University of the Philippines Manila

College of Medicine-Philippine General Hospital

Department of Otorhinolaryngology

A SELF-INSTRUCTIONAL MANUAL ON DISORDERS OF

HEARING: DIAGNOSIS, PATHOPHYSIOLOGY AND
PRINCIPLES OF THERAPY
FOR YEAR LEVEL IV MEDICAL STUDENTS

SYNOPSIS

This module will enable the year level IV student to adopt a basic approach to the
diagnosis and principles of management of disorders of hearing by correlating signs,
symptoms and pathophysiology.

H-1
 INTRODUCTION

“HEARING → LANGUAGE → COMMUNICATION → KNOWLEDGE”

Dear Medical Student,

In year level III, you learned about sound, the structures of the ear, how it collects
and amplifies sound and its pathway from the external ear to the brain. This time,
you will learn how to “think through” the approach to diagnosis and principles of
therapy of disorders of hearing.

Although the organ system concerned with hearing is also the center for balance, we
shall concentrate on disorders of hearing as they occur from the external and middle
ear to the inner ear and central pathways. Generally, the former causes a
mechanical or conductive hearing problem while the latter cause an electrochemical
or sensorineural loss. We will learn how to obtain a good clinical history and conduct
an otologic physical examination to differentiate the two main causes of disordered
hearing, correlating the signs and symptoms with underlying pathophysiologic
processes. Where needed, we will learn how to order ancillary examinations such as
diagnostic imaging to confirm presumptive diagnosis beyond the capabilities of the
history and physical examination. This is an activity workbook. As you solve each
case and complete each activity, pieces of the puzzle fell into place. You must
discover the “how’s and the why’s” of disorders of hearing before you can arrive at
the “what’s” and perform the “how’s”.

TABLE OF CONTENTS
INTRODUCTION AND TABLE OF CONTENTS H-2
LESSON 1: The Tools of Our Trade: History, Physical Examination H-3
and Diagnostic Tests
LESSON 2: Conductive Hearing Loss H-15
LESSON 3: Sensorineural Hearing Loss H- 25
LESSON 4: Mixed Hearing Loss H- 42
REFERENCES H- 47

H-2
 Lesson 1
The Tools of Our Trade:
History, Physical Examination and Diagnostic Tests

Purpose: At the end of this lesson, the student should be able to:
1. obtain a systematic and comprehensive clinical history and perform an
adequate physical examination on a patient with hearing loss
2. demonstrate the clinical tests that will aid in determining the diagnosis
3. differentiate conductive, sensorineural and mixed hearing loss
4. list the most likely differential diagnosis and arrive at a single most likely
(parsimonious) cause of disease
5. enumerate some of the ancillary diagnostic tests for evaluating disorders
of hearing

A good clinical history and physical examination separates the would be

doctor from the skilled technician and may help you clinch the right diagnosis and
devise the proper management “90%” of the time.

CLINICAL HISTORY: Tool Kit Guide for “Hearing loss”

Chief Complaint Please match the chief complaint with
- use the patient’s own words: the following terms:
- a. “nabibingi”, “mahina ang ____ dizziness
pandinig” ____ vertigo
- b. “nahihilo” ____ decreased hearing, hearing loss
- c. “umiikot ang paligid” ____ ear discharge
- d. “sipon sa tainga, luga” ____ tinnitus
- e. “umuugong”, “humuhuni ang
tainga” Others: ear fullness, speech delay, can
hear but not understand words, ear
pain, ear deformity, etc.
Onset: “bigla”, “dahan-dahan”, Sudden occurrence vs progressive,
“pabago-bago” waxing and waning vs persistent or
Age at present and age of onset progressive; duration, character,
Presentation resolution, etc.
Unilateral Very important not to mix this up ☺
Bilateral
Symmetry (which is the better ear)
Previous Surgery or Procedures You want to know if the basic anatomy
is altered…

H-3
Nasal Problems like Recent URTI You can blame the nose and throat for a
(Upper Respiratory Tract Infection), lot of problems of the ear. While pinching
nasal obstruction or discharge, etc. your nose and keeping your mouth
closed:
1. blow your nose until your ears “pop”
1. swallow repetitively; do your ears
pop?
Trauma What kind: cotton buds?, stick? Blast
injury? Pressure changes? Blunt trauma
to the head?
Ear Infections Dig deeper into presentation and course
☺
Otorrhea Means ear discharge: watery or mucoid,
purulent, foul smelling, bloody, etc
Otalgia Ear pain: (may also be “referred pain”,
e.g. from the temporomandibular joint,
tongue, pharynx and tonsils); What is the
pathway and nerves involved?
Tinnitus (character and duration) A “ringing”, “blowing” or “buzzing” sound
in the ear, high or low pitched, pulsatile,
intermittent or persistent,
Vertigo Sensation of spinning of self or
surroundings (must be differentiated from
dizziness), headache, blurring of vision,
syncope
Family History Hearing loss can be congenital…
Some risk factors may be inherited…
Risk Factors Past Medical and Personal Social History:
Medical Diabetes, hypertension, syphilis, and
Occupational other disease; smoking, alcohol, sexual
Environmental practices, noise exposure
*For Congenital Hearing Loss All newborns must undergo hearing
- family history screening.
- failed universal hearing screening
- Newborn with high risk factors:
- Asphyxia (low APGAR<3)
- Bacterial meningitis
- Congenital Perinatal Infection
(TORCHES)
- Defects of the Head and Neck
- Elevated Bilirubin
- Family History
- Gram birth weight <1.5 kg

H-4
Did you know???
The “Valsalva” maneuver is actually of two kinds, one is holding your breath
and exerting by contracting your tummy like you want to defecate, the other one is by
pinching and blowing your nose to pop your ears. “Toynbee” maneuver on the other
hand is the opposite. One pinches the nose and swallows air. This results in you
swallowing air from the nasopharynx and oropharynx. You actually “reverse popped”
your ear. Hence, you exercise your eardrum inward and outward in the process.

Activity 1

Interview a friend or relative who you think may have hearing loss (e.g., grandparent,
policeman or drummer friend) and practice your history taking skills. Summarize your
clinical history in the space provided. Exchange workbooks with a classmate and
comment on each other’s histories. You may be asked to share your history during
the group discussion. _________________________________________________
___________________________________________________________________
___________________________________________________________________
___________________________________________________________________
___________________________________________________________________
___________________________________________________________________
___________________________________________________________________
___________________________________________________________________
___________________________________________________________________

PHYSICAL EXAMINATION TOOL BOX

Otoscope Pneumatoscope Tuning Fork (512 Hz)

Pneumatoscope (optional): this evaluates how well the eardrum moves when
pressure inside the ear canal is changed and can be modified using an asepto bulb
and/or Intravenous plastic tubing and superglue. Construct one as shown in the
diagram. Just be careful using this so as not to rupture the ear drum or cause noise
induced hearing loss.

H-5
Materials: Pediatric intravenous plastic tubing and aseptosyringe or ear syringe
Method: Cut around a foot of the distal end of the IV tubing and attach to the tip
of the syringe. You can attach the distal end to your otoscope like the one
above and you have an improvised pneumatoscope. Now you can put it in the
ear canal of the patient making sure that it snugly fits and introduce pressure by
pressing on the bulb.

PHYSICAL EXAMINATION TOOL KIT

External and Middle Ear Examination and Otoscopy Findings: Take note of the
normal pinna and skin. Some common abnormalities include preauricular pits and
sinuses, skin tags, microtia.

Activity 2
Very quickly, which of these are preauricular sinus, skin tags and microtia?

a. b. c.

H-6
Otoscopy examines the outer (cartilaginous) and the inner (bony) external ear canal,
the tympanic membrane and the bony impressions of the ossicles. In presence of a
perforation like in this example, one may examine the middle ear mucosa and exposed
ossicles. Look at the (hopefully) normal ear of a classmate and compare with the
illustrations.

Activity 3
Now match the following common conditions with these illustrations.
____ 1) Otitis externa showing a narrowed canal and pus
____ 2) Chronic suppurative otitis media with brownish dried discharge
____ 3) Acute otitis media, hyperemic stage
____ 4) Attic perforation with cholesteatoma formation

a. b. c. d.

Obstruction, discharge (quality and smell, etc),

Ear Canal (Lumen and skin
surface)
Tympanic Membrane Smoothness, intactness, retracted or bulging, size
and location of perforation, scarring, etc.
Compliance and mobility can be assessed using a
pneumatoscope.
Middle Ear Space Seen through the perforation: smooth or polypoid,
edematous mucosa; parts of ossicles; discharge
and quality; presence of mass; etc.
Tuning Fork Test Use 512 Hz tuning fork and recall findings for
Weber, Rinne, Schwabach normal versus a conductive, mixed and
sensorineural hearing loss.
Craniofacial deformities Microtia, cleft lip and palate, micrognathia,
(auscultate for bruits too) macroglossia, etc.
Language Delays AGE versus LANGUAGE skills
Cranial Nerve Examination Tumors, vascular lesions (you can auscultate for
Neurologic Examinations bruits), cortical or brainstem injuries from
(Perform your 10-minute Neuro compression, infarcts, trauma, bleed, etc may
Exam) manifest as neurological deficits

H-7
Activity 4

Now go back to your chosen “subject” from whom you obtained a clinical history. Draw
your findings in the space provided. Compare these drawings with your classmate,
questioning each other’s physical findings. Do they correlate with or explain the problem
you identified in the clinical history?
HOW to DRAW your findings:
1. Draw the “anterior canal bulge” and borders of your eardrum.
2. Emphasize the anterior malleolar fold.
3. Outline well the malleus handle-taking note of the lateral process. Is it prominent or
not?
4. Complete your drawing by showing the posterior malleolar fold and adding the cone
of light. The anterior and posterior malleolar folds border the “attic”. The cone of
light gives you the impression of the surface of the pars tensa. (Imagine a light
reflection of a convex and a flat TV screen. It’s as if you’re looking at a concave TV
screen with reflected light.)

1. 2. 3. 4.

Right ear or Left ear? And is the

perforation at the pars tensa or pars
flaccida?

DIAGNOSTIC EXAMINATIONS TOOLKIT

TUNING FORK TESTS
The most basic and easiest diagnostic tests that you should do in all patients with
hearing loss are Tuning Fork Tests. For this, a 512 Hz tuning fork is recommended
because it is more sound producing than vibratory. The patient should be examined in a
quiet room, preferably in a sound proof booth. There are several tuning fork tests, but the
most commonly used are the Weber, Rinne and the Schwabach Tests. Recall your Year
Level III Manual and fill in the blanks:

Example: Conductive Hearing loss of the right ear

Weber: Lateralizes to the ___________(right or left)
Rinne: Right - AC __ BC (>, < or =)
Left – AC __ BC (>, <, or =)
Schwabach: Right- patient’s ear ___ examiner’s ear (>, <, v=)
Left – patient’s ear ___ examiner’s ear (>, <, =)

Clue: Assuming you have normal ears, plug your right ear canal with a cotton ball and do
the tests on yourself (except for Schwabach).

H-8
 Tuning fork tests provide a rough estimate of a patient’s hearing. In the absence
of machine to measure hearing, the tuning fork tests may suffice. Knowing the sound
intensity of a tuning fork, this basic screening tool can be used even in the primary
setting. The Ear Institute of the National Institutes of Health is utilizing tuning fork tests to
detect mild hearing loss in elementary school children. With this simple test administered
by the school nurses, students with moderate to profound hearing loss are identified.

Activity 5
Perform tuning fork tests on your chosen “subject” (yes, the same “victim” who
agreed to your history taking and physical examination). In the space below, write down
the diagnostic tests you think should be requested for your “patient,” briefly explaining
why. If no anciliary tests are warranted, explain why also. Exchange workbooks with
your classmate, and discuss each others’ findings. You may discuss this with your
preceptor individually or during group discussion sessions. Once clear on your
recommendations, you may actually communicate these with your “patient” (or refer
them for a real consultation which you may observe to confirm your findings and see
how your consultant(s) obtain the history and perform the otologic examination.
______________________________________________________________________

Aside from the tests mentioned, there are other ancillary diagnostic tests that need
special equipment.
OTHER DIAGNOSTIC HEARING TESTS THAT NEED SPECIAL EQUIPMENT

The tuning fork test can only grossly determine hearing loss and is applicable to
cooperative patients. Other hearing tests that can be employed to determine status of hearing
are the following. These are behavioral tests and are therefore subjective

Item Behavioral Visual Play Audiometry Adult Audiometry

Observation Reinforcemnt
Audiometry Audiometry
Age < 6 mos 6-24 mos 2-4 yrs >5 yrs
Environment Quiet room Sound booth Sound booth Sound booth

Stimulus Speech, Warble Speech, Warble Speech, Pure tones Speech,Pure tones
tones, rattles, tones
horns
Delivery Sound field Sound field Headphones Headphones

Response Eyes; startle Head turning Task (blocks) Raise hand

Reliability Poor to fair Good Good to Excellent

excellent
Adapted from Lapena. 2003. Do you hear what I hear? Special senses screening in ENT. Unpublished lecture

H-9
 Objective hearing assessment also involves special equipment. However, these
tests do not require active participation from the patient. Thus, these tests are
usually used in children and infants.

Otoacoustic Emissions are responses of the ear to introduction of sound.

This test can be done in a few seconds and detects mild to profound hearing
loss. This is further discussed in Lesson 3 Sensorineural Hearing Loss.

Auditory Brainstem Response involves placing scalp electrodes on the

patient then detecting changes in brainwave patterns on introduction of clicks.
This examination takes a few minutes, requires that a child be stilland and
usually necessitates sedating the child. This examination detects mild to
profound hearing loss and can localize site of lesion.

Tympanometry is done much like OAE and lasts for a few seconds. This test
evaluates compliance of the eardrum, eustachian tube function, identification
of middle ear fluid and detection of pinpoint perforations. Though this test
does not evaluate hearing loss per se, it can identify factors contributing or
causing the hearing loss.

Let us now move to pure tone audiometry, the most commonly requested among
these diagnostic tests;
Pure Tone Audiometry

Pure tone audiometry is an objective test where patient cooperation is needed. The
patient is placed in a soundproof booth and headphones applied on both ears. Pure tones
of different frequencies (high pitch and low pitch) are successively introduced to one (test)
ear at different intensities (soft and loud volumes) until the minimum (threshold) sound
volume in decibels (dB) the subject hears in each frequency is arrived at.
The frequency of sound is then plotted on the x-axis while the threshold in hearing
for each is plotted on the y -axis. A mark is placed at each frequency and minimum
intensity that the patient hears. With the aid of pure tone audiometry, one can
quantitatively measure hearing loss in each ear. It can also distinguish conductive,
sensorineural or mixed hearing loss.
H-10
 The speech frequencies or the frequencies of sound usually used in speech are
the 500 KHz, 1000 KHz and 2000 KHz. The average hearing levels for these
frequencies are used to indicate the severity of the hearing impairment of the patient.
In plotting the values obtained, red marks correspond to the R ear and blue or
black marks for the left ear. A “0” mark is air conduction while “x” is bone conduction.
The severity of the hearing loss is obtained by getting the threshold intensities average
of the speech frequencies classified as follows (for adult pure tone audiometry):
Normal 0-25 dBs
Mild 26-40
Moderate 41-55
Moderately severe 61-75
Severe 76-90

Activity 6

Reading Pure Tone Audiometry

Below is an example of a pure tone audiometry result. Classify the severity of the
hearing loss of the patient using the table given above

H-11
 Imaging

The imaging for the ear has evolved from plain x-rays (Towne’s view,
Mayer’s, Schullers and Law’s view) to high resolution computed tomography
and magnetic resonance imaging.

Towne’s View

Schuller’s view

Law’s view

Mayer’s view

Caparas et al;Basic Otolaryngology, 1986

.

H-12
 High resolution computed tomography (or HRCT scans) and magnetic
resonance imaging (MRI) provide details of the anatomy and gross
pathologies of the temporal bone and adjacent soft tissues. CT scan is best for
studying bony relations, while MRI is best for soft tissues. Of course these are
not very cheap examinations hence; you must individualize and know the
indications for these tests. If your clinical history, physical examination and
hearing tests are not enough to determine a diagnosis and severity of
pathology, ancillary procedures can be indicated. Imaging may narrow down if
not determine your diagnosis, determine extent of disease process, and
dictate the indications and conduct of management.

CT Scan of the Temporal Bone MRI of the temporal bone

Now you’re equipped with REVIEW OF ANATOMY AND PHYSIOLOGY

CLINICAL HISTORY KIT
PHYSICAL EXAMINATION KIT
DIAGNOSTIC KIT

We will now learn to apply our tools to actual or simulated patients with
common causes of hearing loss. The following algorithm illustrates how to “think
through” your approach to the problem, beginning with the external (conductive,
mechanical) and proceeding to the internal (sensorineural, electrochemical) causes
of hearing loss. As you will discover, there maybe an overlap of both in some
patients. This results in both “conductive” and “sensorineural” or simply “mixed”
hearing loss.

H-13
H-14
 Lesson 2
Conductive Causes of Hearing Loss

Purpose: At the end of this lesson, the student should be able to:
1.describe the physical examination findings (signs) and information from the
clinical history (symptoms) that may point to a conductive hearing loss.
2.enumerate the common causes of conductive hearing loss and explain the
pathophysiology of each
3. describe the appropriate ancillary diagnostic examinations relevant to the
different common causes of conductivehearing loss.
4. discuss principles of therapy for the different common causes of conductive
hearing loss.
5. demonstrate how to instruct patients/ caregivers to perform simple
therapeutic recommendations such as ear wicking, otic drops, aural toilet and
antibiotic treatment.

Sound is successfully conducted from the environment to the inner ear by the
proper functioning of the following structures: pinna, external auditory canal,
tympanic membrane, ossicles, round window, oval window. Go back to your year
level 3 SIM and review the anatomy of the external and the middle ear. Anything that
disrupts the function of any of these structures may cause a conductive (mechanical)
hearing loss.

History Physical Examination

The following are some of the The following are some of the
information/ symptoms that may be signs that a patient with
present in a patient with conductive conductive hearing loss may
hearing loss: present with:

1. Otalgia 1. Grossly deformed pinna

2. Tinnitus 2. Narrowed external
3. Ear fullness auditory canal
4. Otorrhea 3. Swollen, erythematous
5. Fever external auditory canal.
6. Nasal obstruction/ discharge 4. Ear pain upon
7. Ear manipulation or foreign manipulation of the pinna,
body in the ear tragal tenderness
8. Trauma, including noise 5. Erythematous tympanic
exposure and barotrauma membrane
(flying and diving) 6. Middle ear fluid
7. Tympanic membrane
perforation
8. Foreign body in the
external auditory canal
H-15
 Activity 7
What results will you expect to find in a patient with a conductive hearing loss when
doing the tuning fork tests? Why?

1. Weber-
___________________________________________________________
2. Rinne-
___________________________________________________________
3. Schwabach-

Key Points:
1. Get a good history. Is there a history of swimming, ear cleaning, using cotton
buds? Travel? Trauma?

Why ask about the use of cotton buds for cleaning the ear? It is common practice for
most people to clean the ears with cotton buds, but is it sensible to do so? Why or
why not? Review SILM III.

Why ask about travel? Air travel in particular?

Aside from direct trauma (as when the arm is bumped while cleaning the ear using
cotton buds), what other trauma can damage the eardrum?

2. If there is ear discharge, get a detailed history about it. Has there been an episode
of ear discharge prior to the present one? Frequency? Amount? Duration?

H-16
 Why is it important to ask about the frequency, duration, and amount?

Ask about the consistency. Is it serous, mucoid, or purulent?

What is the significance of the consistency? Clue: review the histology of the lining
of the outer and middle ear. If you have serous discharge, where can it be coming
from? Why? What about mucoid ear discharge? Can it come from the outer ear,
middle ear? Why or why not? What does purulent ear discharge mean?

Ask about the odor or the ear discharge

What would foul smelling ear discharge indicate?

3. If there is otalgia, ask the patient to describe it. Is it worsened by the manipulation
of the pinna? Is there tragal tenderness?
What will tragal tenderness and pain upon pinna manipulation indicate? How
does this differ from pain over the mastoid bone?

4. Don’t focus only on signs and symptoms expected in patients with conductive
hearing loss. Remember, some illnesses causing conductive hearing loss may
progress and cause sensorineural hearing loss as well. In these cases, the patient
will have a mixed hearing loss.

5. Perform a thorough physical exam.

H-17
We now discuss the various conditions causing conductive hearing loss.

A. Congenital & Developmental Causes of Conductive Hearing Loss

Aural atresia (Figure 1) and external auditory canal stenosis (Figure 2) can both
cause conductive hearing loss. Aural atresia is the failure of development of the
aural opening. It can be congenital or acquired. Failure of canalization of the of the
epithelial plug portion can lead to congenital aural atresia while chronic otitis externa
and trauma of the cartilaginous or bony canal can lead to acquired congenital
atresia. External auditory canal stenosis is the narrowing or constriction of the canal.
This can be due to fibrotic thickening of the walls brought about by chronic
infections. Both conditions can cause conductive hearing loss because sound
cannot reach the tympanic membrane through the external auditory canal.

How does this happen? The surface ectoderm in the region of the upper end of
the first pharyngeal groove (dorsal) thickens during the 8th week age of gestation
(AOG). This solid mass of epithelium continues to grow toward the middle ear. With
this, the concha cavum deepens to form the outer one third of the external auditory
canal and this begins to recoil and hollow out to form a channel by the 21st week
AOG. It is this innermost layer of the ectoderm that remains to become the
superficial layer of the tympanic membrane. At the 28th week AOG, the tympanic
membrane appears and derived its layers: ectoderm – squamous layer, mesoderm –
fibrous layer, endoderm – mucosal layer (Figure 3)

FIGURE 1 FIGURE 2

Adapted from: http://www.entrusa.com/Ear_photos

FIGURE 3

Adapted from: http://hope4hearing.org/drum.gif

H-18
 Activity 8
Match the embryologic description with
the correct part of the ear. A. External auditory
____1) It contains sebaceous and canal
ceruminous glands and has an
epithelial lining. The outer one thirds is B. Tympanic
cartilaginous and the inner two thirds is membrane
bony.
____2) Its anteromedial two thirds is C. Eustachian tube
cartilaginous and its posterolateral one
third is bony. It is lined by
pseudostratified columnar ciliated
epithelium.
___3) It consists of squamous, fibrous,
mucosal layers and appears
semitranslucent, pearly white and
made up of pars tensa and pars
flaccida.

B. Inflammatory & Infectious Causes of Conductive Hearing Loss

1. Otitis externa

A history of ear manipulation with subsequent otalgia and pain on ear

manipulation suggests a diagnosis of otitis externa. There’s usually the presence of
an erythematous or hyperemic or swollen ear canal. It can involve a spectrum of
infectious, non-infectious and inflammatory conditions. This happens when the lipid
layer covering the skin of the ear canal is removed by moisture or local trauma
resulting in edema, obstruction of glands, aural fullness and itching. Staphylococcus
aureus is the usual infecting organism in localized otitis externa while diffuse otitis
externa is usually caused by the Pseudomonas group. Otic preparations containing
neomycin or polymyxin as well as a steroid can relieve inflammation. Quinolones
have also been shown to be effective against gram (+) and gram (-) organisms.

Tips
How else will you treat otitis externa? How does an ear wick work?
Otitis externa can cause the external auditory canal to be edematous
preventing adequate delivery of medications to the ear canal. A short strip of gauze
impregnated with the desired otic antibiotic can be placed as a wick in the ear
canal. The wick must be thin and wet enough to slip into the occluded ear using
microforceps or a small mosquito clamp. It is left in place for 24-72 hours and
serves to stent the canal open, preventing further collapse facilitating delivery of
otic drops into the ear canal.

H-19
 2. Otomycosis

Otomycosis or externa mycotica is a fungal infection of the external auditory

canal. Symptoms are similar to otitis externa however pruritus is more prominent
rather than otalgia. On examination, you may visualize fungal mycelia. Moisture,
high temperature, poor hygiene and immunosuppression appear to contribute to the
development of this condition. The offending organisms usually are Aspergillus
albicans, Aspergillus niger and Pitysporum. Treatment may involve application of
acetic acid and isopropyl alcohol solution of equal amounts (1:1 preparation). Otic
preparations with anti fungal properties such as nystatin-containing preparations and
clotrimazole (example of which is the Canesten solution) can also be used.

3. Acute Otitis Media

Acute otitis media is an infection of the middle ear of less than 3 weeks
duration. It may present with pain, fever, malaise and sometimes headache in
addition to the otalgia. On ear examination, the tympanic membrane may be red and
bulging either in part or in its entirety. It can be caused by infection from the upper
respiratory tract spreading to the middle ear. The common etiologic agents are: It
has 4 stages: stage of hyperemia, exudative stage, suppurative stage, stage of
resolution or complication.

Stage Pathophysiology Symptoms Signs Treatment

Hyperemic Reflux from Otalgia, ear Hyperemic, *Amoxicillin for
primary infection fullness, fever, bulging or gram (+)
in the upper otalgia retracted organisms,
respiratory tract tympanic analgesics,
into middle ear membrane antipyretics,
via Eustachian increase oral
tube fluid intake)
Exudative Increase Severe otalgia, Bulging Antibiotics,
intercapillary fever, eardrum, decongestants,
pressure versus worsened loss of light myringotomy if
middle ear space hearing loss reflex, fluid with no
and submucosa behind improvement
intact
eardrum
Suppurative Tympanic Discharge, Perforated Cleaning the
membrane fever, tympanic ear canal with
ruptures causing worsening membrane ear wick,
a lot of discharge hearing loss, suction or
decrease or cotton swab,
relief of pain systemic
antibiotics

H-20
 Resolution Resolves in 24-48 hours if with proper treatment or it may lead to
or intracranial or extracranial complications (i.e. meningitis, abscess,
Complication labyrinthitis)

Gloria-Cruz, 2001, unpublished lecture on “Common Otologic Disorders"

Activity 9

Match the stage with the corresponding picture:

______1. Hyperemic Stage
______2. Exudative Stage
______3. Suppurative Stage

A. B.

C.

Microbiology

The following usually causes acute otitis media: Streptococcus

pneumoniae, Haemophilus influenzae, Moraxella catarrhalis. Management of
this condition must therefore be towards these organisms.

H-21
 Tips
What can you do for the active discharge?
Ear wicking with tissue paper is a cost-effective method of soaking up
ear discharge that you can teach patients and caregivers to perform especially
before administering otic drops. Fold a square of tissue paper as follows until
you have a “tissue spear.” Insert into the ear canal and allow it to soak-up
discharge. Discard and repeat until clear.

You can also do aural toilet with hydrogen peroxide. The head is tilted
to the opposite side, 3 drops of the solution is placed in the ear and wait for
around a minute before the head is straightened up. Instilling otic preparations
usually follows this. 3 drops of the otic solution is placed in the ear. The
important thing is to massage the area anterior to the tragus so as to facilitate
the delivery of the solution.
C. Traumatic & Toxic Causes of Conductive Hearing Loss

1. Mechanical Trauma via the External Canal

There are a variety of insults that an ear can receive in the attempt to alleviate
the sense of pruritus or to remove cerumen. Ear manipulation can lead to external
auditory canal abrasion or laceration. This disruption in the integrity of the epithelial
lining of the external canal can predispose a patient to infection. Patient may present
with a bleeding ear, ear discharge, ear pain and hearing loss. On otoscopy, you may
not be able to fully visualize the tympanic membrane if there’s active bleeding or
presence of blood clots. Otherwise, you’ll be able to see an erythematous or
hyperemic ear canal or a canal laceration if there are any. Cleaning is necessary
and hydrogen peroxide can be used.
A. B.

Adapted from: http://images.google.com.ph/imgres

H-22
 Activity 10
Identify from the figures:
_____1) which perforation is caused by infection?
_____2) which is caused by trauma?

2. Foreign Body

The presence of foreign body in the ears is common among the pediatric
population. Any object small enough to fit the external auditory canal can be a
potential culprit: toy gun pellets, beads as well as insects. Otoscopic evaluation and
appropriate instruments are needed to remove it. We can make use of instruments
such as alligator or ear spoons. For beads and pellets, we can flush the ear with
water but it is a no-no for vegetative foreign bodies because it may expand resulting
in more difficult extraction of the material. For cockroaches, it is better to kill it first by
drowning it in oil, hydrogen peroxide or even lidocaine. It is important to assess the
ear canal and tympanic membrane after the removal of the foreign body.

Did you know???

A patient presenting with ear fullness and otalgia without any other related
pathology usually may have impacted cerumen. Cerumen (ear wax) is produced
by ceruminous glands of the external auditory canal. It is composed of fatty acids,
lysozyme and immunoglobulin components and these are believed to have
inhibitory or bactericidal qualities. When the cerumen production is excessive,
normal outward migration is not optimal and wax accumulates. More often, this
results when individuals clean their ears with cotton buds or cotton tip applicators,
forcing the cerumen further into the external ear canal and causing impaction.
Hearing loss can result from this obstruction thus the need to remove it. It is
usually softened with the use of baby oil or commercial preparations such as
Cerumenex or Debrox. Once softened, removal can be done with warm water
irrigation. We can also make use of ear curettes to remove impacted wax.

3. Barotrauma

Barotrauma is damage to tissues caused by changes in barometric pressure

that occur during diving or flying. The middle ear is the most common site because
of the complexity of the Eustachian tube. As environmental pressure increases, the
air in the middle tube and the eustachian tube is compressed. The build-up of
pressure within the tympanic membrane may cause the small vessels to rupture
causing hemotympanum that may lead to possible rupture of the membrane. There’s
usually pain, feeling of fullness or decreased hearing. On otoscopy, eardrum is
retracted and may exhibit hemorrhagic blebs within the drum or blood behind the
drum. Occasionally, the tympanic membrane may be seen perforated. Treatment
may involve the use of decongestants, cessation of flying or diving. Antibiotic

H-23
treatment is not warranted unless there’s the presence of tympanic membrane
perforation.
4. Neoplastic Causes of Conductive Hearing Loss

Exostoses are benign periosteal outgrowths that occur in the bony canal and
are associated with multiple exposures to swimming in cold water. They may appear
as nodules next to the annulus and frequently are multiple and bilateral. They
usually don’t require treatment, although they may result in more frequent canal
obstruction by cerumen in some individuals. Osteomas, on the other hand, are
benign tumors of the external canal wall which are usually single, unilateral, and
firm, rounded growths attached by a smaller bony pedicle to the bony portion of the
canal wall. Both exostoses and osteomas may cause conductive hearing loss.
Treatment usually involves excision of the lesion and preserving as much of the ear
canal. There are also primary malignant tumors affecting the external auditory canal,
such as ceruminomas and adenoid cystic carcinomas, but these are rare.

5. Systemic/ Degenerative Causes of Conductive Hearing Loss

Otosclerosis is an autosomal dominant disorder due to abnormality of bone

metabolism and turnover affecting the otic capsule of the ossicles. Stiffening and
fixation of the stapes footplate occur secondary to the dense sclerosis and causes
conductive hearing loss. The hearing loss is usually noticed when a threshold level
of 40 decibel or greater is reached. Aside from hearing loss, patient may experience
dizziness, balance problems and tinnitus. Physical examination is usually normal.
This is usually diagnosed through audiometry (shows a person’s hearing sensitivity)
and tympanometry (shows how well the middle ear functions to conduct sound).
Surgery need not be done for those with mild hearing loss. However, a
stapedectomy can be an option for treatment.

Trivia

What is Schwartze’s Sign?

The tympanic membrane shows hyperemia of the promontory mucosa
that is due to increase vascularization but it is only seen in a minority of
patients with otosclerosis.

H-24
Now consider the following cases. Give your diagnosis based on the given
situations.

Activity 11

1) A 16-year-old male came to you with decreased hearing and fullness

of the right ear after swimming. There is occasional otalgia but no other
complaints. On physical examination, you see this.

2) A 30-year-old female complained of excruciating ear pain and

decreased hearing in the left ear. On review of history, all you can determine is
that she cleaned her ear with cotton buds the previous day. The patient
complained of severe pain as you gently pull the pinna outwards to perform
otoscopy. You see this.

Lesson 3
Sensorineural Hearing Loss

Purpose: At the end of this lesson, the student should be able to…
1.enumerate the signs and symptoms that may point to a sensorineural
hearing loss
2.enumerate the common causes of sensorineural hearing loss and explain
the pathophysiology of each
3.discuss the appropriate diagnostic test relevant to each of the common
causes of sensorineural hearing loss

H-25
 4.discuss principles of therapy for each of the common causes of
sensorineural hearing loss and
5. demonstrate how to communicate history and PE findings, diagnosis, and
therapeutic recommendations to a patient or a care giver

After sound is successfully conducted into the middle ear, it travels from the
cochlea to the auditory cortex. Go back to the year level III SIM and review the
pathway of hearing. Insults affecting this part of the hearing apparatus will give rise
to sensorineural hearing loss.

Ossicles

Cochlea

Netter F. Atlas of Human Anatomy. 1997. New Jersey.

Semicircular canals

Vestibule

Netter F. Atlas of Human Anatomy. 1997. New Jersey.

Unfurling the cochlea will give you this figure

H-26
 History Physical Examination

The following are some of the The following are some of the
information/ symptoms that may be signs that a patient with
present in a patient with sensorineural hearing loss may
sensorineural hearing loss: present with:

1. Pre-term birth 1. Normal looking external

2. Pre-natal illness in mother auditory canal/tympanic
3. Birthing complications membrane
4. Speech delay/developmental 2. Nystagmus
delay 3. Other neurologic signs
5. Childhood viral infections (ex. 4. Congenital anomalies
Measles, chickenpox, mumps, 5. Poor response to loud
etc) sounds/name calling
6. Ototoxic drug intake 6. Tuning fork tests with the
7. Acute exposure to a very loud following results:
sound
8. Chronic noise exposure Weber: lateralizes to the
9. Unusually loud speaking voice better hearing ear
10. Old age Rinne: Air conduction>bone
11. History of systemic illnesses conduction in the poorer
such as hypertension, hearing ear
diabetes, etc Schwabach’s: poorer
hearing ear- examiner>
patient

Key points:
1. Hearing loss is quite difficult to ascertain. Most patients insist that their
hearing is fine, and that it is other people who insist that they have hearing
loss. Listen to the patient’s voice. An inappropriately loud voice suggests
impaired hearing because of inadequate feedback (observe how loudly
people speak after a rock concert). Ask about inability to use the telephone,
or difficulty understanding conversations when there is competing noise. Ask
about difficulty in hearing certain consonants (f, s, th) or difficulty in hearing a
female speaker. This may be indicative of a high-pitched sensorineural
hearing loss.

What part of the cochlea is damaged in people with low-pitched hearing

loss? High pitched hearing loss?
High pitched: base of cochlea (hair cells which perceive
high pitched sounds are concentrated at the base)
Low pitched: apex of cochlea cochlea (hair cells which
perceive low pitched sounds are concentrated at the base)

H-27
2. In children, ask about speech delay. Children with hearing loss will have
difficulty developing speech. Ask about the child’s reaction to loud sounds
such as thunder, loud music, banging doors. Does the child respond to name-
calling even without visual clues (ex. Calling the child from behind, as
opposed to calling the child while motioning for the child to come closer)?

3. Get a good personal/social history. Ask about chronic noise exposure at

work or at home, use of ear plugs, etc.

4. Get a good past medical history. Any disease that may give rise to hearing
loss (measles, diabetes, hypertension, etc)? Any intake of ototoxic drugs?

5. For children, take a good birth and maternal history, family medical history.
TORCH, especially congenital rubella, is a common cause of hearing loss.

Did you know???

• In Asia, .06% of all children have some degree of hearing loss
• 1:1000 children are clinically deaf
• Only 1% of deaf children are enrolled in primary education,
schools for the deaf numbering less than 20 in the Philippines

Environmental Health Perspectives Volume 102, Number 11, November 1994

Given the high incidence of deafness among infants, most first world countries
have included hearing screening in their new born screening programs. The
otoacoustic emissions (OAE) test is the preferred examination.

OAE test: - This was discussed in the diagnostics tool kit. The presence of OAEs
assures that if sensorineural hearing loss is indeed present, it is not greater than
40dB. If OAEs are absent, there is a possibility that there is sensorineural
hearing loss, but this is not enough to label a child deaf. Other examinations are
needed. It is a very simple test, but it requires that the patient be very quiet and
still.

Did you know???

Otoacoustic Emissions (OAE) were discovered by David Kemp in 1978
when, out of boredom, he inserted a microphone into his ear and heard
sounds coming out if it.

2. Tinnitus is most frequently associated with sensorineural hearing loss.

Usually, there is worsening of the tinnitus in a quiet environment. It usually
becomes bothersome during bedtime. Ask if the tinnitus is pulsatile of not.

H-28
 Pulsatile tinnitus may be indicative of a vascular lesion, and will, thus,
warrant further work up to determine the source.

3. Dizziness is another symptom that may accompany sensorineural hearing

loss. It is important to ascertain whether the dizziness is vestibular in
origin or not. Please refer to the module on vertigo for more information.

4. A full neurologic examination is required in patients with sensorineural

hearing loss. Pay close attention to cranial nerves in close proximity to the
eighth nerve

Activity 11
What results will you expect to find in a patient with sensorineural hearing loss
when doing the tuning fork tests? Why?

1. Weber-
_________________________________________________________
2. Rinne-
_________________________________________________________
3. Schwabach
_________________________________________________________

We now discuss common conditions causing sensorineural hearing loss.

A. Congenital & Developmental Causes of Sensorineural Hearing Loss

H-29
Congenital deafness may be genetic or non- genetic in origin.

1. Congenital Deafness of Genetic Origin

- may occur alone, or it may be associated with other diseases. Below
are some examples:

1.1 Congenital deafness of genetic origin, which occur alone

DISORDER DESCRIPTION MODE OF

INHERITANCE

Micheal Dysplasia There is total lack of Autosomal dominant

development of the
inner ear
Mondini Dysplasia There is partial Autosomal dominant
aplasia of both the
membranous and
bony labyrinth. The
cochlea appears
flattened, with only 1
½ turns, as opposed
to the usual 2 ½ turns.
The deafness is
caused by the
dysgenesis of the
Organ of Corti.
Scheibe Dysplasia Most common of all Autosomal recessive
congenital genetic
causes of deafness;
the bony labyrinth is
fully developed, but
the pars inferior
(saccule and cochlear
duct) is undeveloped.
The most affected is
the Organ of Corti and
adjacent ganglion
cells of the basal coil
of the cochlea, giving
rise to high frequency
hearing loss. There
may be residual low
frequency hearing

H-30
 1.2. Congenital deafness of genetic origin which occur with other
abnormalities
• Waardenburg’s Syndrome- features include: lateral
displacement of the medial canthi, flat nasal root,
hyperplasia of the eyebrows, heterochromia of the irides,
partial albinism (white forelock), and congenital deafness in
around 25% of patients. Autosomal dominant
• Pendred’s Syndrome- nonendemic goiter; accounts for an
estimate of 10% of cases of recessive hereditary deafness.
There is abnormal iodine metabolism resulting in thyroid
enlargement appearing during adolescence, with nodular
development in adulthood. Patients are usually born with
severe hearing loss

Molecular Structure

A defect in the gene locus known as DFNB1 has been found in a high
proportion of non syndromic autosomal recessive deafness. The gene involved
is GJB2. This gene encodes for the gap junction protein connexin. Connexins
allow rapid transport of ions and small molecules between cells. The exact role
of connexins in deafness is yet to be determined

The Connexin Deafness Homepage

http://www.crg.es/deafness

2. Congenital Deafness of Non-genetic Origin

Congenital deafness of non-genetic origin may or may not be associated with

other abnormalities. The most common cause of non-genetic congenital deafness is
CONGENITAL RUBELLA, otherwise known as German measles. In a pregnant
woman who contracts rubella during the first 3 months of pregnancy, the probability
of her child having sensorineural hearing loss is 5-10%.

H-31
 Pathophysiology

Rubella is caused by an enveloped RNA virus belonging to the Rubivirus

genus of the family Togaviridae. This virus is known for its propensity to infect a
fetus. During the first trimester, maternal infection has an 80% chance of
transmission to the fetus. During the second trimester, there is 50% chance of
transmission. The rubella virus acts as a teratogen. The mother, once infected,
experiences a viremic phase wherein the placenta becomes a target. The virus
then reaches fetal circulation, (and thus fetal tissues) and the amniotic fluid. The
infected amniotic fluid is then aspirated and/or swallowed by the fetus, later
reaching fetal circulation, and fetal tissues. The mechanism by which this virus
causes fetal damage is poorly understood. One theory is that the infection causes
vasculitis resulting into tissue necrosis. Another theory is that there is direct
damage to the infected cells.
Inner Ear Infections, St. George ENT,
http://www.ent.com.au/INNER%20EAR%20INFECTIONS%20Mar%202003.htm

Activity 12

List down the signs and symptoms that may be seen in patients with congenital
rubella. _______________________________________

Congenital rubella may be prevented by giving women in the child- bearing age
MMR (measles, mumps, rubella) immunization. Immunization will be discussed in
greater detail in your Pediatrics modules.

Examples of non-genetic congenital deafness, which may occur alone, include

deafness secondary to prolonged labor, hypoxia, and premature birth. The pathology
of these diseases has not been well studied.

B. Infectious & Inflammatory Causes of Sensorineural Hearing Loss

Middle ear infections may cause sensorineural hearing loss when metabolic
products pass from the middle ear into the cochlea through the oval or round
window. The type of hearing loss seen in middle ear disease is not purely
sensorineural. Go back to the section on conductive hearing loss and review how a
patient with middle ear disease develops hearing loss. Mixed hearing loss will be
discussed in more detail later.

H-32
 Microbiology

Viral infections may cause hearing loss, and is usually seen in children.
Etiologic agents include the following: mumps, chicken pox, measles, influenza,
herpes zoster, and adenovirus. Mumps is the most common cause of unilateral
acquired hearing loss in children. Measles is the most common cause of bilateral
hearing loss. The reason behind hearing loss being unilateral or bilateral has not
been fully determined yet.
Inner Ear Infections, St. George ENT,
http://www.ent.com.au/INNER%20EAR%20INFECTIONS%20Mar%202003.htm

Herpes Zoster-caused by the Varicella Zoster virus (also causes chicken

pox). In patients who have had chicken pox, the virus may remain latent in nerve
roots. When there is reactivation of latent virul in the geniculate ganglion, this may
result to Herpes zoster oticus, also known as Ramsay Hunt Syndrome. There is
intense pain of the ears, vesicles appear on the external auditory canal, and may
also appear on the face. Hearing loss, vertigo, facial nerve paralysis may occur,
depending on the severity.

Pathophysiology
Reactivation of the virus in the geniculate ganglion causes vesicles to form on the
facial nerve. The inflammation may then spread to the eighth cranial nerve, and to
the labyrinth.

Inner Ear Infections, St. George ENT,

http://www.ent.com.au/INNER%20EAR%20INFECTIONS%20Mar%202003.htm

Measles is caused by the paramyxovirus rubeola. Cough, maculopapular

rashes, Koplik’s spots, etc characterize this. Less than 1 per 1,000 measles patients
develops hearing loss. The hearing loss is usually abrupt, bilateral, and manifests at
the time of the rash. Pathophysiology is still not clear.
Pathology
There is degeneration of the Organ of Corti, spiral ganglion and vestibular sense
organs.
Inner Ear Infections, St. George ENT,
http://www.ent.com.au/INNER%20EAR%20INFECTIONS%20Mar%202003.htm

In mumps, or viral parotitis, patients who develop deafness usually

experience the hearing loss towards the end of the disease. It is usually bilateral,
profound, and permanent.
Pathophysiology
The pathophysiology is still not clear. It is hypothesized that during the viremic
phase, the virus reaches the CSF, then the perilymph. This may then cause
inflammation or direct damage to the inner ear.
Inner Ear Infections, St. George ENT,
http://www.ent.com.au/INNER%20EAR%20INFECTIONS%20Mar%202003.htm

H-33
 Meningitis is another possible cause of hearing loss. It usually manifests as
bilateral sensorineural hearing loss, but unilateral sensorineural hearing loss may
also be seen. It usually occurs in patients who have been comatose for some period
of time. Hearing loss usually occurs early due to ossification of the cochlea, and
does not resolve with the institution of appropriate antibiotic therapy. It is postulated
that the infection reaches the inner ear through spread from the CSF to the
perilymph.

C. Toxic and Traumatic Causes of Sensorineural Hearing Loss

1. Drug Induced Hearing Loss

Ototoxic Hearing loss- in general, any drug/chemical that causes renal
toxicity is also ototoxic. The ototoxicity may be reversible or irreversible. For most
cases, the only way to stop, or to reverse the damage is to stop taking the offending
drug. Below is a list of some drugs, which are ototoxic.

DRUG PROPERTIES
Aminoglycosides These are the best known and the
Ex. Streptomycin. gentamycin most studied ototoxic drugs. These
drugs may preferentially damage the
vestibular system, the cochlea, or both.
In terms of cochlear damage, the outer
hair cells at the basal end of the organ
of Corti are damaged first, causing high
pitched hearing loss.
Loop Diuretics When taken in large doses (dose not
ex. Furosemide (Lasix) specified in sources), these may affect
a wide range of frequencies. These
rarely cause irreversible auditory
sensitivity. These interfere with ion
transport in the stria vascularis. They
don’t directly affect the hair cells
Salicylates Rarely cause irreversible hearing loss.
Ex. Aspirin (Aspilet) Causes mild to moderate hearing loss
(<40 decibels). It is usually reversible
after stopping the drug for 72 hrs. The
mechanism by which these drugs
cause hearing loss has not been fully
determined yet.
Environmental Health Perspectives Volume 102, Number 11, November 1994

2. Ototoxic Chemicals and Heavy Metals- these may be present in the

workplace. Examples of which are:

Chemical/Heavy Metal PROPERTIES

Trichloroethylene This is a non-corrosive agent used in

H-34
 dry cleaning, spot removers, rug
cleaners, paints, waxes, etc
Associated with vestibular and auditory
nerve impairment.
Toluene This is a frequently used solvent, seen
in paints, lacquers, thinners, adhesives,
etc. Primarily affects the central
nervous system. Hair cell damage in
the cochlea has been reported
Mercury Early stages in poisoning may cause
cochlear lesions. Hearing loss in the
later staqges of intoxication is usually
due to neurological damage.
Environmental Health Perspectives Volume 102, Number 11, November 1994

3. Noise

Exposure to loud sounds may damage auditory hair cells, resulting to hearing
loss. Factors which contribute to the effects of noise on hearing include sound levels
(loudness), spectral composition (frequencies), time distribution of noise exposure
during the day (continuous exposure the whole day? few minutes per day?) and
cumulative exposure over days, weeks, months. It may be classified as: acoustic
trauma, temporary threshold shift, permanent threshold shift.
The ear protects itself from loud noises through the acoustic reflex wherein an
acoustic stimulus (loud sound) induces middle ear muscles, the stapedius and the
tensor tympani, to contract, causing the ossicular chain to stiffen and dampen the
sound. The function of the acoustic reflex as a protective mechanism is just a theory.
The functional role of the middle ear muscles is still not completely clear. Although a
protective function is a possibility, its long latency and the absence of intense
sounds in nature argues against a purely protective function.

Did you know???

NATURE HAS PROVIDED MAN WITH EAR PLUGS

Nature's ear plugs? Immunocytochemical labeling marks heat shock protein 72,
which may protect hearing after noise, as black dots in outer hair cells. Photo:
Richard Altschuler in Environmental Health Perspectives Volume 102, Number 11, November
1994

H-35
 Several studies suggest that a major function of HSPs is to facilitate
cellular repair and to protect cells from further injury. HSPs are widely distributed
and respond rapidly to changing conditions, a finding consistent with the basic
emergency response of cells.

Did you know???

Hearing loss is one of the most prevalent occupational diseases in first world
countries. Environmental Health Perspectives Volume 102, Number 11, November 1994
The statistics in the Philippines are not yet known. But in a country where
safety precautions are often neglected, hearing loss may be expected to be a
prevalent occupational disease as well.

3.1. Acoustic Trauma- this is when exposure to a loud/ intense, short duration
sound (ex. Gunshot) causes sudden, permanent hearing loss. This may damage any
or all parts of the inner ear, especially the Organ of Corti, which may be torn apart.

3.2. Temporary Threshold Shift- this is caused by exposure to moderate

intensity noise. Hearing threshold becomes higher than normal, temporarily. During
this period, the sound a person perceives as “normal” is actually louder that usual.
Observe people who have just been to a rock concert. They speak louder than usual
because of inadequate feedback. Talk to them using your usual voice volume, and
they can hardly hear you. Hearing threshold returns to normal in 18 to 24 hours.

In a double blind placebo controlled clinical trial entitled: “The Effect of

Betahistine Mesylate on Transient Threshold Shift Secondary to Discotheque
Music”, sixty subjects were exposed to 90 – 100 db disco music for two hours. It
was shown that administration of betahistine mesylate, 12mg single dose, 30 min
before exposure to disco music ameliorated transient threshold shift and
promoted earlier full recovery.
Chiong et al, “The Effect of Betahistine Mesylate on Transient Threshold Shift Secondary to Discotheque Music,” 1999.

3.3. Noise induced hearing loss- this is when constant exposure to moderate
intensity noise causes permanent deterioration (permanent threshold shift) of
auditory thresholds. Regardless of the frequency of the noise exposed to, hearing
loss usually begins at 4000 Hz, and then spreads slowly to higher and lower
frequencies.

H-36
Adapted from Environmental Health Perspectives Volume 102, Number 11, November 1994

Did you know??? Did you know???

In Elizabethan England, men In ancient Rome, it was
had to refrain from beating their forbidden to use chariots in the
wives at night to avoid disturbing evening due to noise it creates
their neighbors.

Did you know???

In Australia, you may be arrested by the police
for playing your stereo loud enough to disturb
your neighbors at night.

H-37
 Histology of Damaged Hair Cells
(Right) Synaptic damage. All
afferent dendrites are
disconnected from the inner
hair cells due to excitotoxic
injury.
CHEMICALS
Carbon monoxide

(Left) Damaged outer hair

cells. These hair cells are
entering apoptosis and are
destined to die.

Environmental Health Perspectives Volume 102, Number 11, November 1994

4. Barotrauma

This is damage to tissues caused by changes in barometric pressures which

occur during diving or flying. It may occur in the ears. This was discussed in the
lesson on conductive hearing loss, but barotrauma may cause, though rarely
sensorineural hearing loss as well. This occurs when the pressure change causes
disruption of the round or oval window, allowing perilymph to leak into the middle ear
space. Patients will suddenly experience hearing loss and vertigo. There is no
reliable test to determine the presence of a perilymph fistula, not even surgical
exploration. A patient suspected to have this is asked to refrain from straining. In
some cases, the fistula may be plugged with fat grafts.
Activity 13
Boyle’s law explains Barotrauma. What is Boyle’s law?_____________________
_________________________________________________________________
_____________________________________________________

H-38
D. Neoplasms Causing Sensorineural Hearing Loss

Tumors which affect any of the components of the sensorineural pathway of

hearing may cause sensorineural hearing loss. Of course, in these cases, diagnosis,
or, at least localization, may be done by doing a thorough history and physical exam,
paying close attention to the neurologic exam.

Manterand Gatz. Clinical Neuroanatomy.1994.McGraw Hill Companies.

All patients with unilateral or asymmetric hearing loss should be screened for
acoustic neuroma. Acoustic neuromas are the most common inner ear tumors
causing hearing loss. These are benign tumors of Schwann cells covering the eighth
nerve.

Activity 15
What does the suffix –oma imply? __________________________________

note: there are exceptions to this rule. Ex: a hepatoma is a malignant hepatic tumor
5. Systemic/ Degenerative Cause of Sensorineural Hearing Loss

H-39
E. Systemic/ Degenerative Cause of Sensorineural Hearing Loss

Activity 16
What does the prefix presby- imply? ________________________________

Presbycusis is hearing loss due to the aging mechanism of the inner ear. Any
of four parts of the inner ear may be affected first: hair cells, cochlear neurons, stria
vascularis, and basilar membrane. The exact nature of the pathologic process is not
yet clear.

TREATMENT

Principles of therapy are founded on making accurate diagnoses. In many

cases, “therapy” assumes public health dimension by emphasizing disease
preventive and health promotive aspects. Genetic counseling for families with
hearing impaired individuals, immunization against rubella, measles, and mumps,
universal newborn hearing screening, and occupational noise protection, are among
the important campaigns to safeguard hearing. Where an identifiable etiology is
medically or surgically treatable, appropriate therapy should be instituted. For
instance, surgery for acoustic neuromas will remove the tumor and prevent further
damage (ex. Facial nerve paralysis), though it may not restore hearing. Finally,
hearing can be aided by prescribing appropriate hearing aids.

Hearing aids amplify sound. They cannot amplify voices alone without
amplifying background noise. The hearing aid amplifies the sound, and the
patient’s auditory system processes it. They do not restore the patient’s normal
hearing; it merely “aids” the patient’s auditory system. It takes time for a patient to
adjust to a hearing aid; therefore, counseling is very important. Most patients
expect instant improvement in hearing. Without counseling, a patient may get
discouraged easily.
There are 5 types of hearing aid: body aid, behind the ear, in the ear, in the
canal, and completely in the canal. Size is not necessarily proportional to quality
of sound produced.
There are hearing aids which, through digital technology, can be
programmed to adapt to different listening environments. Such aids can help
improve understanding in noisy environments.

H-40
 See how man’s efforts to improve hearing have led to the modern hearing aid.

Did you know???

Through the years, man has continually invented contraptions to

improve hearing. Here are some examples:

Initially, sailors who needed to communicate with

each other from a distance used ear trumpets. Later,
the hearing impaired used ear trumpets. After some
time, it became fashionable to use ear trumpets.

Acoustic Appliances, Hearing Catalog, Sears, Roebuck & Co., Chicago Ill

Auricles and cornets were created as an

alternative to ear trumpets. These were less
conspicuous.

Acoustic Appliances, Hearing Catalog, Sears, Roebuck & Co., Chicago Ill

Did you know???

Acoustic thrones of various
types were popular with many
European royal families during
the 18th and 19th centuries.
Elaborately designed armrests
had openings that served as
mouthpieces through which
courtiers speak. Resonators
were concealed inside, and
sound was conveyed from the Hearing devices,
arms to an earpiece that was which made use of
fitted on the end of a tube. bone conduction,
Acoustic Appliances, Hearing Catalog, Sears, were also created.
Roebuck & Co., Chicago Ill

H-41
Did you know???

Electrical hearing aids were introduced just after 1900. The earliest electric aids offered the
same amplification as ear trumpets did, but covered a wider frequency range — sometimes
as much as 500 to 1800 Hz. These include the carbon type and vacuum tube hearing aids.

Later models with multiple microphones provided 25-30 dB of amplification, and the
introduction of amplifiers in the 1920s increased the range to 45-50 dB. Hearing aids today
can provide substantially more amplification than that, and can be individually tailored to
address each wearer's particular hearing loss.

Acoustic Appliances, Hearing Catalog, Sears, Roebuck & Co., Chicago Ill

Lesson 4
Mixed Hearing Loss

At the end of this lesson, the student should be able to

1. enumerate by history and physical examination, the signs and symptoms
that may point to a mixed hearing loss.
2. explain the pathophysiology of chronic suppurative otitis media being the
most common cause of mixed hearing loss.
3. enumerate some other causes of mixed hearing loss.
4. discuss principles of diagnosis and therapy for common causes of mixed
hearing loss.

Mixed hearing loss is a combination of both conductive and sensorineural

hearing loss.

This hearing disorder can occur when a there is a pathology that targets the
conductive and the sensorineural components of hearing. Such disease process
may involve external, middle and inner ear structures at the same time, or in
succession. Inflammatory process, neoplastic, congenital and even traumatic causes
can result to mixed hearing loss. You can go back to page H-14 to compare causes
of conductive and sensorineural hearing loss and note pathologies common for the
two.

H-42
 The most common cause of mixed hearing loss is chronic suppurative otitis
media. This condition causes sensorineural hearing loss when toxic by-products of
the infections from the middle ear reach the inner ear through the round or oval
window or when there is direct erosion of the inner ear structures secondary to
cholesteatoma.

Other conditions producing mixed hearing loss are the following: trauma,
neoplasms, congenital conditions, barotraumas etc.

Some of these conditions producing mixed hearing loss are discussed below.
Given the pathophysiology, how can these conditions produce mixed hearing loss?

1. Temporal Bone Fracture Secondary to Trauma

Temporal bone trauma usually is the sequela of blunt head injury. Injury to the
temporal bone may result in fracture, hemorrhage, or disruption of the inner ear
structures. Hearing loss may be either conductive and/or sensorineural. Conductive
hearing loss usually is seen with longitudinal fracture and ossicular disruption.
Sensorineural hearing loss usually is seen with transverse fracture and fracture of
the vestibulocochlear apparatus.

2. Ear Tumors

Benign tumors are usually located on the external ear, most usually producing
primarily conductive hearing loss. While very unusual, acoustic neuromas or
metastatic cancer (particularly breast) can be a source of hearing loss. Typically
there is a combination of a hearing problem or either dizziness or imbalance.
Audiometry generally shows an asymmetrical sensorineural hearing loss

Cancer in the external auditory meatus ( the most common being squamous
cell carcinoma, basal cell carcinoma ,adenocarcinoma ,acinic cell carcinoma and
adenoid cystic carcinoma ) can invade posteriorly ,erosion through the posterior
bony canal provides access to the mastoid cavity. Tumor growth medially along the
external ear canal can extend through the tympanic membrane and bony tympanic
ring, allowing invasion into the middle ear.

In the middle ear or mastoid, tumors spread easily via the eustachian tube,
round and oval windows, neurovascular structures, and extensive air spaces of the
mastoid cavity.

3. Congenital Conditions

As mentioned in page H-14, there are a number of congenital conditions that

produce either conductive or sensorineural hearing loss. A number of these
conditions may occur in one patient producing mixed hearing loss.

Congenital microtia, for example, may occur as part of a syndrome. So aside

from the external ear anomaly producing the conductive hearing loss, any of the

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inner ear conditions discussed in Lesson 3 can be present as well, giving you mixed
hearing loss.

4. Barotrauma

Barotrauma refers to injury sustained from failure to equalize the pressure of an

air-containing space with that of the surrounding environment. The most common
examples of barotrauma occur in air travel and scuba diving. Symptoms of
barotrauma include "clogging" of the ear, ear pain, hearing loss, dizziness, ringing of
the ear (tinnitus), and hemorrhage from the ear ( pointing to conductive hearing
loss).

Dizziness (or vertigo) may also occur during diving from a phenomenon known
as alternobaric vertigo. It is caused by a difference in pressure between the two
middle ear spaces, which stimulates the vestibular (balance) end organs
asymmetrically, thus resulting in vertigo. The alternobaric response can also be
elicited by forcefully equalizing the middle ear pressure Politzer maneuver, which
can cause an unequal inflation of the middle ear space.

Inner ear injury during descent is directly related to impaired ability to equalize
the middle ear pressure on the affected side. Sudden, large pressure changes in the
middle ear can be transmitted to the inner ear, resulting in damage to the delicate
mechanisms of the inner ear. This can cause severe vertigo and even deafness.

4. Chronic Suppurative Otitis Media

CSOM is the most common cause of mixed hearing loss. This is usually a
sequelae of untreated acute otitis media, usually in the first five years of life, and is
associated with poor socioeconomic conditions .Its natural history, without treatment,
is continuous or intermittent purulent ear discharge for months or even years with
destruction of the bones of the middle ear and increasing hearing impairment.
Occasionally it may lead to serious infective complications such as chronic
mastoiditis, meningitis and cerebral abscess. When it occurs during the first two
years of life, the consequent hearing loss is likely to have serious effects on the
critical period of a young child's language development, and later it can cause
significant delays in school progress.

This is a common cause of consult among poor populations where it is

thought that having “sipon sa tenga” is normal.

Helpful Herbs

Local remedy for draining ears is the sap of the plant soro-soro. Aside from
treating discharging ears, itchiness (presumably otomycosis and/or CSOM) can
be treated with this local medication.

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 The more common pathogens in CSOM, as found in local rural school children in
a study by Chiong et al, 19: are the following Proteus mirabilis, Pseudomonas putida
and Proteus vulgaris. From international literature, Pseudomonas aeruginosa,
Staphylococcus aureus, Proteus species, Klebsiella pneumoniae, and diphtheroids
are the most common bacteria cultured from chronically draining ears. Anaerobes
and fungi may grow concurrently with the aerobes in a symbiotic relationship.

Five to 10% of infections are polymicrobial in etiology, often demonstrating a

combination of gram-negative organisms and S aureus, which is also recovered
regularly. The anaerobes (Bacteroides, Peptostreptococcus, Peptococcus) and fungi
(Aspergillus, Candida) complete the spectrum of colonizing organisms in this
disease. The anaerobes make up 20-50% of the isolates in CSOM and tend to be
associated with cholesteatoma. Fungi have been reported in up to 25% of cases.

This condition can result from acute otitis media, eustachian tube obstruction,
mechanical trauma, thermal or chemical burns, or blast injuries. It can be divided
into two major categories, depending on the type of perforation: (1) those with
central perforations of the pars tensa, and (2) those with the more dangerous attic
perforations of the pars flaccida or marginal perforations of the pars tensa.

Perforations of the tympanic membrane

• Central perforations usually involve the pars tensa only and are usually
treated medically i.e. with aural toilette and ear drops
• Marginal perforations usually occur in the posterior-superior portion of the
pars tensa, with no substance of the tympanic membrane between the
edge of the perforation and the bony sulcus tympanicus
• Attic perforations of the pars flaccida lead into the epitympanum

Marginal and attic perforations are usually associated with formation of

cholesteatoma (discussed earlier). Treatment of CSOM may be medical or surgical.
The table below may serve as a guide to whether the patient has a surgical or a
medical ear.

Safe/ Medical ear Dangerous/ Surgical ear

Discharge Non-foul smelling Foul-smelling
Perforation Central Marginal, attic, total
Intra and extracranial None Specially with
complications cholesteatoma
Response to medical (+) (-)
treatment

Patients with CSOM respond more frequently to topical than to systemic

therapy. Successful topical therapy consists of 3 important components: selection of
an appropriate antibiotic drop, regular aggressive aural toilet, and control of
granulation tissue.

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 The antibiotic should have an appropriate spectrum of activity that includes
gram-negative organisms, especially pseudomonas, and gram-positive organisms,
especially S aureus. The antibiotics that meet this initial criterion are the
aminoglycosides and the fluoroquinolones. Topical antibiotic drops containing
aminoglycosides have been marketed and used for more than 20 years.

Pathophysiology
In patients presenting with otitis media, the algorithm below can be used to
guide a clinician on the proper management of such common disease entities
whether it be acute or chronic knowing that the organisms involved are different.

Otitis Media

Intact tympanic membrane Perforated tympanic membrane

(Suppurative Otitis Media)

Resolve Persistent fluid Acute (ASOM) Chronic (CSOM)

(Effusion)

Otitis media with effusion (OME) Oral medication Otic solution

+/- otic solution +/- oral
medication

Acute Chronic

(+) culture (-) culture

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 REFERENCES

1. Lee, KJ. 1999. Essential Otolaryngology, Head and Neck Surgery. 7th Edition.
Appleton and Lange, Connecticut.

2. Chiong, CM, A. Chan, EC Yap, RA del Roasario, TR Pajarillo, GT Abes, JC Jamir,

R de Mesa, M Velmonte. The Microbiology of Chronic Otitis Media Among School
Children in a Rural Community. Acta Medica Philippina.

3. Environmental Health Perspectives Volume 102, Number 11, November 1994

4. Linstrom CJ, Lucente FE. Infections of the External Ear. In: Bailey BJ, et al, eds.
Head and Neck Surgery-Otolaryngology. Philadelphia: JB Lippincott, 1993: 117.

5. Cummings CW, Fredrickson, JM, Harker, LA. 1998. Otolaryngology Head & Neck
Surgery. 3rd Edition. Philadelphia

6. Boies LR, Adams G, Hilger PA. Fundamentals of Otolaryngology. 6th Edition. WB

Saunders Company.

7. Acoustic Appliances, Hearing Catalog, Sears, Roebuck & Co., Chicago Ill.

8. http://www.ent.com.au/inner%20ear%20infections%20mar%202003.htm

9. http://images.google.com.ph/imgres

10. Chiong et al, “The Effect of Betahistine Mesylate on Transient Threshold Shift
Secondary to Discotheque Music,” 1999.

11. http://www.crg.es/deafness

12. http://hope4hearing.org/drum.gif

13. Gloria-Cruz, 2001, Unpublished Lecture on “Common Otologic Disorders"

14. http://www.entrusa.com/Ear_photos

15. Caparas et al. Basic Otolaryngology, 1986

16. Lapena. 2003. Do you hear what I hear? Special senses screening in ENT.
Unpublished Lecture

17. Netter F. Atlas of Human Anatomy. 1997. New Jersey.

18. http://www.emedicine.com/radio/topic678.htm

19. http://www.dizziness-and-balance.com/disorders/hearing/hearing.html

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