IMAGING OF THE SOUND

CONDUCTING AND SENSORY

NEURAL SYSTEM
 ANATOMY
EXTERNAL EAR
MIDDLE EAR
INNER EAR
 EXTERNAL EAR
PINNA ( AURICLE ):
MEMBRANOUS / BONY FORM
EXTERNAL AUDITORY CANAL
MASTOID ANTRUM
 MIDDLE EAR
TYMPANIC MEMBRAN CAVITY
3 OSSICLES ( MALLEUS , INCUS ,
STAPES )
EUSTACHIAN TUBE
MUSCLE & TENDON
3 OSSICLES :
MALLEUS (HEAD, BODY, MANUBRIUM)
INCUS (SHORT, LONG, LENTICULAR
PROCESS)
STAPES (HEAD, NECK, CRURA, FOOT
PLATE TYMPANIC / MEDIAL SURFACE
 INNER EAR
MEMBRANOUS LABYRINTH
- VESTIBULE
SEMICIRCULAR CANALS
ENDOLYMPHATIC SAC / DUCT
COCHLEAR , COCHLEAR DUCT
HAIR CELLS
AUDITORY SENSORY NETWORK

BONY LABYRINTH
 HOW WE HEAR
WE HEAR SOUNDS TROUGH 2 BASIC
PHYSIOLOGIC PATHWAYS :

1. AIR CONDUCTION PATHWAY

2. BONE CONDUCTION PATHWAY
 AIR CONDUCTION PATHWAY

SOUND WAVES EXTERNAL AUDITORY CANAL

VIBRATION TYMPANIC MEMBRANE
by OSSICLES
VIBRATION ARE TRANSMITTED TO INNER EAR

STAPES FOOTPLATE VIBRATES VIBRATION MOVES

FLUIDS WITHIN INNER EAR CREATES CHANGES IN
THE SENSORY CELLS (TURN STIMULATE NEURAL
IMPULSES)

BRAIN
CREATING THE SENSATION WE RECOGNIZE
AS HEARING
 BONE CONDUCTION
PATHWAY:
PEOPLE ALWAYS HEAR THEIR OWN VOICES BY BONE
CONDUCTION WHEN THEY SPEAK.
VIBRATION CARRIED THROUGH MANDIBLE & JAW
TRANSMITTED DIRECTLY
MOVE FLUIDS WITHIN INNER EAR

CREATES CHANGES IN SENSORY CELLS

BRAIN
CREATING SENSATION WE RECOGNIZE AS HEARING
SOUND TRANSMITTED BY AIR AND
BONY CONDUCTED VIBRATIONS ARE
PERCEIVED BY THE INDIVIDUAL AS
PRECISELY THE SAME SOUND.
 HEARING LOSS

POSSIBLE CAUSES :
CONGENITAL
INFECTION
TRAUMATIC
HEARING LOSSES
CONDUCTIVE
SENSORINEURAL
MIXED
HEARING LOSS
PERIPHERAL HEARING PROBLEM
CENTRAL HEARING PROBLEM
 CONDUCTIVE
HEARING LOSS
INTERFRENCE ANY SORT IN
TRANSMISSION OF SOUND FROM
EXTERNAL AUDITORY CANAL TO
INNER EAR CAUSES CONDUCTIVE
HEARING LOSS
SOUND VIBRATION IS UNABLE TO
STIMULATE COCHLEA VIA NORMAL
AIR CONDUCTION PATHWAY
 SOUND CONDUCTED TO INNER EAR
DIRECTLY BY BONY VIA THE SKULL &
TEMPORAL BONY ARE HEAR
NORMALLY

REQUIRE MEDICAL / SURGICAL

TREATMENT
 SENSORINEURAL
HEARING LOSS
DAMAGE / ANOMALY :
SENSORY END ORGAN
HAIR CELLS WITHIN COCHLEA
DYSFUNCTION AUDITORY NERVES
NEARLY ALWAYS PERMANENT AND
IRREVERSIBLE

MIXED HEARING LOSS :

BOTH SENSORNEURAL & CONDUCTIVE LOSS
 ANOMALIES OF THE SOUND
CONDUCTING SYSTEM
EXTERNAL AUDITORY CANAL :
CONGENITAL AURAL ATRESIA
MICROTIA

MASTOID
MIDDLE EAR CAVITY
ANOMALY OSSICLES
ANOMALY LABYRINTHINE WINDOW
 ANOMALIES OF THE SENSORY NEURAL
SYSTEM :
(MALFORMASI COCHLEOVESTIBULAR)

COCHLEA
VESTIBULAR AQUEDUCT ABNORMALITY
SEMICIRCULAR CANALS & VESTIBULE
ANOMALIES INTERNAL AUDITORY CANAL
 CONGENITAL AURAL
ATRESIA
CONGENITAL HYPOPLASIA/APLASIA
EXTERNAL AUDITORY CANAL
OFTEN ASSOCIATED WITH
ANOMALIES MIDDLE EAR & MASTOID
 IN ALL CASES COURSE OF FACIAL
NERVE MUST IDENTIFIED TO
PREVENT INJURY DURING
RECONSTRUCTIVE SURGERY
SURGICAL IS TREATMENT OF CHOICE
 CLASSIFIED 4 TYPES :
TYPE A:
MEATALSTENOSIS IN CARTILAGINOUS
PORTION
TYPE B:
PARTIALATRESIA OF CARTILAGENOUS /
BONY PORTIONS
OFTEN ASSOCIATED WITH ANOMALY
OSSICLES
 TYPE C :
COMPLETE ATRESIA
WELL PNEUMATIZED TYMPANIC CAVITY
PARTIAL/COMPLETE ATRETIC PLATE
ANOMALY OSSICLE
TYPE D :
COMPLETE ATRESIA
REDUCED PNEUMATIZED TYMPANIC CAVITY
ANOMALY OF BONY LABYRINTH & ABNORMAL
COURSE OF FACIAL NERVE
 IMAGING FEATURES

CT IS PERFORMED TO IDENTITY THE

TYPE & EXTENT OF ABNORMALITY &TO
DETERMINE IF THE LESSION IS
SURGICALLY CORRECTABLE.
THE CT FINDING :
TYPE A & B ARE :
STENOSISEXTERNAL AUDITORY CANAL
NORMALLY FORMED MIDDLE EAR CAVITY
 TYPE C&D
THICKIRREGULAR ATRETIC BONY PLATES
OFTEN ASSOCIATED WITH OSSICULAR
ANOMALIES
ABNORMAL COURSE OF FACIAL NERVE
MALLEUS & INCUS ARE MALFORMED FUSED,
ROTATED OR ABSENT
 HEAD OF MALEUS USUALLY FUSED
WITH SEGMEN TYMPANI OR BONY
ATRETIC PLATE
STAPES USUALLY IS NOT INVOLVE
MIDDLE EAR CAVITY USUALLY
SMALL
OVAL & ROUND WINDOWS
HYPOPLASTIC / ABSENT
COMPLETE ATRESIA USUALLY
ABSENT TYMPANIC MEMBRANE,
MICROTIA, MASTOID
PNEUMATIZATION
 MICROTIA
DEFORMITY AURICLE
OFTEN ASSOCIATED WITH DISPLASIA
EXTERNAL AUDITORY CANAL
IN AGENESIS EXTERNAL SMALL
AUDITORY CANAL
OFTEN SMALL TISSUE TAG / SMALL
PIT
 MASTOID PNEUMATIZATION

RADIOGRAPHY REVEALS DEGREE

MASTOID PNEUMATIZATIION

PNEUMATIZATION CAN BE:

COMPLETELY ABSENT
LIMITED SMALL MASTOID ANTRAL CELL
COMPLETELY NORMAL
Konvensional
 AXIAL CT :
PNEUMATIZATION
DEGREE
SINUS SIGMOID
POSITION IN
RELATION TO
CANAL EXTERNAL
ANTRUM & MIDDLE
EAR
 CORONAL CT : RELATION TEGMENTAL
PLATE TO ANTRUM & MIDDLE EAR
 ANOMALIES OF THE MIDDLE EAR :

DEGRE OF DEVELOPMENT & AERATION IS

DETERMINED BY AXIAL & CORONAL CT
SECTION

MINOR HYPOPLASIA COMPLETE

AGENESIS
 ANOMALIES OF THE OSSICLES :

RELATIVELY COMMON CAUSE OF CONDUCTIVE

HEARING LOSS

ANOMALIES OF MALLEUS, INCUS & STAPES

OCCUR IN VARYING DEGREE

MOST COMMONLY ;INCUDO STAPEDIAL

DISCONNECTION: LONG PROCESS INCUS TO
HEAD STAPES
 EXTERNAL AUDITORY CANAL AGENESIS WITH
RELATIVELY NORMAL MIDDLE EAR CAVITY
DEVELOPMENT:

MALLEUS & INCUS ARE FUSSED & FIXED TO

ATRETIC BONE PLATE (AT LEVEL MALEUS
NECK)

LONG PROCESS OF INCUS IS NORMAL

 HYPOPLASTIC MIDDLE EAR CAVITY :
MALLEUS & INCUS AS A AMALGUM
LONG PROCESS INCUS IS SHORTENED OR
ABSENT
EXTERNAL AUDITORY CANAL, MIDDLE EAR ,
OSSICLES ARE WELL FORMED , MALLEUS OR
INCUS ARE FUSED TO EPITYMPANIC WALL
(MALLEUS IS MORE COMMONLY)

PATIENTS WITH ISOLATED CONGENITAL

OSSICULAR CANDIDATES FOR PROTHESES
 ANOMALIES OF THE SENSORINEURAL SYSTEM
(MALFORMATION COCHLEOVESTIBULAR):

MOST CASES OF CONGENITAL

SENSORINEURAL DEAFNESS ARE CAUSED
BY ABNORMAL DEVELOPMENT OF THE
MEMBRANOUS LABYRINTH

MOST ARE BILATERAL & SYMMETRIC

ONLY 5%-15% OF THE CONGENITALLY

DEAF CHILDREN HAVE
RADIOGRAPHYCALLY DETECTABLE
ABNORMALITIES
 CT OF THE LABYRINTH IS
INDICATED FOR :
1. DIAGNOSIS : STATUS OTIC CAPSULE
2. DETECTION ENDOLYMPHATIC SAC
3. DETECTION OTHER INNER EAR
STRUCTURES
4. SELECTION CANDIDATES FOR
COCHLEAR IMPLANTS
 COCHLEA

VARIOUS ANOMALIES AFFECTING :

THE SIZE : NORMAL TO COMPLETE
ABSENCE

THE LUMEN : NARROWED, OBLITERATED,

GROSSLY DILATED

BONY PARTITIONS BETWEEN COCHLEAR

COILS ARE ABSENT APPEAR EMPTY
COCHLEA
THE FOLLOWING COCHLEAR
MALFORMATION HAVE ABNORMALITIES
DETECTABLE BY CT :

1. Michel Malformation
2. Cochlear Aplasia
3. Cochlear Hypoplasia
4. Mondini Malformation
 1. MICHEL MALFORMATION :

MOST SEVERE MALFORMATION INVOLVING THE

OSSEOUS & MEMBRANEOUS LABYRINTH

CHARACTERIZED:
COMPLETE ABSENT OF THE MEMBRANOUS
LABYRINTH (COCHLEA, VESTIBULE,SEMICIRCULARIS
CANAL)
OFTEN OF SINGLE LABYRINTHINE CAVITY
WHEN FACIAL NERVE FUNCTION IS NORMAL THE
INTERNAL AUDITORY CANAL IS PRESENT
THE EXTERNAL & MIDDLE EAR OFTEN NORMALLY
 2. COCHLEAR APLASIA

ABSENCE COCHLEA
PARTIALLY / WELL FORMED
VESTIBULE & SEMICIRCULARIS CANAL
USUALLY BILATERAL
3. COCHLEAR HYPOPLASIA

SMALL COCHLEAR BUD (1-3mm)

MALFORMED SEMICIRCULARIS CANAL IN

50% PATIENTS

VARIABLE HEARING LOSS : DEPENDS ON

DEGREE MEMBRANOUS LABYRINTH
DEVELOPMENT WITHIN HYPOPLASTIC
COCHLEA
4. MONDINI MALFORMATION

MOST COMMON TYPE OF

COCHLEAR ANOMALY
HYPOPLASTIC/NORMAL SIZE
EMPTY COCHLEA (HYPOPLASTIC
OR ABSENCE BONY PARTITION)
 OFTEN ASSOCIATED WITH:
ABNORMALITY OF:
VESTIBULAR AQUADUCT (SHORT &
DILATED)
THE VESTIBULE :LARGE / MORE
GLOBULAR
THE SEMICIRCULAR CANAL

UNILATERAL / BILATERAL
 THE MALFORMED SEMICIRCULAR
CANALS & VESTIBULE
ISOLATED
IMAGING (CT SCAN ):
CYSTIC DILATATION SEMICIRCULAR CANALS
(MOST COMMONLY AFFECTED HORIZONTAL
CANAL)
MAY WITH DILATED VESTIBULE, ENLARGED
VESTIBULAR AQUEDUCT
EXTERNAL AUDITORY CANAL & MIDDLE EAR
USUALLY NORMAL
40% PATIENT COCHLEAR MALFORMATION
WITH DISPLASIA LATERAL SEMICIRCULAR
CANAL
 LARGE VESTIBULAR AQUEDUCT
SYNDROME

SENSORINEURAL HEARING LOSS

MOST COMMONLY CAUSE OF
CONGENITAL HEARING LOSS
60% ASSOCIATED WITH MALFORMATION
INNER EAR
40% ARE ISOLATED
 MORE COMMON BILATERAL

CONTAINS :ENDOLYMPHATIC DUCT /SAC

THE ENDOLYMPHATIC SAC PARTICIPATES IN

PRESSURE EQUALIZATION BETWEEN CSF &
ENDOLYMPH
 CT FINDING
VESTIBULAR AQUEDUCT >1,5mm
NORMAL : 1,5mm
MAY NOT BE VISUALIZED

MOSTLY : BILATERAL
ASSOCIATED WITH INNER
EAR ABNORMALITY
 MR T2 Weighted :

SHOW THE FLUID CONTENTS OF THE

ENDOLYMPHATIC DUCT & SAC ( HIGH
SIGNAL INTENSITY IN REGION OF
BONY AQUEDUCT)

DUCT & SAC DILATATION

 ANOMALIES OF THE AUDITORY
CANAL

WHENEVER A PATIENT WITH BILATERAL

INVOLVEMENT COCHLEAR IMPLANT
CANDIDATES

MR : DETERMINE THE COCHLEAR NERVE,

WHICH IS : IN SEVERE HYPOPLASIA INTERNAL
AUDITORY CANAL : COCHLEAR NERVE OFTEN
ABSENT
 COCHLEAR IMPLANTATI0N

CONSIDERED PEOPLE WITH PROFOUND

SENSORINEURAL HEARING LOSS

ATTEMPT TO : REPLACE NON

FUNCTIONAL INNER EAR HAIR CELL
TRANSDUCER SYSTEM BY CONVERTING
MECHANICAL SOUND INTO ELECTRICAL
SIGNALS THAT CAN BE TRANSMITTED TO
COCHLEAR NERVE
 COCHLEAR IMPLANT BY PASS THE HAIR
CELLS AND DIRECTLY STIMULATE THE
SPIRAL GANGLION CELLS.

CI , PATIENT WITH :
COCHLEAR AGENESIS (MICHEL
DYSPLASIA)
NARROW INTERNAL AUDITORY CANAL
 CT SCAN :
DETERMINE THE SIZE / DEVELOPMENT
MASTOID

MIDDLE EAR

ROUND WINDOW

COCHLEA
 FACIAL NERVE CANAL

HYPOPLASI INTERNAL AUDITORY

CANAL INDICATES LACK OF
DEVELOPMENT ACOUSTIC NERVE,
IMPLANT INFEASIBLE

POSTOP. : IDENTIFYING EXACT

ELECTRODE LOCATION