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 Classification System Published Classification of Headache Disorder

International Classification 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, Tension typed headache, Cluster headache/TACs

2. Part 2: the secondary headaches

Headache attributed to . (specific causes)

3. Part 3: painful cranial neuropathies

cranial neuralgias

Migraine with cranial autonomic symptoms

Vestibular migraine

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

Otalgia

Migraine introduction

Different phases of Migraine

Genetic

Environmental and factor

Cause Pathophysiology of acute migraine

Pathophysiology of episodic and chronic migraine

- Genetic: FHM, TREK!-Trigger factor

Pathophysiology- Aura- vasodilatation- neurogenic inflammation- peripheral and central sensitization- Trigemino vascular systemNeurotransmitter- Serotonergic system- Dopaminergic systemStructural and functional brain change- Brain stem activation

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

episodic

constant

episodic become chronic

acute on chronic

Evolution of Migraine

ICHD-III Beta, 2013

Tension-type headache

Dull aching, constant, mild to moderate painBilateral 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 flushing - 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) reflex

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

Specific triggers can not be counted as migraine/sinus headache

Tension-type headache

Pressure-tightening-constant-frontal pain: mis-diagnosed as sinus headache

Hints: location, sinus symptoms, response to medication

The diagnostic criteria for sinus headache

IHS (2004) diagnostic criteria for!

Headache attributed to rhinosinusitis!

A. Frontal headache accompanied by pain in one or more regions of the face, ears or teeth and fulfilling 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 acute-on-chronic rhinosinusitis

IHS (1998) diagnostic criteria for!

Acute sinus headache!

A. Purulent discharge in nasal passage either spontaneous or by suction

B. Pathological finding 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 disorders-headache 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 fulfilling 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 specific diagnosis => emerging vestibular migraine

Eggers SD. Curr Pain Headache Rep 2007;11:217-26Furman JM et al. Lancet Neurol 2013;12:706-15

Migraine with brain stem aura !(old term; basilar-type migraine)A. At least two attacks fulfilling 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 five episodes fulfilling 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

peripheralinner ear -> TVS innervation endolymp homeostasis change

Vestibular migraine pathway centralshared vestibular system and migraine generator

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

Treatment of Migraine and Migraine Vertigo

Non-specific medication

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

Migraine- specific medication

(Imigran)

(Zomig)

(Relpax)

Ergotamine/Caffeine

1 mg/100 mg Caffeine B

2000 Guideline!The Quality Standards Subcommittee of the AAN

Group 1!Anti-epileptic drug!- Na valproate - Topiramate Anti-depressants!- Amitryptyline Beta-blockers!- Metoprolol - Propranolol - Timolol Other !-Petasites (butterbur) !+ Ca blocker: flunarizine in EFNS guideline

Vestibular migraine treatment

Few studies

acute; zolmitriptan

anti-vertigo agent: promethazine, dimemhydrinate, meclozine

prophylactic;

nortriptylline, verapamil, metoprolol, topiramate, flunarizine, 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 parieto-occipital region. It may develop without apparent cause or as a complication of Herpes zoster

Pain in the throat and mouthGlossopharyngeal 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 BPPV Central PPV

Latency following precipitating positioning manoeuvre

1-15 sec (shorter in h-BPPV) 0-5 sec

Duration of attack 5-6 sec (longer in h-BPPV) 5-

Neuro-otalgic syndrome

Hearing lossCentral hearing loss Peripheral hearing loss

Conduction HL Sensorineural HL

Genetic Acquire- Syndromic: Alport syndrome,

Treacher-Collin syndrome, Usher syndrome etc

- Neurofibromatosis type 2 (NF 2) - Mitochondrial disease

- Cortical deafness

Clinical features of mitochondrial syndromes associated with deafness

Condition/syndrome

Neuro-otologic syndrome

Main clinical features Additional features Epidemiology

Laboratory markers

MELASCochlear origin; symmetric gradual onset SNHL

Encephalopathy (seizures+/-dementia); stroke like; mitochondrigl myopathy

Short stature;normal early psychomotor development; recurrent headache

Usually first decade;sometimes 10-40 years

Ragged red fibres on muscle biopsy;increase lactate

MERRFSymmetric gradual onset SNHL

Myoclonus;epilepsy;cerebellar myopathy

Short statue; dementia;optic atrophy;cardiomyopathy;WPW synd; neuropathy

Usually childhood onset, but may be adults

Ragged red fibres on muscle biopsy;increase lactate

KSSSymmetric gradual onset SNHL

Retinitis pigmentosa;aphthalmoplegia

Cardiac conduction block;cerebellar syndrome;short stature;impair intellect

Onset

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