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Otosclerosis

Otoscleroses

Otosclerosis is a common cause of hearing impairment.


Presentation

Hearing loss is the primary sign of otosclerosis. In the preliminary stages, the individuals suffer from partial hear loss which gets worse over time and results in complete deafness. In addition to it, the condition presents itself with the following signs and symptoms:

Broad Thumb
  • Mutations in the NOG gene are known to be associated with a variety of rare stapes ankylosis syndromes including stapes ankylosis with broad thumbs and toes, multiple synostoses syndrome, and proximal symphalangism.[ncbi.nlm.nih.gov]
Collapse
  • Collapse of the ductus reuniens and dilated saccule was seen in three temporal bones.[ncbi.nlm.nih.gov]
Intravenous Administration
  • PURPOSE: Increased cochlear lymph fluid signals on three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) images obtained several minutes after intravenous administration of a single dose of gadolinium-based contrast agent (IV-SD-GBCA) in a[ncbi.nlm.nih.gov]
Tinnitus
  • CONCLUSION: Low pitch tinnitus is more likely to be resolved after stapedotomy for patients with otosclerosis. High pitch tinnitus may not resolve even after closure of the Air-Bone Gap at tinnitus frequencies.[ncbi.nlm.nih.gov]
  • Hearing loss and tinnitus are the main symptoms of otosclerosis. Little is known about the cause of tinnitus in otosclerosis and the factors influencing the effect of surgery on tinnitus.[ncbi.nlm.nih.gov]
  • Otosclerosis may also be associated with annoying tinnitus, and tinnitus management is important in the rehabilitation process.[ncbi.nlm.nih.gov]
  • In the thulium group, there were four patients with sensorineural hearing loss (4.4%) and three with tinnitus (3.1%) compared with none in the CO2 group.[ncbi.nlm.nih.gov]
  • Also, see eMedicineHealth's patient education article Tinnitus.[emedicine.medscape.com]
Hearing Impairment
  • Otosclerosis is caused by an abnormal bone homeostasis of the otic capsule and represents a frequent cause of hearing impairment.[ncbi.nlm.nih.gov]
  • Hearing Impairment from Otosclerosis If we were able to examine the inner ear bone under a microscope before a hearing impairment developed, we would see minute areas of both softening and hardening of the bone.[epichearing.com]
  • These cases demonstrate the use of hearing aid amplification in patients with mixed-type hearing impairment due to otosclerosis.[ncbi.nlm.nih.gov]
  • She had been suffering from progressive hearing impairment since childhood.[ncbi.nlm.nih.gov]
  • The mental and physical summary component scores correlated significantly to hearing disability measured by the SSQ but not to hearing impairment.[ncbi.nlm.nih.gov]
Hearing Problem
  • The condition may not need to be treated until you have more serious hearing problems. Using some medicines such as fluoride, calcium, or vitamin D may help to slow the hearing loss.[nlm.nih.gov]
  • Problems Fungal Ear Infections Outer Ear Infections Middle Ear Infections Tinnitus Can You Prevent Tinnitus Links Between Hearing Loss And Tinnitus Tinnitus Causes Tips For Dealing With Tinnitus About What Makes Us Different Contact Us Dr Hilary Bio[hiddenhearing.co.uk]
  • The surgical replacement of the stapes has become a common procedure to improve conductive hearing problems. During this operation, called a stapedectomy, the stapes is removed and replaced with an artificial device.[encyclopedia.com]
  • The condition may not need to be treated until you have more serious hearing problems. Medications such as fluoride, calcium, or vitamin D may help to slow the hearing loss. However, the benefits of these treatments have not yet been proven.[nicklauschildrens.org]
Visual Hallucination
  • However, on both occasions the transformation from one form to another occurred during an alcohol withdrawal syndrome characterized mainly by vivid visual hallucinations.[ncbi.nlm.nih.gov]
Dizziness
  • […] have hearing loss You develop fever, ear pain, dizziness, or other symptoms after surgery Otospongiosis; Hearing loss - otosclerosis House JW, Cunningham CD.[nlm.nih.gov]
  • The post-operative course was uneventful in 12 of 14 cases with no EH in the vestibule, and the other two cases had a short period of dizziness, but two cases with EH in the vestibule had a long period of dizziness.[ncbi.nlm.nih.gov]
  • A statistically significant interaction was found between calorics and dizziness in relation to PTA thresholds. No relationship was found between calorics and dizziness with VEMPs responses.[ncbi.nlm.nih.gov]
  • Post-surgical complications such as bleeding, blood clot, infection, dizziness and pain. Unusual taste in the mouth. The exact cause that triggers such an abnormal bone remodeling mechanism is unknown.[symptoma.com]
  • […] fever, ear pain, dizziness, or other symptoms after surgery visHeader References House JW, Cunningham CD III.[nicklauschildrens.org]
Vertigo
  • METHODS: Fifty otosclerosis patients comprising 27 patients with vertigo (Group A) and 23 patients without vertigo/dizziness (Group B) were enrolled.[ncbi.nlm.nih.gov]
  • However, a longer follow-up may help in understanding the behaviour of cVEMPs in post-stapes surgery vertigo.[ncbi.nlm.nih.gov]
  • Secondary outcome measures were the incidence of intraoperative (bleeding and fractured footplate) and postoperative (vomiting, vertigo, sensorineural hearing loss, tinnitus, facial nerve paralysis, and hospital stay) morbidities.[ncbi.nlm.nih.gov]
  • This paper presents a combined procedure for the management of intractable benign paroxysmal positional vertigo (BPPV) and profound hearing loss in a patient with far advanced otosclerosis.[ncbi.nlm.nih.gov]
  • The study, of 140 patients with otosclerosis who underwent stapedial surgery, found that, while 12 patients [8.6%] reported having vertigo prior to surgery and no vertigo after the operation, 36 patients [25.7%] who had no vertigo before surgery reported[emedicine.medscape.com]
Speech Disorders
  • Alessia Zanon, Flavia Sorrentino, Leonardo Franz and Davide Brotto, Gender-related hearing, balance and speech disorders: a review, Hearing, Balance and Communication, 10.1080/21695717.2019.1615812, (1-10), (2019).[doi.org]

Workup

A normal examination of the ear using auriscope would usually not show any abnormalities. Individuals should therefore visit an ENT specialist to get a differential diagnosis done. Specialized instruments such as tympanometer would help to evaluate the movement of the bones inside the middle ear. This is usually a painless procedure and yields quick results.

In certain conditions, imaging studies of the head may also be required, such as temporal bone computed tomography [3]. Temporal bone computed tomography can further be enhance with the use of 18F-fluoride positron emission tomography to elucidate bone metabolism of the inner ear [4]. The relative hypodensity in otosclerosis of the round window during CT scan can help surgeons in counselling and in deciding whether to proceed for surgical stapedectomy [5].

Treatment

In the preliminary stages, the hearing loss is mild and does not require any treatment. However, over a time of time, the condition can worsen and individuals are then immediately put on treatment. The following are the several methods employed for treating otosclerosis:

  • Hearing aids are meant for treating moderate hearing loss. But, when the condition turns severe then hearing aids are not of much help.
  • Supplements of fluoride, calcium and vitamin D can be of some help in slowing down the progression of the disease [6]. However, not enough research has been conducted in this regard and efficacy of nutritional supplements is yet to be proved.
  • Surgery is often the treatment of choice to restore hearing ability. In this, the part of the ear known as stapes is replaced by prosthesis which can restore hearing function. In many cases, a total replacement may be required and such a procedure is known as stapedectomy [7]. Operative intervention of the middle ear may require local or general anesthesia with sedation [8]. Individuals often recover well with surgery and hearing ability is restored [9]. This is however a very delicate operative procedure and in certain cases can even damage the neighboring nerves calling for complications. The risk though small, can contribute towards development of unpleasant conditions.
  • Advances in ear surgery has implored the use of endoscopic surgery to significantly lower risk and morbidity [10]. 

Prognosis

If prompt treatment is not initiated then the condition may worsen over a period. Individuals can suffer from total deafness for the rest of the life. Surgical procedures can be carried out to restore the hearing function to certain extent; but may not always be successful.

Complications

Possible complications of otosclerosis include the following conditions:

Etiology

The exact cause that triggers such an abnormal bone remodeling mechanism is unknown. However, there have been some pieces of evidence suggesting that otosclerosis is an inherited condition and can be passed from one generation to other. In otosclerosis the bone remodeling function is disrupted as a result of which the sound waves are unable to travel to the inner ear from the middle ear. Such a condition causes hearing loss.

Some other theories point towards the fact that otosclerosis develops as a result of measles virus. The exact theory states that individuals may be born with certain kind of genetic defect and a viral infection may further trigger the onset of the disorder. In addition to this, low levels of fluoride can also have some kind of link with development of otosclerosis.

Epidemiology

Otosclerosis is a common condition affecting about 3 million Americans. Women are more prone to contract this condition than males. This condition is often diagnosed in mid adulthood and is a common cause of hearing loss amongst the young adult population. It has also been estimated that Caucasians are more affected by this disease condition than individuals of other regions.

Sex distribution
Age distribution

Pathophysiology

Under normal conditions, for individuals to be able to hear, sound waves must travel from the middle ear to the inner ear. Such sequence of events does not take place in otosclerosis due to an abnormal bone growth in the middle ear.

Bone remodeling is an ongoing process wherein the bone tissues are periodically renewed by replacing the old with new. However, due to abnormal remodeling such an activity does not take place and the sound waves are unable to travel to the inner ear. There is an observable fixation of stapes ossicle to the oval window causing a disruption in the sound transmission [1]. Otosclerosis can affect one or both ears.

Prevention

It is not possible to prevent otosclerosis. It is a genetic defect that occurs spontaneously thus making it difficult to prevent the development of such a disease condition. However, there have been small pieces of evidence suggesting fluoride tablets and measles vaccination as preventive measures against otosclerosis. There is still dearth of information in this regard, and more research needs to be carried out.

Summary

Otosclerosis is a condition characterized by abnormal development of bone in the middle ear. Such a condition can contribute to hearing loss and other associated abnormalities. It is a serious condition demanding prompt diagnosis. Otosclerosis develops due to abnormal remodeling of the bone. Such an event interrupts the travelling of the sound waves from the middle ear to inner ear contributing to hearing loss. It is not an uncommon condition and affects millions of individuals across the globe.

Patient Information

Definition

Otosclerosis is a condition characterized by abnormal growth of bone in the middle ear region. This causes gradual ear loss which can lead to complete deafness over a period of time.

Cause

The exact cause that triggers the development of otosclerosis is unknown. However heredity and genetic factors are known to play foul. Researchers also believe that measles virus and low fluoride can also cause otosclerosis.

Symptoms

Partial or complete hearing loss is the major symptom of otosclerosis. In addition to this, individuals can also experience tinnitus, dizziness and loss of balance.

Diagnosis

Diagnosis of otosclerosis is done using specialized instruments that can measure the activity of the bone inside the middle ear. Individuals need to consult an ENT specialist for appropriate diagnosis of the condition. In addition to this, CT scan of the head may also be required.

Treatment

Individuals with mild hear loss do not usually require any treatment. Those with moderate hear loss require hearing aids to deal with the problem. However, in severe cases, hearing aids are of no help and surgical procedures are the only resort.

References

Article

  1. Toynbee J. Pathological and surgical observations of the diseases of the ear. Trans Med Chir Soc Lond. 1841; 24:190-196.
  2. Jack FL. Remarkable improvement of hearing by removal of the stapes. Trans Am Otol Soc. 1893; 284:474-89.
  3. Virk JS, Singh A, Lingam RK. The role of imaging in the diagnosis and management of otosclerosis. Otol Neurotol. Sep 2013; 34(7):e55-60.
  4. Waterval JJ, Vallinga M, Brans B, Winkens B, Stokroos RJ. 18F-fluoride PET/CT scan for quantification of bone metabolism in the inner ear in patients with otosclerosis--a pilot study. Clin Nucl Med. 2013; 38(9):677-85 
  5. Mansour S, Nicolas K, Ahmad HH. Round window otosclerosis: radiologic classification and clinical correlations. Otol Neurotol. 2011; 32(3):384-92
  6. Liktor B, Szekanecz Z, Batta TJ, Sziklai I, Karosi T. Perspectives of pharmacological treatment in otosclerosis. Eur Arch Otorhinolaryngol. 2013; 270(3):793-804 
  7. Jack FL. Remarkable improvement of hearing by removal of the stapes. Trans Am Otol Soc. 1893; 284:474-89.
  8. Lempert J. Improvement in hearing in cases of otosclerosis: A new, one-stage surgical technic. Arch Otolaryngol. 1938; 28:42-97.
  9. Thomeer HG, Kunst HP, Cremers CW. Congenital stapes ankylosis associated with another ossicular chain anomaly: surgical results in 30 ears. Arch Otolaryngol Head Neck Surg. Sep 2011; 137(9):935-41.
  10. Kojima H, Komori M, Chikazawa S, Yaguchi Y, Yamamoto K, Chujo K, et al. Comparison between endoscopic and microscopic stapes surgery. Laryngoscope. May 13 2013.

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Last updated: 2019-07-11 22:23