otosclerosis - orl.lf1.cuni.cz · otosclerosis important cause of auditory and/or vestibular...
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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