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Progressive Deafness with Stapes Fixation

Stapedo-vestibular ankylosis


Presentation

  • Differential diagnosis Differential diagnosis should include minor ossicular malformations presenting with a normal otoscopy, a conductive hearing loss, and abolished stapedial reflexes.[orpha.net]
  • Presents new contributors for 14 chapters to provide you with authoritative coverage and the dynamic perspectives of leaders in the field.[books.google.de]
Physician
  • Written for practicing clinicians, this volume is an excellent reference for physicians, audiologists, and other professionals working with individuals with hearing loss and their families, and can also serve as a text for clinical training programs and[books.google.com]
  • This information is not intended to be patient education, does not create any patient-physician relationship, and should not be used as a substitute for professional diagnosis and treatment.[diseaseinfosearch.org]
  • In-Depth Information The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers.[rarediseases.info.nih.gov]
  • Your physician will suggest selected laboratory and x-ray studies based on the physical examination and history involving your particular child.[pediatric-ent.com]
Surgical Procedure
  • Treatments The hearing can be restored with a surgical procedure called stapedectomy. Hearing aids can be helpful in patients who do not desire surgical treatment.[texasearcenter.com]
  • In a surgical procedure called stapedectomy (or more correctly stapedotomy), the top portion of the stapes bone is removed by breaking it or vaporizing it with a LASER.[seanpalaciosmd.com]
  • Surgery (stapedectomy): A stapedectomy is a surgical procedure that involves either a total or partial removal of the stapes bone. A prosthesis is then used to restore function.[marshfieldclinic.org]
  • If the vertigo is not medically controlled, then various surgical procedures are used to eliminate the vertigo.[hearingloss.org]
  • A stapedotomy is a surgical procedure similar to a stapedectomy except that the surgeon uses the laser to cut a hole in the stapes in order to insert the prosthesis rather than removing the stapes.[surgeryencyclopedia.com]
Inflammation
  • Tympanosclerosis is a sequela of middle ear chronic inflammation. Diagnostic methods Otoscopy is normal in otosclerosis, Paget's disease, and osteogenesis imperfecta. It reveals middle ear lesions in tympanosclerosis.[orpha.net]
  • Learn More About Topic Swimmer's Ear Swimmer’s ear (also called acute otitis externa) is a painful condition that affects the outer ear and ear canal that is caused by infection, inflammation, or irritation.[entnet.org]
  • Corticosteroids may also be used to reduce cochlea hair cell swelling and inflammation after exposure to loud noise.[hearingloss.org]
Chronic Infection
  • Postinfective tympanosclerosis usually follows chronic infection. Paget's disease of bone can cause a clinically indistinguishable syndrome, but will usually have other features of the disease. Ask about change in hat size and bony pain.[patient.info]
  • Chronic middle-ear infection Chronic infection of the middle ear occurs when there is a permanent perforation of the tympanic membrane that allows dust, water, and germs from the outer air to gain access to the middle-ear cavity.[britannica.com]
Respiratory Disorders
  • Disorder 624 Häufige Begriffe und Wortgruppen Bibliografische Informationen Titel Saunders Comprehensive Review for the NCLEX-PN Examination - E-Book Autor Linda Anne Silvestri Ausgabe 5, überarbeitet Verlag Elsevier Health Sciences, 2012 ISBN 1455759562[books.google.de]
Failure to Thrive
  • Fred Ferri’s popular "5 books in 1" format provides quick guidance on menorrhagia, Failure to Thrive (FTT), Cogan’s syndrome, and much more.[books.google.de]
Ankylosis
  • Affiliated tissues include bone, and related phenotypes are bilateral conductive hearing impairment and stapes ankylosis[malacards.org]
  • Congenital stapes ankylosis: study of 28 cases and surgical results.. Laryngoscope. 2006; 116(7):1153-1157. . Hara A, Ase Y, Kusakari J, Kurosaki Y. Arch Otolaryngol Head Neck Surg. 1997; . Accessed 12/11/2009.[rarediseases.info.nih.gov]
  • The otosclerotic focus may be asymptomatic, or if present in the area of foot plate of stapes it may give rise to ankylosis of foot plate with resultant conductive deafness.[drtbalu.co.in]
  • INTRODUCTION Otosclerosis is a disease of otic capsule in which new vascular spongy bone formation causes ankylosis or fixation of the foot plate of the stapes and progressive conductive deafness.  Otosclerosis can result in conductive and/or sensorineural[slideshare.net]
  • [oto- G. sklērōsis, hardening] otosclerosis /oto·scle·ro·sis/ ( -sklĕ-ro sis ) a condition in which otospongiosis may cause bony ankylosis of the stapes, resulting in conductive hearing loss.otosclerot ic otosclerosis (ō′tō-sklə-rō′sĭs) n.[medical-dictionary.thefreedictionary.com]
Progressive Hearing Loss
  • Diagnosis - Progressive hearing loss stapes fixation Prognosis - Progressive hearing loss stapes fixation Not supplied. Treatment - Progressive hearing loss stapes fixation Not supplied.[checkorphan.org]
  • Generally, it is considered a slowly progressive hearing loss. There are some patients who experience a rather rapidly progressive hearing loss with otosclerosis. Pregnancy can accelerate the otosclerosis process, thus accelerating hearing loss.[marshfieldclinic.org]
  • Bilateral progressive hearing loss over several months, also diagnosed as autoimmune inner ear disease, is managed medically with long-term corticosteroids and sometimes with drug therapy.[hearingloss.org]
  • The primary symptom of otosclerosis is a slowly progressive hearing loss beginning anytime between the age of 15 and 45, although it usually starts around age 20.[seanpalaciosmd.com]
  • Natural Course of Otosclerosis Otosclerosis may affect one or both ears of an individual and gradually causes progressive hearing loss as the bony growth gets larger. The speed at which the otosclerotic bone develops in an individual one.[bcm.edu]
Hearing Impairment
  • Affiliated tissues include bone, and related phenotypes are bilateral conductive hearing impairment and stapes ankylosis[malacards.org]
  • X-linked Disorders Alport syndrome X-linked mixed hearing impairment with stapes fixation and perilymphatic gusher. Hereditary causes of hearing loss are suspected in all cases of hearing impairment.[pediatric-ent.com]
  • CAUSES OF HEARING IMPAIRMENT If there is some difficulty in the external (outer) or middle ear, a conductive hearing impairment occurs.[potomacaudiology.com]
  • Affected females manifest bilateral, moderate hearing impairment that affects the high frequencies ( Fig. 2 C).[academic.oup.com]
  • Synonym: otospongiosis Otosclerosis (OTSC) is the single most common cause of hearing impairment.[patient.info]
Hearing Impairment
  • Affiliated tissues include bone, and related phenotypes are bilateral conductive hearing impairment and stapes ankylosis[malacards.org]
  • X-linked Disorders Alport syndrome X-linked mixed hearing impairment with stapes fixation and perilymphatic gusher. Hereditary causes of hearing loss are suspected in all cases of hearing impairment.[pediatric-ent.com]
  • CAUSES OF HEARING IMPAIRMENT If there is some difficulty in the external (outer) or middle ear, a conductive hearing impairment occurs.[potomacaudiology.com]
  • Affected females manifest bilateral, moderate hearing impairment that affects the high frequencies ( Fig. 2 C).[academic.oup.com]
  • Synonym: otospongiosis Otosclerosis (OTSC) is the single most common cause of hearing impairment.[patient.info]
Suggestibility
  • Access full-color images and more than 90 online-only topics at Expert Consult, as well as EBMs, Suggested Reading, Patient Teaching Guides, and additional algorithms.[books.google.de]
  • A type A D tympanogram suggests ossicular discontinuity. Often, as incus necrosis progresses, the union between the incus and the stapes is replaced by a dense fibrous band.[emedicine.medscape.com]
  • Bedside: The otologic clinician should confirm audiological testing suggesting a conductive hearing loss with bedside examination.[dizziness-and-balance.com]
  • While there are other theories suggesting that it is a metabolic bone disorder the exact cause remains elusive.[health.ucsd.edu]
Meningism
  • Ferri’s popular "5 books in 1" format provides quick guidance on short QT syndrome, microscopic polyangiitis, fungal meningitis, and much more. This medical reference makes the answers you need even easier to find - anytime, anywhere.[books.google.de]
  • The proximity of the brain cavity to the mastoid air cells is such that an infection, if severe and untreated, may lead to meningitis (inflammation of the covering of the brain) or brain abscess.[britannica.com]
  • […] committee on infant hearing has identified ten risk factors for newborn hearing loss: Family history of hearing loss, In utero infection, Craniofacial anomalies, Hyperbilirubinemia requiring exchange transfusion, Birth weight less than 1500 grams, Bacterial meningitis[pediatric-ent.com]
  • ., meningitis) In utero infection (e.g., toxoplasmosis, rubella, cytomegalovirus infection, herpes, syphilis) Neonatal indicators: hyperbilirubinemia requiring exchange transfusion, persistent pulmonary hypertension associated with mechanical ventilation[aafp.org]
  • These include the following: acute otitis media, suppurative labyrinthitis and meningitis, vertigo, reparative granuloma, perilymph fistula, facial paralysis, fluctuating conductive hearing loss, persistent perforation of the tympanic membrane, taste[asams.org]

Treatment

  • CLOSE Medical Disclaimer The medical information on this site is provided as an information resource only, and is not to be used or relied on for any diagnostic or treatment purposes.[diseaseinfosearch.org]
  • You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.[rarediseases.info.nih.gov]
  • The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]

Prognosis

  • […] results with minimal complications. [4] [7] Last updated: 1/30/2017 Surgical treatment of isolated deafness with stapes fixation allows good functional results regardless of age or type of surgery. [4] If other symptoms or complications are present, prognosis[rarediseases.info.nih.gov]
  • Diagnosis - Progressive hearing loss stapes fixation Prognosis - Progressive hearing loss stapes fixation Not supplied. Treatment - Progressive hearing loss stapes fixation Not supplied.[checkorphan.org]
  • Prognosis OTSC treated surgically has a good prognosis in the vast majority of cases. The rate of hearing deterioration with time after stapedotomy does not exceed that due to presbyacusis [ 21 ] .[patient.info]
  • Prognosis In 90 of 100 patients, the operation is completely successful, in that the hearing is markedly improved. In 8 out of 100 patients, the hearing is improved, but not quite as much as hoped for.[bcm.edu]
  • Prognosis People with otosclerosis almost never become totally deaf, and will usually be able to hear with a hearing aid or with surgery plus a hearing aid.[medical-dictionary.thefreedictionary.com]

Etiology

  • Etiology The etiology of otosclerosis is still unknown. Otosclerosis appears to be a multifactorial disease. Autosomal dominant and a low penetrance (40%) transmission is observed in familial cases.[orpha.net]
  • What are 3 etiologies of sudden SNHL? Vascular occlusion Viral etiology Vestibular schwannoma 1% cases What is presbycusis? What causes it? Bilateral SNHL. Symmetric, slowly progressive. Cumulative effect of aging.[quizlet.com]
  • Etiology Pathophysiology Clinical features Symptoms may increase during pregnancy or following menopause because of hormonal changes! References: [1] [2] Diagnostics References: [1] [2] [4] Treatment[amboss.com]
  • "The etiology of otosclerosis: A combination of genes and environment". The Laryngoscope. 120 (6): 1195–202. doi : 10.1002/lary.20934. PMID 20513039.[en.wikipedia.org]

Epidemiology

  • Chapters on epidemiology, embryology, non-syndromic hearing loss, and syndromic forms of hearing loss have all been updated with particular attention to the vast amount of new information on molecular mechanisms, and chapters on clinical and molecular[books.google.com]
  • Summary Epidemiology The prevalence of symptomatic otosclerosis is estimated as 0.1 to 0.5% of the population in the United-States and Western Europe, thus this is not a rare disease.[orpha.net]
  • Epidemiology References: [1] [2] Epidemiological data refers to the US, unless otherwise specified. Etiology Pathophysiology Clinical features Symptoms may increase during pregnancy or following menopause because of hormonal changes![amboss.com]
  • Epidemiology Histological OTSC is present in around 2.5% of people [ 5 ]. However, the clinical manifestations are seen in only about 0.3%.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • Etiology Pathophysiology Clinical features Symptoms may increase during pregnancy or following menopause because of hormonal changes! References: [1] [2] Diagnostics References: [1] [2] [4] Treatment[amboss.com]
  • One genome-wide analysis associates otosclerosis with variation in RELN gene. [9] Loci include: Pathophysiology [ edit ] The pathophysiology of otosclerosis is complex.[en.wikipedia.org]
  • Pathophysiology: The primary pathological change occurs in the bony labyrinth with secondary effects upon middle ear and inner ear function.[drtbalu.co.in]
  • Pathophysiology of otosclerosis. Otol Neurotol 22:249–257. McKenna MJ, Kristiansen AG, Bartley ML, Rogus JJ, Haines JL.1998. Association of COL1A1 and otosclerosis: evidence for a shared genetic etiology with mild osteogenesis imperfecta.[dizziness-and-balance.com]

Prevention

  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.de]
  • […] characterized by hearing loss, fixation of stapes (ear bone which causes conductive deafness if it becomes fixed and immovable) and reduced vestibular response Symptoms - Progressive hearing loss stapes fixation Causes - Progressive hearing loss stapes fixation Prevention[checkorphan.org]
  • Although hearing improvement can be immediate, packing placed into the ear canal at the end of the surgery prevents patients from experiencing their new hearing for a week or so.[health.ucsd.edu]
  • In selected cases, medication may be prescribed in an attempt to prevent further hair cell loss and subsequently prevent further inner ear damage. This medication is sodium fluoride.[potomacaudiology.com]

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