Otospongiosis
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Otospongiosis
Otosclerosis is a condition of the middle ear where portions of the dense enchondral layer of the bony labyrinth remodel into one or more lesions of irregularly-laid spongy bone. As the lesions reach the stapes the bone is resorbed, then hardened ( sclerotized), which limits its movement and results in hearing loss, tinnitus, vertigo or a combination of symptoms. The term otosclerosis is something of a misnomer: much of the clinical course is characterized by lucent rather than sclerotic bony changes, so the disease is also known as otospongiosis. Etymology The word ''otosclerosis'' derives from Greek ὠτός (''ōtos''), genitive of οὖς (''oûs'') "ear" + σκλήρωσις (''sklērōsis''), "hardening". Presentation The primary form of hearing loss in otosclerosis is conductive hearing loss (CHL) whereby sounds reach the ear drum but are incompletely transferred via the ossicular chain in the middle ear, and thus partly fail to reach the inner ear (cochlea). This can af ...
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Bony Labyrinth
The bony labyrinth (also osseous labyrinth or otic capsule) is the rigid, bony outer wall of the inner ear in the temporal bone. It consists of three parts: the vestibule, semicircular canals, and cochlea. These are cavities hollowed out of the substance of the bone, and lined by periosteum. They contain a clear fluid, the perilymph, in which the membranous labyrinth is situated. A fracture classification system in which temporal bone fractures detected by computed tomography are delineated based on disruption of the otic capsule has been found to be predictive for complications of temporal bone trauma such as facial nerve injury, sensorineural deafness and cerebrospinal fluid otorrhea. On radiographic images, the otic capsule is the densest portion of the temporal bone. In otospongiosis, a leading cause of adult-onset hearing loss, the otic capsule is exclusively affected. This area normally undergoes no remodeling in adult life and is extremely dense. With otospongiosis, the ...
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Schwartz Sign
Schwartz sign, also known as Flemingo's pink sign, is a diagnostic indicator for otosclerosis, a disease of the bones of the middle or inner ear. In clinical examination of the ear drum, increased vascularity of the promontory may be seen through the ear drum. This sign is known as Flemingo's flush sign or Schwartz's sign. This indicates otospongiosis (active otosclerosis). In about 10% of cases of otosclerosis, there is a redness of the promontory of the cochlea seen through the tympanic membrane due to prominent vascularity associated with an otospongiotic focus. Be aware of the similar, Brown's sign. This is a red retro-tympanic bulge that blanches on pressure via pneumatic otoscopy. This is secondary to paragangliomata of the middle ear. References {{reflist Diseases of inner ear ...
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Stapes
The ''stapes'' or stirrup is a bone in the middle ear of humans and other animals which is involved in the conduction of sound vibrations to the inner ear. This bone is connected to the oval window by its annular ligament, which allows the footplate to transmit sound energy through the oval window into the inner ear. The ''stapes'' is the smallest and lightest bone in the human body, and is so-called because of its resemblance to a stirrup ( la, Stapes). Structure The ''stapes'' is the third bone of the three ossicles in the middle ear and the smallest in the human body. It measures roughly , greater along the head-base span. It rests on the oval window, to which it is connected by an annular ligament and articulates with the ''incus'', or anvil through the incudostapedial joint. They are connected by anterior and posterior limbs ( la, crura). Development The ''stapes'' develops from the second pharyngeal arch during the sixth to eighth week of embryological life. The ce ...
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Sensorineural Hearing Loss
Sensorineural hearing loss (SNHL) is a type of hearing loss in which the root cause lies in the inner ear or sensory organ (cochlea and associated structures) or the vestibulocochlear nerve (cranial nerve VIII). SNHL accounts for about 90% of reported hearing loss . SNHL is usually permanent and can be mild, moderate, severe, profound, or total. Various other descriptors can be used depending on the shape of the audiogram, such as high frequency, low frequency, U-shaped, notched, peaked, or flat. ''Sensory'' hearing loss often occurs as a consequence of damaged or deficient cochlear hair cells. Hair cells may be abnormal at birth or damaged during the lifetime of an individual. There are both external causes of damage, including infection, and ototoxic drugs, as well as intrinsic causes, including genetic mutations. A common cause or exacerbating factor in SNHL is prolonged exposure to environmental noise, or noise-induced hearing loss. Exposure to a single very loud noise such as ...
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Pure Tone Audiometry
Pure-tone audiometry is the main hearing test used to identify hearing threshold levels of an individual, enabling determination of the degree, type and configuration of a hearing loss and thus providing a basis for diagnosis and management. Pure-tone audiometry is a subjective, behavioural measurement of a hearing threshold, as it relies on patient responses to ''pure tone'' stimuli. Therefore, pure-tone audiometry is only used on adults and children old enough to cooperate with the test procedure. As with most clinical tests, standardized calibration of the test environment, the equipment and the stimuli is needed before testing proceeds (in reference to ISO, ANSI, or other standardization body). Pure-tone audiometry only measures audibility thresholds, rather than other aspects of hearing such as sound localization and speech recognition. However, there are benefits to using pure-tone audiometry over other forms of hearing test, such as click auditory brainstem response (ABR). ...
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Oval Window
The oval window (or ''fenestra vestibuli'' or ''fenestra ovalis'') is a membrane-covered opening from the middle ear to the cochlea of the inner ear. Vibrations that contact the tympanic membrane travel through the three ossicles and into the inner ear. The oval window is the intersection of the middle ear with the inner ear and is directly contacted by the ''stapes''; by the time vibrations reach the oval window, they have been reduced in amplitude and increased in force due to the lever action of the ossicle bones. This is not an amplification function, as often incorrectly reported. Rather, it is an impedance-matching function, allowing sound to be transferred from air (outer ear) to liquid (cochlea). It is a reniform (kidney-shaped) opening leading from the tympanic cavity into the vestibule of the internal ear; its long diameter is horizontal and its convex border is upward. It is occupied by the base of the ''stapes'', the circumference of which is fixed by the annular l ...
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Measles Virus
''Measles morbillivirus'' (MeV), also called measles virus (MV), is a single-stranded, negative-sense, enveloped, non-segmented RNA virus of the genus ''Morbillivirus'' within the family ''Paramyxoviridae''. It is the cause of measles. Humans are the natural hosts of the virus; no animal reservoirs are known to exist. Disease The virus causes measles, a highly contagious disease transmitted by respiratory aerosols that triggers a temporary but severe immunosuppression. Symptoms include fever, cough, runny nose, inflamed eyes and a generalized, maculopapular, erythematous rash and a pathognomic Koplik spot seen on buccal mucosa opposite to lower 1 st and 2 nd molars . The virus is spread by coughing and sneezing via close personal contact or direct contact with secretions. Replication cycle Entry The measles virus has two envelope glycoproteins on the viral surface – hemagglutinin (H) and membrane fusion protein (F). These proteins are responsible for host cell ...
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Hypervascular
Hypervascularity is an increased number or concentration of blood vessels. In Graves disease, the thyroid gland is hypervascular, which can help in differentiating the condition from thyroiditis. 90% of thyroid papillary carcinoma cases are hypervascular. See also * Angiogenesis Angiogenesis is the physiological process through which new blood vessels form from pre-existing vessels, formed in the earlier stage of vasculogenesis. Angiogenesis continues the growth of the vasculature by processes of sprouting and splitting ... References Hematology Oncology Angiology {{circulatory-stub ...
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Paget's Disease Of Bone
Paget's disease of bone (commonly known as Paget's disease or, historically, osteitis deformans) is a condition involving cellular remodeling and deformity of one or more bones. The affected bones show signs of dysregulated bone remodeling at the microscopic level, specifically excessive bone breakdown and subsequent disorganized new bone formation. These structural changes cause the bone to weaken, which may result in deformity, pain, bone fractures, fracture or arthritis of associated joints. The exact cause is unknown, although leading theories indicate both genetic and acquired factors (see Paget's disease of bone#Causes, Causes). Paget's disease may affect any one or several bones of the body (most commonly pelvis, tibia, femur, lumbar vertebrae, and skull), but never the entire skeleton, and does not spread from bone to bone. Rarely, a bone affected by Paget's disease can transform into a Osteosarcoma, malignant bone cancer. As the disease often affects people differently, ...
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Temporal Bone
The temporal bones are situated at the sides and base of the skull, and lateral to the temporal lobes of the cerebral cortex. The temporal bones are overlaid by the sides of the head known as the temples, and house the structures of the ears. The lower seven cranial nerves and the major vessels to and from the brain traverse the temporal bone. Structure The temporal bone consists of four parts— the squamous, mastoid, petrous and tympanic parts. The squamous part is the largest and most superiorly positioned relative to the rest of the bone. The zygomatic process is a long, arched process projecting from the lower region of the squamous part and it articulates with the zygomatic bone. Posteroinferior to the squamous is the mastoid part. Fused with the squamous and mastoid parts and between the sphenoid and occipital bones lies the petrous part, which is shaped like a pyramid. The tympanic part is relatively small and lies inferior to the squamous part, anterior to the mast ...
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OMIM
Online Mendelian Inheritance in Man (OMIM) is a continuously updated catalog of human genes and genetic disorders and traits, with a particular focus on the gene-phenotype relationship. , approximately 9,000 of the over 25,000 entries in OMIM represented phenotypes; the rest represented genes, many of which were related to known phenotypes. Versions and history OMIM is the online continuation of Dr. Victor A. McKusick's ''Mendelian Inheritance in Man'' (MIM), which was published in 12 editions between 1966 and 1998.McKusick, V. A. ''Mendelian Inheritance in Man. Catalogs of Autosomal Dominant, Autosomal Recessive and X-Linked Phenotypes.'' Baltimore, MD: Johns Hopkins University Press, 1st ed, 1996; 2nd ed, 1969; 3rd ed, 1971; 4th ed, 1975; 5th ed, 1978; 6th ed, 1983; 7th ed, 1986; 8th ed, 1988; 9th ed, 1990; 10th ed, 1992. Nearly all of the 1,486 entries in the first edition of MIM discussed phenotypes. MIM/OMIM is produced and curated at the Johns Hopkins School of Medicine ...
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Reelin
Reelin, encoded by the ''RELN'' gene, is a large secreted extracellular matrix glycoprotein that helps regulate processes of neuronal migration and positioning in the developing brain by controlling cell–cell interactions. Besides this important role in early development, reelin continues to work in the adult brain. It modulates synaptic plasticity by enhancing the induction and maintenance of long-term potentiation. It also stimulates dendrite and dendritic spine development and regulates the continuing migration of neuroblasts generated in adult neurogenesis sites like the subventricular and subgranular zones. It is found not only in the brain but also in the liver, thyroid gland, adrenal gland, Fallopian tube, breast and in comparatively lower levels across a range of anatomical regions. Reelin has been suggested to be implicated in pathogenesis of several brain diseases. The expression of the protein has been found to be significantly lower in schizophrenia and psycho ...
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