Latest revision as of 06:13, 5 November 2021

Wikidoc

Nasal polyp

Diagnosis

History and symtoms

Nasal polyps don't cause any symptoms and could be an incidental finding if it's smaller in size. Larger polyps or clusters cause many symptoms of obstruction like lack of smell or taste, stuffy nose, facial pain or headache, sneezing, and runny nose, postnasal dripping, itchiness around the eyes, and snoring.

Physical examination

Laboratory findings

Electrocardiogram

XRAY

CT

MRI

Ultrasound

Other Diagnostic studies

Pathophysiology

PMID: 8521175 Even though it is considered an idiopathic condition, it has been considered that frequent infections and inflammations could cause polyps originating from the lining of ethmoidal or maxillary sinuses. Histopathological study on the polyp shows epithelial damage, edema, fibrosis, hyperplastic or metaplastic changes of epithelium, and basement thickening.PMID: 8922142 These nasal polyps are also abundant in eosinophils, mast cells, T lymphocytes, cytokines, chemokines, interleukins, TNF-α, and adhesion molecules. Multiple studies have been performed to establish how genetic factors correlate with the development and persistence of mucosal inflammation that causes nasal polyps. Several genes and single nucleotide RYD5 polymorphism has been studied to be found to be a potential cause.PMID: 26204469 It has been postulated that subjects with HLA-DQA1*0201-DQB1*0201 haplotype expression increase the risk of having nasal polyp by 5.53 times.PMID: 10718431 Weakened mucosal innate immunity and impaired immune response also contribute to the pathogenesis of the disease.PMID: 18416964 A zinc metalloenzyme, Carbonic Anhydrase(CA) functions by transporting water and electrolyte across the epithelium. Hence, a downregulated expression of CA plays a role in edema in nasal polyps, and studies on the genes and variants that cause this reduced expression can help in with diagnosis and management of the disease. PMID: 18622311

Fungal particles usually reside in the nasal mucosa of healthy individuals. However, the same fungal particles can act as antigens in immunocompromised individuals. This antigen response leads to increased eosinophils and inflammatory markers. Superinfections stem from translocating germinating spores in contrast to fungus balls which are irritant to the neighboring cells. Most common species involved in nasal polyposis is Aspergillus and Alternaria."""PMID: 16210651"""

Biofilm:

Etiology

Although the etiology of the disease is unclear, it is a common disease affecting 4% of the population. It is often associated with many pathogenic conditions like aspirin sensitivity(36%), asthma(7%), cystic fibrosis(20%) and especially chronic rhinosinusitis. Other conditions with nasal polyps are Churg-Strauss Syndrome(eosinophilic granulomatosis with polyangiitis), allergic fungal sinusitis, and cilia dyskinetic syndrome, (Kartagener's),Young Syndrome and Nasal mastocytosis. Nasal polyps are found more commonly in non allergic asthma than in allergic asthma.

Cystic fibrosis (CF) is the most common cause of nasal polyps in children. Therefore, any child under 12 to 20 years old with nasal polyps should be tested for CF. Half of the people with CF will experience extensive polyps leading to nasal obstruction and requiring aggressive management.-- management

Differential Diagnosis

Most of the nasal masses might look like a nasal polyp. Hence,some of the differential diagnosis among benign masses include encephalocele, glioma,antrochoanal polyp, angiofibroma and inverted papilloma . While malignant masses include squamous cell carcinoma (SCC), esthesioneuroblastoma, nasal lymphoma and rhabdomyosarcoma .

Epidemology

Nasal polyposis is a common disease in the population and has been estimated to as 1-4%. Nasal polyposis occurs more in men compared to women. The prevalence of nasal polyps increases substantially between 40 to 60 years of age. Since the exact etiology of nasal polyp is unclear, studies have postulated multiple theories including genetic associations and increased incidence among asthmatic patients. Patients with Chronic rhinosinusitis(10%-54%) especially allergic fungal rhinusinusitis aspirin sensitivity (40%-80%) and cystic fibrosis (36%) found to have nasal polyp. Hence, children with nasal polyps should get a mandatory cystic fibrosis workup to rule it out.

Risk Factors

Risk factors for Nasal polyps
Asthma Allergies Cystic fibrosis Aspirin insensitivity Kartagener's syndrome Young's syndrome Churg strauss syndrome

Screening

Natural History,complications and prognosis

Classification

Primary classification

Ethmoidal Polyps: Mostly found in adults. These are small and grape-like masses, originating from ethmoidal sinuses and grows into the middle meatus.
Antrochoanal Polyp: They are common in children and present as a large solitary polyp. They originate from the maxillary sinus but grow backward to the posterior choana and hang down behind the soft palate.

Histopathological Classification of Nasal polyp PMID:8922142

Eosinophilic edematous(allergic): It is the most common nasal polyp which is characterized by edema, goblet cell hyperplasia of the epithelium, basement membrane thickening. They are abundant with eosinophils and leukocytes too.
Fibroinflammatory: These nasal polyps are a result of chronic inflammation and metaplasia.
Polyp with Hyperplasia seromucinous glands: Rare
Polyp with Atypical stroma: Rare

Phenotypical classification of Chronic Rhinosinusitis with Nasal polyp :

Eosinophilic
Neutrophilic
Non Eosinophilic Non-Neutrophilic

Otosclerosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: CSN, M.D.

Synonyms and keywords: Otospongiosis

Overview

Otosclerosis -https://commons.wikimedia.org/wiki/File:Gray919.png#/media/File:Gray919.png

Otosclerosis is a word derived from the Greek word "sklērós" meaning hardening and oto meaning ear. Otosclerosis is an osseous dyscrasia of the otic capsule, which should be an endochondral dense bony part in the labyrinth, replaced by irregularly laid spongy bone leading to the fixation of stapes. It is one of the most common causes of conductive deafness with normal tympanic membranes. Thereby affecting sound transmission to the inner ear at the level of the oval window Though majority of cases are observed at the oval window, otosclerosis can also affect the round window, cochlear apex, posterior to the oval window, posterior and anterior wall of the internal auditory canal (IAC), cochlear aqueduct, semicircular canals, and within the stapes footplate. It can be asymptomatic or in advanced cases extend into the bony labyrinth and affect cochlea which causes mixed conductive and sensorineural hearing loss depending on the position of foci. Otosclerosis is common in Caucasians and predominantly women. Hormonal fluctuations in women like during pregnancy and menopause may aggravate the disease. Many genetic factors contribute to the development of otosclerosis. And it is an autosomal dominant inheritance with incomplete penetrance. Measles was found to be one of the reasons for otosclerosis. Other factors include inflammatory cytokine and cytotoxic mediators secondary to inflammation. (CT) scanning of the temporal bone can often demonstrate foci of demineralization in the otic capsule in cases of cochlear otosclerosis. The treatment for hearing loss is essentially stapes surgery with successful improvement of 90%-95% in the first 5 years; decreases to 63% after 30years and rarely need revision surgery. Additionally, hearing aid and cochlear implants could be used along with surgery.

Historical perspective

1704:Antonio Maria Valsalva identified fixation of stapes as a cause of hearing loss
1841:Toyn bee stated, "osseous ankylosis of the stapes to the fenestra ovalis as one of the causes of deafness".
1876:Johannes Kessel described stapes surgery as the treatment.
1930-1950: Used Julius Lempert’s single-stage fenestration of stapes as treatment.
1956:John Shea modernized stapedectomy.

Classification

Based on Symons/fanning grading system

Grade 1: Fenestral Otosclerosis
Grade 2: Cochlear Otosclerosis
- Grade 2A:Basal cochlear turn Otosclerosis
- Grade 2B:Middle/Apical turn Otosclerosis
- Grade 2C:Both Basal and Middle/apical Otosclerosis
Grade 3:Diffuse confluent cochlear involvement (with or without fenestral involvement)

Embryology

Table explains the embryological process for the development of bony labyrinth

Gestational week	Developmental process
4th week	Development of otic canal from mesenchyme of otic vesicle by forming an otic capsule around it
8th week	Initiation of the cartilaginous framework
16th week	Endochondrial replacement of the cartilaginous framework.

In certain cases, completion of the third stage of bone formation doesn't occur leading to cartilages between bony structures. During labyrinth bone formation, the anterior to the oval window is usually the last area to develop. This area is the most common site for otosclerosis.

Pathophysiology

Accumulation of bone deposits because of increased bone remodeling which is bone resorption followed by bone deposition in the otic capsule result in otosclerosis. Audiological findings are directly proportionate to the extent of bone remodeling. Bone remodeling happens in 3 phases:

Phase	Mechanism of action
Otospongiosis	Increase in both osteoclast activity and microvascularity
Transitional phase	begins with deposits of spongy bone by osteoblasts in areas of previous bone reabsorption
Otosclerotic phase	characterized by spongy bone deposits developing into dense bone that narrows the microcirculation previously developed in the otospongiosis phase

The lesions occur mostly in the anterior to the oval window by calcifying of annular ligament or by involving stapes footplate (80%). While 30% of cases have the lesion at the round window,21% have it at pericochlear region, and 19% at the anterior segment of the internal auditory canal.

Etiology

Though the exact cause of otosclerosis is unknown and multiple research studies are still ongoing, several etiologies are the following:

Embryological Cause: During the maturation of the otic capsule of the labyrinth, certain places skip the complete conversion to endochondral, leading to irregularly laid spongy bone. This most commonly is seen at fissula ante fenestram.

Genetic Cause: Studies have been conducted and found Type 1 collagen gene(COL1A1), a component essential for bone metabolism plays a role in otosclerosis. TGF-beta 1(BMP 2 and BMP 4 gene and Angiotensin II (AGT M235T and ACE I/D genes) are also found associated with otosclerosis. Other genetic causes for otosclerosis include sex hormones, autoimmune reaction, human leucocyte antigen, inflammatory and regulatory cytokines, parathyroid hormone, and expression of parathyroid hormone-related peptides receptors, and oxidative stress
Gender: Women are more likely to have otosclerosis than men , suggesting the role of sex hormones.
Ethinicity: Otosclerosis is more common in Caucasians and Japanese compared to African populations.
Age: Even though otosclerosis could be seen in patients at their twenties and thirties, it usually causes hearing loss only in or after their forties.
Pregnancy: There is no strong evidence why pregnancy worsens hearing loss in some patients with otosclerosis.However 33% women with otosclerosis are found to deteriote in hering after one pregnancy.
Measles: RNA of measles virus was detected at the footplate of stapes in otosclerotic patients , suggesting its role in casuing it, while incidence has reduced significantly in vaccinated populations.

Trauma and Major surgeries also cause otosclerosis.

Autoimmune: Though research on this is in the initial stages, inflammatory cytokine like TNF-alpha and cytotoxic mediators were found at the otosclerotic bone suggesting its role in the pathology.

Differentiating otosclerosis from other diseases

Conditions mimicking Otosclerosis
Serous otitis media Adhesive otitis media Congenital stapes fixation Meniere disease Tympanosclerosis Attic fixation of head of the malleus Ossicular discontinuity Paget's disease

Epidemiology and demographics

Though a variable pattern of inheritance is observed, 60% of patients report a family with a history of otosclerosis. Thus researchers conclude this condition as an autosomal dominant with incomplete penetrance.
Greater preponderance in women compared to men in a ratio of 2:1.
Even though the disease begins in the second and third decade of life, it doesn't result in hearing loss until the fourth decade.
Clinical prevalence of otosclerosis is found to be higher in Caucasians by 0.04%-1% while the histological incidence of otosclerosis increases to 10% in the same..The incidence of histological otosclerosis is 1% and 5% in African and Asian population respectively.

Risk Factors

Genetic factor: Otosclerosis is an autosomal dominant disease with incomplete penetrance. When 60% of patients were found with family history, 40-50% of patients have it with variable patterns of inheritance. But hearing loss in otosclerosis is found only with family history of the same.^[1]
Hormonal conditions: Puberty, pregnancy, and menopause increase the occurrence of hearing loss in pre-existing otosclerosis.
Measles: Persistent measles virus infection of stapes footplate results in activated osteoclast and inflammatory pathways by TNF-alpha mRNA. The protective function of osteoprotegerin at the otic capsule is inhibited by TNF-alpha and its action on RANK production

Screening

Even though otosclerosis appears to be genetic deafness, early prevention can help in combating the disorder and its prevention.

Systematic Impedance-audiometry screening: The purpose of this screening is to detect a possible diphasic impedance change (on-off effect) by eliciting stapedius reflex. It is done in school going-children and helps in the early detection of stapedial fixation.
Systematic Bone-conducted audiometry screening: It is done in young children with a family history of otosclerosis.

Natural History, complications and Prognosis

Prognosis

Most of the patients find improvement in their hearing ability after the surgery. Only a few don't have any change post-surgery and rarely hearing loss worsens. There have been cases where the prosthesis was found displaced from its original position, causing conductive hearing loss again in some patients. The second surgery was observed to be less successful than the first.

Complications

Post-op complications:

Perilymph fistula (3-10%): Progressive hearing loss, tinnitus, or vertigo.
Taste disturbance(9%)
Facial nerve injury (< 1%)
Sensorineural Hearing loss(< 1% chance)
Poststapedectomy granulomas
Vertigo: Usually mild, subsides over time.

Clinical presentaion

Patients present with the following:

Most cases are asymptomatic and diagnosed as an incidental finding in temporal autopsies.
Bilateral(80%) or unilateral involvement earlier in the disease.
Progressive hearing loss, which might worsen with lower tone and frequencies like male voices and vowels.
vertigo(10%)
tinnitis (50%)
Schwartz sign or flemingo's pink sign(not necessary for diagnosis): redness along the promontory of tymphanic membrane on otoscopic examination

Diagnosis

Physical Examintaion

Tuning fork

Pros of using a tuning fork for diagnosis:
- Negative Rinne test
- Weber test lateralizes to the ear with severe conductive loss.
Cons of using tuning fork for diagnosis
- Rinne test cannot differentiate between sensorineural hearing loss and normal hearing loss.
- Weber test's inability to differentiate a bilateral hearing loss.

Whisper test

Audiometry and tympanometry

Caharts notch: Bone conduction depression in threshold at 2000Hz. After stapedectomy caharts notch diappears.
Tympanometry normal at early disease. In severe cases, flattening and stiffening of curve representing low complaince of ossicular chain and tympanic membrane. Good test to differentiate between otosclerosis and pathologies with low resonance.

CT Imaging

Otosclerosis fenestral - Case courtesy of Assoc Prof Frank Gaillard, Radiopaedia.org, rID: 5568

High resolution computed tomography (CT) of the temporal bones is the imaging technique of choice in the diagnosis of otosclerosis.
However, preoperative CT has little to add in establishing otosclerosis diagnosis and may not even be necessary to confirm diagnosis.
CT is recommended to be reserved for those patients with presumed additional abnormalities, for specific preoperative planning, or for legal necessity.
CT Finding: increased bony radiolucency in the otic capsule around the anterior footplate, thickening of the stapes, and widening of the oval window.
Early sign on CT: halo sign; demineralized area outlining the cochlea.

Treatment

Medical Management
- Sodium flouride: Though controversial, it is used to slow the progression of otosclerosis.
- Bisphosphonates: By inducing osteoclastic apoptosis, bisphosphates cause antiresorptive action, with promising results.
Hearing aid:It is used alone or in combination with other treatments.
Surgical Management:Stapedotomy or Stapedectomy along with placement of a prosthesis, has shown a good prognosis with improved hearing ability. Only rare cases have shown no benefit. Revision surgery is controversial but proposed for patients with no improvement of hearing loss, facial nerve damage, or persistent vertigo.Most common cause of stapedectomy failure is prosthesis displacement with or without incus erosion. Revision surgery is also recommended post surgery for patients with footplate fixation, perilymph fistula, otosclerotic regrowth. Post surgery, some are still encouraged to use a hearing aid..

Prevention

Otosclerosis cannot be prevented but early detection of the disease can help in combatting the disease better.

References

↑ Rudic M, Keogh I, Wagner R, Wilkinson E, Kiros N, Ferrary E; et al. (2015). "The pathophysiology of otosclerosis: Review of current research". Hear Res. 330 (Pt A): 51–6. doi:10.1016/j.heares.2015.07.014. PMID 26276418.

[pmid26276418-1] Rudic M, Keogh I, Wagner R, Wilkinson E, Kiros N, Ferrary E; et al. (2015). "The pathophysiology of otosclerosis: Review of current research". Hear Res. 330 (Pt A): 51–6. doi:10.1016/j.heares.2015.07.014. PMID 26276418.

[1]

@@ Line 1: / Line 1: @@
 Wikidoc
+=Nasal polyp=
+==Diagnosis==
+===History and symtoms===
+Nasal polyps don't cause any symptoms and could be an incidental finding if it's smaller in size. Larger polyps or clusters cause many symptoms of obstruction like lack of smell or taste, stuffy nose, facial pain or headache, sneezing, and runny nose, postnasal dripping, itchiness around the eyes, and snoring.
+===Physical examination===
+===Laboratory findings===
+===Electrocardiogram===
+===XRAY===
+===CT===
+===MRI===
+===Ultrasound===
+===Other Diagnostic studies===
+==Pathophysiology==
+PMID: 8521175
+Even though it is considered an idiopathic condition, it has been considered that frequent infections and inflammations could cause polyps originating from the lining of ethmoidal or maxillary sinuses. Histopathological study on the polyp shows epithelial damage, edema, fibrosis, hyperplastic or metaplastic changes of epithelium, and basement thickening.'''PMID: 8922142'''
+These nasal polyps are also abundant in eosinophils, mast cells, T lymphocytes, cytokines, chemokines, interleukins, TNF-α, and adhesion molecules. Multiple studies have been performed to establish how genetic factors correlate with the development and persistence of mucosal inflammation that causes nasal polyps. Several genes and single nucleotide RYD5 polymorphism has been studied to be found to be a potential cause.'''PMID: 26204469'''
+It has been postulated that subjects with HLA-DQA1*0201-DQB1*0201 haplotype expression increase the risk of having nasal polyp by 5.53 times.'''PMID: 10718431'''
+Weakened mucosal innate immunity and impaired immune response also contribute to the pathogenesis of the disease.'''PMID: 18416964'''
+A zinc metalloenzyme, Carbonic Anhydrase(CA) functions by transporting water and electrolyte across the epithelium. Hence, a downregulated expression of CA plays a role in edema in nasal polyps, and studies on the genes and variants that cause this reduced expression can help in with diagnosis and management of the disease. '''PMID: 18622311'''
+Fungal particles usually reside in the nasal mucosa of healthy individuals. However, the same fungal particles can act as antigens in immunocompromised individuals. This antigen response leads to increased eosinophils and inflammatory markers. Superinfections stem from translocating germinating spores in contrast to fungus balls which are irritant to the neighboring cells. Most common species involved in nasal polyposis is Aspergillus and Alternaria."""PMID: 16210651"""
+Biofilm:
+==Etiology==
+Although the etiology of the disease is unclear, it is a common disease affecting 4% of the population. It is often associated with many pathogenic conditions like aspirin sensitivity(36%), asthma(7%), cystic fibrosis(20%) and especially chronic rhinosinusitis. Other conditions with nasal polyps are Churg-Strauss Syndrome(eosinophilic granulomatosis with polyangiitis), allergic fungal sinusitis, and cilia dyskinetic syndrome, (Kartagener's),Young Syndrome and Nasal mastocytosis. Nasal polyps are found more commonly in non allergic asthma than in allergic asthma.
+''Cystic fibrosis (CF) is the most common cause of nasal polyps in children. Therefore, any child under 12 to 20 years old with nasal polyps should be tested for CF. Half of the people with CF will experience extensive polyps leading to nasal obstruction and requiring aggressive management.''-- management
+==Differential Diagnosis==
+Most of the nasal masses might look like a nasal polyp. Hence,some of the differential diagnosis among benign masses include encephalocele, glioma,antrochoanal polyp, angiofibroma and inverted papilloma . While
+malignant masses include squamous cell carcinoma (SCC), esthesioneuroblastoma, nasal lymphoma and rhabdomyosarcoma .
+==Epidemology==
+Nasal polyposis is a common disease in the population and has been estimated to as 1-4%. Nasal polyposis occurs more in men compared to women. The prevalence of nasal polyps increases substantially between 40 to 60 years of age. Since the exact etiology of nasal polyp is unclear, studies have postulated multiple theories including genetic associations and increased incidence among asthmatic patients. Patients with Chronic rhinosinusitis(10%-54%) especially allergic fungal rhinusinusitis  aspirin sensitivity (40%-80%) and cystic fibrosis (36%) found to have nasal polyp. Hence, children with nasal polyps should get a mandatory cystic fibrosis workup to rule it out.
+==Risk Factors==
+{| class="wikitable" style="text-align:center"
+|-
+! style="background:#4479BA; color: #FFFFFF;" |Risk factors for Nasal polyps
+|-
+|
+*Asthma
+*Allergies
+*Cystic fibrosis
+*Aspirin insensitivity
+*Kartagener's syndrome
+*Young's syndrome
+*Churg strauss syndrome
+|}
+==Screening==
+==Natural History,complications and prognosis==
+=Classification=
+*<u>Primary classification</u>
+#Ethmoidal Polyps: Mostly found in adults. These are small and grape-like masses, originating from ethmoidal sinuses and grows into the middle meatus.
+#Antrochoanal Polyp: They are common in children and present as a large solitary polyp. They originate from the maxillary sinus but grow backward to the posterior choana and hang down behind the soft palate.
+*<u>Histopathological Classification of Nasal polyp</u> '''PMID:8922142'''
+#Eosinophilic edematous(allergic): It is the most common nasal polyp which is characterized by edema, goblet cell hyperplasia of the epithelium, basement membrane thickening. They are abundant with eosinophils and leukocytes too.
+#Fibroinflammatory: These nasal polyps are a result of chronic inflammation and metaplasia.
+#Polyp with Hyperplasia seromucinous glands: Rare
+#Polyp with Atypical stroma: Rare
+*<u>Phenotypical classification of Chronic Rhinosinusitis with Nasal polyp  :</u>
+#Eosinophilic
+#Neutrophilic
+#Non Eosinophilic Non-Neutrophilic
-__NOTOC__
 =Otosclerosis=
-{{CMG}} {{CSN}}
+{{CMG}}; {{AE}} {{CSN}}
-[[User:Christina Ninan|Christina Ninan, M.D.]]
@@ Line 12: / Line 91: @@
 ==Overview==
 [[Image:Gray919.png|thumb|right|500px|Otosclerosis -https://commons.wikimedia.org/wiki/File:Gray919.png#/media/File:Gray919.png]]
-Otosclerosis is a word derived from the Greek word "sklērós" meaning hardening and oto meaning ear. Otosclerosis is an osseous dyscrasia of the otic capsule, which should be an endochondral dense bony part in the labyrinth, replaced by irregularly laid spongy bone leading to the fixation of stapes. It is one of the most common causes of conductive deafness with normal tympanic membranes. Thereby affecting sound transmission to the inner ear at the level of the oval window <ref name="pmid32499972">{{cite journal| author=Rajput MSA, Arain AA, Rajput AA, Adeel M, Suahil A, Awan MS| title=Otosclerosis: Experience With Stapes Surgery. | journal=Cureus | year= 2020 | volume= 12 | issue= 5 | pages= e7927 | pmid=32499972 | doi=10.7759/cureus.7927 | pmc=7265776 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32499972  }} </ref> Though majority of cases are observed at the oval window, otosclerosis can also affect the round window, cochlear apex, posterior to the oval window, posterior and anterior wall of the internal auditory canal (IAC), cochlear aqueduct, semicircular canals, and within the stapes footplate. It can be asymptomatic or in advanced cases extend into the bony labyrinth and affect cochlea which causes mixed conductive and sensorineural hearing loss depending on the position of foci. Otosclerosis is common in Caucasians and predominantly women. Hormonal fluctuations in women like during pregnancy and menopause may aggravate the disease. Many genetic factors contribute to the development of otosclerosis. And it is an autosomal dominant inheritance with incomplete penetrance. Measles was found to be one of the reasons for otosclerosis. Other factors include infammatory cytokine and cytotxic mediators secondary to inflammation.<ref name="pmid26276418">{{cite journal| author=Rudic M, Keogh I, Wagner R, Wilkinson E, Kiros N, Ferrary E | display-authors=etal| title=The pathophysiology of otosclerosis: Review of current research. | journal=Hear Res | year= 2015 | volume= 330 | issue= Pt A | pages= 51-6 | pmid=26276418 | doi=10.1016/j.heares.2015.07.014 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26276418  }} </ref>
+Otosclerosis is a word derived from the Greek word "sklērós" meaning hardening and oto meaning ear. Otosclerosis is an osseous dyscrasia of the otic capsule, which should be an endochondral dense bony part in the labyrinth, replaced by irregularly laid spongy bone leading to the fixation of stapes. It is one of the most common causes of conductive deafness with normal tympanic membranes. Thereby affecting sound transmission to the inner ear at the level of the oval window  Though majority of cases are observed at the oval window, otosclerosis can also affect the round window, cochlear apex, posterior to the oval window, posterior and anterior wall of the internal auditory canal (IAC), cochlear aqueduct, semicircular canals, and within the stapes footplate. It can be asymptomatic or in advanced cases extend into the bony labyrinth and affect cochlea which causes mixed conductive and sensorineural hearing loss depending on the position of foci. Otosclerosis is common in Caucasians and predominantly women. Hormonal fluctuations in women like during pregnancy and menopause may aggravate the disease. Many genetic factors contribute to the development of otosclerosis. And it is an autosomal dominant inheritance with incomplete penetrance. Measles was found to be one of the reasons for otosclerosis. Other factors include inflammatory cytokine and cytotoxic mediators secondary to inflammation.
 (CT) scanning of the temporal bone can often demonstrate foci of demineralization in the otic capsule in cases of cochlear otosclerosis. The treatment for hearing loss is essentially stapes surgery with successful improvement of 90%-95% in the first 5 years; decreases to 63% after 30years and rarely need revision surgery. Additionally, hearing aid and cochlear implants could be used along with surgery.
 ==Historical perspective==
-*1704:Antonio Maria Valsalva identified fixation of  stapes as a cause of hearing loss<ref name="pmid20195188">{{cite journal| author=Makarem AO, Hoang TA, Lo WW, Linthicum FH, Fayad JN| title=Cavitating otosclerosis: clinical, radiologic, and histopathologic correlations. | journal=Otol Neurotol | year= 2010 | volume= 31 | issue= 3 | pages= 381-4 | pmid=20195188 | doi=10.1097/MAO.0b013e3181d275e8 | pmc=2880664 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20195188  }} </ref>
-*1841:Toyn bee stated, "osseous ankylosis of the stapes to the fenestra ovalis as one of the causes of deafness".<ref name="pmid29502722">{{cite journal| author=Nazarian R, McElveen JT, Eshraghi AA| title=History of Otosclerosis and Stapes Surgery. | journal=Otolaryngol Clin North Am | year= 2018 | volume= 51 | issue= 2 | pages= 275-290 | pmid=29502722 | doi=10.1016/j.otc.2017.11.003 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29502722  }} </ref>
+*1704:Antonio Maria Valsalva identified fixation of  stapes as a cause of hearing loss
-*1876:Johannes Kessel described stapes surgery as the treatment.<ref name="pmid18652164">{{cite journal| author=Wielgosz R, Mroczkowski E| title=[History of endaural surgery]. | journal=Otolaryngol Pol | year= 2008 | volume= 62 | issue= 3 | pages= 348-50 | pmid=18652164 | doi=10.1016/S0030-6657(08)70269-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18652164  }} </ref>
+*1841:Toyn bee stated, "osseous ankylosis of the stapes to the fenestra ovalis as one of the causes of deafness".
-*1930-1950: Used Julius Lempert’s single-stage fenestration of stapes as treatment.<ref name="pmid9760779">{{cite journal| author=Pietruski J| title=[Juliusz Lempert (1890-1959): the author of the fenestration technique]. | journal=Otolaryngol Pol | year= 1998 | volume= 52 | issue= 3 | pages= 341-6 | pmid=9760779 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9760779  }} </ref>
+*1876:Johannes Kessel described stapes surgery as the treatment.
-*1956:John Shea modernized stapedectomy.<ref name="pmid29397948">{{cite journal| author=Cheng HCS, Agrawal SK, Parnes LS| title=Stapedectomy Versus Stapedotomy. | journal=Otolaryngol Clin North Am | year= 2018 | volume= 51 | issue= 2 | pages= 375-392 | pmid=29397948 | doi=10.1016/j.otc.2017.11.008 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29397948  }} </ref>
+*1930-1950: Used Julius Lempert’s single-stage fenestration of stapes as treatment.
+*1956:John Shea modernized stapedectomy.
 ==Classification==
-;Based on Symons/fanning grading system<ref name="pmid19321627">{{cite journal| author=Lee TC, Aviv RI, Chen JM, Nedzelski JM, Fox AJ, Symons SP| title=CT grading of otosclerosis. | journal=AJNR Am J Neuroradiol | year= 2009 | volume= 30 | issue= 7 | pages= 1435-9 | pmid=19321627 | doi=10.3174/ajnr.A1558 | pmc=7051554 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19321627  }} </ref>
+;Based on Symons/fanning grading system
 *'''Grade 1''': Fenestral Otosclerosis
 *'''Grade 2''': Cochlear Otosclerosis
@@ Line 39: / Line 121: @@
 |-
-! style="background:#4479BA; color: #FFFFFF;" | Gestational week
+! style="background:#4479BA; color: #FFFFFF;" |Gestational week
-! style="background:#4479BA; color: #FFFFFF;" | Developmental process
+! style="background:#4479BA; color: #FFFFFF;" |Developmental process
 |-
-| style="background:#DCDCDC;" align="center" | 4th week
+| style="background:#DCDCDC;" align="center" |4th week
 |
@@ Line 53: / Line 135: @@
 | style="background:#DCDCDC;" align="center" |8th week
 |
 *Initiation of the cartilaginous framework
 |-
-| style="background:#DCDCDC;" align="center" | 16th week
+| style="background:#DCDCDC;" align="center" |16th week
 |
 *Endochondrial replacement of the cartilaginous framework.
 |}
@@ Line 66: / Line 148: @@
 ==Pathophysiology==
-Accumulation of bone deposits because of increased bone remodeling which is bone resorption followed by bone deposition in the otic capsule result in otosclerosis. Audiological findings are directly proportionate to the extent of bone remodeling.<ref name="pmid30975972">{{cite journal| author=Wiatr A, Składzień J, Świeży K, Wiatr M| title=A Biochemical Analysis of the Stapes. | journal=Med Sci Monit | year= 2019 | volume= 25 | issue=  | pages= 2679-2686 | pmid=30975972 | doi=10.12659/MSM.913635 | pmc=6475125 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30975972  }} </ref>
+Accumulation of bone deposits because of increased bone remodeling which is bone resorption followed by bone deposition in the otic capsule result in otosclerosis. Audiological findings are directly proportionate to the extent of bone remodeling.
 Bone remodeling happens  in 3 phases:
 {| class="wikitable" style="text-align:center"
@@ Line 72: / Line 154: @@
 ! style="background:#4479BA; color: #FFFFFF;" |Phase!! style="background:#4479BA; color: #FFFFFF;" |Mechanism of action
 |-
-| style="background:#DCDCDC;" align="center" | Otospongiosis||  Increase in both osteoclast activity and microvascularity
+| style="background:#DCDCDC;" align="center" |Otospongiosis||Increase in both osteoclast activity and microvascularity
 |-
-| style="background:#DCDCDC;" align="center" |Transitional phase||  begins with deposits of spongy bone by osteoblasts in areas of previous bone reabsorption
+| style="background:#DCDCDC;" align="center" |Transitional phase||begins with deposits of spongy bone by osteoblasts in areas of previous bone reabsorption
 |-
-| style="background:#DCDCDC;" align="center" |Otosclerotic phase|| characterized by spongy bone deposits developing into dense bone that narrows the microcirculation previously developed in the otospongiosis phase
+| style="background:#DCDCDC;" align="center" |Otosclerotic phase||characterized by spongy bone deposits developing into dense bone that narrows the microcirculation previously developed in the otospongiosis phase
 |}
-The lesions occur mostly in the anterior to the oval window by calcifying of annular ligament or by involving stapes footplate (80%). While 30% of cases have the lesion at the round window,21% have it at pericochlear region, and 19% at the anterior segment of the internal auditory canal.<ref name="pmid17245019">{{cite journal| author=Arnold W| title=Some remarks on the histopathology of otosclerosis. | journal=Adv Otorhinolaryngol | year= 2007 | volume= 65 | issue=  | pages= 25-30 | pmid=17245019 | doi=10.1159/000098665 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17245019  }} </ref>
+The lesions occur mostly in the anterior to the oval window by calcifying of annular ligament or by involving stapes footplate (80%). While 30% of cases have the lesion at the round window,21% have it at pericochlear region, and 19% at the anterior segment of the internal auditory canal.
 ==Etiology==
 Though the exact cause of otosclerosis is unknown and multiple research studies are still ongoing, several etiologies are the following:
-;Embryological Cause: During the maturation of the otic capsule of the labyrinth, certain places skip the complete conversion to endochondral, leading to irregularly laid spongy bone. This most commonly is seen at fissula ante fenestram.<ref name="pmid26276418">{{cite journal| author=Rudic M, Keogh I, Wagner R, Wilkinson E, Kiros N, Ferrary E | display-authors=etal| title=The pathophysiology of otosclerosis: Review of current research. | journal=Hear Res | year= 2015 | volume= 330 | issue= Pt A | pages= 51-6 | pmid=26276418 | doi=10.1016/j.heares.2015.07.014 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26276418  }} </ref>
+;Embryological Cause:During the maturation of the otic capsule of the labyrinth, certain places skip the complete conversion to endochondral, leading to irregularly laid spongy bone. This most commonly is seen at fissula ante fenestram.
+;Genetic Cause:Studies have been conducted and found Type 1 collagen gene(COL1A1), a component essential for bone metabolism plays a role in otosclerosis. TGF-beta 1(BMP 2 and BMP 4 gene and Angiotensin II (AGT M235T and ACE I/D genes) are also found associated with otosclerosis. Other genetic causes for otosclerosis include sex hormones, autoimmune reaction, human leucocyte antigen, inflammatory and regulatory cytokines, parathyroid hormone, and expression of parathyroid hormone-related peptides receptors, and oxidative stress
+;Gender:Women are more likely to have otosclerosis than men , suggesting the role of sex hormones.
+;Ethinicity:Otosclerosis is more common in Caucasians and Japanese compared to African populations.
+;Age:Even though otosclerosis could be seen in patients at their twenties and thirties, it usually causes hearing loss only in or after their forties.
+;Pregnancy:There is no strong evidence why pregnancy worsens hearing loss in some patients with otosclerosis.However 33% women with otosclerosis are found to deteriote in hering after one pregnancy.
+;Measles:RNA of measles virus was detected at the footplate of stapes in otosclerotic patients , suggesting its role in casuing it, while incidence has reduced significantly in vaccinated populations.
-;Genetic Cause:Studies have been conducted and found Type 1 collagen gene(COL1A1), a component essential for bone metabolism plays a role in otosclerosis. TGF-beta 1(BMP 2 and BMP 4 gene and Angiotensin II (AGT M235T and ACE I/D genes) are also found associated with otosclerosis. Other genetic causes for otosclerosis include sex hormones, autoimmune reaction, human leucocyte antigen, inflammatory and regulatory cytokines, parathyroid hormone, and expression of parathyroid hormone-related peptides receptors, and oxidative stress<ref name="pmid24170657">{{cite journal| author=Bittermann AJ, Wegner I, Noordman BJ, Vincent R, van der Heijden GJ, Grolman W| title=An introduction of genetics in otosclerosis: a systematic review. | journal=Otolaryngol Head Neck Surg | year= 2014 | volume= 150 | issue= 1 | pages= 34-9 | pmid=24170657 | doi=10.1177/0194599813509951 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24170657  }} </ref><ref name="pmid11170898">{{cite journal| author=Van Den Bogaert K, Govaerts PJ, Schatteman I, Brown MR, Caethoven G, Offeciers FE | display-authors=etal| title=A second gene for otosclerosis, OTSC2, maps to chromosome 7q34-36. | journal=Am J Hum Genet | year= 2001 | volume= 68 | issue= 2 | pages= 495-500 | pmid=11170898 | doi=10.1086/318185 | pmc=1235283 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11170898  }} </ref><ref name="pmid17213839">{{cite journal| author=Thys M, Van Den Bogaert K, Iliadou V, Vanderstraeten K, Dieltjens N, Schrauwen I | display-authors=etal| title=A seventh locus for otosclerosis, OTSC7, maps to chromosome 6q13-16.1. | journal=Eur J Hum Genet | year= 2007 | volume= 15 | issue= 3 | pages= 362-8 | pmid=17213839 | doi=10.1038/sj.ejhg.5201761 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17213839  }} </ref><ref name="pmid16885748">{{cite journal| author=Karosi T, Jókay I, Kónya J, Szabó LZ, Pytel J, Jóri J | display-authors=etal| title=Detection of osteoprotegerin and TNF-alpha mRNA in ankylotic Stapes footplates in connection with measles virus positivity. | journal=Laryngoscope | year= 2006 | volume= 116 | issue= 8 | pages= 1427-33 | pmid=16885748 | doi=10.1097/01.mlg.0000225928.35838.e5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16885748  }} </ref>
-;Gender: Women are more likely to have otosclerosis than men , suggesting the role of sex hormones.<ref name="pmid11240433">{{cite journal| author=Ben Arab S, Besbes G, Hachicha S| title=[Otosclerosis in populations living in northern Tunisia: epidemiology and etiology]. | journal=Ann Otolaryngol Chir Cervicofac | year= 2001 | volume= 118 | issue= 1 | pages= 19-25 | pmid=11240433 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11240433  }} </ref>
-;Ethinicity: Otosclerosis is more common in Caucasians and Japanese compared to African populations.<ref name="pmid14172803">{{cite journal| author=JOSEPH RB, FRAZER JP| title=OTOSCLEROSIS INCIDENCE IN CAUCASIANS AND JAPANESE. | journal=Arch Otolaryngol | year= 1964 | volume= 80 | issue=  | pages= 256-62 | pmid=14172803 | doi=10.1001/archotol.1964.00750040266004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14172803  }} </ref>
-;Age: Even though otosclerosis could be seen in patients at their twenties and thirties, it usually causes hearing loss only in or after their forties.
-;Pregnancy: There is no strong evidence why pregnancy worsens hearing loss in some patients with otosclerosis.However 33% women with otosclerosis are found to deteriote in hering after one pregnancy.<ref name="pmid6883784">{{cite journal| author=Gristwood RE, Venables WN| title=Pregnancy and otosclerosis. | journal=Clin Otolaryngol Allied Sci | year= 1983 | volume= 8 | issue= 3 | pages= 205-10 | pmid=6883784 | doi=10.1111/j.1365-2273.1983.tb01428.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6883784  }} </ref>
-;Measles: RNA of measles virus was detected at the footplate of stapes in otosclerotic patients , suggesting its role in casuing it, while incidence has reduced significantly in vaccinated populations.<ref name="pmid18235207">{{cite journal| author=Niedermeyer HP, Arnold W| title=Otosclerosis and measles virus - association or causation? | journal=ORL J Otorhinolaryngol Relat Spec | year= 2008 | volume= 70 | issue= 1 | pages= 63-9; discussion 69-70 | pmid=18235207 | doi=10.1159/000111049 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18235207  }} </ref>
 '''Trauma and Major surgeries''' also cause otosclerosis.
-;Autoimmune: Though research on this  is in the initial stages, inflammatory cytokine like TNF-alpha and cytotoxic mediators were found at the otosclerotic bone suggesting its role in the pathology.<ref name="pmid26276418">{{cite journal| author=Rudic M, Keogh I, Wagner R, Wilkinson E, Kiros N, Ferrary E | display-authors=etal| title=The pathophysiology of otosclerosis: Review of current research. | journal=Hear Res | year= 2015 | volume= 330 | issue= Pt A | pages= 51-6 | pmid=26276418 | doi=10.1016/j.heares.2015.07.014 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26276418  }} </ref>
+;Autoimmune:Though research on this  is in the initial stages, inflammatory cytokine like TNF-alpha and cytotoxic mediators were found at the otosclerotic bone suggesting its role in the pathology.
 ==[[Differentiating otosclerosis from other diseases|Differentiating otosclerosis from other diseases]]==
 {| class="wikitable" style="text-align:center"
 |-
-! style="background:#4479BA; color: #FFFFFF;" | Conditions mimicking Otosclerosis
+! style="background:#4479BA; color: #FFFFFF;" |Conditions mimicking Otosclerosis
 |-
 |
 *Serous otitis media
 *Adhesive otitis media
@@ Line 112: / Line 196: @@
 ==Epidemiology and demographics==
 *Though a variable pattern of inheritance is observed, 60% of patients report a family with a history of otosclerosis. Thus researchers conclude this condition as an autosomal dominant with incomplete penetrance.
-*Greater preponderance in women compared to men in a ratio of 2:1.<ref name="pmid30540696">{{cite journal| author=Crompton M, Cadge BA, Ziff JL, Mowat AJ, Nash R, Lavy JA | display-authors=etal| title=The Epidemiology of Otosclerosis in a British Cohort. | journal=Otol Neurotol | year= 2019 | volume= 40 | issue= 1 | pages= 22-30 | pmid=30540696 | doi=10.1097/MAO.0000000000002047 | pmc=6314447 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30540696  }} </ref>
+*Greater preponderance in women compared to men in a ratio of 2:1.
-*Even though the disease begins in the second and third decade of life, it doesn't result in hearing loss until the fourth decade.<ref name="pmid3883726">{{cite journal| author=Fitzgerald DC| title=The aging ear. | journal=Am Fam Physician | year= 1985 | volume= 31 | issue= 2 | pages= 225-32 | pmid=3883726 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3883726  }} </ref>
+*Even though the disease begins in the second and third decade of life, it doesn't result in hearing loss until the fourth decade.
-*Clinical prevalence of otosclerosis is found to be higher in Caucasians by 0.04%-1% while the histological incidence of otosclerosis increases to 10% in the same.<ref name="pmid17245017">{{cite journal| author=Declau F, Spaendonck MV, Timmermans JP, Michaels L, Liang J, Qiu JP | display-authors=etal| title=Prevalence of histologic otosclerosis: an unbiased temporal bone study in Caucasians. | journal=Adv Otorhinolaryngol | year= 2007 | volume= 65 | issue=  | pages= 6-16 | pmid=17245017 | doi=10.1159/000098663 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17245017  }} </ref>.The incidence  of histological otosclerosis is 1% and 5% in African and Asian population respectively.<ref name="pmid6074235">{{cite journal| author=Tato JM, Tato JM| title=Otosclerosis and races. | journal=Ann Otol Rhinol Laryngol | year= 1967 | volume= 76 | issue= 5 | pages= 1018-25 | pmid=6074235 | doi=10.1177/000348946707600512 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6074235  }} </ref>
+*Clinical prevalence of otosclerosis is found to be higher in Caucasians by 0.04%-1% while the histological incidence of otosclerosis increases to 10% in the same..The incidence  of histological otosclerosis is 1% and 5% in African and Asian population respectively.
 ==Risk Factors==
-;Genetic factor: Otosclerosis is an autosomal dominant disease with incomplete penetrance. When 60% of patients were found with family history, 40-50% of patients have it with variable patterns of inheritance. But hearing loss in otosclerosis is found only with family history of the same.<ref name="pmid26276418">{{cite journal| author=Rudic M, Keogh I, Wagner R, Wilkinson E, Kiros N, Ferrary E | display-authors=etal| title=The pathophysiology of otosclerosis: Review of current research. | journal=Hear Res | year= 2015 | volume= 330 | issue= Pt A | pages= 51-6 | pmid=26276418 | doi=10.1016/j.heares.2015.07.014 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26276418  }} </ref>
-;Hormonal conditions:  Puberty, pregnancy, and menopause increase the occurrence of hearing loss in pre-existing otosclerosis.<ref name="pmid15513522">{{cite journal| author=Imauchi Y, Lainé P, Sterkers O, Ferrary E, Bozorg Grayeli A| title=Effect of 17 beta-estradiol on diastrophic dysplasia sulfate transporter activity in otosclerotic bone cell cultures and SaOS-2 cells. | journal=Acta Otolaryngol | year= 2004 | volume= 124 | issue= 8 | pages= 890-5 | pmid=15513522 | doi=10.1080/00016480310017081 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15513522  }} </ref>
+;Genetic factor:Otosclerosis is an autosomal dominant disease with incomplete penetrance. When 60% of patients were found with family history, 40-50% of patients have it with variable patterns of inheritance. But hearing loss in otosclerosis is found only with family history of the same.<ref name="pmid26276418">{{cite journal| author=Rudic M, Keogh I, Wagner R, Wilkinson E, Kiros N, Ferrary E | display-authors=etal| title=The pathophysiology of otosclerosis: Review of current research. | journal=Hear Res | year= 2015 | volume= 330 | issue= Pt A | pages= 51-6 | pmid=26276418 | doi=10.1016/j.heares.2015.07.014 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26276418  }} </ref>
-;Measles: Persistent measles virus infection of stapes footplate results in activated osteoclast and inflammatory pathways by TNF-alpha mRNA. The protective function of osteoprotegerin at the otic capsule is inhibited by TNF-alpha and its action on RANK production<ref name="pmid16885748">{{cite journal| author=Karosi T, Jókay I, Kónya J, Szabó LZ, Pytel J, Jóri J | display-authors=etal| title=Detection of osteoprotegerin and TNF-alpha mRNA in ankylotic Stapes footplates in connection with measles virus positivity. | journal=Laryngoscope | year= 2006 | volume= 116 | issue= 8 | pages= 1427-33 | pmid=16885748 | doi=10.1097/01.mlg.0000225928.35838.e5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16885748  }} </ref>
+;Hormonal conditions:Puberty, pregnancy, and menopause increase the occurrence of hearing loss in pre-existing otosclerosis.
+;Measles:Persistent measles virus infection of stapes footplate results in activated osteoclast and inflammatory pathways by TNF-alpha mRNA. The protective function of osteoprotegerin at the otic capsule is inhibited by TNF-alpha and its action on RANK production
 ==Screening==
-Even though otosclerosis appears to be genetic deafness, early prevention can help in combating the disorder and its prevention.<ref name="pmid6577559">{{cite journal| author=Causse JR, Causse JB| title=Early detection of otosclerosis by impedance-audiometry screening. | journal=Scand Audiol Suppl | year= 1983 | volume= 17 | issue=  | pages= 47-54 | pmid=6577559 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6577559  }} </ref>
+Even though otosclerosis appears to be genetic deafness, early prevention can help in combating the disorder and its prevention.
 #Systematic Impedance-audiometry screening: The purpose of this screening is to detect a possible diphasic impedance change (on-off effect) by eliciting stapedius reflex. It is done in school going-children and helps in the early detection of stapedial fixation.
 #Systematic Bone-conducted audiometry screening: It is done in young children with a family history of otosclerosis.
@@ Line 128: / Line 216: @@
 ==Natural History, complications and Prognosis==
 ===Prognosis===
-Most of the patients find improvement in their hearing ability after the surgery. Only a few don't have any change post-surgery and rarely hearing loss worsens. There have been cases where the prosthesis was found displaced from its original position, causing conductive hearing loss again in some patients. The second surgery was observed to be less successful than the first.<ref name="pmid16985478">{{cite journal| author=Vincent R, Sperling NM, Oates J, Jindal M| title=Surgical findings and long-term hearing results in 3,050 stapedotomies for primary otosclerosis: a prospective study with the otology-neurotology database. | journal=Otol Neurotol | year= 2006 | volume= 27 | issue= 8 Suppl 2 | pages= S25-47 | pmid=16985478 | doi=10.1097/01.mao.0000235311.80066.df | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16985478  }} </ref>
+Most of the patients find improvement in their hearing ability after the surgery. Only a few don't have any change post-surgery and rarely hearing loss worsens. There have been cases where the prosthesis was found displaced from its original position, causing conductive hearing loss again in some patients. The second surgery was observed to be less successful than the first.
 ===Complications===
 Post-op complications:
-*Perilymph fistula (3-10%): Progressive hearing loss, Tinnitis, or vertigo.<ref name="pmid5440713">{{cite journal| author=Moon CN| title=Perilymph fistulas complicating the stapedectomy operation. A review of forty-nine cases. | journal=Laryngoscope | year= 1970 | volume= 80 | issue= 4 | pages= 515-31 | pmid=5440713 | doi=10.1288/00005537-197004000-00001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5440713  }} </ref>
-*Taste disturbance(9%)<ref name="pmid22326118">{{cite journal| author=Guder E, Böttcher A, Pau HW, Just T| title=Taste function after stapes surgery. | journal=Auris Nasus Larynx | year= 2012 | volume= 39 | issue= 6 | pages= 562-6 | pmid=22326118 | doi=10.1016/j.anl.2011.10.020 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22326118  }} </ref>
+*Perilymph fistula (3-10%): Progressive hearing loss, tinnitus, or vertigo.
-*Facial nerve injury (< 1%)<ref name="pmid15138427">{{cite journal| author=Neff BA, Lippy WH, Schuring AG, Rizer FM| title=Stapedectomy in patients with a prolapsed facial nerve. | journal=Otolaryngol Head Neck Surg | year= 2004 | volume= 130 | issue= 5 | pages= 597-603 | pmid=15138427 | doi=10.1016/j.otohns.2004.01.011 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15138427  }} </ref>
+*Taste disturbance(9%)
+*Facial nerve injury (< 1%)
 *Sensorineural Hearing loss(< 1% chance)
-*Poststapedectomy granulomas<ref name="pmid4604445">{{cite journal| author=Burtner D, Goodman ML| title=Etiological factors in poststapedectomy granulomas. | journal=Arch Otolaryngol | year= 1974 | volume= 100 | issue= 3 | pages= 171-3 | pmid=4604445 | doi=10.1001/archotol.1974.00780040179003 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4604445  }} </ref>
+*Poststapedectomy granulomas
 *Vertigo: Usually mild, subsides over time.
 ==Clinical presentaion==
 Patients present with the following:
 *Most cases are asymptomatic and diagnosed as an incidental finding in temporal autopsies.
-*Bilateral(80%) or unilateral involvement earlier in the disease.<ref name="pmid21916215">{{cite journal| author=Thomas JP, Minovi A, Dazert S| title=Current aspects of etiology, diagnosis and therapy of otosclerosis. | journal=Otolaryngol Pol | year= 2011 | volume= 65 | issue= 3 | pages= 162-70 | pmid=21916215 | doi=10.1016/S0030-6657(11)70670-9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21916215  }} </ref>
+*Bilateral(80%) or unilateral involvement earlier in the disease.
 *Progressive hearing loss, which might worsen with lower tone and frequencies like male voices and vowels.
-*vertigo(10%) <ref name="pmid21358194">{{cite journal| author=Ealy M, Smith RJH| title=Otosclerosis. | journal=Adv Otorhinolaryngol | year= 2011 | volume= 70 | issue=  | pages= 122-129 | pmid=21358194 | doi=10.1159/000322488 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21358194  }} </ref>
+*vertigo(10%)
-*tinnitis (50%)<ref name="pmid21358194">{{cite journal| author=Ealy M, Smith RJH| title=Otosclerosis. | journal=Adv Otorhinolaryngol | year= 2011 | volume= 70 | issue=  | pages= 122-129 | pmid=21358194 | doi=10.1159/000322488 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21358194  }} </ref>
+*tinnitis (50%)
-*Schwartz sign or flemingo's pink sign(not necessary for diagnosis): redness along the promontory of tymphanic membrane on otoscopic examination<ref name="pmid10205935">{{cite journal| author=Sellari-Franceschini S, Ravecca F, De Vito A, Berrettini S| title=[Progressive sensorineural hearing loss in cochlear otosclerosis]. | journal=Acta Otorhinolaryngol Ital | year= 1998 | volume= 18 | issue= 4 Suppl 59 | pages= 59-65 | pmid=10205935 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10205935  }} </ref>
+*Schwartz sign or flemingo's pink sign(not necessary for diagnosis): redness along the promontory of tymphanic membrane on otoscopic examination
 ==Diagnosis==
-===Tuning fork<ref name="pmid16434632">{{cite journal| author=Bagai A, Thavendiranathan P, Detsky AS| title=Does this patient have hearing impairment? | journal=JAMA | year= 2006 | volume= 295 | issue= 4 | pages= 416-28 | pmid=16434632 | doi=10.1001/jama.295.4.416 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16434632  }}  [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=&cmd=prlinks&id=17076020 Review in: Evid Based Nurs. 2006 Oct;9(4):120]  [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=&cmd=prlinks&id=17213129 Review in: Evid Based Med. 2006 Aug;11(4):116] </ref>===
+===Physical Examintaion===
+===='''<u>Tuning fork</u>'''====
 *Pros of using a tuning fork for diagnosis:
 **Negative Rinne test
@@ Line 155: / Line 247: @@
 **Rinne test cannot differentiate between sensorineural hearing loss and normal hearing loss.
 **Weber test's inability to differentiate a bilateral hearing loss.
-===Whisper test<ref name="pmid16434632">{{cite journal| author=Bagai A, Thavendiranathan P, Detsky AS| title=Does this patient have hearing impairment? | journal=JAMA | year= 2006 | volume= 295 | issue= 4 | pages= 416-28 | pmid=16434632 | doi=10.1001/jama.295.4.416 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16434632  }}  [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=&cmd=prlinks&id=17076020 Review in: Evid Based Nurs. 2006 Oct;9(4):120]  [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=&cmd=prlinks&id=17213129 Review in: Evid Based Med. 2006 Aug;11(4):116] </ref>===
+====<u>Whisper test</u>====
 ===Audiometry and tympanometry===
 *Caharts notch: Bone conduction depression in threshold at 2000Hz. After stapedectomy caharts notch diappears.
 *Tympanometry normal at early disease. In severe cases, flattening and stiffening of curve representing low complaince of ossicular chain and tympanic membrane. Good test to differentiate between otosclerosis and pathologies with low resonance.
 ===CT Imaging===
-*High resolution computed tomography (CT) of the temporal bones is the imaging technique of choice in the diagnosis of otosclerosis. <ref name="pmid23921926">{{cite journal| author=Virk JS, Singh A, Lingam RK| title=The role of imaging in the diagnosis and management of otosclerosis. | journal=Otol Neurotol | year= 2013 | volume= 34 | issue= 7 | pages= e55-60 | pmid=23921926 | doi=10.1097/MAO.0b013e318298ac96 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23921926  }} </ref>
+[[Image:Otosclerosis-fenestral.jpg|thumb|right|500px|Otosclerosis fenestral - Case courtesy of Assoc Prof Frank Gaillard, Radiopaedia.org, rID: 5568]]
-*However, preoperative CT has little to add in establishing otosclerosis diagnosis and may not even be necessary to confirm diagnosis. <ref name="pmid26649602">{{cite journal| author=Wegner I, van Waes AM, Bittermann AJ, Buitinck SH, Dekker CF, Kurk SA | display-authors=etal| title=A Systematic Review of the Diagnostic Value of CT Imaging in Diagnosing Otosclerosis. | journal=Otol Neurotol | year= 2016 | volume= 37 | issue= 1 | pages= 9-15 | pmid=26649602 | doi=10.1097/MAO.0000000000000924 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26649602  }} </ref>
-*CT is recommended to be reserved for those patients with presumed additional abnormalities, for specific preoperative planning, or for legal necessity.<ref name="pmid26649602">{{cite journal| author=Wegner I, van Waes AM, Bittermann AJ, Buitinck SH, Dekker CF, Kurk SA | display-authors=etal| title=A Systematic Review of the Diagnostic Value of CT Imaging in Diagnosing Otosclerosis. | journal=Otol Neurotol | year= 2016 | volume= 37 | issue= 1 | pages= 9-15 | pmid=26649602 | doi=10.1097/MAO.0000000000000924 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26649602  }} </ref>
+*High resolution computed tomography (CT) of the temporal bones is the imaging technique of choice in the diagnosis of otosclerosis.
-*CT Finding: ''increased bony radiolucency in the otic capsule around the anterior footplate, thickening of the stapes, and widening of the oval window''.<ref name="pmid23921926">{{cite journal| author=Virk JS, Singh A, Lingam RK| title=The role of imaging in the diagnosis and management of otosclerosis. | journal=Otol Neurotol | year= 2013 | volume= 34 | issue= 7 | pages= e55-60 | pmid=23921926 | doi=10.1097/MAO.0b013e318298ac96 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23921926  }} </ref>
+*However, preoperative CT has little to add in establishing otosclerosis diagnosis and may not even be necessary to confirm diagnosis.
+*CT is recommended to be reserved for those patients with presumed additional abnormalities, for specific preoperative planning, or for legal necessity.
+*CT Finding: ''increased bony radiolucency in the otic capsule around the anterior footplate, thickening of the stapes, and widening of the oval window''.
 *Early sign on CT: halo sign; demineralized area outlining the cochlea.
 ==Treatment==
 *'''Medical Management'''
-**Sodium flouride: Though controversial, it is used to slow the progression of otosclerosis.<ref name="pmid20089010">{{cite journal| author=Uppal S, Bajaj Y, Coatesworth AP| title=Otosclerosis 2: the medical management of otosclerosis. | journal=Int J Clin Pract | year= 2010 | volume= 64 | issue= 2 | pages= 256-65 | pmid=20089010 | doi=10.1111/j.1742-1241.2009.02046.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20089010  }} </ref>
+**Sodium flouride: Though controversial, it is used to slow the progression of otosclerosis.
-**Bisphosphonates: By inducing osteoclastic apoptosis, bisphosphates cause antiresorptive action, with promising results.<ref name="pmid22935809">{{cite journal| author=Quesnel AM, Seton M, Merchant SN, Halpin C, McKenna MJ| title=Third-generation bisphosphonates for treatment of sensorineural hearing loss in otosclerosis. | journal=Otol Neurotol | year= 2012 | volume= 33 | issue= 8 | pages= 1308-14 | pmid=22935809 | doi=10.1097/MAO.0b013e318268d1b3 | pmc=3442123 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22935809  }} </ref>
+**Bisphosphonates: By inducing osteoclastic apoptosis, bisphosphates cause antiresorptive action, with promising results.
 *'''Hearing aid''':It is used alone or in combination with other treatments.
-*'''Surgical Management''':Stapedotomy or Stapedectomy along with placement of a prosthesis, has shown a good prognosis with improved hearing ability. Only rare cases have shown no benefit. Revision surgery is controversial but proposed for patients with no improvement of hearing loss, facial nerve damage, or persistent vertigo.Most common cause of stapedectomy failure is prosthesis displacement with or without incus erosion. Revision surgery is also recommended post surgery for patients with footplate fixation, perilymph fistula, otosclerotic regrowth. Post surgery, some are still encouraged to use a hearing aid.<ref name="pmid23336672">{{cite journal| author=Redfors YD, Hellgren J, Möller C| title=Hearing-aid use and benefit: a long-term follow-up in patients undergoing surgery for otosclerosis. | journal=Int J Audiol | year= 2013 | volume= 52 | issue= 3 | pages= 194-9 | pmid=23336672 | doi=10.3109/14992027.2012.754957 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23336672  }} </ref><ref name="pmid20456195">{{cite journal| author=Bajaj Y, Uppal S, Bhatti I, Coatesworth AP| title=Otosclerosis 3: the surgical management of otosclerosis. | journal=Int J Clin Pract | year= 2010 | volume= 64 | issue= 4 | pages= 505-10 | pmid=20456195 | doi=10.1111/j.1742-1241.2009.02047.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20456195  }} </ref><ref name="pmid29502731">{{cite journal| author=McElveen JT, Kutz JW| title=Controversies in the Evaluation and Management of Otosclerosis. | journal=Otolaryngol Clin North Am | year= 2018 | volume= 51 | issue= 2 | pages= 487-499 | pmid=29502731 | doi=10.1016/j.otc.2017.11.017 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29502731  }} </ref>.
+*'''Surgical Management''':Stapedotomy or Stapedectomy along with placement of a prosthesis, has shown a good prognosis with improved hearing ability. Only rare cases have shown no benefit. Revision surgery is controversial but proposed for patients with no improvement of hearing loss, facial nerve damage, or persistent vertigo.Most common cause of stapedectomy failure is prosthesis displacement with or without incus erosion. Revision surgery is also recommended post surgery for patients with footplate fixation, perilymph fistula, otosclerotic regrowth. Post surgery, some are still encouraged to use a hearing aid..
+==Prevention==
+Otosclerosis cannot be prevented but early detection of the disease can help in combatting the disease better.
 ==References==
+<references />

Sandbox:Christina: Difference between revisions

Latest revision as of 06:13, 5 November 2021

Nasal polyp

Diagnosis

History and symtoms

Physical examination

Laboratory findings

Electrocardiogram

XRAY

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Pathophysiology

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Differential Diagnosis

Epidemology

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Classification

Otosclerosis

Overview

Historical perspective

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Embryology

Pathophysiology

Etiology

Differentiating otosclerosis from other diseases

Epidemiology and demographics

Risk Factors

Screening

Natural History, complications and Prognosis

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Clinical presentaion

Diagnosis

Physical Examintaion

Tuning fork

Whisper test

Audiometry and tympanometry

CT Imaging

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