Auditory-vestibular sensory

system

Assoc. prof. T. Chamova, MD, PhD

 Cranial nerves- overview
I N. olfactorius
II N. opticus
III N. oculomotorius
IV N. trochlearis
V N. trigeminus
VI N. abducens
VII N. facialis
VIII N.vestibulocochlearis
IX N. glossopharyngeus
X N. vagus
XI N. accessorius
XII N. hypoglossus
Anatomic and Physiologic Considerations

• Vestibulocochlear nerve
(VIII)
– Cochlear nerve- subserves
hearing or acoustic
function
– Vestibular nerve-
concerned with
equilibrium (balance) and
orientation of the body
and eyes to the
surrounding world
Anatomic and Physiologic Considerations

• Perilymph
-located
between the
wall of the
bony labyrinth
and the
membranous
labyrinth
• Endolymph-
located within
the
membranous
labyrinth
 Acoustic system- anatomy
• Perception of sounds and speech
• Humans perceive sound waves 20 Hz-20 kHz
 Acoustic system- anatomy
• Membranous labyrinth
– Scala vestibuli- oval small window
– Scala tympani- circular small
window
– Ductus cochlearis
• Spiral organ of Corti- end
organ of hearing, wherein
sound is transduced into nerve
impulses
– 15,000 neuroepithelial (hair) cells
on the basilar membrane-
transforming mechanical
vibrations in nerve impulses
– Stereocilia- embedded in the
tectorial membrane
– Sound causes the basilar
membrane to vibrate
 Acoustic system- anatomy
• Tonotopic organisation of
the auditory receptors in
the cochlea
– Close to the oval window-
high frequencies
– Apical cochlear part- low
frequencies
 Acoustic system- anatomy
• The first neuron- in the spiral ganglion of the
cochlea
• Bipolar cells
– With peripheral processes which convey auditory
impulses from the specialized neuroepithelium of
the inner ear
– Axons, forming the auditory part of VIII
 Acoustic system- anatomy

• Vestibulocochlear
nerve enters the
cranial cavity
through internal
auditory meatus
• It appears in
pontocerebellar
angle
Acoustic system- anatomy
• Dorsal and ventral cochlear
nuclei- second neuron
• Crossing and ascending
pathways to inferior colliculi
via the lateral lemnisci
• Secondary acoustic fibers
project via the trapezoid
body and lateral lemniscus to
the medial geniculate bodies
• Some fibers terminate in the
trapezoid body and superior
olivary complex and subserve
such reflex functions as
auditory attention, sound
localization, auditory startle,
and oculopostural
orientation to sound
 Acoustic system- anatomy
• From the medial geniculate bodies (3rd-4th neuron), fibers project
to the cortex via the auditory transverse gyri of Heschl (41) and
other associative cortical areas (22 and 42 Brodmann area)
• Bilateral auditory afferentation
• Cortical deafness in bilateral temporal lobe lesions involving the
geniculocortical fasciculi
 Acoustic system- reflexes
• Startle reflex- motor reactions in case of sudden
sounds (reticular formation)
• Rapid contractions of m. stapedius and m. tensor
tympani in loud sounds (n. V, n. VII)
• Reflective orientation of eyes and head towards
a loud sound (colliculi sup. and inf.)
 Hearing examination
• Otoscopy
• Audiometry- continuous and interrupted tones are presented at
various frequencies
• Tuning-fork tests for bone conduction
• Differentiation of deafness:
– Conductive, caused by a defect in the mechanism by which sound is
transformed (amplified) and conducted to the cochlea (disorders of the
external or middle ear—obstruction by atresia or cerumen, thickening of
the tympanic membrane from infection or trauma, chronic otitis media,
otosclerosis and obstruction of the eustachian tube)
– Sensorineural (disease of the cochlea or of the cochlear division of the
eighth cranial nerve)
– Mixed
– Central, caused by lesions of the cochlear nuclei and their connections with
the primary auditory receptive areas in the temporal lobes
 Hearing examination
• Speech discrimination- list of 50 phonetically balanced
monosyllabic words (e.g., thin, sin) at suprathreshold levels
– The speech-discrimination score
– Marked reduction (less than 30 percent) in the speech-discrimination
scores- characteristic of eighth nerve (retrocochlear) lesions
• Loudness recruitment- depending on the selective destruction
of low-intensity elements subserved by the external hair cells
of the organ of Corti with preservation of high-intensity
elements
– In recruiting deafness (lesion in the organ of Corti—e.g., Ménière
disease), the more affected ear gains in loudness and may finally be
equal to the better one
– Recruitment- intensity of the stimulus that causes discomfort, about
100 dB (decibels) in normal persons
 Hearing examination
• Brainstem auditory evoked potentials, or response (BAEP, or BAER)- very
refined information as to the integrity of primary and secondary
auditory pathways from the cochlea to the superior colliculus.
– detecting small acoustic and vestibular schwannomas
– in localizing brainstem lesions such as those caused by demyelination
– in corroborating the state of brain death, in which all waves, except occasionally
the eighth nerve (wave I), responses are abolished
– in assessing sensorineural damage in neonates who have had meningitis or
been exposed to ototoxic medications

• The acoustic-stapedial reflex- used as a measure of conduction in the

auditory (and the facial) nerve.
– It normally protects the cochleas from excessively loud sound
– In loud sounds 70 to 90 dB above threshold hearing reaches the inner ear, the
stapedius muscles on both sides contract reflexly, relaxing the tympanum and
offering impedance to further sound
 Pathology- tinnitus
• Tinnitus aurium- "ringing of the ears"
• Sounds originating in the ear
– Buzzing
– Humming
– Whistling
– Roaring
– Hissing
– Clicking
– Chirping
– Pulse-like sounds
 Pathology- tinnitus
• Tonal- subjective tinnitus, it can be heard only by the
patient- cochlear damage, impairment of tympanic
membrane, ossicles of the middle ear, inner ear, or eighth
nerve
• The nontonal form- sometimes objective, produced in the
inner ear or in some other part of the head and neck
– Turbulent flow of blood in the large vessels of the neck or in an
arteriovenous malformation or glomus jugulare tumor
– Pseudotumor cerebri or raised intracranial pressure of any type,
noise, due to a pressure gradient between the cranial and
cervical venous structures and the resulting venous turbulence
– Gentle compression of the jugular vein on the symptomatic
side- decrease of the noise
 Treatment of tinnitus
• Vascular decompression of the eighth nerve
• Antiepileptic drugs and tocainide hydrochloride
• Small doses of amitriptyline at night
 Middle ear deafness
• Otosclerosis
• Otitis media
• Trauma
 Cochlear damage
• Antimicrobial drugs (aminoglycoside group and
vancomycin) damage cochlear hair cells
• Neonatal cochlear damage by rubella in the
pregnant mother
• Mumps, acute purulent meningitis
• Presbyacusis
 Acoustic nerve impairment
• Tumors of the
cerebellopontine angle
• Mycotic, lymphomatous,
carcinomatous,
tuberculous, or other
types of chronic meningitis
and sarcoidosis
• Lymphomatous meningitis
has a particular
predilection to cause
unilateral hearing loss
 Acoustic nerve impairment
• Carcinomatous meningitis
• Solid tumors- schwannomas, neurofibromas,
meningiomas, dermoids, and metastatic
carcinoma
• In central neurofibromatosis (type II), the
involvement by vestibular and acoustic
schwannomas is typically bilateral
• Unilateral deafness- demyelinative plaques,
infarction, or tumor involving the cochlear
nerve fibers or nuclei in the brainstem
 Pathology
• Hyperacusis- lesions of the facial nerve (m.
stapedius)
• Paracusis, a condition in which a sound, tune, or
a voice is repeated for several seconds
• Complex auditory hallucinations- as part of
temporal lobe seizures arising from a variety of
temporal lobe lesions- tumors, strokes
• Sudden deafness – impaired blood flow in a.
labyrinthi (a. cerebelli inf.)
 Vestibular system anatomy
• The semicircular ducts for angular
accelerations of head and body
• Utricle, and saccule for the detection
linear acceleration
• The sensory epithelium is located:
– On hillocks (cristae) in the dilated openings
of the semicircular ducts, covered by a sail-
shaped gelatinous mass called a cupula
– In the utricle and saccule (maculae
acusticae), covered by the otolithic
membrane, composed of calcium
carbonate crystals embedded in a
gelatinous matrix
• 1st neuron in vestibular ganglion in the
internal auditory meatus
 Vestibular system anatomy
• 2nd neuron- four vestibular nuclei: superior
(Bechterew), lateral (Deiters), medial
(triangular, or Schwalbe), and inferior (spinal,
or descending)
• Direct projections from the semicircular canals
flocculonodular lobe and adjacent vermian
cortex (vestibulocerebellum)
 Vestibular system anatomy
Connections of vestibular nuclei

• The lateral and medial vestibular nuclei- with the spinal

cord, mainly via the uncrossed lateral vestibulospinal
tract and the crossed and uncrossed medial
vestibulospinal tracts- effects on posture and muscle tone

• The nuclei of the III, IV, and VI cranial nerves by the

medial longitudinal fasciculus- synchronous eye
movements, eye fixation, synchronization between eye
movements and head position
 Vestibular system anatomy
Connections of vestibular nuclei
• Afferent and efferent connections with the
pontine reticular formation and nucl. dorsalis n.
vagi- autonomic symptoms, accompanying
peripheral vestibular lesions
• Afferent and efferent connections with the
cerebellum- coordination
• To cerebral cortex, specifically to the regions of
the intraparietal sulcus and superior Sylvian
gyrus
 Vestibular system- reflexes
• Vestibulo-ocular reflexes

• Vestubulo-spinal reflexes

• Vestibulo-vegetative reflexes
 Vestibular examination- Nystagmus
• Electronystagmography and videooculography
• Nystagmus- periodic rhythmic ocular oscillation of the
eyes
• Slow phase
• Quick phase- determines the direction
– Direction- horizontal, vertical, rotational, monocular and
combined
– Grade of nystagmus
• I- at end-most position
• II in looking onward
• III- opposite direction
 Vestibular examination- Nystagmus
• Amplitude- mild, moderate and severe
• Spontaneous acquired nystagmus- always
pathological- suppression in case of fixation
• Congenital nystagmus- two identical phases,
more expressed with fixation
Vestibular examination- provoked nystagmus

• Rapid head impulse" test

– Patient fixates on a target and then for the examiner
to rotate the patient's head quickly by 10 degrees
– Ocular instability- when the patient turns his head
toward the side of the affected labyrinth
• Rotational tests with Bárány chair- comparison
the direction, duration and severity of nystagmus
• Caloric testing
– Warm water- ny towards the irrigated ear
– Cold water- ny towards the opposite ear
 Symptoms of vestibular dysfunction
• Vertigo- a sensation of rotation or movement of one's self
(subjective vertigo) or of one's surroundings (objective vertigo) in
any plane
– Systemic with defined direction
– Non-systemic- unspecified direction+dizziness
• Deformation of space
• Osciloscopia
• Nystagmus
• Autonomic dysfunction- nausea, vomitting, sweating, change in
the heart rate, blood pressure
 Vestibular syndromes
Peripheral vestibular
impairment
• Vertigo contralateral to the
affected labyrinth
• Ny contralateral to the
affected ear
• Ataxia- homolateral
• Autonomic dysfunction
Central vestibular
impairment
• Spontaneous Ny,
depending on the
location of the lesion
• Absence of systemic
vertigo and
autonomous symptoms
• Preserved hearing
• Other brainstem
symptoms
 Ménière Disease
• Recurrent attacks of vertigo
associated with fluctuating tinnitus
and deafness
• Affection of both sexes equally
• Frequent onset around the fifth
decade of life
• Increase in the volume of endolymph
and distention of the endolymphatic
system (endolymphatic hydrops)
 Ménière Disease
• Abrupt attacks of vertigo that last for several
minutes to an hour or longer
• Whirling or rotational in type
• So severe that the patient cannot stand or walk
• Nausea and vomiting, low-pitched tinnitus, a
feeling of fullness in the ear and a diminution in
hearing
• Nystagmus- horizontal in type, usually with a
rotary component and with the slow phase to the
side of the affected ear
 Ménière Disease- treatment
• Antihistaminic agents Dymenhidrinate 3 x 1
tabl.
• Antiemetics- Metoclopramid hydrochloride-
i.v.
• An endolymphatic–subarachnoid shunt is the
operation favored by some surgeons
 Benign Positional Vertigo
• Paroxysmal vertigo and nystagmus
• Occur only with the assumption of certain positions of the
head, particularly lying down or rolling over in bed, bending
over and straightening up, and tilting the head backward
• Individual episodes last- less than a minute
• Dix and Hallpike- moving the patient from the sitting
position to recumbency, with the head tilted 30 to 40
degrees over the end of the table and 30 to 45 degrees to
one side
• Reversed direction of vertigo and nystagmus (position-
changing nystagmus)- the most certain sign that the disorder
originates in the labyrinth
 Benign Positional Vertigo- treatment
• Epley canalith repositioning maneuver
 Vestibular Neuritis (Neuronitis)

• Mainly in young to middle-aged adults

• Prior history of an antecedent upper respiratory
infection of nonspecific type
• Abrupt onset of vertigo
• Persistence of the symptoms for a day or more
• Severe vertigo, associated with nausea,
vomiting, and the need to remain immobile
 Other Causes of Vertigo of Vestibular Nerve Origin

• Vestibular schwannoma
– Deafness affecting the high-frequency tones initially,
– Mild chronic imbalance rather than vertigo
– Additional cranial nerve palsies (the seventh, fifth,
and tenth nerves),
– Ipsilateral ataxia of limbs
– Headache
• Vascular irritation or compression by a small
branch of the basilar artery
Thank you for your attention!