DISEASES

 OF  THE  EAR:  

EVALUATION  &  MANAGEMENT  

DEPARTMENT OF OTORHINOLARYNGOLOGY - HEAD & NECK SURGERY

FACULTY OF MEDICINE & SURGERY
UNIVERSITY OF SANTO TOMAS
CARDINAL  SYMPTOMS  OF  EAR  DISEASE  
•  Tinnitus - sensation of sound in the absence of
any external acoustic stimulation
•  Hyperacusis - oversensitive hearing ability

•  Hearing  Loss  
•  Hypoacusis - diminished hearing ability
•  Dysacusis - distortion of hearing, imprecise
comprehension, sound distortion
•  VerAgo - an illusion of movement self or of the
environment
 CAUSES  OF  TINNITUS  
•  Subjective tinnitus – heard only by the patient
§  Otologic: hearing loss, Meniere's disease, acoustic
neuroma
§  Ototoxic: medications or substances
§  Neurologic: multiple sclerosis, head injury
§  Metabolic: thyroid disorder, hyperlipidemia, vitamin
B12 deficiency
§  Psychogenic: depression, anxiety, fibromyalgia

•  Objective tinnitus - heard by patient and examiner

§  Vascular: arterial bruit, venous hum, arteriovenous
malformation, vascular tumors
§  Non-vascular: TMJ, palatal myoclonus, middle ear
muscle
Crummer  RW,    2004  
DIAGNOSTIC
ALGORITHM
NZ Best Practice
Journal, 2012
EVALUATION  OF  TINNITUS  
Medical   Onset, laterality, pattern, pitch characteristics,
History   associated symptoms, exposures, exacerbating/
alleviating factors, medical history (use validated
questionnaires – THI)

Physical   Vital signs, palpation of periauricular and cervical

ExaminaAon   area, TMJ, oral/orophrayngeal cavity, otoscopy,
neurotologic and vestibular exam

Audiologic   PTA, speech tests, loudness discomfort levels,

EvaluaAon   tympanometry, acoustic reflex, OAE, ABR

Imaging   Only for pulsatile (vascular) tinnitus, unilateral

Studies   hearing loss, suspicion of mass lesion

Laboratory   If indicated - CBC, lipid & thyroid profile, FBS, GTT,

Tests   immunologic studies (ANA, ESR), FTA-ABS
 TINNITUS  TINNITUS MANAGEMENT GUIDE
MANAGEMENT  GUIDE  

TINNITUS

MILD SEVERE

ACUTE CHRONIC
•  Elimination of
cause •  Pharmacotherapy MASKABLE UNMASKABLE

•  Explanation & •  Acoustic Therapy

•  Pharmacotherapy
reassurance •  Counseling •  Masking
device •  Counseling/Retraining
•  Stress Therapy
Reduction •  Acoustic •  Physiological Stimulation
Therapy (Neuromonics,
•  Diet/Lifestyle
Transmagnetic etc.)
modification
•  Self-help Groups
HEARING  LOSS  

SITE OF LESION TYPE OF LOSS

Outer Ear
Conductive
Middle Ear

Inner Ear Sensory/Cochlear Sensorineural

Auditory Nerve to Neural/Retrocochlear

Cortex

TIME OF INJURY Prenatal Postnatal
(Conception to Birth) (Birth to Death)

TYPE OF LOSS Congenital Acquired

CONGENITAL  HEARING  LOSS  

•  Hearing impairment is the single most common

condition affecting newborns
§  1-3 per 1,000 in well-baby
§  2-4 per 100 in the NICU

•  Republic Act 9709

§  Universal Newborn Hearing Screening and
Intervention Act of 2009
§  RULE: Screening (1st month), Identification
(3rd month) and Intervention (6th month)
EFFECTS  OF  HEARING  LOSS  IN  CHILDREN  

Congenital or acquired hearing loss in infants and children

has been linked with:
•  Lifelong deficits in speech and language acquisition
•  Poor academic performance
•  Personal and social maladjustments
•  Emotional difficulties
HIGH-­‐RISK  REGISTRY  
•  Apgar <5 in 5 minutes
•  Bacterial meningitis
•  Congenital infection (TORCHES)
•  Defects of head and neck
•  Elevated bilirubin level
•  Family history
•  Gram weight <1,500 g
•  History of NICU enrollment
•  Intake of ototoxic drugs
AUDITORY  MILESTONES  

Age (Months) Description

0-3 Startles at a loud sound or noise
Stops moving or crying when you call
3-6 Turns head or moves eyes to a familiar
sound
6-10 Responds to his/her own voice
10-15 Repeats simple words and sounds you
make
15-18 Understand simple phrases and can point to
body parts
18-24 Should have at least 150 spoken vocabulary
CONGENITAL  HEARING  LOSS  

Usher Stickler
Pendred Norrie
Jervell and Lange-Nielsen CHARGE
Goldenhar Treacher Collins
Syndromic (30%) Waardenburg Neurofibromatosis
Branchio-Oto-Renal Osteogenesis
Imperfecta
GENETIC (50%)
Autosomal Recessive (77%) DFNB1 – DFNB30
Non-Syndromic (70%) Autosomal Dominant (22%) DFNA1 – DFNA3
X-Linked (1%) DFN1 – DFN8
Mitochondrial (<1%)

Maternal TORCHES Infection

Meningitis
Hyperbilirubinemia
ENVIRONMENTAL (25%)
Prematurity
Low birthwieght
Ototoxicity

IDIOPATHIC (25%)

EVALUATION  OF  CONGENITAL  HEARING  LOSS  
Pediatric History   Onset of hearing loss and progression of symptom,
history of exposure to risk factors, history of
pregnancy, delivery and postnatal period,
developmental milestones, family history of deafness  
 
Clinical Measurement of height, weight and head
Examination   circumference; Inspection of craniofacial region;
Examination of the ears, neck, skin and nails, limbs,
chest, abdomen and gait  

Age-Specific Screening: OAE/AABR  

Audiological Below 6 months – ABR
Assessment   36 months to 6 years – Play audiometry  
6 to 36 months – Visual Reinforcement Audiometry
6 years and above – PTA  
Medical Referral to Pediatrics, ORL-HNS, Ophthalmology, etc.  
Evaluation  
EVALUATION  OF  PEDIATRIC  HEARING  LOSS  
Imaging Studies   Assess the anatomy (inner ear malformations in 20%;
ossicular chain integrity; facial nerve)  
Identify cause (narrow IAC; skull base/inner ear
trauma)  
Provide prognostic information on potential treatments
(fibrous or osseous labyrinth obstruction)  
Genetic testing   Syndrome is suspected; multiple system
abnormalities; opinion required on interpretation of
genetic mutation testing; after completion of
investigations if a genetic disorder is diagnosed;
parental request  
Expanded TORCH titers; Connexin 26; Electrocardiography;
Laboratory Work CBC; Urinalysis; ANA; ESR; BUN; Creatinine; FTA;
Up Protocol   Glucose; Thyroid function; Perchlorate; Liver function
test; Radiographic studies (renal USG)  
 graph serves as a guideline for finding an ideal solution for your
child’s hearing
MANAGEMENT   OF  Closs.
ONGENITAL  HEARING  LOSS  
LOW FREQUENCY (Hz) HIGH

125 250 500 1000 2000 4000 8000

SOFT

-10

NORMAL
0 HEARING
NO AMPLIFICATION NECESSARY
10

20

30 MILD
LOUDNESS (dBHL)

40

50
INDICATION FOR A HEARING AID MODERATE
60

70

80 SEVERE

90

100
INDICATION FOR A COCHLEAR IMPLANT PROFOUND
110

LOUD 120
HEARING  DEVICES  

COCHLEAR  IMPLANT  

HEARING  AIDS  
COMMON  CAUSES  OF  ACQUIRED  HEARING  LOSS  

CONDUCTIVE HEARING LOSS Cerumen

Middle Ear Infection
SENSORINEURAL Known Noise-Induced
HEARING LOSS Etiology Ototoxicity
Acute Labyrinthtis
Unknown Sudden Sensorineural
Etiology Hearing Loss
Age-Related
(Presbycusis)

CONDUCTIVE and/or Temporal Bone Fracture

SENSORINEURAL HEARING LOSS
 IMPACTED CERUMEN - associated with symptoms
CERUMEN   (itching, pain, hearing loss, tinnitus, dizziness,
discomfort, and increased infection risk), AND/OR
prevents needed assessment of the ear

•  Therapeutic Interventions:
§  Observation
§  Cerumenolytic agents
§  Irrigation
§  Manual removal with a curette, probe,
hook, forceps, or suction
WET  
§  Combination of the above

•  Under direct visualization with headlight,

otoscope, or microscope

WATCH OUT FOR: allergy, diabetes mellitus,

immunocompromised state, anticoagulant therapy,
previous ear surgery, perforated TM
DRY  
 Otitis Media - inflammation of the middle ear
MIDDLE  EAR   Acute Less than 3 week course
INFECTION   Subacute 3 weeks to 3 months
Chronic 3 months or longer

Host Related Risk Factors (Non-

Modifiable): age, gender, race,
genetics

Environment Related Risk

Factors (Modifiable): large
numbers of siblings, low socio-
economic status, smoke
exposure, attendance in day care
centers, milk-formula feeding
 •  History of acute onset of signs and
ACUTE  OTITIS  MEDIA   symptoms
Most common bacterial pathogens: •  Presence of middle ear effusion
Streptococcus pneumoniae •  Signs and symptoms of middle ear
Hemophilus influenzae inflammation
Moraxhella catarrhalis
Diagnosis: History and otoscopy (pneumatic)

Treatment
•  Observation in uncomplicated AOM
•  Management of pain during the first 24 hours
(Paracetamol or Ibuprofen)
•  Antibiotic therapy may be initiated based on
severity of illness, as defined by symptoms,
otoscopic findings and presence of risk factors
(Amoxicillin is drug of choice)
•  Myringotomy for severe cases and those with
complications
OTITIS  MEDIA  WITH  EFFUSION  (OME)  
•  Fluid in the middle ear without signs or symptoms of acute ear
infection
•  Tympanic membrane is cloudy with distinctly impaired mobility,
and an air-fluid level or bubble may be visible in the middle ear
Diagnostic Considerations
Thorough history
Pneumatic otoscopy as primary diagnostic method
Tympanometry (Type B) used to confirm the
diagnosis
Hearing test recommended when OME persists for
3 months or longer

Treatment
•  A child NOT at high risk for speech &
language delay managed with watchful
waiting
•  Myringotomy and ventilation tube insertion
CHRONIC  OTITIS   ME inflammation with persistent
or recurrent ear discharge over
MEDIA  (COM)   3 months through a perforation
of the TM

COM without Cholesteatoma - chronic perforation of

eardrum and purulent discharge

COM with Cholesteatoma - chronic perforation of

eardrum that has cholesteatoma (epidermal inclusion
cysts of the middle ear or mastoid that contains
desquamated debris) and purulent discharge

Inactive COM - chronic perforation of eardrum that

are dry, or discharge only occasionally, and have no
sign of active infection
CHRONIC  OTITIS  MEDIA  (COM)  
Diagnostic Considerations
Thorough history and otoscopic examination
Pure tone audiometry and speech testing
Radiographic imaging with CT scan:
•  Medically unresponsive
•  Presence of cholesteatoma (lesion mapping & surgical planning)
•  Atypical course/high risk patients
•  Presence of intra-temporal or intracranial complications

Medical Therapy
•  Aural toilet is an essential part of treatment in all patients
•  Topical antibiotics (otic drops)
Surgical Therapy
•  Mastoidectomy – eradication of disease by drilling affected mastoid
bone and removal of cholesteatoma
•  Tympanoplasty – patching of TM perforation; ossicular
reconstruction
COMPLICATIONS  OF  OTITIS  MEDIA  
Pathways of Infection Spread
•  Bone erosion due to cholesteatoma
•  Vascular extension (retrograde thrombophlebitis)
•  Extension along preformed pathways: congenital
dehiscences, fracture lines, round window membrane,
labyrinth, dehiscences due to previous surgery

Extracranial Complications Intracranial Complications

•  Mastoiditis
•  Cholesteatoma
•  Post-auricular abscess
•  Facial nerve paralysis
•  Labyrinthine fistula
•  Labyrinthitis
•  Petrous apicitis
•  Meningitis
•  Cerebral abscess
•  Sigmoid sinus thrombosis
•  Otitic hydrocephalus
•  Subdural abscess
•  Epidural abscess
NOISE-­‐INDUCED
 HEARING
 LOSS
 
  Permanent cochlear damage
(OccupaAonal
 or
 RecreaAonal)
  due to intense sound exposure
Temporary threshold shift (TTS) - reversible loss from sound exposure
Permanent threshold shift (PTS) - permanent change in hearing

•  History of long-term exposure to

dangerous noise levels (i.e., >90
dBA for 8 hours/day)
•  Impairment involves symmteric mid-
frequency range, primarily 3 to 6
kHz, with “4-kHz notch”
NIHL Prevention:
•  Noise exposure monitoring
•  Engineering and administrative
controls
•  Audiometric evaluation
•  Use of hearing protection devices
•  Education and motivation
ACOUSTIC  TRAUMA   Single exposure to a severe sound
causing violent changes in air pressure

•  Caused by a single, short-

lasting exposure to a very
intense sound (e.g.,
accidental explosive blast,
shooting, firecrackers)
•  Sudden, usually painful loss
of hearing, tinnitus
•  Asymmetric “4-kHz notch”
•  Prevention: stop exposure,
hearing protection devices
 Degeneration of the inner ear and of the
OTOTOXICITY   eighth cranial nerve due to drug effects

•  Toxicity generally occurs only after days or weeks

of exposure
•  May continue after cessation of therapy due to
active metabolites
•  Bilateral high-frequency hearing loss initially
progressing to lower frequencies
•  E.g. aminoglycosides, diuretics, NSAID’s/
salicylates, Quinine, Cisplatin
•  Prevention: monitoring using hearing tests and drug
peak-and-trough levels, substitution with nontoxic
drugs
 IDIOPATHIC  SUDDEN  SENSORINEURAL  
HEARING  LOSS  (ISSNHL)  
•  Decrease in hearing of > to 30 decibels, affecting at
least 3 consecutive frequencies, occurring over a 3
day period
•  Idiopathic - attributed to vascular, viral, or autoimmune
•  32% to 65% of cases may recover spontaneously
•  Recovery dependent on time between onset and
treatment, patient age, presence of vertigo at onset,
degree of hearing loss, and audiometric configuration
•  Management: corticosteroids as initial therapy;
hyperbaric oxygen therapy within 3 months, hearing
amplification
AGE-­‐RELATED  HEARING  LOSS  (PRESBYCUSIS)  
•  Progressive, bilateral, symmetrical, sensorineural
hearing loss
•  A multi-factorial complex disorder (environmental
and genetic factors)
•  Diagnosis is by exclusion
•  High frequencies are usually affected first, but the
hearing loss can be “flat” across all frequencies
•  Poor word recognition and speech comprehension
due to central degeneration
•  Can coexist with other forms of hearing loss
ACUTE  LABYRINTHITIS  
•  Inflammation of the labyrinth
•  Cardinal symptoms: Hearing loss, tinnitus, vertigo

Serous Labyrinthitis
•  Inflammation of labyrinth from mediators or bacterial
toxins; labyrinth not infected
Suppurative Labyrinthitis
•  Inflammation of labyrinth through direct extension from
middle ear infection; more rapid and serious symptoms

•  Treatment: high-dose antibiotics, steroids, close and

continuous monitoring for symptoms, surgery for
cholesteatoma
SIGNS  &  SYMPTOMS  OF  
TEMPORAL  BONE  FRACTURE  

•  Battle’s sign
•  Hemotympanum
•  Raccoon eyes
•  Otorrhea or rhinorhea
•  Facial nerve palsy/
paralysis
•  Persistent dizziness,
vertigo
TEMPORAL  BONE  FRACTURE  
When the head trauma is of sufficient magnitude
to fracture the skull, 14% to 22% of injured
patients sustain a temporal bone fracture

Otic Capsule Otic Capsule

Sparing Disrupting
94.2 - 97.5% of 2.5 - 5.8% of
cases cases
OTIC  CAPSULE  SPARING   Blow to Blow to occipital
temporoparietal region
region
CN VII paralysis CN VII paralysis
in 6-14% in 30-50%
Conductive or Sensorineural
Mixed hearing hearing loss
loss
CSF leak less CSF leak 2 to 4
likely times more likely
OTIC  CAPSULE  DISRUPTING  
COMPLICATIONS  OF  TEMPORAL  BONE  FRACTURE    
CEREBROSPINAL  FLUID  OTORRHEA
Diagnosis: Halo sign; β2 transferrin,
HRCT or intrathecal flourescein
Management:
•  Observation, elevate  head  of  bed,  
bed  rest,  stool  so7eners,  prophylac<c  
an<bio<cs,  lumbar  drain  
•  Surgical  closure  if  leak  persists  for  
7-­‐10  day  
FACIAL  NERVE  PARALYSIS
Diagnosis: Electroneurography (EnoG)
Management:
•  Observation and steroids
•  Facial nerve surgical decompression:
95% denegeration on EnoG
MANAGEMENT  OF  HEARING  LOSS  
HEARING LOSS TYPE RECOMMENDED MANAGEMENT
Conductive • Medications
• Surgery
• Hearing Aids
• Implantable Devices (BAHA, Middle ear implant)

Cochlear • Medications (for ISSNHL)

• Hearing Aids
• Cochlear Implant (for severe to profound HL)
• Speech Therapy for children

Neural • Hearing Aids (?)

• Cochlear Implant (?)
• Auditory Brainstem or Midbrain Implant
• Speech Therapy for children

Central • Auditory Processing Rehabilitation Therapy

• Frequency Modulation Systems


TYPES  OF  DIZZINESS  

VerAgo   Presyncope   Disequilibrium   Other  

Subtypes  
•  An illusion of •  A feeling of •  A sense of •  Swimming or
movement, faintness or loss unsteadiness in floating
usually of the lower body sensations
rotations consciousness •  More felt in the •  Feelings of
•  More felt in lower extremities dissociation
the head •  Relieved when •  Difficult to
sitting down describe

Drachman  DA,  Hart  CW.  Neurology  1972  

NYSTAGMUS  
COMMON  CAUSES  OF  VERTIGO  

SUBTYPE LIKELY CAUSE

Position-induced BPPV
Cervical vertigo
Acute-onset persistent with Stroke
neurologic signs Tumors
Degenerative diseases
Acute-onset persistent Labyrinthitis
without neurologic signs Vestibular neuritis
Recurrent with no neurologic Meniere’s disease
signs Migraine

Eggers SD, Zee DS, 2010.

DIAGNOSIS  OF  VERTIGO:  TWO  BY  TWO  TABLE  
Two Important Questions to Ask Patients:
1. Is the vertigo INTERMITTENT or PERSISTENT?
2. Is there associated HEARING LOSS?

INTERMITTENT PERSISTENT
With
MENIERE’S VESTIBULAR
Hearing
DISEASE SCHWANOMMA
Loss
No
VESTIBULAR
Hearing BPPV
NEURITIS
Loss
BENIGN  PAROXYSMAL  POSITIONAL  VERTIGO  (BPPV)  
PRESENTATION  
•  Brief, episodic, and transient vertigo
induced by a rapid change in head
position
•  Caused by canalithiasis/cupulithiasis
in the posterior SCC commonly
•  Risk factors: head trauma, vestibular
neuritis, infection, surgery, prolonged
bed rest
DIAGNOSIS  
•  Dix-Hallpike Maneuver
•  Normal PTA, speech test, caloric exams
MANAGEMENT: Particle repositioning therapy (Epley or
Semont maneuver)
DIX-­‐HALLPIKE  &  EPLEY  MANEUVER  
MENIERE’S  DISEASE   PRESENTATION  
•  Two or more spontaneous episodes of
vertigo, each lasting 20 minutes to 12
hours
•  Fluctuating aural symptoms (hearing
loss, tinnitus or fullness) in the
affected ear
DIAGNOSTICS  
•  PTA: low to medium frequency
sensorineural hearing loss in one
ear, on at least one occasion before,
during or after one of the episodes of
vertigo
MANAGEMENT
•  Low salt diet
•  Betahistine for 2 to 3 months
•  Diuretics
VESTIBULAR  NEURITIS   PRESENTATION  
•  Sudden vertigo with
unsteadiness, nausea or
vomiting. Etiology may be viral
•  Persistent vertigo (days to
weeks)
•  No auditory deficits & neurologic
symptom

PHYSICAL  FINDINGS  
•  Spontaneous nystagmus to the contralateral ear for first 3 days
•  (+) saccades on head impulse test

DIAGNOSTICS  
•  PTA & speech test: normal
•  Calorics: reduced or absent caloric response in one ear
MANAGEMENT  OF  VESTIBULAR  NEURONITIS  
•  Continuous
vertigo •  Vertigo
•  Bedridden weeks to
with gradual months
ACUTE   recovery •  Increased
discomfort
REHABILITATION  
PHASE  
PHASE  
•  Postural of activity
imbalance that
•  Horizontal involves
spontaneous motion
nystagmus

ACUTE  PHASE:  STEROIDS   REHAB  PHASE:  

VesAbular  suppressant  for   VesAbular  
symptomaAc  relief  ONLY     RehabilitaAon  Therapy  
VESTIBULAR  SCHWANOMMA  
PRESENTATION  
•  Unsteadiness rather than vertigo
•  Unilateral hearing loss or
tinnitus
•  May have cranial nerve deficits
(e.g. CN V- decreased corneal
reflex)
DIAGNOSIS  
•  PTA: > 20dB threshold average between ears; Speech Test:
15% SDS difference; ABR: IA wave V difference > 0.3 msec.
•  Calorics: reduced or absent caloric response in one ear
•  Gold Standard: MRI with gadolinium
MANAGEMENT  
•  Observation and monitoring
•  Stereotactic (gamma knife)/vestibular schwanomma surgery
DIAGNOSTIC
ALGORITHM
Edmonds M. Adelaide
Emerg Phys. 2014