Neuro-otology

Surat Tanprawate, MD, MSc(Lond.), FRCP(T) Neurology Division, Faculty of Medicine Chiang Mai University

Outline

Headache and facial pain vs ENT conditions Central vertigo Neuro-otologic syndrome

Headache and facial pain for ENT

The Headache Classication System Published

! Classication of Headache Disorder" ! International Classication in Headache Disorder (ICHD)" ! ICHD-I: 1988" ! ICHD-II: 2004

Last updated ICHD-III Beta version (Boston 2014)

Jes Olesen, MD, PhD University of Copenhagen, Glostrup Hospital, Denmark

Headache and facial pain vs ENT conditions

ICHD-III Beta: 2013

1. Part 1: the primary headaches

Migraine with cranial autonomic symptoms Vestibular migraine

Migraine, Tension typed headache, Cluster headache/TACs

2. Part 2: the secondary headaches

11. Headache or facial pain attributed to disorder of the Headache attributed to . (specic causes) cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other 3. Part 3: painful cranial neuropathies facial or cervical structure

cranial neuralgias

Otalgia

Migraine introduction

Different phases of Migraine

Cause
Genetic

Pathophysiology of acute migraine

Pathophysiology of episodic and chronic migraine

episodic

Environmental and factor

- Genetic! : FHM, TREK! ! -Trigger factor

Pathophysiology! - Aura! - vasodilatation! - neurogenic inammation! - peripheral and central sensitization! - Trigemino vascular system! Neurotransmitter! - Serotonergic system! - Dopaminergic system! Structural and functional brain change! - Brain stem activation

constant

episodic become chronic

acute on chronic

Clinical: chronic and transform migraine, allodynia, neck pain! ! Anatomical: PAG, central sensitization

Evolution of Migraine

ICHD-III Beta, 2013

Tension-type headache

Dull aching, constant, mild to moderate pain Bilateral location Featureless headache

Cluster headache and Trigeminal autonomic cephalalgias (TACs)

Unilateral headache severe Ipsilateral cranial autonomic symptoms - conjunctival injection/ lacrimation - nasal congestion/ rhinorrhoea - eyelid edema - forehead and facial ushing - Sensation of fullness of ear - Miosis/ptosis

Headache with sinus symptoms

!

Headache with vestibular symptoms

Sinus headache as a misdiagnosis

Sinus headache often self-diagnosed or diagnosed in primary care setting 810 pts with diagnosed as migraine; 78% stated that they were having sinus headache 100 self-diagnosed sinus headache pt.; 86% met criteria for migraine (only 3% had acute sinusitis)

Schreiber CP et al. Arch Intern Med 2004;164:176972.

How common of unilateral autonomic (UA) symptoms in migraine

841 subjects had migraine, out of which 226 reported accompanying unilateral autonomic symptoms 26.9%

M Obermann Cephalalgia 2007; 27:504509

Trigemovascular system and Trigeminoautonomic (Trigeminoparasympathetic) reex

SSN = superior salivatory nucleus

Goadsby PJ. Lancet Neurol 2002; 1: 25157

What should we look for?

Migraine features:

Pain: throbbing/dull aching Location: unilateral/bilateral/alternate site Associated symptoms: photo-/phonophobia, nausea/vomiting

Specic triggers can not be counted as migraine/ sinus headache

Tension-type headache

Pressure-tightening-constant-frontal pain: misdiagnosed as sinus headache Hints: location, sinus symptoms, response to medication

The diagnostic criteria for sinus headache

IHS (2004) diagnostic criteria for!

IHS (1998) diagnostic criteria for!

Headache attributed to rhinosinusitis! Acute sinus headache! A. Frontal headache accompanied by pain in one or more regions of the face, ears or teeth and fullling criteria C and D B. Clinical, nasal endoscopic, CT and/or MRI imaging and/or acute-on-chronic rhino sinusitis C. Headache and facial pain develop simultaneously with onset or acute exacerbation of rhino sinusitis D. Headache and/or facial pain resolve within 7 days after remission or successful treatment of acute or acuteon-chronic rhinosinusitis

A. Purulent discharge in nasal passage either spontaneous or by suction B. Pathological nding in one or more tests; X-ray, transillumination, CT/MRI C. Simultaneous onset of headache and sinusitis D.Headache location; 1. frontal, 2. maxillary, ethmoiditis, sphenoiditis E. Headache disappears after treatment of acute sinusitis

11. Headache or facial pain attributed to disorder of the cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structure

Clinical, nasal endoscopic and/or imaging evidence Temporal relation/waxes and wanes with degree of congestion Exacerbated by pressure applied over paranasal sinus Ipsilateral to unilateral rhinosinusitis chronic pathology causes persistent headache?: controversy

Sino-nasal disordersheadache relation?

Deviation of nasal septum Hypertrophy of turbinates Atrophy of sinus membranes Mucosal contact disease

Nasal contact point headache (A11.5.1)

Intermittent pain localised to the periorbital and medial canthal or temporozygomatic regions and fullling criteria C and D Clinical, nasal endoscopic and/or CT imaging evidence of mucosal contact points without acute rhinosinusitis Evidence that the pain can be attributed to mucosal contact based on at least one of the following:

pain corresponds to gravitational variations in mucosal congestion as the patient moves between upright and recumbent postures abolition of pain within 5 minutes after diagnostic topical application of local anaesthesia to the middle turbinate using placebo- or other controls1

Pain resolves within 7 days, and does not recur, after surgical removal of mucosal contact points

ICHD-II 2004

Migraine vs Vertigo

Fact about migraine and vestibular symptoms

1. Vestibular symptoms as a migraine subcategories; benign paroxysmal vertigo (BPV) 2. Migraine with brainstem aura; vertigo and other brains stem symptoms 3. Most migraine pt. with vestibular symptoms do not have a recognised independent vestibular disorder; Menieres disease, BPPV, vestibular neuritis 4. Many migraine pt. with vestibular symptoms dont have specic diagnosis => emerging vestibular migraine
Furman JM et al. Lancet Neurol 2013;12:706-15 Eggers SD. Curr Pain Headache Rep 2007;11:217-26

Migraine with brain stem aura ! (old term; basilar-type migraine)

A. At least two attacks fullling criteria B-D B. Aura consisting of visual, sensory, a/o speech/language, each fully reversible, but no motor or retinal symptoms C. At least two of the following brainstem symptoms: 1. dysarthria, 2. vertigo, 3. tinnitus, 4, hypacusis, 5. diplopia, 6. ataxia, 7. decreased level of consciousness D. At least two of the following 4 characteristics: 1. at least one aura symptom spreads gradually over > 5 mins, a/o two or more symptoms occur in succession 2. each individual aura symptoms lasts 5-60 mins 3. at least one aura symptom is unilateral 4. the aura is accompanied or followed within 60 minutes, by headache E. Not better accounted for by another ICHD-3 diagnosis, and transient ischaemic attack has been excluded
ICHD-III Beta, 2013

Migrainous vertigo: a diagnostic criteria !

proposed by Neuhauser and Lampert 2004

Neuhauser H and Lempert T. Neurology 2004;24:83-91

New diagnostic criteria of Vestibular migraine (A1.1.6) : ICHD-III Beta version 2013
A. At least ve episodes fullling criteria C and D" B. A current or past history of 1.1 Migraine without aura or 1.2 Migraine with aura" C. Vestibular symptoms of moderate or severe intensity, lasting between 5 minutes and 72 hours D. At least 50% of episodes are associated with at least one of the following three migrainous features:"
1. headache with at least two of the following four characteristics: unilateral location, pulsating quality, moderate or severe intensity, aggravation by routine physical activity" 2. Photophobia/phonophobia" 3. Visual aura"

E. Not better accounted for by another ICHD-III diagnosis or by another vestibular disorder ICHD-III Beta 2013

Truth about VM

VM may be not associated with migraine headache Temporal relation of vestibular symptoms - headache is variable Duration of dizziness/vertigo range from seconds to days Vestibular symptoms; spontaneous vertigo/gait instability/ visual motion sensitivity/dizziness induced by head movement During an attack-nystagmus is common

Know basis of migraine mechanism

Vestibular migraine pathway

inner ear -> TVS innervation endolymp homeostasis change

peripheral

Vestibular migraine pathway

central
shared vestibular system and migraine generator

posterior insular cortex anterior insular orbitofrontal cortex posterior and anterior cingulate gyri

Treatment of Migraine and Migraine Vertigo

Non-specic medication

Migraine- specic medication

Ergotamine/ Caffeine 1 mg/100 mg Caffeine B

(Imigran)

(Zomig)

(Relpax) Ever S, Afra J. Eur J Neurol 2009, 16:968-981

Group 1! Anti-epileptic drug! - Na valproate - Topiramate Anti-depressants! - Amitryptyline Beta-blockers! - Metoprolol - Propranolol - Timolol Other ! -Petasites (butterbur)
!

+ Ca blocker: unarizine in EFNS guideline

2000 Guideline! The Quality Standards Subcommittee of the AAN

Vestibular migraine treatment

Few studies acute; zolmitriptan

anti-vertigo agent: promethazine, dimemhydrinate, meclozine

prophylactic;

nortriptylline, verapamil, metoprolol, topiramate, unarizine, valproic acid, lamotrigine CAI: acetazolamine

Otalgia

Headache attributed to disorder of ears

No pathology of the ear can cause headache without concomitant otalgia Primary otalgia+/- headache: structural lesion of pinna, external auditory canal, tympanic membrane or middle ear

most common symptoms of AOM is earache

About 50% of earache is not ear origin (referred otalgia)

Sources of referred otalgia

5th CN (mandibular division)

teeth, oral cavity, TMJ

7th CN (nervus intermedius branch)

middle ear

9th CN (Jacobsons nerve)

nasopharynth, eustachian tube, palatine tonsil, tongue

10th CN (Arnolds branch)

hypopharynth, larynth, nasopharynth

2nd & 3rd cervical roots (great auricular nerve and lesser occipital nerve)

base of skull

Painful cranial neuropathies and other facial pains

13.3 Nervus intermedium (facial nerve) neuralgia

Description:

A rare disorder characterized by brief paroxysmal of pain felt deeply in the auditory canal, sometimes radiating to the parietooccipital region. It may develop without apparent cause or as a complication of Herpes zoster

Pain in the throat and mouth

Glossopharyngeal neuralgia!

A severe, transient, stabbing, unilateral pain experienced in the ear, base of the tongue, tonsillar fossa and/or beneath the angle of the jaw. It is commonly provoked by swallowing, talking and/or coughing, and may remit and relapse in the fashion of classical trigeminal neuralgia.

Burning mouth syndrome (BMS); stomatodynia!

an intraoral burning sensation, recurring daily for more than 2 hours per day over more than 3 months, without clinically evident causative lesion

Neck-Tongue Syndrome

Vertigo

Vertigo: central vs peripheral

Time-course-onset: acute, chronic recurrent Otological symptoms: hearing loss Neurological symptoms: neuro signs Ophthalmologic symptoms: nystagmus Associated symptoms: headache, nausea/vomiting Triggers: position, Special test: MRI, Audiogram, nystagmography

Acute vestibular syndrome

Rapid onset of sustained vertigo, nausea and vomiting (in association with nystagmus, unsteady gait, and and head motion intolerance) days to weeks Classical symptoms

Common-peripheral: vestibular neuritis (VN); labyrinthitis

acute central vestibular syndrome (VPN)

Unilateral (fascicular) lesion of the entry zone of the eight nerve, vestibular nucleus lesions, vestibulocerebellar lesions No sign of other brain stem lesions

BPPV vs Pseudo PPV

Features; latency of onset of symptoms after positioning, duration of nystagmus bouts, course of nystagmus during an attack, vertigo Paroxysmal downbeat, upbeat, or torsional nystagmus -> lesion of central origin

Benign paroxysmal positioning nystagmus vs Central positioning nystagmus and vertigo (pseudo-BPPV)
Features Latency following precipitating positioning manoeuvre Duration of attack Direction of nystagmus Fatigability Course of nystagmus and vertigo in an attack Vertigo Nausea/vomitting Natural course of the condition Associated neurological s/s Brain imagining BPPV 1-15 sec (shorter in h-BPPV) 5-6 sec (longer in h-BPPV) During stimulation in the plane of the affected canal Typical, rare in h-BPPV Crescendo-decrescendo typical, no common in h-BPPV Typical
Rare on single precipitating manoeuvre (associated with intense nystagmus

Central PPV 0-5 sec 5-<60 sec

Pure vertical; pure torsional, not attributable to the stimulated canal plane

Possible Crescendo-decrescendo possible Typical

Frequent on single precipitating manoeuvres (not associated with strong nystagmus intensities

Spontaneous recovery within several weeks in 70-80% None Normal

Spontaneous recovery within weeks possible None possible, often cerebellar and other oculomotor sign Normal; lesions of the dorsal vermis a/ o dorsolateral to the fourth ventricle

Neuro-otalgic syndrome

Hearing loss
Central hearing loss
-

Peripheral hearing loss

Cortical deafness

Conduction HL

Sensorineural HL

Genetic
-

Acquire

Syndromic: Alport syndrome, Treacher-Collin syndrome, Usher syndrome etc Neurobromatosis type 2 (NF 2) Mitochondrial disease

Clinical features of mitochondrial syndromes associated with deafness

Condition/ Neuro-otologic syndrome syndrome Main clinical features Additional features Epidemiology Short stature;normal Usually rst early psychomotor decade;sometim development; es 10-40 years recurrent headache Laboratory markers

MELAS

Encephalopathy Cochlear origin; (seizures+/symmetric dementia); stroke gradual onset like; mitochondrigl SNHL myopathy

Ragged red bres on muscle biopsy;increase lactate

MERRF

Symmetric gradual onset SNHL

Short statue; dementia;optic Myoclonus;epilepsy; atrophy;cardiomyo cerebellar myopathy pathy;WPW synd; neuropathy Cardiac conduction Retinitis block;cerebellar pigmentosa;aphthal syndrome;short moplegia stature;impair intellect

Usually childhood onset, but may be adults

Ragged red bres on muscle biopsy;increase lactate

KSS

Symmetric gradual onset SNHL

Onset<20 years;majority are sporadic

Ragged red bres on muscle biopsy;increase lactate, raised CSF protein

MELAS;mitochondrial encephalomyopathy, lactic acidosis and stroke like episodes MERRF; myoclonic epilepsy with ragged red bres KSS; Kearns-Sayre syndrome

Overell J. JNNP 2004;75(supp):iv53-iv59

Cortical auditory disorders

Cortical deafness

unable to hear sounds but has no apparent damage to the anatomy of the human ear (damage to primary auditory cortex)

Other cortical auditory syndrome:

auditory agnosia;

selective/generalized decrease in recognitive of verbal+/-verbal sound

amusia;

melodies lose their musical character

pure word deafness(auditory verbal agnosia, AVA);

inability to recognize speech(still recognise non-verbal sound)

Cortical auditory disorder needs damage bilateral cortical auditory cortex

Image from Netters anatomy

Thank you