Preface

Ménière's disease is an alteration of the inner ear characterized by two groups of symptoms: vestibular symptoms and auditory symptoms. Classical symptoms such as fluctuating hearing loss, tinnitus, atrial fullness, and concomitant dizziness greatly aid the otorhinolaryngologist to diagnose carriers of the disease. But in many patients, their presentation may be different.

In this book we will adopt the term Ménière's disease to follow the prevailing trend among most research groups and discuss the main topics and current and past ideas about the etiopathogenesis, diagnosis, and treatment of Ménière's disease and Ménière's disease associated with migraine.

It is likely that there are genotypic—racial as well as phenotypic—environmental factors that influence the prevalence difference between countries. One of the major problems in this respect is that the initial presentation of the disease is often the cochlear form, which is not clinically recognized, and is again attributed to another specific cause or is presumed to be simply due to aging. Even after the vestibular component becomes obvious, long periods of remission may mask the complete final image of the syndrome with episodic vertigo, fluctuating autistic loss, tinnitus, and aural fullness. Therefore, generally, in clinical practice, only moderate to severe cases have been tabulated in the estimates so far.

Some of the epidemiologically published studies to date have tended to blend different epidemiological concepts. The direction of these studies is mainly retrospective (the themes are identified after a result or illness), and they actually measure only prevalence (existing events or the number of cases of a disease at a given moment divided by the population at risk). Only prospective studies (subjects are identified before a result or illness, and future events are counted) would have the power to adequately measure this incidence. Although more reflective of real life than an artificial experiment, retrospective observational studies are susceptible to bias.

The multiplicity of diagnostic criteria is another problem that makes it difficult to establish the true incidence of Ménière's disease in the general population. In 1972 the Committee on Hearing and Equilibrium of the American Academy of Otorhinolaryngology proposed a specific definition of the disease and guidelines for the evaluation of Ménière's disease in communicating treatment results. In 1985, it was considered that the definition of Ménière's disease needed to be restricted to cases with a complete set of classic signs and symptoms. The 1995 criteria were intended to simplify the definition of Ménière's disease and allow greater flexibility, making it usable in a wide range of studies and classifications. A minimum set of signs and symptoms must be noted in such a way that the degree of certainty of the diagnosis can be established.

Currently, there is no universally accepted theory about the pathophysiology of this disease. Through histopathological studies, it is presumed that endolymphatic hydrops is the most descriptive pathological characteristic of Ménière's disease.

**II**

**Section 6**

Meniere's Disease: Surgical Treatment

Future of Surgical Treatment **107**

**Chapter 7 109**

*by Yetkin Zeki Yilmaz, Begum Bahar Yilmaz and Mehmet Yilmaz*

The pathophysiology of the symptoms is still disputed: ruptures of membranes, increased pressure and mechanical displacement of the peripheral organs as saccule by endolymph accumulation, viral infections, and autoimmune disease in addition to several other theories that have already been reported. It can be seen that in this scheme, currently accepted endolymphatic hydrops is no longer a central etiology but rather one of the manifestations of the syndrome. And the exact mechanism of the etiopathogenesis of the syndrome remains unknown. It is believed that multifactorial inheritance is the best response, in which the necessary conditions are met to lead to malabsorption of the endolymph and, subsequently, to dropsy. Clinical and laboratory evidence supports this concept.

> **Fayez Bahmad Jr.** University of Brasilia, Brasilia, DF, Brazil

> > **1**

Section 1

Introduction

Ophthalmology and Otolaryngology Faculty, University of São Paulo, São Paulo, SP, Brazil

Section 1 Introduction

**3**

**Chapter 1**

Disease (MD)

or the frequency of attacks of vertigo [1–4].

are of fundamental importance [1, 2].

have been reported [1–3].

disease were identified:

disease.

[1, 2–6].

*Fayez Bahmad Jr*

**1. Introduction**

Introductory Chapter: Ménière's

Ménière's disease (MD) is probably a multifactorial disorder where the genetics and environmental factors determine the onset of the disease. This disease have been related to the accumulation of endolymph in the cochlear duct and the vestibular organs in histopathological studies, although endolymphatic hydrops (EH) per se does not explain all clinical features, including the progression of hearing loss

Dizziness and vertigo are frequent symptoms in the otolaryngologist's practice, and all efforts towards the better comprehension of this system and its pathology

Currently, there is no universally accepted theory about the pathophysiology of the disease. Through histopathological studies, it is assumed that endolymphatic hydrops is the most descriptive pathological feature of Ménière's disease. The pathophysiology of symptoms is still widely disputed: membrane ruptures, increased pressure, and mechanical displacement of peripheral organs such as endolymph accumulation, viral infections, autoimmune disease, and several other theories that

Great advances have been made in neuro-otology, and increasing knowledge in the field of molecular biology, genetics, and neurosciences has substantially modified the approach of the patient with balance complaints. This book studies the most interesting and controversial of these vestibular diseases, the Ménière's

The Classification Committee for the International Classification of Vestibular

Disorders (ICVD) nominated by the Bárány Society, 2009, standardized the nomenclature of vestibular symptoms (SV) in four groups. One of the most important is the episodic vestibular syndrome: crises of vestibular symptoms interspersed with asymptomatic periods, such as Ménière's syndrome and vestibular migraine

Ménière's disease is an inner ear alteration characterized by two groups of symptoms: vestibular and auditory symptoms. In many patients, their presentation may be unusual or different than the classical symptoms such as tinnitus, fluctuat-

The history of the disease may be progressive or nonprogressive, and, in addition to the typical clinical presentation of Ménière's disease, two variants of the

1. cochlear Ménière's disease—hearing is the predominant symptom; and

2.vestibular Ménière's disease—vestibular symptoms are predominant.

ing hearing loss, aural fullness, and concomitant dizziness [1, 2].

#### **Chapter 1**

## Introductory Chapter: Ménière's Disease (MD)

*Fayez Bahmad Jr*

#### **1. Introduction**

Ménière's disease (MD) is probably a multifactorial disorder where the genetics and environmental factors determine the onset of the disease. This disease have been related to the accumulation of endolymph in the cochlear duct and the vestibular organs in histopathological studies, although endolymphatic hydrops (EH) per se does not explain all clinical features, including the progression of hearing loss or the frequency of attacks of vertigo [1–4].

Dizziness and vertigo are frequent symptoms in the otolaryngologist's practice, and all efforts towards the better comprehension of this system and its pathology are of fundamental importance [1, 2].

Currently, there is no universally accepted theory about the pathophysiology of the disease. Through histopathological studies, it is assumed that endolymphatic hydrops is the most descriptive pathological feature of Ménière's disease. The pathophysiology of symptoms is still widely disputed: membrane ruptures, increased pressure, and mechanical displacement of peripheral organs such as endolymph accumulation, viral infections, autoimmune disease, and several other theories that have been reported [1–3].

Great advances have been made in neuro-otology, and increasing knowledge in the field of molecular biology, genetics, and neurosciences has substantially modified the approach of the patient with balance complaints. This book studies the most interesting and controversial of these vestibular diseases, the Ménière's disease.

The Classification Committee for the International Classification of Vestibular Disorders (ICVD) nominated by the Bárány Society, 2009, standardized the nomenclature of vestibular symptoms (SV) in four groups. One of the most important is the episodic vestibular syndrome: crises of vestibular symptoms interspersed with asymptomatic periods, such as Ménière's syndrome and vestibular migraine [1, 2–6].

Ménière's disease is an inner ear alteration characterized by two groups of symptoms: vestibular and auditory symptoms. In many patients, their presentation may be unusual or different than the classical symptoms such as tinnitus, fluctuating hearing loss, aural fullness, and concomitant dizziness [1, 2].

The history of the disease may be progressive or nonprogressive, and, in addition to the typical clinical presentation of Ménière's disease, two variants of the disease were identified:

1. cochlear Ménière's disease—hearing is the predominant symptom; and

2.vestibular Ménière's disease—vestibular symptoms are predominant.

Other classifications used frequently are as follows:


Recent studies revealed that there are genotypic and phenotypic factors that influence the prevalence difference between countries [7].

In the majority of these patients, the initial presentation of the disease is often the cochlear form, which is harder to be clinically recognized and frequently is associated with another cause or is presumed to be simply due to aging.

Even after the vestibular component becomes obvious, long periods of remission may mask the complete final image of the syndrome with episodic vertigo, fluctuating autistic loss, tinnitus, and aural fullness. Therefore, generally in clinical practice, only moderate to severe cases are tabulated in the estimates so far.

A multifactorial inheritance is believed to be the best response, where the necessary conditions are met, leading to endolymph malabsorption and subsequently hydrops. Clinical and laboratory evidence supports this concept. Merchant et al. analyzed the temporal bone collection of the Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, USA, with a clinical diagnosis of Ménière's syndrome (28 cases) or a histopathological diagnosis of hydrops (79 cases).

All 28 cases with classic symptoms of Ménière's syndrome had hydrops in at least one ear. However, the reverse is not true. There were 9 cases of idiopathic hydrops and 10 cases with secondary hydrops, in which the patients did not have the classic symptoms of Ménière's syndrome. Endolymph is mainly produced in the stria vascularis. Slowly, endolymph is absorbed into the endolymphatic duct and sac, a biologically active transport structure where absorption occurs mainly and also to a lesser extent secretion occurs. Evidence strongly suggests that longitudinal flow (slow process) and radial flow (fast) occur.

In this book we will adopt the term Ménière's disease to follow the prevailing trend among most research groups and discuss the main topics, current and past ideas about etiopathogenesis, diagnosis, and treatment of Ménière's disease associated with migraine.

Some of the epidemiologically published studies to date have tended to blend different epidemiological concepts. The direction of these studies is mainly retrospective (the themes are identified after a result or illness), and they actually measure only the prevalence (existing events or the number of cases of a disease at a given moment divided by the population at risk). Only prospective studies (subjects are identified before a result or illness, future events are counted) would have the power to adequately measure this incidence. Although more reflective to real life than an artificial experiment, retrospective observational studies are susceptible to bias.

#### **2. Diagnostic failures**

There are many classifications and proposed diagnostic criteria, and it makes difficult to establish the true incidence of Ménière's disease in the general population.

The 1995 and 2015 criteria simplified the definition of Ménière's disease and allowed to be usable in all global studies and so may be able to substitute and unify all the classifications [1, 2].

**5**

*Introductory Chapter: Ménière's Disease (MD) DOI: http://dx.doi.org/10.5772/intechopen.91924*

Almost all the researchers and histopathological works in the past presumed that

endolymphatic hydrops was the pathological characteristic of Ménière's disease. There are still many questions and polemic discussions about the pathophysiology of the symptoms: increased pressure and mechanical displacement of the peripheral organs such as saccule by endolymph accumulation, ruptures of membranes, viral infections, and autoimmune disease, in addition to several other theories that have

Recent consensus accepted that endolymphatic hydrops is no longer a central

Classical Ménière's disease is an excellent example of a condition that can be

When it is the classic or definite form, it is characterized by recurrent and spontaneous episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural fullness. In this case the diagnosis is easy even for the most naive

In 2015, the Hearing and Balance Committee of the American Academy of Otorhinolaryngology-Head and Neck Surgery (AAO-HNS), Bárány Society, and other entities set the parameters for the clinical diagnosis of Ménière's disease. The classification includes two categories: defined Ménière's disease and prob-

Defined Ménière's disease is based on clinical criteria and requires the observation of an episodic vertigo syndrome associated with low- to medium-frequency sensorineural hearing loss and fluctuating auditory symptoms (tinnitus in the ear

The duration of vertigo episodes is limited to a period of between 20 minutes and 12 hours. Probable Ménière's disease is a broader concept defined by episodic vestibular symptoms (vertigo or dizziness) associated with fluctuating aural symp-

• detailed medical history that should include all previous vertigo events;

• imaging tests to aid diagnosis and rule out differential diagnoses of the syn-

• cochlear and vestibular, audiological, and electrophysiological examinations.

• laboratory tests to rule out differential diagnoses of the syndrome;

toms that occur over a period of 20 minutes to 24 hours [1, 2]. The clinical evaluation then includes the following [8, 9]:

The most appropriate exams to aid in diagnosis consist of:

etiology but rather as one of the manifestations of the syndrome.

diagnosed on clinical grounds and simple audiometric examinations.

**3. Etiopathogeny**

already been reported.

**4. Diagnostic**

clinician [1, 2].

able Ménière's disease [2].

drome; and

• glycerol dehydration test;

• PEMV or VEMP test.

• electrocochleography (ECochG); and

and/or fullness) in the affected ear.

#### **3. Etiopathogeny**

*Meniere's Disease*

toms; and

Other classifications used frequently are as follows:

influence the prevalence difference between countries [7].

associated with another cause or is presumed to be simply due to aging.

practice, only moderate to severe cases are tabulated in the estimates so far.

2.Ménière's disease: idiopathic cause [1, 2].

(slow process) and radial flow (fast) occur.

ated with migraine.

**2. Diagnostic failures**

all the classifications [1, 2].

population.

1.Ménière's syndrome: known and well-established condition causing symp-

Recent studies revealed that there are genotypic and phenotypic factors that

In the majority of these patients, the initial presentation of the disease is often the cochlear form, which is harder to be clinically recognized and frequently is

Even after the vestibular component becomes obvious, long periods of remission may mask the complete final image of the syndrome with episodic vertigo, fluctuating autistic loss, tinnitus, and aural fullness. Therefore, generally in clinical

A multifactorial inheritance is believed to be the best response, where the necessary conditions are met, leading to endolymph malabsorption and subsequently hydrops. Clinical and laboratory evidence supports this concept. Merchant et al. analyzed the temporal bone collection of the Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, USA, with a clinical diagnosis of Ménière's syndrome (28 cases) or a histopathological diagnosis of hydrops (79 cases).

All 28 cases with classic symptoms of Ménière's syndrome had hydrops in at least one ear. However, the reverse is not true. There were 9 cases of idiopathic hydrops and 10 cases with secondary hydrops, in which the patients did not have the classic symptoms of Ménière's syndrome. Endolymph is mainly produced in the stria vascularis. Slowly, endolymph is absorbed into the endolymphatic duct and sac, a biologically active transport structure where absorption occurs mainly and also to a lesser extent secretion occurs. Evidence strongly suggests that longitudinal flow

In this book we will adopt the term Ménière's disease to follow the prevailing trend among most research groups and discuss the main topics, current and past ideas about etiopathogenesis, diagnosis, and treatment of Ménière's disease associ-

Some of the epidemiologically published studies to date have tended to blend

different epidemiological concepts. The direction of these studies is mainly retrospective (the themes are identified after a result or illness), and they actually measure only the prevalence (existing events or the number of cases of a disease at a given moment divided by the population at risk). Only prospective studies (subjects are identified before a result or illness, future events are counted) would have the power to adequately measure this incidence. Although more reflective to real life than an artificial experiment, retrospective observational studies are susceptible to bias.

There are many classifications and proposed diagnostic criteria, and it makes difficult to establish the true incidence of Ménière's disease in the general

The 1995 and 2015 criteria simplified the definition of Ménière's disease and allowed to be usable in all global studies and so may be able to substitute and unify

**4**

Almost all the researchers and histopathological works in the past presumed that endolymphatic hydrops was the pathological characteristic of Ménière's disease. There are still many questions and polemic discussions about the pathophysiology of the symptoms: increased pressure and mechanical displacement of the peripheral organs such as saccule by endolymph accumulation, ruptures of membranes, viral infections, and autoimmune disease, in addition to several other theories that have already been reported.

Recent consensus accepted that endolymphatic hydrops is no longer a central etiology but rather as one of the manifestations of the syndrome.

#### **4. Diagnostic**

Classical Ménière's disease is an excellent example of a condition that can be diagnosed on clinical grounds and simple audiometric examinations.

When it is the classic or definite form, it is characterized by recurrent and spontaneous episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural fullness. In this case the diagnosis is easy even for the most naive clinician [1, 2].

In 2015, the Hearing and Balance Committee of the American Academy of Otorhinolaryngology-Head and Neck Surgery (AAO-HNS), Bárány Society, and other entities set the parameters for the clinical diagnosis of Ménière's disease.

The classification includes two categories: defined Ménière's disease and probable Ménière's disease [2].

Defined Ménière's disease is based on clinical criteria and requires the observation of an episodic vertigo syndrome associated with low- to medium-frequency sensorineural hearing loss and fluctuating auditory symptoms (tinnitus in the ear and/or fullness) in the affected ear.

The duration of vertigo episodes is limited to a period of between 20 minutes and 12 hours. Probable Ménière's disease is a broader concept defined by episodic vestibular symptoms (vertigo or dizziness) associated with fluctuating aural symptoms that occur over a period of 20 minutes to 24 hours [1, 2].

The clinical evaluation then includes the following [8, 9]:


The most appropriate exams to aid in diagnosis consist of:


#### **5. Treatment**

Different treatment options for Ménière's disease exist with substantial variability between countries. None of the treatment options cure the disease. As many treatments have a significant impact on the functioning of surrounding structures, one should start with noninvasive approaches with the fewest possible side effects and proceed to more invasive steps:


Sodium restriction diet: Low-level evidence suggests that restricting the sodium intake may help to prevent Ménière's attacks.

Betahistine: Substantial disagreement in the medical community about the use of betahistine exists. A Cochrane review found low-level evidence to support the use of betahistine with substantial variability between studies. Medical therapy in many medical centers often starts with betahistine orally.

Intratympanic steroid injections may reduce the number of vertigo attacks in patients with Ménière's disease.

Intratympanic gentamycin injections: Gentamycin has strong ablative properties towards vestibular cells. The side effects are a sensorineural hearing loss because of a certain amount of toxicity towards cochlear cells.

Surgery with vestibular nerve section or labyrinthectomy: Nerve section is a therapeutic option in patients who failed the conservative treatment options and labyrinthectomy when surgical options failed. Labyrinthectomy leads to a complete hearing loss in the affected side.

Clinically, three situations arise in which drug treatment is very helpful:

#### **6. Acute attack drugs**

Aiming at sedating the vestibule-trunk axis is particularly useful in aborting acute attacks. These include cinnarizine, promethazine, and diazepam.

Prolonged use of drugs such as cinnarizine is not advisable due to the risk of extrapyramidal side effects from prolonged use, particularly in the elderly.

#### **6.1 Maintenance treatment**

Dietary salt restriction and the use of diuretics such as furosemide, amiloride, and hydrochlorothiazide are attempts to prevent endolymphatic hydrops. The basis

**7**

remission.

*Introductory Chapter: Ménière's Disease (MD) DOI: http://dx.doi.org/10.5772/intechopen.91924*

preferred means of ensuring medical treatment.

**7. Ablative treatment**

afferents.

*Protocol of use*:

**7.1 Intratympanic gentamicin**

have been recognized for decades.

patient's symptoms are refractory to the above measures.

poor, but the vertigo presented by the patient is disabling.

Ménière's disease is therefore very promising [10].

• Provide for college entrance compensation

• Repeat the VENG at the end of the sessions

• Patient rests for 1 hour after application

• Intratympanic gentamicin (40 mg/ml)

• Repeat audiometry weekly

• Topical anesthesia

**7.2 Surgical treatment**

• Initial reference (VENG before GIT)

• Complete battery of vestibular tests before therapy

• Weekly intervals (up to three to four applications)

for this is historical rather than scientific, as the data from the few controlled stud-

Betahistine has been subject to some scientific scrutiny, and several controlled clinical studies have shown significant improvement in vertigo, hearing loss, and tinnitus in the short term. Betahistine, with or without a diuretic, is currently the

Drugs such as cinnarizine, propranolol (particularly if the patient has a history of migraine), and corticosteroids are also used empirically by some doctors if the

The toxic effects of aminoglycosides on the inner ear sensory neuroepithelium

Chemical labyrinthectomy through intratympanic gentamicin (GIT) controls vertigo and has been helpful in mainly unilateral Ménière's disease when hearing is

The attending otologist should properly remind and advise the patient that from 3 days after the first application, fiber differentiation begins to occur and this usually leads to severe vestibular symptoms between 7 and 10 days after application. And it is a phenomenon expected by the chemical destruction of vestibular nerve

Several series have a vertigo control rate of about 90%, although a cochleotoxic effect is seen in 15–25% of cases. The future for intratympanic aminoglycosides in

Whether as a result of medical treatment or as a consequence of the clinical

course of Ménière's disease, about 90% of patients have a long period of

ies that exist are conflicting and the placebo effect is clinically significant.

#### *Introductory Chapter: Ménière's Disease (MD) DOI: http://dx.doi.org/10.5772/intechopen.91924*

for this is historical rather than scientific, as the data from the few controlled studies that exist are conflicting and the placebo effect is clinically significant.

Betahistine has been subject to some scientific scrutiny, and several controlled clinical studies have shown significant improvement in vertigo, hearing loss, and tinnitus in the short term. Betahistine, with or without a diuretic, is currently the preferred means of ensuring medical treatment.

Drugs such as cinnarizine, propranolol (particularly if the patient has a history of migraine), and corticosteroids are also used empirically by some doctors if the patient's symptoms are refractory to the above measures.

#### **7. Ablative treatment**

*Meniere's Disease*

**5. Treatment**

• conservative;

• diet;

• diuretics;

and proceed to more invasive steps:

• labyrinth suppressors;

• invasive procedures;

• labyrinthectomy; and

• vestibular neurectomy.

patients with Ménière's disease.

hearing loss in the affected side.

**6. Acute attack drugs**

**6.1 Maintenance treatment**

• intratympanic gentamicin;

• endolymphatic sac decompression surgery;

intake may help to prevent Ménière's attacks.

many medical centers often starts with betahistine orally.

a certain amount of toxicity towards cochlear cells.

Different treatment options for Ménière's disease exist with substantial variability between countries. None of the treatment options cure the disease. As many treatments have a significant impact on the functioning of surrounding structures, one should start with noninvasive approaches with the fewest possible side effects

Sodium restriction diet: Low-level evidence suggests that restricting the sodium

Betahistine: Substantial disagreement in the medical community about the use of betahistine exists. A Cochrane review found low-level evidence to support the use of betahistine with substantial variability between studies. Medical therapy in

Intratympanic steroid injections may reduce the number of vertigo attacks in

Surgery with vestibular nerve section or labyrinthectomy: Nerve section is a therapeutic option in patients who failed the conservative treatment options and labyrinthectomy when surgical options failed. Labyrinthectomy leads to a complete

Aiming at sedating the vestibule-trunk axis is particularly useful in aborting

Prolonged use of drugs such as cinnarizine is not advisable due to the risk of

Dietary salt restriction and the use of diuretics such as furosemide, amiloride, and hydrochlorothiazide are attempts to prevent endolymphatic hydrops. The basis

acute attacks. These include cinnarizine, promethazine, and diazepam.

extrapyramidal side effects from prolonged use, particularly in the elderly.

Clinically, three situations arise in which drug treatment is very helpful:

Intratympanic gentamycin injections: Gentamycin has strong ablative properties towards vestibular cells. The side effects are a sensorineural hearing loss because of

**6**

#### **7.1 Intratympanic gentamicin**

The toxic effects of aminoglycosides on the inner ear sensory neuroepithelium have been recognized for decades.

Chemical labyrinthectomy through intratympanic gentamicin (GIT) controls vertigo and has been helpful in mainly unilateral Ménière's disease when hearing is poor, but the vertigo presented by the patient is disabling.

The attending otologist should properly remind and advise the patient that from 3 days after the first application, fiber differentiation begins to occur and this usually leads to severe vestibular symptoms between 7 and 10 days after application. And it is a phenomenon expected by the chemical destruction of vestibular nerve afferents.

Several series have a vertigo control rate of about 90%, although a cochleotoxic effect is seen in 15–25% of cases. The future for intratympanic aminoglycosides in Ménière's disease is therefore very promising [10].

*Protocol of use*:


#### **7.2 Surgical treatment**

Whether as a result of medical treatment or as a consequence of the clinical course of Ménière's disease, about 90% of patients have a long period of remission.

This implies that 10% of patients continue to have clinically important episodes of vertigo, and surgical treatment should be considered for them.

The various surgical procedures advocated for Ménière's disease continue to raise considerable controversy among otolaryngologists. The decision to operate and the choice of procedure are often dictated by the understanding and experience of a particular technique and the surgeon's individual threshold for surgical intervention. Generally, surgical procedures for Ménière's disease are classified as destructive or nondestructive with regard to hearing [8–10].

#### **7.3 Endolymphatic sac surgery**

Endolymphatic sac surgery was first described in 1927 by Portmann, and no other aspect of Ménière's disease has elicited further debate or controversy. Just as the exact role of the endolymphatic sac in the development of hydrops is not yet known, the precise mechanism by which surgery works remains undefined. However, endolymphatic sac decompression surgery is still widely performed [10].

#### **7.4 Vestibular nerve section**

In the vestibular nerve section, no attempt is made to modify the underlying pathophysiology. The objective is to dissociate the offensive maze from the trunk, preserving the patient's hearing.

The procedure is uniformly effective, with vertigo control in 90–95% of patients according to some series. However, it is a surgery with considerable risks inherent in any posterior fossa neurosurgical procedure [10].

#### **7.5 Surgical labyrinthectomy**

Labyrinth extirpation is indicated in patients with severe symptoms who have virtually useless hearing. Disturbance of the inner ear thus invariably leads to permanent anacusis. However, the ear on the opposite side may have subclinical hydrops, and we should be naturally concerned that the progress of the disease in the patient's contralateral ear may aggravate and make it bilaterally deaf. This is probably the reason for the widespread choice of nondestructive inner ear procedures [10].

#### **7.6 Cochlear implant**

Over the past decade, the hearing rehabilitation of certain profoundly deaf people has been transformed by cochlear implants.

Patients with severe bilateral Ménière's disease and severe to profound bilateral sensorineural deafness will end up with an indication for hearing rehabilitation with cochlear implant. Surgeons with patients with symptoms whose disease is refractory to clinical treatment have several surgical options.

We should always start with the use of intratympanic aminoglycosides as the least aggressive option.

When intratympanic gentamicin does not work, there are three management strategies: proponents of endolymphatic sac surgery as the first surgical step, reserving revision surgery, or vestibular neurectomy for patients who continue to have vertigo.

Patients who have not yet achieved clinical improvement after the endolymphatic sac decompression operation, the otologist who has no experience or staff to subject the patient to vestibular neurectomy is faced with the option of performing surgical labyrinthectomy [10].

**9**

**Author details**

Fayez Bahmad Jr 1,2

São Paulo, SP, Brazil

1 University of Brasilia, Brasilia, DF, Brazil

provided the original work is properly cited.

\*Address all correspondence to: fayezbjr@gmail.com

2 Ophthalmology and Otolaryngology Faculty, University of São Paulo (FMUSP),

© 2020 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium,

*Introductory Chapter: Ménière's Disease (MD) DOI: http://dx.doi.org/10.5772/intechopen.91924* *Introductory Chapter: Ménière's Disease (MD) DOI: http://dx.doi.org/10.5772/intechopen.91924*

*Meniere's Disease*

This implies that 10% of patients continue to have clinically important episodes

The various surgical procedures advocated for Ménière's disease continue to raise considerable controversy among otolaryngologists. The decision to operate and the choice of procedure are often dictated by the understanding and experience of a particular technique and the surgeon's individual threshold for surgical intervention. Generally, surgical procedures for Ménière's disease are classified as destruc-

Endolymphatic sac surgery was first described in 1927 by Portmann, and no other aspect of Ménière's disease has elicited further debate or controversy. Just as the exact role of the endolymphatic sac in the development of hydrops is not yet known, the precise mechanism by which surgery works remains undefined. However, endolymphatic sac decompression surgery is still widely performed [10].

In the vestibular nerve section, no attempt is made to modify the underlying pathophysiology. The objective is to dissociate the offensive maze from the trunk,

The procedure is uniformly effective, with vertigo control in 90–95% of patients according to some series. However, it is a surgery with considerable risks inherent in

Labyrinth extirpation is indicated in patients with severe symptoms who have virtually useless hearing. Disturbance of the inner ear thus invariably leads to permanent anacusis. However, the ear on the opposite side may have subclinical hydrops, and we should be naturally concerned that the progress of the disease in the patient's contralateral ear may aggravate and make it bilaterally deaf. This is probably the reason for the widespread choice of nondestructive inner ear procedures [10].

Over the past decade, the hearing rehabilitation of certain profoundly deaf

Patients with severe bilateral Ménière's disease and severe to profound bilateral sensorineural deafness will end up with an indication for hearing rehabilitation with cochlear implant. Surgeons with patients with symptoms whose disease is

We should always start with the use of intratympanic aminoglycosides as the

When intratympanic gentamicin does not work, there are three management strategies: proponents of endolymphatic sac surgery as the first surgical step, reserving revision surgery, or vestibular neurectomy for patients who continue to

Patients who have not yet achieved clinical improvement after the endolymphatic sac decompression operation, the otologist who has no experience or staff to subject the patient to vestibular neurectomy is faced with the option of performing

of vertigo, and surgical treatment should be considered for them.

tive or nondestructive with regard to hearing [8–10].

**7.3 Endolymphatic sac surgery**

**7.4 Vestibular nerve section**

preserving the patient's hearing.

**7.5 Surgical labyrinthectomy**

**7.6 Cochlear implant**

least aggressive option.

surgical labyrinthectomy [10].

have vertigo.

any posterior fossa neurosurgical procedure [10].

people has been transformed by cochlear implants.

refractory to clinical treatment have several surgical options.

**8**

#### **Author details**

Fayez Bahmad Jr 1,2

1 University of Brasilia, Brasilia, DF, Brazil

2 Ophthalmology and Otolaryngology Faculty, University of São Paulo (FMUSP), São Paulo, SP, Brazil

\*Address all correspondence to: fayezbjr@gmail.com

© 2020 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

### **References**

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**11**

Section 2

Etiopathogenesis
