Otosclerosis - orl.lf1.cuni.cz · Otosclerosis Important cause of auditory and/or vestibular...

Otosclerosis

Prof.MUDr.J. Fajstavr,DrSc.

ENT clinic,UK,2nd Fac.of Med.

Otosclerosis

Bone disease unique to the

otic capsule

Otosclerosis

Important cause of auditory

and/or vestibular symptoms

The term was introduced by

Adam Politzer

150 years ago

Similar term:

• Tympanosclerosis - calcium deposits

• and scars in the middle ear mucosa

• of inflammatory origin

• > loss of hearing

Development of otic capsule

• Ossification starts at the 15th week of

pregnancy -- in 3 layers:

• Periosteal layer on the outer surfice

• Endosteal layer on the inner surfice

• Enchondral layer – the thickest one:

• 14 ossification centers in the cartillaginous

part of the capsule

The ossification is finished at

birth

Sporadic chondrocytes even in

adults:

Fissula ante fenestram (slot)

Fossula post fenestram (groove)

Labyrinthine windows

Site of oval (vestibular) window:

Where the basal part of stapes

impinges on the wall of vestibule:

The portion of otic capsule becomes

part of the stapes base and annular

ligament ( 9th week)

Round (cochlear) window

Develops in about 10th week

embryo

Result: membrana tympani

secundaria

Incidence

In whites: in about 10% of temporal

bones, but only 1% with hearing loss

(Guild, 1944)

About 70%: women

Bilaterally – in >90%

Hearing loss: clinically distinct

between puberty and 30 years

Genetics

• Hereditary nature of otosclerosis:

• Toynbee 1861

• Recent studies:

• About 70% - hereditary basis.

Inheritance: autosomal dominant

(Nowadays: genes on 6 chromosomes: 15q,7q,6p,16q,3q,6q)

• Penetrance: 25 – 40%

Coincidental occurrence:

Otosclerosis + osteogenesis

imperfecta

Symptoms:

Pathological bone fractures

Hearing impairment

Blue sclera

(syndroma van der Hoeve, de Klejn)

Sy van der Hoeve, de Klejn

Achondroplasia-dwarfish figure

Factors infuencing

manifestation and course of

otosclerosis

• Hormonal changes (in women):

• Puberty (first symptoms – after pub.)

• Delivery (significantly hastens the

hearing loss)

Histopathology

Otosclerosis begins as discrete foci

of abnormal bone that enlarge and

may coalesce

Two phases: spongiotic

sclerotic

Spongiotic phase

Bone is resorbed around the wessels

and is replaced by cellular, fibrous

connective tissue

Highly vascular, less dense, loose

spongy bone replaces the normal

bone

(It stains blue with H.E.)

Sclerotic phase

The new bone is resorbed and

replaced with osseous tissue

containing many collagen fibers and

little ground substance. Calcium

deposits – the tissue stains red.

Location (Anywhere in temporal bone)

70 – 90%:

foci anterior to oval window

Round window is involved in

30 – 50%

Stapes fixation

• Is the cause of hearing impairment

• The focus of otosclerosis involves the

anterior margin of oval window and

gradually spreads to the footplate

Clinical presentation

• History: gradually progressive hearing loss,

• Age on onset: 11 to 45years, average: 20

• Progress: initial conductive loss becomes mixed

and later on, the higher frequences worsen

(labyrinthisation of otosclerosis)

• Pregnancy hastens the hearing loss

• Paracusis willisii

• Affection of the second ear in later course

Diagnosis

• History – see clinical presentation

• Normal pneumatization of mastoid

• No or only seldom ataks of OMA

• Tinnitus in 75 – 100% of patients

• Seldom dizziness

Testing

• Audiometry: air – bone gap

• Tympanometry: curve A, low compliance, stapes reflex absent

• Tuning fork tests: special test: Gellé

• CT

Vzdušná sprcha

Aktivní provzdušnění

Treatment

• Surgery:

• Tympanotomia, stapedectomia or

stapedotomia

• Replacement of stapes by Schuknecht

prosthesis or by piston

m. stapedius třmínek

ploténka

dlouhé raménko kovadlinky

Schucknechtova

protézka

Acute acoustic trauma

2 different forms:

Sudden clash (gunshot trauma)

Blast trauma

Gunshot trauma

• Sudden sound (noise): the peak of the

sound pressure wave lasts less than

• 1,5 milisec

• ( =shorter time period than is the latency

of the reaction of middle ear muscles)

• Comprises high frequencies around 4 kHz

Pathogenesis

• The excessive travel wave affects the

microcirculation causing partially

reversible damage to the sensory cells

• (due to the spasm of the branches of

arteria labyrinthi)

Symptoms

• Short piercing pain in the ear

• Tinnitus (high whistling, hissing

sounds)

• Impaired hearing

Therapy

• Vasodilating drugs by means of intravenous infusion

• High pressure oxygen therapy

• The result depends on the time period between the trauma and the therapy

Blast trauma

• Impairment of the ear due to the explosion

• The injuring factor is the wave of the high airpressure

• The concomitant noise could be less important

• High airpressure could damage the conductive apparatus (drum, ossicles)

Symptoms

• Sharp pain in the ear

• Impaired hearing

• Bleeeding from the middle ear

Therapy

• Uncomplicated perforation of the

drum: sterile closure by means of

prosthesis (e.g. a disc of moistened

cigarette paper)

• Tympanoplasty

Chronic noise trauma

Damage to the inner ear by the long

lastig exposure to the noise

The severity of the hearing loss

depends:

on the time of exposure

on the individual inner ear

sensitivity to the noise

Hazardous factor

• The long lasting noise above the level

• of 80 dB

• = dangerous place of work

Symptoms

Initially, the higher frequencies are

affected (especially 4 kHz)

Later on, the deafness spreads to the

speech frequences

The outer hair cells degenerate first,

the inner cells last.

Conditions for the work in

the noise

• Normal hearing level (audiogram)

• Normal hearing weariness (special test)

• The infrasound (vibrations) and toxic agents

could also affect the health of workers

Protection of hearing

• Ear protectors:

• External canal plugs (cotton wool plugs are

inefective!)

• Headphone protectors (diminish the laudness by

40 dB)

• Helmet protectors (obligatory at the noise >120 dB)

Toxically damaged hearing

• smoking (nikotin - ganglionic poison –

spasms of vessels)

• Aminoglykosidal ATB

• Organic solvents

• quinine

The

End

Dizziness - vertigo

J Fajstavr

ENT clinic, UK, 2nd Med.Fac.

Head:doc.MUDr.Z.Kabelka,PhD.

Complex system of balance control

- vestibular system

- oculomotor (visual) system

- proprioceptiv - kinesthetic system

- cerebellum

- higher sections of CNS - cortex

Balance disorders are rare in pediatric patients

In childtren it is difficult to recognise them since most of the times children

cannot describe precisely what they are feeling

Balance disorders are hardly evaluated in children who still have not

developed their balance mechanisms

Spine, brainstem, midbrain, cortex

I. Spinal and/or brainstem development:

Apedal, prone or supine infant with primarily primitive

(unconditioned) reflexes (sucking, grasping)

II. Development of the midbrain region:

Quadrupedal, crawling or sitting child with development of

head and body „righting“ reactions – coordination of nuchal

muscles, trapezius and voluntary sight fixation (basis for

voluntary grasping)

4 levels of CNS development in infants and children

III. integration of the cortical level of development:

Bipedal, standing and walking child – with the emergence of

equilibrium reactions, initiated esp. by the trunk muscles

IV.maturation of the CNS

Proceeds to involve ever higher levels; complex of

acute postural reflexes develops, conditions of equilibrium

demanding actions

(ball games, biking, skating etc.)

Structure of the peripheral vestibular

system

semicircular canals

lateral, anterior vertical, posterior

vertical

static maculae of saccule and utricule

Organs of perception

semicircular canals

cristae ampullares – cupulae ampullares

(cilia of sensory cells imbeded in a gelatinous matter)

static maculae :

cilia of sensory cells: also imbeded in a gelatinous matter

containing crystals of Aragonit (otoliths, otoconia)

• .

1. Ramus superior: (nervus utriculo-ampularis) bbranches: r. ampullae superioris r. ampullae lateralis r. utriculi 2. Ramus inferior (nervus sacculo-ampularis) : branches:r. ampullae posterioris r. sacculi

Vestibular nerve

Bipolar neurons of vestibular ganglion in meatus acusticus internus send peripheral

fibers in two bundles:

In area vestibularis -

floor of the 4. ventricle:

n. dorsalis (Bechterev),

n. lateralis (Deiters),

n. medialis (Schwalbe)

n. inferior (Roller).

Nuclei of vestibular nerve

Direct sensory cerebellar tract:

- tractus vestibulo-floccularis.

Vestibular tracts

1. Tractus vestibulo-cerebellaris 2. Tractus vestibulo-longitudinalis 3. Directly to nucl. reticularis tegmenti: tractus vestibulo-reticularis. 4. tractus vestibulo-tectalis to corp. quadrigem. 5. to thalamus: tractus vestibulo-thalamicus 6. tractus vestibulo-spinalis (Deitero-spinalis)

Impulses from the vestibular end organs

lead to the vegetative centers of

mezencephalon, from here pupilla, blood

circulation, digestive trakt are influenced

Function of vestibular

system • Semicircular canals perceive angular acceleration

and/or deceleration of the movement

• Maculae staticae perceive linear change of movement and control the position of the head in the gravitational field

• (reverse rotation of eyeballs - J.E.Purkyně)

• Sensory cell of ampullar crist possesses about 60 stereociliae and 1 kinocilia

Origin of the assymetrical

flow of impulses

• Increased and/or decreased flow of impulses depends on the direction of deflection of ampullar cupula

• deflection toward kinocilia (to ampulla): depolarization (increased flow of impulses)

• Opposite deflection: polarization

• Quiescence:

• 80 mV - 10 impulses/sec

• Deflection towards kinocilia:

• 60 mV - 60 impulses/sec

• Deflection in opposite direction:

• 120 mV - 3 impulses/sec

Factor initiating displacement of

ampullar cupula

• Stream of endolymph in the

semicircular canal

• Ampulopetal: depolarization

• Ampulofugal: polarization

Klid: 10 imp.

ampulpetal

60 imp.

ampulofugal

3 imp.

Endolymph flow

could be due to:

movement - changes of

temperature (caloric test)

•

Subjective symptom of

vestibular imbalance

Vertigo

• Vertigo is a subjective symptom:

• Perception of imaginary movement.

• Vertigo of periferal vestibular origin:

• Sensation of one´s own rotation and/or rotation of the environment, always

connected with Ny,

• mostly with impaired hearing and

• with vegetative symptoms (vagus)

J.E.Purkyně:

Dizziness

Disorder of balance of non-

vestibular – peripheral-

origin

Non-vestibular „vertigo“

• Orthostatic hypotension

• Dizziness due to hypoglycaemia

• Aura before migraine

• Abuse of alcohol, drugs

• unfitting glasses

Objective symptoms of

vestibular imbalance

• Nystagmus

• Vestibulo-spinal reflexes

Nystagmus

• Conjugated coordinated eye movement

• with slow and fast component.

• Basic characteristics:

• - Direction – determined by the fast component –

• horizontal, rotatory, vertical, alternating, mixt

• - intensity – (I. II. III. degree)

• - frequency

• - amplitude

Nystagmus

Slow component of Ny:

originated from the stimulated labyrinth with higher flow

of impulses and is directed to the labyrinth with lower

activity.

Fast component of Ny:

Is of central origin -it returns the bulb to neutral

position.

Labyrinths = two fighters

They struggle to push the rival aside

The more active labyrinth sending higher number

of impulses wins

The direction of pushing is

against the weaker labyrinth = slow phase of Ny

Nystagmus

• Irritative

• (fast component to the

stimulated labyrinth)

• Extinguishing

• (fast component from the

damaged labyrinth)

Examination of a child with balance

disorders

• Clinical history,

• Physical examination

• Blood tests

• Audiological, vestibular, EEG

• evaluations

• Imaging methods

History

Family history:

Parent´s diseases: esp. of ENT region, neurological disorders –

migraine, epilepsy, Menière´s disease,

diabetes,congenital disorders

Prenatal history

intrauterine infections,

Rh compatibility,

ototoxic drugs, smoking,

alcoholism

Neonatal, postnatal

history

Abortion, asphyxia, jaundice,

respiratory failure,

craniofacial anomalies,

septicemia, viral,

bacterial infections,

head injuries

Description of balance disorders

Children´s, parents´ description:

Frequent falls, inability to roller-scate,

to ride on a skateboard or a bike

Observation of nystagmus

Detailed description of balance

disorders

Vertigo: rotatory, determinable direction

Circumstances of the onset, duration,

frequence, intensity,

Accompanying symptoms:

(headache, hearing disorders, tinnitus,

nausea, vomitus)

Physical examination

• Oriented to three main causes of

• balance disorders:

• Otogenic, neurological, systemic

• Exams: general, otolaryngological,

vestibular, neurological, imaging

methods

Basic otoneurological

examination

The patient will be examined while

sitting, standing, lying, at rest, in motion

Vestibulo-spinal reflexes-balance tests:

Romberg, Babinski, Fukuda, Hautant

The balance tests

• Romberg: • basic position, open/closed eyes

• Babinski-Weil:

• Walking test on the straight line

• Unterberger, Fukuda:

• Stepping tests

• Hautant:

• Deviation of forward stretched arms

Vestibulo-cerebellar connections

• Index finger – nose test

• Heel – knee test

• = taxis

• Hands prone – supine test

• = diadochokinesis

• Muscle tone reactions

Vestibulo- ocular connections

Examination of spontaneous and

evoked nystagmus

In various sight directions

Head shaking nystagmus

Sight palsy

Spontaneous nystagmus

Ny without vertigo:

of central origin

Pendulous Ny:

Ocular origin

Optokinetic Ny

The influence of oculomotor

systém only

• Caloric testing:

• Bilateral

• Unilateral

• Irrigation of the external canal with water at 30º or 44ºC (20ml per 10 sec)

• 30º: ampullofugal flow: Ny to the other side

• 44º: ampullopetal flow: Ny to the irrigated ear

Turning test

• Sitting position, head in the axis of rotation, bent 30º forward, 10 turnings per 20 sec. Slow start, sudden stop. At the beginning of rotation: Ny in the direction of rotation. At the stop: Ny against the direction of rotation

Peripheral (harmonic) vestibular

syndrome

• Vertigo

• Feeling of rotatory movement with determinable direction –

• always in the direction of the slow component of nystagmus

• Nystagmus

• Always simultaneously with vertigo, mostly horizontal, of the III degree

Harmonic vestibular sy

• Sudden onset

• of symptoms,long duration of the attack

• (hours, days)

• Hearing disorders

• Vestibulo-spinal symptoms

• Correspond with the slow component of Ny

• Vegetative symptoms

The most frequent causes of

balance disorders • 1. Middle ear inflammations

• 2. Head injuries

• 3. BPPV (benign paroxysmal positional vertigo)

• 4. Vestibular neuronitis (neuritis)

• 5. Menière´s disease (M.M.)

• 6. Sudden senzorineural hearing loss (vessels!)

• 7. Labyrinthitis (esp. caused by meningitis)

• 8. Vertebrogenic vertigo

• 9. Phobic vertigo

• !! Endocranial tumors cause 3% of balance disorders in children!!

Middle ear pathology

• Spread of toxins into the labyrinth

• Defects of the bony wall of the labyrinth (labyrinthitis circumscripta)

• Assymmetric caloric reaction (drum perforation)

• Dysfunction of the Eustachian tube (negative

• middle ear pressure, displacement of the round window membrane)

Injuries

• Concussion of the labyrinth

• Fractures of the temporal bone

• (esp. through internal canal and labyrith)

• Perilymph fistula

• (symptoms: sudden vertigo, longlasting and/or fluctuating hearing loss – symptoms immitating M.M.)

•

Cricket-ball stroke

Broken stapes

Direct injury by the skewer of lollipop

Stapes footplate perforation

BPPV

• Benign paroxysmal positional vertigo

• Short attacs of rotatory vertigo of sudden onset, provoked by sudden change of the head position

• In children: transient vascular disturbance,

• Relation to migraine

• In adults: canaliculolithiasis –

• otoliths released from macula utriculi spread into the posterior semicircular canal

Dix - Hallpike test

• Rapid vigorous position change from sitting to supine, head-hanging position with the head turned to the right or left side

After latence of 5 to 10 sec appears Ny and V, lasting several sec

Vestibular symptoms disepear during repeteated tests

Vestibular neuronitis

(neuritis) • Preceded by viral infections of the upper airwais

• First vestibular symptoms: in the 6th – 10th day:

• Instability, vertigo, HR nystagmus, tinnitus, nausea, vomitus

• Intensity of symptoms increases during several hours (not suddenly)

• The examination gives evidence for the peripheral vestibular syndrome

• The symptoms disappear during 5 to 10 days (but

balance disorders could last even weeks)

Menière´s disease

Hydrops of cochlear duct

First symptoms:

Feeling of pressure in the ear

Tinnitus

Hearing disorder

Menière´s disease

• Main sy:

• Attacks of violent rotatory vertigo

• lasting hours or days

• Accompanied by Ny

• Between attacs without any symptoms

• After repeated attacs the hearing loss

worsens

Vertebrobasilar insufficiency

• Transient ischemic attacs within the vertebrobasilar system

• Embolisation of the a. labyrinthi

• Bleeding into the labyrinth

• First symptoms: hearing loss

• Longlasting Oxygen deficiency:

• Vestibular damage

Cervical syndrome

• Symptoms:

• brief attacks of dizziness, dictated by

• the position of the head,

• tinnitus,

• pain in the nape of the neck radiating

• to the occiput and forhead area

•

• dysphagia,

• impaired hearing

Cervical syndrome

• Painful outputs of the cervical plexus branches (occipital nerves)

• RTG

• Straightened cervical spine lordosis,

• Restriction of the movement

• Cause

• Lesion of the joints of the cervical spine and of the muscles of the back of the neck,

• the dysfunction is mostly in the region of

• C1 to C3

The end