extraaxial altered signal intensity lesion of size approx 22 x 18 x 20 mm involving right cerebello pontine angle with its internal acoustic component measuring 11 mm causing widening of right internal acoustic meatus . The lesion appears hypointense on T1WI and heterogeneously hyperintense on T2W and FLAIR images. The lesion abuts the cerebellar hemisphere , middle , lower cerebellar peduncle and pons on right side with preserved fat plane. On post contrast study the lesion does not shows any significant post contrast enhancement.  

36. Vestibular schwanoma / Acoustic neuroma / Neruliemmoma 80% CPA tumour Bengin , encapsulated extremely slow growing. After eroding and widening internal acoustic canal it grows into the CP angle Anterosuperiorly , it involves the CN V and inferiorly involves the CN IX, X and XI, which lie in in jugular foramen.

37. Classification of CP angle tumors

38. Mangement Surgery Middle cranial fossa Supralabyrinthine Translabyrinthine Retrolabyrinthine Suboccipital / retrosigmoid Gamma knife surgery High dose of ionizing radiation to the tumor and minimizes its effect on the surrounding neural tissue, arrest of tumor growth and reduction in size.

39. Good surgeons know how to operate, better ones when to operate, and the best when not to operate We have two diseases in the body which are potentially life threatening Which do we treat first?

40. The Journal of Laryngology & Otology  ,  Volume 123  ,  Issue 5  , May 2009 , pp. 502 - 508 Analysis of causes for late presentation of Indian patients with vestibular Schwannoma Delay between first consultation and final diagnosis: 32.2 ± 38.9 months vs 15 months

41. Several disorders and environmental substances can influence the corneal blink reflex Infectious diseases  Type 1 herpes simplex   Varicella zoster virus   Mycobacterium leprae  Fungal infectionsAutoimmune disorder  Diabetes mellitus  Grave’s disease   Sjögren’s syndromeOphthalmic procedures and surgeries  Corneal transplant  Laser and other ocular surgeries including laser-assisted in situ keratomileusis (LASIK)  Photorefractive keratectomy (PRK)Ophthalmic medication   Antiglaucoma topical medication especially topical beta-adrenergic antagonists   Benzalkonium chlorideAge  Advanced age

42.  Notwithstanding, the presence of cholesteatoma decreased the effectiveness of surgical gain and indicated a poor prognosis after surgery. It is thought that direct inflammation of the nerve, by bacteria or by neurotoxic substances secreted from the cholesteatoma matrix, is the leading factor causing deterioration. In conclusion, COM resulting in facial nerve paralysis is most frequently due to cholesteatoma , and the presence of cholesteatoma decreased the effectiveness of surgical gain indicating poor prognoses after surgery. 

43. Hemifacial spasm is a movement disorder affecting the muscles innervated by the facial nerve. The result is involuntary tonic or clonic contractions of the facial muscles, which are almost always unilateral. The cause in most cases is a compression of the facial nerve in its root-exit zone from the brainstem by a vessel with an aberrant or ectatic blood vessel. The diagnosis is led by the clinical features. Important in deciding treatment is the differential diagnostic differentiation from other craniofacial movement disorders. Magnetic resonance imaging using CISS sequence is helpful for diagnosing a possible vascular compression or other intracranial cause. The most important symptomatic treatment is local injection of botulinum toxin. In more than 85% of patients this leads to a notable alleviation of symptoms. The disadvantage associated with his procedure is the fact that repeated administration at intervals of several months is necessary. Microvascular decompression surgery is the only causal treatment option with a success rate of about 85% in terms of permanent freedom of symptoms.

44. In contrast to the strictly unilateral symptoms of hemifacial spasm, blepharospasm presents with bilateral, involuntary, mostly synchronous, symmetrical contractions of the eyelids. Oromandibular dystonia presents with involuntary, repeated, sustained muscle contractions that affect primarily the lower part of the face, the mouth, the mandible and maxilla, the tongue, and the pharynx. Facial nerve tic presents with more complex, coordinated, multifocal movement patterns and switches between the right and the left of the face. In contrast to hemifacial spasm, the tics can typically be suppressed ( 6 ). Simple focal seizures can also be confused with hemifacial spasm if they affect the facial muscles. Hemimasticatory spasm describes painful contractions of the muscles of mastication. Synkinesias after facial nerve paralysis also lead to activation of several muscles innervated by the facial nerve. Typically this only occurs in the context of voluntary movement.

45. Table 61.1Location of Central Trigeminal Lesions Functional lossLocationStructure (s)Pain, temperature, touch over entire body, including face ipsilaterallyLateral rostral pons and aboveSpinothalamic ventral trigeminal tracts contralaterallyMasticatory muscle paralysis and pain, temperature, touch over face ipsilaterallyMidponsMain sensory nucleus, motor nucleus, and entering root fibers ipsilaterallyPain , temperature over face ipsilaterally ; pain, temperature over body (and occasionally face) contralaterallyLateral inferior pons or lateral medullaSpinal tract and spinal tract nucleus ipsilaterally ; spinothalamic tract and occasionally ventral trigeminal tract contralaterally

46. The nasociliary and supraorbital branch of the ophthalmic division of the trigeminal nerve gives origin to the afferent innervation for the corneal and blink reflexes, respectively, while the efferent motor response is interceded via branch of the facial nerve to the orbicularis oculi muscle. Hence, these reflexes are essential instruments for assessment of the integrity of the trigeminal and facial cranial nerves which comprise the reflex arc 

47. Additional methods to find a diagnosis include electromyography (EMG) ( 13 ) and magnetic resonance imaging (MRI). MRI is also a useful modality to exclude pathological changes in the cerebellopontine angle, such as tumors or brainstem lesions. To display possible vascular compression, high-resolution, T2-weighted sequences are particularly useful—for example, an axial-plane CISS (constructive interference in steady state) sequence, since this yields images with a high contrast between cerebrospinal fluid, nerves, and vessels

48. Interpretation No direct response  - absent blink in the tested eyeIpsilateral facial nerve (VII) lesion No consensual response  - absent blink in the opposite eyeContralateral facial nerve (VII) lesion No afferent response  - absent blink in one eye when testing either eyeIpsilateral ophthalmic nerve (V1) lesion No response  - absent blink in both eyes when testing either eyePons or medullary lesion

49. Other causes of facial paralysis Bell’s palsy Infection or inflammation of the facial nerve head or neck tumor stroke Head trauma, skull fracture or injury to the face Lyme disease Ramsay-Hunt Syndrome/ herpes zoster oticus Autoimmune diseases  multiple sclerosis, and  Guillain-Barré syndrome

50. Why did this history surprise us? Frequency of facial nerve dysfunction in COM ranges from 0.16 to 5.1%. Proposed etiologic factors Osteitis of bone surrounding facial nerve Erosion of facial canal External compression by disease Edema and inflammation of the nerve Ref: Facial Nerve Paralysis due to Chronic Otitis Media: Prognosis in Restoration of Facial Function after Surgical Intervention Yonsei Med J. 2012 May 1; 53(3): 642–648.

51. Treatment: Urgent surgical clearance of disease Facial nerve decompression Steroids Antibiotics Prognosis is poor- may take months to recover The bottom line is…. 1. Transient facial nerve dysfunction due to cholesteatoma is rare 2. Reversal of facial paralysis due to chlosteatoma without surgical intervention is unlikely

52. Possibilities … Hemifacial spasm Blepharospasm - only eyelids,bilateral Oromandibular dystonia - Hemimasticatory spasm- lower part of face Facial nerve tics- typically can be suppressed Synkinesias after facial nerve paralysis- occures in context of voluntary movement Focal seizures