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:
- Dizziness is often a common accompaniment with hearing loss.
- Tinnitus is a condition characterized by ringing or buzzing in the ears [2].
- Balance problems.
Entire Body System
- Rigor
Other more rigorous trials have reported similar results (Bretlau et al, 1989). Some advocate using sodium fluoride for cochlear otosclerosis (Lippy and Berkowitz, 2008). [dizziness-and-balance.com]
Jaw & Teeth
- Metallic Taste
Taste - sometimes bruising can occur to the small nerve concerned with taste which runs just under the ear drum. As a result, some patients experience a metallic taste on the side of the tongue for a month or two after the operation. [tinnitus.org.uk]
Symptoms include a dry mouth, sore tongue, and a metallic taste. Symptoms usually subside in 3 - 4 months. Stretching the chorda is more likely to result in symptoms than simply dividing the nerve. [audiologyonline.com]
Taste Disturbance A temporary taste disturbance is common after stapedectomy/stapedotomy. The disturbance is usually reported as a "tinny" or "metallic" taste. [earcentergreensboro.com]
Sacrificing the nerve better than stretching it Injury leads to hypogeusia and dysgeusia Stretching leads to metallic taste, altered taste to various food, altered taste CHORDA TYMPANI INJURY 125. Immediate post op period Worrisome Serous [slideshare.net]
Musculoskeletal
- Fracture
The stapes suprastructure is then "down-fractured" leaving the footplate still frozen within the oval window. A laser and drill are used to make a small hole in the footplate. A prosthesis is placed over the incus and into the hole in the footplate. [umms.org]
To repair small focus of otosclerosis in the anterior crus of the stapes, in 1960, Hough suggested the implementation of a technique in which part of the anterior crus is fractured and the stapes turned. [ncbi.nlm.nih.gov]
Otosclerosis is often associated with osteogenesis imperfect (van der Hoeve syndrome) in a classic triad of hearing loss (conductive, mixed, or sensorineural), spontaneous bone fractures, and blue sclera. [emedicine.medscape.com]
Ears
- 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]
Also, see eMedicineHealth's patient education article Tinnitus. [emedicine.medscape.com]
- Hearing Impairment
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]
If you feel a significant degree of hearing impairment following stapedectomy, revision surgery can be performed with good odds of success. [hillsent.com.au]
Types of Hearing Impairment The external ear and the middle ear conduct sound; the inner ear receives it. If there is some difficulty in the external or middle ear, a conductive hearing impairment occurs. [michiganear.com]
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 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]
Extensive hearing tests will confirm the diagnosis. Treatment People with otosclerosis often benefit from a properly fitted hearing aid. The surgical replacement of the stapes has become a common procedure to improve conductive hearing problems. [encyclopedia.com]
With over 16 years of experience serving patients in the Orange County and Long Beach area, we are proud to offer all of our patients a complete, all-in-one solution to any hearing problem or medical related ear condition. [eardoctor.org]
Hearing Loss Genetic Hearing Loss Noise Induced Hearing Loss Bilateral Hearing Loss Unilateral Hearing Loss Conductive Hearing Loss High Frequency Hearing Loss Low Frequency Hearing Loss Sensorineural Hearing Loss Hearing Problems Fungal Ear Infections [hiddenhearing.co.uk]
Neurologic
- Dizziness
Patients sometimes experience dizziness and nausea after stapedectomy surgery. Some mild unsteadiness and brief dizziness with head turning is not uncommon. If dizziness is marked, do not get up without assistance. [tampabayhearing.com]
[…] 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]
- 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]
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]
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.
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
- Toynbee J. Pathological and surgical observations of the diseases of the ear. Trans Med Chir Soc Lond. 1841; 24:190-196.
- Jack FL. Remarkable improvement of hearing by removal of the stapes. Trans Am Otol Soc. 1893; 284:474-89.
- 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.
- 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
- Mansour S, Nicolas K, Ahmad HH. Round window otosclerosis: radiologic classification and clinical correlations. Otol Neurotol. 2011; 32(3):384-92
- Liktor B, Szekanecz Z, Batta TJ, Sziklai I, Karosi T. Perspectives of pharmacological treatment in otosclerosis. Eur Arch Otorhinolaryngol. 2013; 270(3):793-804
- Jack FL. Remarkable improvement of hearing by removal of the stapes. Trans Am Otol Soc. 1893; 284:474-89.
- Lempert J. Improvement in hearing in cases of otosclerosis: A new, one-stage surgical technic. Arch Otolaryngol. 1938; 28:42-97.
- 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.
- 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.