Otosclerosis Medicine - Essay Example

? Otosclerosis Otosclerosis is one of the diseases that normally lead to deafness. This paper aims at describing the disease in termsof its location as well as some of the major causes. Moreover, the paper also highlights the major signs and symptoms that typify the condition in addition to the major tissues and anatomical parts that tend to be affected by the disease. Karosi and Sziklai (2010, p.1337) define Ostosclerosis as a hereditary inflammatory disorder that is characterized by bone remodeling leading to a progressive conducive or sensoneural hearing loss. The disease is perceived to cause an inflammation of the human otic capsule as a result of persistent attack by the measles virus. This is supported by the presence of RNA and antigens commonly associated with the virus in the perilymph of patients diagnosed with the disease. Hereditary play a huge role in the etiopathogenesis of the disease, whereby, Battta, Karosi and Sziklai (2009, p.1171) assert that the disease is passed to generations as an autosomal dominant gene. Cowan (2006) presents that otosclerosis is regarded as the common conductive hearing loss and is secondary to a pathologic change of the bones in the middle ear. In normal cases, the ossicles are usually hard, however, following an otosclerosis infection, the ossicles starts to soften, highly vascularised, spongy and may even become partially or permanently fixed. The fixation in turn results in a reduction of the transmission of the source waves to the fluids in the inner ear. Of the three bones that are found in the middle ear, the disease normally affects the stapes, which is are ossicles in the middle ear that transmit sound vibrations to the fenestra ovalis. According to deSouza and Glasscock (2002, p.245) the disease is the cause of nearly 10% of reported hearing loss cases and an average of 20% of conductive hearing loss cases. Moreover, White, Duncan and Laumie (2010, p.369) posit that the disease is usually common among adults than children with women being highly inclined to be infected. Karosi and Sziklai (2011, p.1337) points that the bone remodeling in otosclerosis disorder usually shows some form of organotropism to the otic capsule. Similarly, they further reveal that focal points of the disease tend to be limited to the temporal bones of the middle ear. In virtually all clinically reported cases of the disease, no lesions have been found outside the ear. It is in the otic capsule within the ear where enchondral ossification develops as well as the footplates of the stapes. The effect of the disease on the otic capsule implies that the three bone layers that form the capsule are also affected in the process. These three layers are the endosteal layer, interosseal globules as well as the periosteal layer. The interosseal layer has been identified as the site of the earliest otosclerotic foci. Karosi and Sziklai (2011, p.1338) further document that the common histopathological features of the disease are usually visible in areas such as those adjacent to oval and round windows, which accounts for 90% and 40% respectively, the pericochlear 35%, and the stapes footplate 95%. Additionally, numerous fibroblasts, osteoclasts and proliferating endothelial cells also tend to be affected by the disease. According to Gristwood and Venables (2003, p.399-341) otosclerosis is histologically divided into two distinct phases. The first phase is majorly characterized by bone resorption and elevated levels of vascularity. This in turn leads to the diminishing of the mature collagen content of the bone thereby making the bone to acquire a spongy appearance. In the second and the late stage of the disease, the reabsorbed phase is then replaced with a rather dense and sclerotic bone hence the derivation of the name otosclerosis. In instances when the disease affects the stapes, otosclerosis commonly begins from the fissula fenestrum from where it progresses to complete footplate involvement and may sometimes cover the entire oval window in advanced cases of the disease (Alfio, et al. 2003, p.741). The primary clinical manifestation of the disease mainly includes slight changes in hearing as well as low-pitched tinnitus. As the disease progresses, it tends to become difficult for patients to differentiate a whisper from normal high-pitched tone, which is normally presented in the third decade. In addition, such patients will find it difficult to hear or have a meaningful conversation in crowded areas. Clinically, the disease has been identified to manifest the Carhart notch, which is a 10-30 drop in dB of the bone line as a result of mechanical factors linked with stapes fixation. Simply, patient suffering from the disease have also been reported to have not stapedial reflex as is the case with uninfected people. Also, Alfio, et al (2003,p.742) reports that, the disease can also be manifested by the presence of the Schwartze sign, a hyperemia of the promontory mucosa as a result of the underlying bone remodeling, which can be seen through the tympanic membrane as a pink hue. Some patients also tend to complain about dizziness, although such vestibular symptoms are rare. The disease can be diagnosed by frequent occurrences of sequel infections of the middle ear such as perforations or scars. The diagnosis of the disease mainly begins with the Weber and Rinne tuning fork tests. Unilateral or bilateral conductive hearing loss can also be an indication of the disease, which should be accompanied by the Rinne test that determines bone and air conduction. A negative result of the test indicating that bone conduction is greater than air conduction can be an indication of the otosclerosis, especially as a result of mechanical inhibition of the movement of the basilar membrane arising from fixation of the footplate. According to White (2010, p.1117) the treatment of otosclerosis is limited to three options. Depending on the individual desire, risks, and economic status of an individual, some individuals obtain periodic audiometry to evaluate the progression of the disease. Alternatively, one can also choose to use a hearing aid without necessary undergoing a surgery or other medical treatment. This is mainly preferred for conventional amplification or in patients with primary inner ear involvement otherwise referred to as cochlear otosclerosis. Common hearing aids include the Bone Anchored Hearing Aid. Similarly, in some mild cases, the disease has been managed by treatment with high doses of sodium fluoride. The methodology is based on the principle that sodium fluoride increase remineralization of otospongic foci. The other choice is surgical management of the disease with an outpatient procedure referred to as stapedectomy. Stapedoctomy is deemed as the most widely preferred surgical technique for improving hearing loss caused by otosclerosis. The procedure can be achieved under either local anesthesia or general anesthesia. It generally entails making surgical incisions in the posterior ear canal prior to removal of the stapes and subsequent replacement through implantation of plastic prosthesis. A non-invasive removal of the stapes can also be done by the use of laser. In this procedure, the tendons supporting the stapes are vaporized the remnants removed by the use of micro instruments (Adunka and Buchm, 2011, p.310). A small opening is then opened to allow implantation of the prosthetic plastic pistons capable of transmitting vibrations into the inner ear. According to Coatesworth, Bajjaj and Uppal (2010, p.258) surgery for otosclerosis may entail four main procedures. The stapes may be surgically removed followed by placement of a piston or bucket-handle prosthesis. Also, stapedoctomy may entail the replacement of the stapes with Schuknecht’s prosthetics. In some cases, stapedoctomy may also involve the implantation of active middle ear implants. The disease can also be managed through stapes mobilization which is another operation for otosclerosis involving surgery around the ankylosed stapes. This procedure is considered as one of the simplest procedures for treatment of the disease in terms of duration of the operation (Cowan, 2006). Also tympanoplasty or microscopically controlled removal of adhesions and dead bone for partial relief of deafness in the middle ear can also be done to alleviate the seriousness of the effect of the disease. Toner, Kerr and Stevenson (2001, p113) contend that surgical treatment of the disease by stapedoctomy has been the main stay of treatment of the disease. The description of the surgical procedure was advanced in 1958 by Shea. The procedure is nowadays easy to perform with the continuing development of refined instruments as well as techniques for carrying out the procedure. As therefore seen, otosclerosis is generally a disease that affects the middle ear, especially the stapes as a result of remodeling of the bone structure that becomes spongy and highly vascularised. The disease is purported to be caused by measles virus and can be spread hereditary to subsequent generations. It mainly leads to conductive hearing loss. Main tissues affected are those found within the otic capsule. There are a number of diagnostic procedures such as otoscopy, the Weber and Rinne tuning fork test as well as trypanometry. The disease is mainly managed and treated through surgical procedures; however, hearing aids and sodium fluoride are also some of the alternative means of managing the disease. References Adunka, Oliver, and Craig Buchm. Otology, Neurotology, and Lateral Skull Base Surgery: An Illustrated Handbook. New York: Theme, 2011. Alfio, Ferlito, Arnold Wolfgang, Rinaldo, Alessan, and Alexis Bozorg. "Viruses and Otosclerosis: Chance Association or True Causal Link?" Acta Oto-Laryngologica 231, no. 6 (2003): 741-746. Batta, Tamaz Jozsef, Istvan Sziklai, and Tamas Karosi. "Otosclerosis: an organ-specic inflammatory disease with sensorineural hearing loss." Eur Arch Otorhinolaryngol 266 (2009): 1711–1718. Coatesworth, A, Y Bajjaj, and S Uppal. "Otosclerosis 2: the medical management of otosclerosis." International Journal of Clinical Practice 64, no. 2 (2010): 256-265. Cowan, Alan L. "Otosclerosis." UMTB . October 26, 2006. http://www.utmb.edu/otoref/grnds/Otosclerosis-061018/Otosclerosis-061018.htm (accessed April 18, 2012). deSouza, Christopher, and Michael E Glasscock. Otosclerosis and Stapedectomy: Diagnosis, Management,. NewYork: Theme Publications, 2002. Gristwood, Ronald E, and William N Venables. "Otosclerosis and Chronic Tinnitus." Annals of Otology, Rhinology & Laryngology, 112, no. 5 (2003): 398-404. Karosi, Tamas, and Istvan Sziklai. "Etiopathogenesis of otosclerosis." Eur Arch Otorhinolaryngol 267 (2010): 1337–1349. Raut, V V, G Toner, and M Stevenson. "Management of otosclerosis in the UK." Clinical Otolaryngol 27 (2002): 113–119. Valdez, Edith, E J Ramirez, and Manuel Martin. "Surgical and Medical Treatment of Hearing Loss in." Annals of Otology, Rhinology & Laryngology 118, no. 12 (2009): 859-865. White, Lois, Gena Duncan, and Wendy Baumle. Foundations of Adult Health Nursing. Mason, Ohio: Cengage Learning, 2010. Read More