Sensorineural Deafness
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Sensorineural Deafness
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 ...
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Otorhinolaryngology
Otorhinolaryngology ( , abbreviated ORL and also known as otolaryngology, otolaryngology–head and neck surgery (ORL–H&N or OHNS), or ear, nose, and throat (ENT)) is a surgical subspeciality within medicine that deals with the surgical and medical management of conditions of the head and neck. Doctors who specialize in this area are called otorhinolaryngologists, otolaryngologists, head and neck surgeons, or ENT surgeons or physicians. Patients seek treatment from an otorhinolaryngologist for diseases of the ear, nose, throat, base of the skull, head, and neck. These commonly include functional diseases that affect the senses and activities of eating, drinking, speaking, breathing, swallowing, and hearing. In addition, ENT surgery encompasses the surgical management of cancers and benign tumors and reconstruction of the head and neck as well as plastic surgery of the face and neck. Etymology The term is a combination of New Latin combining forms ('' oto-'' + '' rhino ...
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Presbycusis
Presbycusis (also spelled presbyacusis, from Greek πρέσβυς ''presbys'' "old" + ἄκουσις ''akousis'' "hearing"), or age-related hearing loss, is the cumulative effect of aging on hearing. It is a progressive and irreversible bilateral symmetrical age-related sensorineural hearing loss resulting from degeneration of the cochlea or associated structures of the inner ear or auditory nerves. The hearing loss is most marked at higher frequencies. Hearing loss that accumulates with age but is caused by factors other than normal aging ( nosocusis and sociocusis) is not presbycusis, although differentiating the individual effects of distinct causes of hearing loss can be difficult. The cause of presbycusis is a combination of genetics, cumulative environmental exposures and pathophysiological changes related to aging. At present there are no preventive measures known; treatment is by hearing aid or surgical implant. Presbycusis is the most common cause of hearing loss, affec ...
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X-linked
Sex linked describes the sex-specific patterns of inheritance and presentation when a gene mutation (allele) is present on a sex chromosome (allosome) rather than a non-sex chromosome (autosome). In humans, these are termed X-linked recessive, X-linked dominant and Y-linked. The inheritance and presentation of all three differ depending on the sex of both the parent and the child. This makes them characteristically different from autosomal dominance and recessiveness. There are many more X-linked conditions than Y-linked conditions, since humans have several times as many genes on the X chromosome than the Y chromosome. Only females are able to be carriers for X-linked conditions; males will always be affected by any X-linked condition, since they have no second X chromosome with a healthy copy of the gene. As such, X-linked recessive conditions affect males much more commonly than females. In X-linked recessive inheritance, a son born to a carrier mother and an unaffected f ...
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Dominant Gene
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 incomplet ...
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Recessive
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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Congenital
A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can range from mild to severe. Birth defects are divided into two main types: structural disorders in which problems are seen with the shape of a body part and functional disorders in which problems exist with how a body part works. Functional disorders include metabolic and degenerative disorders. Some birth defects include both structural and functional disorders. Birth defects may result from genetic or chromosomal disorders, exposure to certain medications or chemicals, or certain infections during pregnancy. Risk factors include folate deficiency, drinking alcohol or smoking during pregnancy, poorly controlled diabetes, and a mother over the age of 35 years old. Many are believed to involve multiple factors. Birth defects may be v ...
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American Academy Of Otolaryngology–Head And Neck Surgery
The American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS) is one of the largest of the world's many professional associations for medical specialists, with nearly 12,000 specialists in the area of otolaryngology (otorhinolaryngology) - caring for the ears, nose, and throat and surgery of the head and neck. The medical disorders treated by these physicians are among the most common that afflict all Americans, young and old. They include chronic ear infection, sinusitis, snoring and sleep apnea, hearing loss, allergies and hay fever, swallowing disorders, nosebleeds, hoarseness, dizziness, and head and neck cancer. Membership The Academy represents the interests of more than 12,000 ENT physicians worldwide and their patients. Membership categories range from board certified Physicians to Residents. Special rates are available for Military/Government and International Members. Membership benefits include a subscription to a scientific peer-monthly journal, th ...
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Meningitis
Meningitis is acute or chronic inflammation of the protective membranes covering the brain and spinal cord, collectively called the meninges. The most common symptoms are fever, headache, and neck stiffness. Other symptoms include confusion or altered consciousness, nausea, vomiting, and an inability to tolerate light or loud noises. Young children often exhibit only nonspecific symptoms, such as irritability, drowsiness, or poor feeding. A non-blanching rash (a rash that does not fade when a glass is rolled over it) may also be present. The inflammation may be caused by infection with viruses, bacteria or other microorganisms. Non-infectious causes include malignancy (cancer), subarachnoid haemorrhage, chronic inflammatory disease (sarcoidosis) and certain drugs. Meningitis can be life-threatening because of the inflammation's proximity to the brain and spinal cord; therefore, the condition is classified as a medical emergency. A lumbar puncture, in which a needle is ins ...
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Cochlear Implant
A cochlear implant (CI) is a surgically implanted neuroprosthesis that provides a person who has moderate-to-profound sensorineural hearing loss with sound perception. With the help of therapy, cochlear implants may allow for improved speech understanding in both quiet and noisy environments. A CI bypasses acoustic hearing by direct electrical stimulation of the auditory nerve. Through everyday listening and auditory training, cochlear implants allow both children and adults to learn to interpret those signals as speech and sound. The implant has two main components. The outside component is generally worn behind the ear, but could also be attached to clothing, for example, in young children. This component, the sound processor, contains microphones, electronics that include digital signal processor (DSP) chips, battery, and a coil that transmits a signal to the implant across the skin. The inside component, the actual implant, has a coil to receive signals, electronics, and an ar ...
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Audiology
Audiology (from Latin , "to hear"; and from Greek , ''-logia'') is a branch of science that studies hearing, balance, and related disorders. Audiologists treat those with hearing loss and proactively prevent related damage. By employing various testing strategies (e.g. behavioral hearing tests, otoacoustic emission measurements, and electrophysiologic tests), audiologists aim to determine whether someone has normal sensitivity to sounds. If hearing loss is identified, audiologists determine which portions of hearing (high, middle, or low frequencies) are affected, to what degree (severity of loss), and where the lesion causing the hearing loss is found ( outer ear, middle ear, inner ear, auditory nerve and/or central nervous system). If an audiologist determines that a hearing loss or vestibular abnormality is present, they will provide recommendations for interventions or rehabilitation (e.g. hearing aids, cochlear implants, appropriate medical referrals). In addition to d ...
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Audiometry
Audiometry () is a branch of audiology and the science of measuring hearing acuity for variations in sound intensity and pitch and for tonal purity, involving thresholds and differing frequencies. Typically, audiometric tests determine a subject's hearing levels with the help of an audiometer, but may also measure ability to discriminate between different sound intensities, recognize pitch, or distinguish speech from background noise. Acoustic reflex and otoacoustic emissions may also be measured. Results of audiometric tests are used to diagnose hearing loss or diseases of the ear, and often make use of an audiogram. History The basic requirements of the field were to be able to produce a repeating sound, some way to attenuate the amplitude, a way to transmit the sound to the subject, and a means to record and interpret the subject's responses to the test. Mechanical "acuity meters" and tuning forks For many years there was desultory use of various devices capable of pro ...
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Tympanometry
Tympanometry is an acoustic evaluation of the condition of the middle ear eardrum (tympanic membrane) and the conduction bones by creating variations of air pressure in the ear canal. Tympanometry is an objective test of middle-ear function. It is not a hearing test, but rather a measure of energy transmission through the middle ear. It is not a measure of TM or middle ear mobility. It is an acoustic measure, measured by a microphone, as part of the ear canal probe, inserted into the ear canal. The test should not be used to assess the sensitivity of hearing and the results of this test should always be viewed in conjunction with pure tone audiometry. Tympanometry is a valuable component of the audiometric evaluation. In evaluating hearing loss, tympanometry permits a distinction between sensorineural and conductive hearing loss, when evaluation is not apparent via Weber and Rinne testing. Furthermore, in a primary care setting, tympanometry can be helpful in making the diagnosi ...
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