1

Chapter I

Introduction

The objectives of the researchers are: (1) educate people

about the autoimmune attack to the inner ear so as to avoid this

disease. This will help our respondent of the research to be

educated and be aware of this type of attack to the immune

system to its own self, particularly to the inner ear. To

promote good health among all people who would be benefited

about the outcome of our research. (2) determine the effects of

the autoimmune attack to the daily life of the people. This will

help the respondent be aware of what action they must do if in

case they will get this disease. (3) to inform the respondent of

the possible treatments. This will help the respondents to know

the options for medical treatments that people who have this

disease can choose from.

Researchers focused on the factors that cause Autoimmune

Attacked to the Inner Ear, because of the taught that autoimmune

attack can lead to the dysfunctional life of a person who

affected by the disease.

Less than 1% of the 28 million Americans who have hearing

loss have it because of the Autoimmune Inner Ear Disease (AIED).

It’s slightly more common in middle – aged women.

 2

Autoimmune Inner Ear Disease (AIED) is the name used to

describe the variety of disorders in which the ear is the sole

target of an inappropriate attack by the immune system. This

disorder differs from other vestibular disorders because medical

treatment can succeed when given early and aggressively.

An early diagnosis is important because treatment can not

only stop the disease progression but in some cases reverse the

damage.

The immune system can attack not just ear, but it can

attack some other body part like eyes, or attack the entire body

including the ear. An autoimmune reaction also creates debris.

Even if the ear is not being directly attacked, it can end up

with debris transported from distant locations and deposited by

the circulation. This debris in the ear can cause problems.

Hearing loss has been viewed historically as the main inner

ear effect of an autoimmune problem, but the vestibular system

can also be attacked. Several factors determine the type of

vestibular symptoms that may be experienced. Those factors

include the speed with which the vestibular loss occurred, the

degree of loss, whether one side or both sides are affected, and

whether the damage has triggered a problem with fluctuating

function. For example, if end lymphatic hydrops developed from

the autoimmune reaction. The symptoms of autoimmune problems can

 3

be similar, even indistinguishable, from other vestibular

disorders.

Variants are bilateral attack of hearing loss and tinnitus

that resemble Meniere’s Disease, and attacks of dizziness

accompanied by abnormal blood tests for antibodies. About 50% of

patients with AIED have symptoms related to balance (dizziness

or unsteadiness).

Tinnitus and aural fullness has 25-50% of the patients also

have a symptom of tinnitus and aural fullness, which can

fluctuate in severity.

Systematic autoimmune disease: Coexisting systematic

autoimmune disease occurs in 15-30% of patients. Diagnoses

include rheumatoid arthritis, ulcerative colitis, systematic

lupus erythematosus, and polyarteritis nodosa.

The immune system is complex and there are several ways

that is can damage the inner ear of the respondent. Both allergy

and traditional autoimmune disease such as ankylosing

spondylitis, systematic lupus erythematosus (SLE), Sjoegren’s

syndrome (dry eye syndrome), Cogan’s disease, ulcerative

colitis, Wegener’s Granulomatosis, Rheumatoid arthritis,

scleroderma, and psoriatic arthritis (Srikumar et al 2004) Can

cause or be associated with AIED. Another multisystem disease,

Bechet’s, commonly has audio vestibular problems.

 4

Allergy is traditionally suspected to be food related, but

there is presently no agreement as to the importance of food

allergy.

Autoimmune Inner Ear Disease (AIED) is rare case of

disease, probably accounting for less than 1% of all cases of

hearing impairment or dizziness (Bovo et al 2009). The precise

incidence is controversial.

A study by Pathak et al suggested that the antioxidant N –

acetyl cysteine (NAC) may be effective against AIED. The

investigators found that peripheral blood mononuclear cells

(PBMCs) in patients with AIED had higher baseline TNF - ∝ and

myeloperoxidase levels that did those of healthy controls.

II. Statement of the Problems

The study focused on the Effects of Autoimmune Inner Ear

Disease to the respondents who affected by it. Specifically,

researchers aim to answer the following questions.

1. What are the factors that cause autoimmune attack to the

inner ear?

2. Why autoimmune attack can lead to dysfunctional life of a

person affected by the disease?

3. Treatments that may possibly apply to the disease.

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III. Hypothesis

The study my anchored on the hypothesis that there is

significant correlation between the immune system to the inner

ear of the people.

IV. Definition of Terms

For clearer understanding of the terms used in the study

the following terms are defined conceptually and operationally.

Autoimmune Disease. In this study, autoimmune disease is the

system of the immune of an organism against its own healthy

cells and tissues. Any disease that results from such an

aberrant immune response is termed by autoimmune.

Inner Ear. In this study, inner ear is the innermost part of the

vertebrate ear. Also called labyrinth of the ear.

Attack. In this research, attack means how aggressive the

disease to the people.

Meniere’s Disease. A condition with recurrent vertigo

accompanied by ringing in the ears (tinnitus) and deafness. Due

to dysfunction of the semi-circular canals (endolymphatic sac)

in the inner ear.

Tinnitus. In this research, it refers to a ringing, whistling or

other sound in the ears.

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Lyme Disease. In this research, it is inflammatory disease

characterized at first by a rash, headache, fever, and chills,

and later by possible arthritis and neurological and cardiac

disorders, caused by bacteria that are transmitted by ticks.

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Chapter II

Related Literature and Studies

This chapter presents the various topics about related

literature and studies taken from different resources such as

books and internet. These resources that the researchers used

become the instrument to achieve the research about the

autoimmune attack to the inner ear.

Autoimmune Attack to the Inner Ear

According to Mj, der Gaag MA, Stokros RJ. (2006). It is

generally felt those anti – cochlear antibodies also called anti

– HSP-70 blood test are not sensitive or specific enough to be

very useful. Antibodies to HSP-70 can also be found in Lyme

Disease, ulcerative colitis, cancers and in about 5% of healthy

individuals. One study suggested that all anti-HSP tests are

directed against the wrong substrate. Autoimmune inner ear

disease is rare, making it difficult to study. One can speculate

that there might be effective treatments that simply have not

been discovered. For example, there are numerous potential

treatments that have not been tried in a formal way. Gamma

globulin infusions, given monthly, are useful in numerous

autoimmune disorders. These treatments are very expensive, which

limits its use. Immune modulating drugs such as are used for
 8

treatment of MS (beta-interferon, alpha-interferon , copaxone)

have not been tried in AIED, to this author’s knowledge. Other

medications that have coincidental suppression of immune

responses, such as minocycline, or other anti-TNF drugs might be

tried.

Dr. George E. Shambaugh, Jr. in Chicag, the American

Hearing Research Foundation serves two vital roles: to fund

significant research in hearing and balance disorders, and to

help educate the public about hearing loss and balance disorders

related to the inner ear.

The AHRF funds 5 to 10 research projects per year, with an

average grant of $20,000. These research projects cover a wide

range of research areas and are conducted with the hopes that we

might better understand how are lose hearing and balance

functions, how we regain them, and most importantly, how to

preserve the function we still have.

(Lopez Escamez et al, 2002) there is evidence that

genetically controlled aspects of the immune system may increase

or otherwise be associated with increased susceptibility to

common hearing disorders such as Meniere’s disease. Bernstein

and associates reported that 44% of patients with Meniere’s,

otosclerosis and striatal presbyacusis had one particular

extended MHC haplotype, compared to only 7% of controls. Sudden

 9

hearing loss in Koreans that does not recover is also associated

with HLA-DRBI*04, DQA1 03 and 05 (Yeo et al, 1995; Yeo et al

2001). The author has also found an association, on the other

hand, a recent study by Lopez – Escamez and other performed in

Spain found no difference in HLA anti gens between 54 patients

with definite MD and 543 normal controls (Lopez – Escamez et al,

2002). The genetic background of HLA studies is important and it

is possible that one group might find HLA differences which are

not found in another. These data are thus conflicted. If there

is needed an association with HLA, at least in certain

populations, it would suggest that more of Meniere’s disease and

other progressive syndromes may be caused by immune dysfunction

than is presently generally thought. It is important to remember

that HLA – typing is relevant when considered in the context of

the patient’s genetic background. In other words, studies of

Korean subjects for example, such as reported by Yeo, may not

apply to persons of non-Korean ethnicity.

Dr. Shambaugh and Dr. Eugene L. Derlacki, et al 2009, a co

– founder of the AHRF and a prominent otolaryngologist, helped

them construct and equip a hearing clinic at Northwestern

University Medical School, the first clinic of its kind where

numerous diagnostic procedures were developed. The establishment

of the clinic led to the creation of Mid-West Hearing Foundation

in 1956, which would later become the American Hearing Research

 10

Foundation. The timeline below lists highlights and

accomplishments of the Foundation, and its evolution under

various names to reflect a broadening in geographical scope. In

1940, a grant from the Chicago Community Trust for $10,000 was

used to construct and equip a laboratory at Wesley Memorial

Hospital, located in Chicago, to perfect the fenestration

technique. In 1942, grants from the John and Mary R. Markle

Foundation of New York kept the lab going and helped establish a

temporal bone histopathology laboratory. The temporal bone is

part of the skull that enclose the hearing and balance systems

within the inner ear.

Cohen et al 2011, Mora et al 2005, larger trials are

necessary before these therapies can be widely practiced.

Enoxaparin affects the clotting system of the blood, and may

place patients at increased risk of bleeding. Transtympanic

delivery of medication may provide better penetration of drug

molecules into the inner ear.

Transtympanic administration of steroids has been shown to

improve hearing and balance symptoms in AIED in a small study

(Garcia – Berrocal et al 2006) larger trails are needed to

determine the true delivery (Alles et al 2006, Rauch 2004).

Etanecept (Enbrel), ant anti - TNF drug, is emerging as a

promising agent for treatment of AIED (Rahman et al 2001). It is

 11

given as an injection twice a week. Transtympanic application of

Enbrel was effective in a pilot study in steroid – dependent

patients and when used in combination with methyl prednisone

(Van Wijk et al 2006). Enbrel is presently very expensive and in

short supply, and the lack of large studies of its use in AIED

presently limit clinician enthusiasm for its use. Nevertheless,

it presently appears to be the most promising agent.

Cell therapy involves transplantation of individual stem

cells capable of developing into inner ear cells in the ear

canal. Gene therapy is the introduction of new genes into native

cells, allowing the cells to produce new proteins that improve

their ability to function. Several laboratories have researched

the possibility of cells or gene therapy to replaced damaged ear

cells in AIED. Laboratory tests with animal models are

promising, but much more research is needed to determine the

effectiveness and safety of cell and gene therapies (Nakagawa &

Ito 2002, Pau & Clarke 2004, Zhou et al 2012, Zhou et al 2011).

In 1989, Luetje studied the use of plasmapheresis in

patients with autoimmune inner ear disease. Improvement in

auditory function occurred in 6 of 8 patients, 3 of whom were

able to discontinue immunosuppressive medication.

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Chapter III

Methodologies

The researchers used the two methodologies, descriptive and

explanatory. Definitely, researchers described what kind of

disease the autoimmune inner ear disease.

Descriptive, because the research needs to explain further,

factually, accurately a situation or problem, it needs to

describe and answer the problems “What is”. Like, what is the

causes and symptoms of the autoimmune inner ear disease.

Lastly, is the explanatory because this research needs to

clarify the “how and why” relationship exist between two or more

aspects of the problem.

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Chapter IV

Result and Discussion

Autoimmune Inner Ear Disease (AIED) is one of the most

aggressive disease that can attack to the body. The immune

system is complex and there are several ways that it can damage

the inner ear. The researchers need to discuss how is Autoimmune

Inner Ear Disease (AIED) diagnosed? Bilateral progressive

hearing loss or progressive vestibular balance loss, audiometry

documenting progressive bilateral sensor neural hearing loss ABR

(if hearing is good enough), or optoacoustic emission testing,

Rotatory chair test, ECOG (electrocochleography).

The diagnosis is based on the history, findings on physical

examination blood test, and the results of hearing and

vestibular test. The symptoms of AIED are sudden hearing loss in

one ear progressing rapidly to the second ear. The hearing loss

can progress over weeks or months. Patients may feel fullness in

the ear and experience vertigo. In addition, a ringing, hissing,

or roaring sound in the ear may be experienced. Diagnosis of

AIED is difficult and is often mistaken for otitis media until

the patients develops a loss in the second ear. One diagnostic

test that is promising is the Western blot immunoassay.

In general, autoimmune disorders occur more frequently in

women than men and less frequently in children and the elderly.
 14

When the ear is attacked, the progression of damage and

functional loss is rapid, occurring over weeks to months and

usually progressing rapidly to the second ear. Not all patients

respond to corticosteroid therapy in the same manner. Some show

improvement in threshold, discrimination scores, or both. Others

with fluctuation and progression before therapy stabilize

without actually improving. Still others actually loss hearing

despite immunosuppressive therapy.

AIED can cause low immune system, dizziness, ringing in the

ears and hearing loss. If one people have AIED then it is the

start of hearing loss of one ear and it possibly spread to the

other part. This may take weeks, or it could happen over a few

months.

The immune cells or system are always on the lookout for

germs trying to invade the body. If they mistake cells in the

inner ear for virus or bacteria, they attack them. This is

called an autoimmune reaction.

To diagnose AIED, the doctor will ask the question about

health and medical history, do a physical exam, and the request

for the hearing test. And also, the doctor tests the respondent

for balance, which can show how well the inner ear is “talking”

to the brain. It might also have a blood work done.

 15

There’s no test that can tell for sure that they have AIED,

but the results may show that their having an autoimmune

reaction. If they do, it’s a good idea to see an

otolaryngologist (ear doctor) who’s also trained in autoimmune

disorders. Since the respondent may not get a clear answer, the

doctor may start the treatment without a sure diagnosis to

prevent damage to the hearing that can’t be fixed. Many

respondents aren’t diagnosed with AIED until they start

treatment and their symptoms get better.

Therefore, the first step in the evaluation of a patients

presenting with hypoacusia is to define whether the hearing loss

is sensorineural or conductive. This is based on the physical

examination, specifically on the otoscopy findings and tuning

forks tests. The magnitude of hearing loss is then defined by an

audiogram where air and bone conduction hearing thresholds are

quantified and compared.

The audio logical assessment is a crucial part of both the

initial diagnostic evaluation and all subsequent follow – ups of

patients with hearing loss, allowing the characterization of the

severity, frequencies involved and symmetry. The two most

important components of the audio logical assessment in patients

with AIED are pure – tone air conduction threshold testing, the

goal of which is to obtain a representation of the softest

 16

intensity heard across frequencies (0.25, 0.5, 1, 2, 3, 4, 6 and

8 khz) comparing observed threshold with normative data pure –

tone average (PTA) is arithmetical average of threshold and

word discrimination, which probes the ability to perceive,

process and verbally reproduce phonological units that comprise

spoken words.

The main risk factor for developing AIED is already having

an autoimmune disease in another part of your body. Autoimmune

diseases such as Addison’s disease, ankylosing spondylitis,

Cogan’s disease, dermatomyositis, Graves’ disease, Guillain-

Barre syndrome, Hashimoto’s thyroiditis, insulin-dependent (Type

1) diabetes mellitus, pernicious anemia, polyarteritis nodosa,

psoriasis, rheumatoid arthritis, sarcoidosis, scleroderma,

Sjoegren’s syndrome (dry eye syndrome), systemic lupus

erythematosus (SLE), ulcerative colitis, and Wegener’s

granulomatosis can cause or be associated with AIED.

It seems that allergies can cause or at least are

associated with AIED. Exactly how these “fit” together is still

unknown? However, a large percentage of the people treated with

immunotherapy for inhalant allergies showed improvement in

vertigo and other Meniere’s symptoms, suggesting an association

between Immunoglobulin E (IgE) disease and inner ear problems.

 17

The treatment most widely used for AIED is corticosteroids

therapy. Corticosteroid carry a risk of side effects, some of

which can cause serious health problems. If know what side

effects are possible, it can take steps to control their impact.

Because oral corticosteroids affect the entire body because of

the high dose, instead of just a particular area, this route of

administration is the most likely to cause significant side

effects.
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Chapter V

Suggestions and Recommendations

The researchers recommend to the respondents who have this

kind of disease, to be aware of how aggressive the AIED. The

doctor probably will give a drug that helps with inflammation.

High doses of steroids have been shown to work well for AIED,

but they have many side effects. So the respondent probably

won’t take them more than a few weeks.

After taking the steroids, the doctors may prescribe a

medication that can slow down the immune system of the one

person. Drugs that are used for chemotherapy, like methotrexate

and cyclophosphamide are sometimes used for this. They don’t

know how long they should prescribe these drugs. Finally, they

don’t have a clue whether these drugs work or not. As a result,

the respondents need to decide to their body from these

dangerous drugs on the chance that it will help their hearing.

They may, and then again, they may not. None of the above are

meant for long – term use unless they like horrible side effects

messing up your body. One person who had experience warns. If

steroids don’t regain or stop the hearing loss, get off them

after just a few weeks.

The researchers like to suggest that if there is no change

within 4 weeks, these drugs are unlikely to help the respondents

 19

that. They should stop before their side effects totally mess up

their body. Also, if the respondent has had a sudden hearing

loss for more than a month or two already, don’t expect the

steroids to really do much to help regain hearing.

 20

Notes

Alexander TH, Weisman MH, Derebery JM, Espeland MA, Gantz BJ, et

al. 2009. Safety of high-dose corticosteroids for the treatment

of autoimmune inner ear disease.

Alles MJ, der Gaag MA, Stokroos RJ. 2006. Intratympanic steroid

therapy for inner ear diseases, a review of the literature. Eur

Arch Otorhinolaryngol 263: 791-7

Brenner M, Hoistad D, Hain TC. Prevalence of thyroid dysfunction

in Meniere's Disease. Archives of Oto HNS, 130/2 226-228 (2004)

Otology & neurotology : official publication of the American

Otological Society, American Neurotology Society [and] European

Academy of Otology and Neurotology 30: 443-8

Campbell, K.C., & Klemens, J. J. (2000). Sudden hearing loss and

autoimmune inner ear disease. Journal of the American Academy of

Audiology, 11, 361–367.

 21

Derebery, J. (2001, November 26). Interview with Jennifer

Derebery, M.D, House Ear Clinic, Autoimmune Inner Ear Disease.

Audiology Online. Available from

McCabe B. “Autoimmune Sensorineural Hearing Loss.” Annals of

Otology, 88:585–589, 1979.

Stone JH, Francis HW “Immune-Mediated Inner Ear Disease.”

Current Opinion in Rheumatology, 12:32–40, 2000.

http://hearinglosshelp.com/blog/autoimmune-inner-ear-disease-

aied-2/

https://www.webmd.com/cold-and-flu/ear-infection/autoimmune-

inner-ear-disease#1

http://www.audiologyonline.com/interview/displayarchives.asp?int

erview_id=92.
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Vitae
 23

KIMBERLY F. ESPLAGO
Address: St. Antonio St.,
Penafrancia Ave., Naga City
Email: kimberlyesplago@gmail.com

Educational Attainment:

Tertiary: Naga College Foundation, Inc.

City of Naga
2013 – Present
Secondary: Partido College
Tambuco, Goa, Camarines Sur
S/Y 2009 – 2013
Elementary: Mabolo Elementary School
Mabolo, Bacoor City, Cavite
S/Y 2002 - 2007

Personal Data:
Age: 22 years old
Birthday: December 11, 1995
Birthplace: Lamon, Goa, Camarines Sur
Sex: Female
Civil Status: Single
Height: 5’1
Weight: 39 kls
Religion: Iglesia ni Cristo
 24

Mackie D. Dollisen
Address: San Rafael,
Pamplona Camarines Sur
Email: dollisenmackie@gmail.com

Educational Attainment:

Tertiary: Naga College Foundation, Inc.

City of Naga
2015 – Present
Secondary: San Rafael National High School
San Rafael, Pamplona
S/Y 2010 – 2014
Elementary: Pamplona Elementary School
Pamplona Camarines Sur
S/Y 2005 - 2010

Personal Data:

Age: 19 years old

Birthday: April 9, 1998
Birthplace: San Rafael Pamplona Cam., Sur
Sex: Female
Civil Status: Single
Height: 4’11
Weight: 47 kls
Religion: Roman Catholic
 25

Mary France F. Royo

Address: Adiangao, San Jose,
Camarines Sur
Email: royo.france_26@yahoo.com

Educational Attainment:

Tertiary: Naga College Foundation, Inc.

City of Naga
2014 – Present
Secondary: San Rafael National High School
San Rafael, Tigaon
S/Y 2010 - 2014

Elementary: Adiangao Elementary School

Adiangao, San Jose, Camarines Sur
S/Y 2005 - 2010

Personal Data:

Age: 19 years old

Birthday: September 26, 1998
Birthplace: BMC, Naga City
Sex: Female
Civil Status: Single
Height: 4’11
Weight: 41 kls
Religion: Roman Catholic