Hearing Loss With Craniofacial Syndromes
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Hearing Loss With Craniofacial Syndromes
Hearing loss with craniofacial syndromes is a common occurrence. Many of these multianomaly disorders involve structural malformations of the outer or middle ear, making a significant hearing loss highly likely. Treacher Collins syndrome Individuals with Treacher Collins syndrome often have both cleft palate and hearing loss, in addition to other disabilities. Hearing loss is often secondary to absent, small or unusually formed ears (microtia) and commonly results from malformations of the middle ear. Researchers have found that most patients with Treacher Collins syndrome have symmetric external ear canal abnormalities and symmetrically dysmorphic or absent ossicles in the middle ear space. Inner ear structure is largely normal. Most patients show a moderate hearing impairment or greater, and the type of loss is generally a conductive hearing loss. Patients with Treacher Collins syndrome exhibit hearing losses similar to those of patients with malformed or missing ossicles (Pr ...
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Outer Ear
The outer ear, external ear, or auris externa is the external part of the ear, which consists of the auricle (also pinna) and the ear canal. It gathers sound energy and focuses it on the eardrum (tympanic membrane). Structure Auricle The visible part is called the auricle, also known as the pinna, especially in other animals. It is composed of a thin plate of yellow elastic cartilage, covered with integument, and connected to the surrounding parts by ligaments and muscles; and to the commencement of the ear canal by fibrous tissue. Many mammals can move the pinna (with the auriculares muscles) in order to focus their hearing in a certain direction in much the same way that they can turn their eyes. Most humans do not have this ability. Ear canal From the pinna, the sound waves move into the ear canal (also known as the ''external acoustic meatus'') a simple tube running through to the middle ear. This tube leads inward from the bottom of the auricula and conducts the ...
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Vestibule Of The Ear
The vestibule is the central part of the bony labyrinth in the inner ear, and is situated medial to the eardrum, behind the cochlea, and in front of the three semicircular canals. The name comes from the Latin ', literally an entrance hall. Structure The vestibule is somewhat oval in shape, but flattened transversely; it measures about 5 mm from front to back, the same from top to bottom, and about 3 mm across. In its lateral or tympanic wall is the oval window, closed, in the fresh state, by the base of the stapes and annular ligament. On its medial wall, at the forepart, is a small circular depression, the recessus sphæricus, which is perforated, at its anterior and inferior part, by several minute holes (macula cribrosa media) for the passage of filaments of the acoustic nerve to the saccule; and behind this depression is an oblique ridge, the crista vestibuli, the anterior end of which is named the pyramid of the vestibule. This ridge bifurcates below to enclose ...
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22q11
Q11 may refer to: * Q11 (New York City bus) * , a survey ship of the Argentine Navy * DZOE-TV, formerly Q-11 * , an armed yacht of the Royal Canadian Navy * Hud (surah) Hud ( ar, هود, ), is the 11th chapter (''Surah'') of the Quran and has 123 verses ('' ayat''). It relates in part to the prophet Hud. Regarding the timing and contextual background of the revelation (''asbāb al-nuzūl''), it is an earlier " ..., of the Quran * Motorola Q11, a Motorola smartphone {{Letter-NumberCombDisambig ...
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Eustachian Tube
In anatomy, the Eustachian tube, also known as the auditory tube or pharyngotympanic tube, is a tube that links the nasopharynx to the middle ear, of which it is also a part. In adult humans, the Eustachian tube is approximately long and in diameter. It is named after the sixteenth-century Italian anatomist Bartolomeo Eustachi. In humans and other tetrapods, both the middle ear and the ear canal are normally filled with air. Unlike the air of the ear canal, however, the air of the middle ear is not in direct contact with the atmosphere outside the body; thus, a pressure difference can develop between the atmospheric pressure of the ear canal and the middle ear. Normally, the Eustachian tube is collapsed, but it gapes open with swallowing and with positive pressure, allowing the middle ear's pressure to adjust to the atmospheric pressure. When taking off in an aircraft, the ambient air pressure goes from higher (on the ground) to lower (in the sky). The air in the middle ear ...
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Velopharyngeal Inadequacy
Velopharyngeal inadequacy is a malfunction of a velopharyngeal mechanism which is responsible for directing the transmission of sound energy and air pressure in both the oral cavity and the nasal cavity. When this mechanism is impaired in some way, the valve does not fully close, and a condition known as 'velopharyngeal inadequacy' can develop. VPI can either be congenital or acquired later in life. Presentation Relationship to cleft palate A cleft palate is one of the most common causes of VPI. Cleft palate is an anatomical abnormality that occurs in utero and is present at birth. This malformation can affect the lip and palate, or the palate only. A cleft palate can affect the mobility of the velopharyngeal valve, thereby resulting in VPI. Causes While cleft is the most common cause of VPI, other significant etiologies exist. These other causes are outlined in the chart below: Diagnosis Classification The most frequent types of cleft palates are overt, submucous, and occult su ...
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Cleft Lip And Palate
A cleft lip contains an opening in the upper lip that may extend into the nose. The opening may be on one side, both sides, or in the middle. A cleft palate occurs when the palate (the roof of the mouth) contains an opening into the nose. The term orofacial cleft refers to either condition or to both occurring together. These disorders can result in feeding problems, speech problems, hearing problems, and frequent ear infections. Less than half the time the condition is associated with other disorders. Cleft lip and palate are the result of tissues of the face not joining properly during development. As such, they are a type of birth defect. The cause is unknown in most cases. Risk factors include smoking during pregnancy, diabetes, obesity, an older mother, and certain medications (such as some used to treat seizures). Cleft lip and cleft palate can often be diagnosed during pregnancy with an ultrasound exam. A cleft lip or palate can be successfully treated with surge ...
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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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Hypoplasia
Hypoplasia (from Ancient Greek ὑπo- ''hypo-'' 'under' + πλάσις ''plasis'' 'formation'; adjective form ''hypoplastic'') is underdevelopment or incomplete development of a tissue or organ.Definition: hypoplasia
Dictionary of Cell and Molecular Biology (11 March 2008) Although the term is not always used precisely, it properly refers to an inadequate or below-normal number of cells.Hypoplasia
Stedman's Medical Dictionary. lww.com
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Auditory Canal
The ear canal (external acoustic meatus, external auditory meatus, EAM) is a pathway running from the outer ear to the middle ear. The adult human ear canal extends from the pinna to the eardrum and is about in length and in diameter. Structure The human ear canal is divided into two parts. The elastic cartilage part forms the outer third of the canal; its anterior and lower wall are cartilaginous, whereas its superior and back wall are fibrous. The cartilage is the continuation of the cartilage framework of pinna. The cartilaginous portion of the ear canal contains small hairs and specialized sweat glands, called apocrine glands, which produce cerumen ( ear wax). The bony part forms the inner two thirds. The bony part is much shorter in children and is only a ring (''annulus tympanicus'') in the newborn. The layer of epithelium encompassing the bony portion of the ear canal is much thinner and therefore, more sensitive in comparison to the cartilaginous portion. Size and sh ...
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Atresia
Atresia is a condition in which an orifice or passage in the body is (usually abnormally) closed or absent. Examples of atresia include: *Aural atresia, a congenital deformity where the ear canal is underdeveloped. * Biliary atresia, a condition in newborns in which the common bile duct between the liver and the small intestine is blocked or absent. * Congenital bronchial atresia, a rare congenital abnormality * Choanal atresia, blockage of the back of the nasal passage, usually by abnormal bony or soft tissue. * Esophageal atresia, which affects the alimentary tract and causes the esophagus to end before connecting normally to the stomach. *Follicular atresia, degeneration and resorption of the ovarian follicles.   * Imperforate anus, malformation of the opening between the rectum and anus. * Intestinal atresia, malformation of the intestine, usually resulting from a vascular accident in utero. * Microtia, absence of the ear canal or failure of the canal to be tubular or fully fo ...
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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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Tympanic Membrane
In the anatomy of humans and various other tetrapods, the eardrum, also called the tympanic membrane or myringa, is a thin, cone-shaped membrane that separates the external ear from the middle ear. Its function is to transmit sound from the air to the ossicles inside the middle ear, and then to the oval window in the fluid-filled cochlea. Hence, it ultimately converts and amplifies vibration in the air to vibration in cochlear fluid. The malleus bone bridges the gap between the eardrum and the other ossicles. Rupture or perforation of the eardrum can lead to conductive hearing loss. Collapse or retraction of the eardrum can cause conductive hearing loss or cholesteatoma. Structure Orientation and relations The tympanic membrane is oriented obliquely in the anteroposterior, mediolateral, and superoinferior planes. Consequently, its superoposterior end lies lateral to its anteroinferior end. Anatomically, it relates superiorly to the middle cranial fossa, posteriorly to the o ...
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