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Otosclerosis
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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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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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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Sclerosis (medicine)
Sclerosis (from Greek σκληρός ''sklērós'', "hard") is the stiffening of a tissue or anatomical feature, usually caused by a replacement of the normal organ-specific tissue with connective tissue. The structure may be said to have undergone sclerotic changes or display sclerotic lesions, which refers to the process of sclerosis. Common medical conditions whose pathology involves sclerosis include: * Amyotrophic lateral sclerosis—also known as Lou Gehrig's disease or motor neurone disease—a progressive, incurable, usually fatal disease of motor neurons. * Atherosclerosis, a deposit of fatty materials, such as cholesterol, in the arteries which causes hardening. * Focal segmental glomerulosclerosis is a disease that attacks the kidney's filtering system (glomeruli) causing serious scarring and thus a cause of nephrotic syndrome in children and adolescents, as well as an important cause of kidney failure in adults. * Hippocampal sclerosis, a brain damage often seen in ...
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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 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 har ..., 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 ...
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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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Conductive Hearing Loss
Conductive hearing loss (CHL) occurs when there is a problem transferring sound waves anywhere along the pathway through the outer ear, tympanic membrane (eardrum), or middle ear ( ossicles). If a conductive hearing loss occurs in conjunction with a sensorineural hearing loss, it is referred to as a mixed hearing loss. Depending upon the severity and nature of the conductive loss, this type of hearing impairment can often be treated with surgical intervention or pharmaceuticals to partially or, in some cases, fully restore hearing acuity to within normal range. However, cases of permanent or chronic conductive hearing loss may require other treatment modalities such as hearing aid devices to improve detection of sound and speech perception. Causes Common causes of conductive hearing loss include: External ear * Cerumen (earwax) or foreign body in the external auditory canal * Otitis externa, infection or irritation of the outer ear * Exostoses, abnormal growth of bone withi ...
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Tinnitus
Tinnitus is the perception of sound when no corresponding external sound is present. Nearly everyone experiences a faint "normal tinnitus" in a completely quiet room; but it is of concern only if it is bothersome, interferes with normal hearing, or is associated with other problems. While often described as a ringing, it may also sound like a clicking, buzzing, hissing or roaring. It may be soft or loud, low- or high- pitched, and may seem to come from one or both ears or from the head itself. In some people, it may interfere with concentration, and in some cases is associated with anxiety and depression. Tinnitus is usually associated with a degree of hearing loss and decreased comprehension of speech in noisy environments. It is common, affecting about 10–15% of people. Most, however, tolerate it well, and it is a significant problem in only 1–2% of all people. It can trigger a fight-or-flight response, as the brain may perceive it as dangerous and important. The word ' ...
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Audiogram
An audiogram is a graph that shows the audible threshold for standardized frequencies as measured by an audiometer. The Y axis represents intensity measured in decibels and the X axis represents frequency measured in hertz. The threshold of hearing is plotted relative to a standardised curve that represents 'normal' hearing, in dB(HL). They are not the same as equal-loudness contours, which are a set of curves representing equal loudness at different levels, as well as at the threshold of hearing, in absolute terms measured in dB SPL (sound pressure level). Audiograms are set out with frequency in hertz (Hz) on the horizontal axis, most commonly on a logarithmic scale, and a linear dBHL scale on the vertical axis. For humans, normal hearing is between −10 dB(HL) and 15 dB(HL), although 0 dB from 250 Hz to 8 kHz is deemed to be 'average' normal hearing. Hearing thresholds of humans and other mammals can be found with behavioural hearing tests or physi ...
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Bone Resorption
Bone resorption is resorption of bone tissue, that is, the process by which osteoclasts break down the tissue in bones and release the minerals, resulting in a transfer of calcium from bone tissue to the blood. The osteoclasts are multi-nucleated cells that contain numerous mitochondria and lysosomes. These are the cells responsible for the resorption of bone. Osteoblasts are generally present on the outer layer of bone, just beneath the periosteum. Attachment of the osteoclast to the osteon begins the process. The osteoclast then induces an infolding of its cell membrane and secretes collagenase and other enzymes important in the resorption process. High levels of calcium, magnesium, phosphate and products of collagen will be released into the extracellular fluid as the osteoclasts tunnel into the mineralized bone. Osteoclasts are prominent in the tissue destruction found in psoriatic arthritis and rheumatological disorders. The human body is in a constant state of bone remod ...
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Autosomal Dominant
In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the effect of a different variant of the same gene on the other copy of the chromosome. The first variant is termed dominant and the second recessive. This state of having two different variants of the same gene on each chromosome is originally caused by a mutation in one of the genes, either new (''de novo'') or inherited. The terms autosomal dominant or autosomal recessive are used to describe gene variants on non-sex chromosomes ( autosomes) and their associated traits, while those on sex chromosomes (allosomes) are termed X-linked dominant, X-linked recessive or Y-linked; these have an inheritance and presentation pattern that depends on the sex of both the parent and the child (see Sex linkage). Since there is only one copy of the Y chromosome, Y-linked traits cannot be dominant or recessive. Additionally, there are other forms of dominance such as incomplete d ...
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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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Middle Ear
The middle ear is the portion of the ear medial to the eardrum, and distal to the oval window of the cochlea (of the inner ear). The mammalian middle ear contains three ossicles, which transfer the vibrations of the eardrum into waves in the fluid and membranes of the inner ear. The hollow space of the middle ear is also known as the tympanic cavity and is surrounded by the tympanic part of the temporal bone. The auditory tube (also known as the Eustachian tube or the pharyngotympanic tube) joins the tympanic cavity with the nasal cavity (nasopharynx), allowing pressure to equalize between the middle ear and throat. The primary function of the middle ear is to efficiently transfer acoustic energy from compression waves in air to fluid–membrane waves within the cochlea. Structure Ossicles The middle ear contains three tiny bones known as the ossicles: '' malleus'', '' incus'', and ''stapes''. The ossicles were given their Latin names for their distinctive shapes; they ar ...
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