Pemicu 4

“Mengapa telingaku tak dapat mendengar?”

Adrian – 405100018 – Blok Penginderaan
LO 1
 Menjelaskan fisiologi pendengaran & keseimbangan
Properties of sound wave
Functions of each structre of the ear
Sound wave vibration at cochlear chambers
Sound transduction
Role of the vestibular apparatus
Balancing mechanism
LO 2
 Menjelaskan infeksi telinga dalam
Otosclerosis
  localized hereditary disorder affecting endochondral
bone of the otic capsule that is characterized by disordered
resorption and deposition of bone
 Clinical otosclerosis
  lesion that involves stapes bone & stapediovestibular joint 
conductive hearing impairment
 Cochlear otosclerosis
  occurrence of pure sensorineural hearing impairment due to
otosclerosis in an ear without any conductive component to hearing
impairment
 Epidemiology
 Etiology
 Genetic predisposition
 Females 2x >, defects in expression of the COL1A1 gene, type 1
osteogenesis imperfecta
 Measles
 Measles RNA has been found in archival & fresh footplate specimen
with otosclerosis, anti-measles antibody >>
 Autoimmune disease
 Antibodies to type II collagen
 Biochemistry
 As a result of reactivation of the arrested secondary remodelling
process within the cartilaginous rest areas of the otic capsule
 Histopathology
 Bone of otic capsule is unique  very little remodelling & contains
small region of immature cartilaginous tissue (globuli interossei)
 Otosclerotic lesions
 Areas of bone resorption, new bone formation, vascular proliferation,
connective tissue stroma (fibroblasts & histiocytes)
 Absence of acute inflammatory cells / PMN
 Blue mantle  areas of the otic capsule that stain more basophilic
 Enlargement of perivascular spaces followed by deposition of immature bone
 Remodelling continues  production of more mature bone
 Proliferation of blood vessels

 Active (spongiotic)  areas of > cellularity, vascularity (schwartze’s sign), &

bone resorption & formation
 Inactive (sclerotic)  focus consisting mineral bone
Distribution of the lesion  anterior of oval window, round window niche, stapes
footplate, posterior of oval window; walls of internal auditory canal, around vestibular
& cochlear aqueducts, semicircular canal, malleus & incus
 Pathology of conductive hearing impairment
 Expansion of the focus anterior to oval window  fibrous
fixation of footplate (conductive impairment 30-40 dB)
 Diffuse bony ankylosis involving the entire circumference of
the annular ligament  conductive loss > 40dB
 Bone remodelling  cytokines & angiogenic factors 
deposition of connective tissue & >> vascularity 
fibrovascular proliferative
 Otosclerotic bone can invade the round window niche
 Pathology of sensorineural hearing impairment
 Sensory & neural elements of cochlea & stria vascularis are
intact
 The focus reaches the endosteum of the cochlea  atrophy of
spiral ligament with impairment of fibrocytes & replacement by
an amorphous eosinophilic substance (hyalinization)
 Cytokines released by remodelling bone  reached the
ligament  disrupt fluid & ion homeostasis within the cochlea
 sensorineural hearing impairment
 Diagnosis
 Otoscopy
 Flamingo flush/schwartz’s sign (vascularity on tympanic membrane of
an active otosclerosis)  rare
 Can also occur when the membrane is abnormal (chronic otitis media)
 Gold standard  surgery
 bone around the oval window may be whiter than normal
 After surgical removal  bony atic wall thicker than normal
 Histological diagnosis
 Pure tone audiometry
 Air-bone gap & Carhart notch
 Radiology
 High resolution CT
 Management
 Fluoridation of drinking water
 Oral fluorides
 Conventional hearing aids
 Effective method of managing conductive hearing impairment
 Bone-anchored hearing aids
 Surgery
 Stapedectomy & mobilization of the stapes
 Fenestration procedures
 Mobilization of the stapes revisited along with stapedectomy with
prostheses in the later half of 20th century
Bacterial labyrinthitis
  inflammation of the labyrinth
 Serous / toxic labyrinthitis
 Sterile inflammation response to the labyrinth to bacterial toxins &
characterized histologically by acidophilic staining of the perilymphatic
fluid
 Result from COM & may be meningitis
 Suppurative otogenic labyrinthitis
 Caused by bacterial invasion to the inner ear & characterized
histologically by collections of PMN in the perilymphatic spaces at the
site of bacterial invasion
 Forming of local precipitate  endolymphatic hydrops  necrosis of
membranous labyrinth + spread to meninges
 Suppurative meningogenic labyrinthitis
 Spread of bacteria from the subarachnoid space into the labyrinth (via
cochlear duct or internal auditory canal
 Etiology
 B-haemolytic streptococci, pneumococci, Staphylococci,
Haemophilus inJluenzae, Proteus vulgaris and Pseudomonas
aeruginosa

 Clinical picture
 Acute / subacute onset of hearing loss
 vertigo with malaise and fever in association with an upper
respiratory infection
 Bacterial invasion  inflammation and tissue destruction
(necrosis) with a fibro-osseous reaction  profound auditory
and vestibular functional loss
 Clinical course
 Dissemination of infection from middle ear space (otitis media
& cholesteatoma)
 Early stages
 Sensorineural hearing loss, but various cytocochlear elements (hair
cells, spiral ganglion, stria vascularis) are intact
  the hearing loss is metabolic in nature
inflammatory cytokines disrupt the integrity of spiral ligament  ion &
fluid maintenance within the cochlea can’t be maintained
the host inflammatory response  hearing loss
 Serous labyrinthitis  reversible hearing loss
 Suppurative labyrinthitis  permanent hearing deficit
 Treatment
 Suppurative labyrinthitis
 Antibiotics + steroid
 timely institution can reverse the sensorineural hearing loss
 Acute labyrinthitis
 Bedrest, IV antibiotics, & sedatives (prochlorperazine)
 Observed carefully for early signs of meningitis
 General condition improved  middle ear & mastoid should be
explored (surgery)
 With suspected labyrinthine fistula  early surgical management to
prevent deterioration of inner ear function
 Complication
 Balance disturbance  1st sign of labyrinthine fistula
(invariably into the lateral canal)
 chronic low grade imbalance with/-out detectable nystagmus, sudden
acute vertigo (rare),
 erosion of the bone overlying the lateral canal  mucosal &
squamous epithelial disease
Viral labyrinthitis
 Clinical presentation
 Upper respiratory infection, associated with an acute disturbance of
auditory and vestibular function with vertigo and nystagmus (3-5
days)
 Varying degrees of permanent hearing loss

 Histopathology
 restricted to the scala media, vestibular labyrinth, internal auditory
canal
 degeneration of the organ of Corti, early encapsulation of the tectorial
membrane, degeneration of the stria vascularis, and round cell infiltration
of the modiolus and contents of the internal auditory canal
 saccule was degenerated with sloughing of the otolithic membrane
 Cystic degeneration of the hair cells with round cell infiltration (typical)
LO 3
 Menjelaskan gangguan pendengaran
Age related sensorineural hearing impairment
  mid- to late-adult onset, bilateral, progressive
sensorinerual hearing loss, where underlying cause have
been excluded
 Exclude
 Loud noise exposure
 Underlying medical condition
 Atherosclerosis, DM, hypertension, Paget’s disease, myxoedema
 Intrinsic otological disease
 Otosclerosis, chronic otitis media, Meniere’s disease
 Head injury
 Ototoxic drugs
 Pathology
 External ear
 Cerumen production >>, epithelial migration <<, hair growth >>, potential collapse of
ear canal, enlargement of pinna
 Middle ear
 Stiffening, thinning, loss of vascularity of tympanic membrane
 Arthritic changes & ossification in ossicles & ossicular joints
 Degeneration of middle ear muscles
 Calcification of cartilaginous support of eustachian tube
 Inner ear
 Sensory type  loss of hair cells at the basal end of the organ of corti
 Neural type  degeneration of neuron of the cochlear nerve
 Vascular / metabolic  atrophy of stria vascularis
 Mechanical / cochlera conductive associated with stiffness of cochlear basilar membrane
 Change of the cochlear duct (intracellular organelles, endolymph composition)
 Mixed
 Other factors
 Genetic
 Homozygotic twins >>
 Ahl genes in mouse chromoseome 10
 Mitochondrial genome mutations / variations
 Environmental
  not clear
 Ex: noise exposure, cigarette smoking, alcohol use, systolic blood
pressure & blood hyperviscosity
 Symptoms
 High-tone hearing loss over the age of 50 to age-related
changes
 Slow & insidious hearing problem
 Difficulty in hearing, conversation (in the presence of other
background/competing sound)
 May be tinnitus

 Examination
 Pure tone audiogram
  mild high-tone hearing loss  progressive loss of middle (1-2
kHz) and low (250 & 500 kHz) frequencies
 Diagnosis
 Over the age of 60
 Normal examination findings
 A symmetrical hearing loss

 Management
 Nonspecific management
 Hearing aids (little benefit if mild high-tone hearing loss)
 Reduction of background noise
 Psychological counselling, lip-reading class
 Specific management
 Hearing aids
 Management of tinnitus
Noise-induced hearing loss
  reduction in auditory acuity associated with noise
exposure
 May be
 temporary threshold shift (TTS)
 Hours to days
 permanent threshold shift (PTS)
 Following repeated TTS or following a single episode of noise exposure
  maximum reduction in sensitivity to stimulation in the range
3-6kHz & recovery at 8kHz
 Pathology
 Cochlear function (metabolic & structural changes)
 Recovery from TTS  role of metabolic mechanisms
 Acoustic overstimulation  excessive release of neurotransmitters
associated with transduction function of the cochlea
 Persistance of PTS  structural change mechanisms
 Change to the micromechanical structures within the cochlea
 Apoptosis & necrosis
 Cessation of voice  apoptotic changes in OHC (nuclear
condensation & cell body shrinkage) in 5 min  necrosis in 30 min
 Predisposing factors
 Smoking, DM, cardiovascular disease
 Recreational drugs; ototoxic agents
 Symptoms
 Acustic shock
 Otalgia, tinnitus, hyperacusis, dizzines, headaches, sleep disturbance,
poor concentration (common)
 Neck pain, shoulder pain, panic attacks (less)
 History of hearing difficulties in the presence of background
noise is encountered
 Lack of clarity rather than loss of volume
 Difficulty of the tv being louder than is comfortable for others
 Telephone conversation may become difficult
 As hearing loss progress  more obvious hearing problem &
frequently have to ask others to repeat themselves
 Examinations
 Pure tone audiogram with both bone & air conduction
 High-tone hearing loss on 4 or 6 kHz with some recovery at 8 kHz
 Tympanometry
 Confirm normal middle ear functioning
 Evoked reflex audiometry
 Objective measure of hearing thresholds  significant asymetry  MRI to
exclude vestibular schwannoma

 Diagnosis
 Clear prolonged history of unprotected exposure to excessive noise
 No evidence of any other otological pathology
 Audiogram  good preservation of low & mid frequencies,
significant high-tone hearing loss (classical notching 4-6 kHz)
 Managements
 Prevention
 Further noise exposure should be kept away as far as possible
 Personal hearing protection
 Earplugs, earmuffs, active noise reduction
 Non specific management
 No way to replace the hearing that has been lost
 Mild high-tone hearing lost  hearing aids
 Reduction of background noise
 More severe hearing loss  directed rehabilitation + psychological
counselling
 Specific management
 Hearing aids & tinnitus retraining therapy
LO 4
 Menjelaskan gangguan keseimbangan
Vestibular neuritis
  disorder in which there is sudden, spontaneous,
isolated, total / subtotal loss of afferent vestibular input
from one labyrinth

 Etiology
 Viral infection of the vestibular nerve
 Latent HSV type 1 infection has been demonstrated in the vestibular
nerve
 Bony canal of superior division of semicircular canal is longer
& narrower  more vulnerable to entrapment when the nerve
is inflammed
 Clinical manifestation
 Acute spontaneous vertigo
 aggravated by head movement, minimized by keeping the head still &
the eyes shut
 Nausea, vomiting
 Postural imbalance
 unsteady when standing  veer towards the side of the affected
labyrinth
 A spontaneous horizontal-torsional nystagmus
 Head impulse test  ‘catch up’ saccades with rotation of the
head toward the affected side
 Rotate towards the affected side when attempting to march on
the spot with their eyes closed (fukuda/unterberger test +)
 Examination
 Subject visual horizontal test
 Electronystagmography
 Caloric testing
 For demonstratong a canal paresis about 3-4 days after onset of symptoms

 DD
 Cerebellar infarction
 the head impulse test is (-), CT, MRI
 Labyrinthine infarction
 Hearing loss (+)
 Autoimmune inner disease
 First attack of meniere disease
 Management
 Corticosteroid (methylprednisolone) & antiviral (NOT
valacyclovir)

 Complications
 Acute peripheral vestibulopathy on the opposite side (bilateral
sequential vestibular neuritis)
 BPPV
Meniere’s disease
  disorder characterized by spontaneous attacks of
vertigo, associated fluctuating sensorinerual hearing loss,
tinnitus, & aural fullness

 Etiology
 Overproduction/malabsorption of endolymph  endolymphatic
hypertension  gross enlargement of the membranous
labyrinth (hydrops)  periodic rupture of membranous
labyrinth  leakage potassium-rich endolymph into perilymph
 meniere attacks
 Clinical manifestations
 Reccurrent attacks of spontaneous vertigo, nause, vomitng
 Lateralized low-frequency hearing loss, tinnitus, aural fullness
 Later stage of the disease  drop attacks
 Patient simply drop to the ground without warning  fracture & serious
injury
 Phase of the attacks
 Irrritative phase
 Horizontal/horizontal-torsional nystagmus, beats towards the affected ear (last
< 1 hour)
 Paretic phase
 Nystagmus beats away from the affected ear (several hours – 1 or 2 days)
  peripheral hypofunction  spontaneous neural activity in the right vestibular
nucleus is <  nystagmus beats toward the left ear
 Recovery phase
 Nystagmus beats towards the affected ear again (last like the 2nd phase)
 Diagnosis
 >=2 spontaneous vertigo
 Hearing loss documented by pure tone audiogram on at least one
occasion
 Tinnitus / aural fullness
 Electrocochleography

 DD
 Vestibular neuritis
 Single spontaneous attack of vertigo
 Migrainous vertigo
 Reccurrent spontaneous attacks of vertigo + NO auditory & vestibular/
auditory loss
 Autoimmune inner ear disease
 Management
 < production of endolymph
 Strict sodium restriction (urinary sodium <50mmol/day)
 Diuretics
 Surgery
 Endolymphatic sac surgery
 Vestibular neurectomy
 Labyrinthectomy
 Hearing <<  hearing aids by cochlear implantation
 Systemic aminoglycosides (streptomycin & gentamycin)

 Complications
 Continue to have vertigo attacks
Benign paroxysmal positional vertigo
  disorder characterized by brief attacks of vertigo,
nystagmus, precipitated by certain changes in head position
with respect to gravity

 Etiology
 Gravity, sequestered otoconia  inappropriate stimulation of the
hair cells
 Otoconia find their way into the duct of an SCC (canalolithiasis) or attach to
cupula (cupulolithiasis)  altering endolymphatic fluid pressure  vertigo
& nystagmus
 Otoconia in the posterior-anterior SCC  vertical torsional nystagmus;
lateral SCC  horizontal nystagmus
 Head trauma
 Vestibular neuritis
 Clinical manifestations
 Reccurrent episodes of vertigo following certain changes in
head position with respect to gravity
 Last 10-20 seconds
 Nausea, vomiting
 Remain unwell between attacks & constantly dizzy
 Nystagmus following Dix-Hallpike manoeuvre

 Diagnosis
 Clinical signs & MRI (exclude structural central cause)
 DD
 Migrainous vertigo
 Posterior fossa tumour
 Malformation or degenerative conditions

 Management
 Epley manoeuvre
LO 5
 Menjelaskan keganasan pada THT
Nasal cavities & paranasal sinus malignancy
 Etiology
 Carcinogenic compounds
 Hard woods in the furniture industry
 Exposure to nickel
 Smoking
 Chromium, polycyclic hydrocarbons
 Alfatoksin
 Mustard gas & thorotrast
 Radiation
 Viral & genetic

 Lymphatic drainage
 Anteroinferior part of nasal cavity  facial, parotid, submandibular nodes
 Remainder of the nose  posterior pathway runs anterior the eustachian
tube  retropharyngeal nodes
 upper deep cervical chain
 Patern of tumor spread
 Local invasion
 Regional spread
 Most common nodes  submandibular & jugulodigastric nodes

 Distant metastase
 Adenocarcinomas 18%
 Squamous cell carcinoma 10%
  bones, brain, liver, lung, skin  poor prognosis
 Staging
 Surgical pathology
 Squamous cell carcinoma
 Polypoid appearance
 Adenocarcinoma
 Adenoid cystic carcinoma
 Olfactory neuroblastoma
 Arises from the basal cells within the olfactory neuroepithelium
 Sinonasal undifferentiated carcinoma
 Melanoma
 Polypoid mass to an area of ulceration
 Haemangiopericytomas
 Presentation
 Maxillary sinus carcinoma
 Facial pain with/-out progressive anesthesia of the cheek by
infiltration of the infraorbital nerve
 Erosion of the medial wall  epistaxis
 Obstruction of the nasolacrimal duct  epiphora
 Destruction of the posterior wall & spread into pterygopalatine &
infratemporal fossa  trismus, maxillary & mandibular trigeminal
nerve deficits
 Inferior wall  loosening of the premolar & molar dentition
 Superior wall  proptosis & diplopia
 Breaks into anterolateral wall  visible swelling/distortion of the
cheek
 Ethmoid sinus carcinomas
 Unilateral nasal obstruction & epitaxes
 Epiphora, orbital swelling, proptosis & diplopia

 Frontal sinus tumours

 Orbital symptoms

 Sphenoid sinus tumours

 Invade the cavernous sinus  infiltrate the contained cranial nerves 
diplopia & facial pain

 Nasal cavity tumours

 Unilateral stuffiness & blockage
 Sinus outflow obstruction  tendency to bleed or spot nasal secretion
 Clinical evaluation
 Endoscopy, imaging (CT & MRI), biopsy

 Management
 General  pre-/postoperative radiotherapy & chemotherapy
 Surgery for maxillary tumors
 Maxillectomy (partial, total, extended)
 Other surgical procedures
 Anterior craniofacial resection
 Lateral craniofacial resection
 Orbital exenteration
 Prognosis
Juvenile angiofibroma
  uncommon, benign & extremely vascular tumour that arises
in the tissues within the sphenopalatine foramen; locally
invasive
 The tumour extends into the nasopharynx, paranasal sinuses,
pterygopalatine & infratemporal fossa
 Larger tumours can involve orbit & cavernous sinus

 Pathology
 Well defined, lobulated, covered by nasopharyngeal mucosa
 Consists of proliferating, irregular vascular channels within a fibrous
stroma
 Tumour blood vessels lack smooth muscle & elastic fibers 
sustained bleeding
 Etiology
 Androgen receptors are present in vascular & stromal elements
of the tumours (75%)
 Vascular endothelial growth factors has been found localized on
both endothelial & stromal cells
 Overexpression of insulin like growth factor II
 Mutations of adenomatous polyposis coli (APC) gene
 Presentation
 Reccurrent severe epitaxes + nasal obstruction
 Early symptoms  swelling of cheek, trismus, hearing loss
secondary to eustachian tube obstruction, anosmia, nasal
intonation
 Invasion of the orbit  proptosis, diplopia, visual loss, facial
pain, headache
 Anterior rhinoscopy
 Abudant mucopurulent secretions in the nasal cavity  obscure the
tumour from vision
 The soft palate often displaced inferiorly by the bulk of tumour
(pink reddish mas that fills the nasopharynx)
 Assessment
 Plain lateral skull radiographic
 Anterior bowing of the posterior wall of the
maxillary sinus
 CT & MRI

 Stagging system (Fisch)

 Treatment
 Preoperative embolization
 Some tumours acquire blood supply from other vessles like internal
carotid artery
 Preoperative chemotherapy
 Flutamide (nonsteroidal androgen receptor blocker)
 Surgical resection
 Endoscopic endonasal techniques
 Open approaches
 Radiotherapy
References
 Sherwood, human physiology
 Scott Brown’s otorhinolaryngology, 7th edition