Auditory Neuropathy
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Auditory Neuropathy
Auditory neuropathy (AN) is a hearing disorder in which the outer hair cells of the cochlea are present and functional, but sound information is not transmitted sufficiently by the auditory nerve to the brain. Hearing loss with AN can range from normal hearing sensitivity to profound hearing loss. A neuropathy usually refers to a disease of the peripheral nerve or nerves, but the auditory nerve itself is not always affected in auditory neuropathy spectrum disorders. Prevalence in the population is relatively unknown. Neonates with high risk factors for hearing loss have a prevalence of up to 40% (Vignesh, Jaya, & Muraleedharan 2016). These high-risk factors are: hypoxia, low birth weight, premature birth, hyperbilirubinemia, jaundice, and aminoglycoside antibiotic treatments (NIDCD, 2018). Possible sites of lesion Based on clinical testing of subjects with auditory neuropathy, the disruption in the stream of sound information has been localized to one or more of three probable l ...
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Cochlea
The cochlea is the part of the inner ear involved in hearing. It is a spiral-shaped cavity in the bony labyrinth, in humans making 2.75 turns around its axis, the modiolus. A core component of the cochlea is the Organ of Corti, the sensory organ of hearing, which is distributed along the partition separating the fluid chambers in the coiled tapered tube of the cochlea. The name cochlea derives . Structure The cochlea (plural is cochleae) is a spiraled, hollow, conical chamber of bone, in which waves propagate from the base (near the middle ear and the oval window) to the apex (the top or center of the spiral). The spiral canal of the cochlea is a section of the bony labyrinth of the inner ear that is approximately 30 mm long and makes 2 turns about the modiolus. The cochlear structures include: * Three ''scalae'' or chambers: ** the vestibular duct or ''scala vestibuli'' (containing perilymph), which lies superior to the cochlear duct and abuts the oval window ** the ty ...
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Decibel
The decibel (symbol: dB) is a relative unit of measurement equal to one tenth of a bel (B). It expresses the ratio of two values of a power or root-power quantity on a logarithmic scale. Two signals whose levels differ by one decibel have a power ratio of 101/10 (approximately ) or root-power ratio of 10 (approximately ). The unit expresses a relative change or an absolute value. In the latter case, the numeric value expresses the ratio of a value to a fixed reference value; when used in this way, the unit symbol is often suffixed with letter codes that indicate the reference value. For example, for the reference value of 1 volt, a common suffix is " V" (e.g., "20 dBV"). Two principal types of scaling of the decibel are in common use. When expressing a power ratio, it is defined as ten times the logarithm in base 10. That is, a change in ''power'' by a factor of 10 corresponds to a 10 dB change in level. When expressing root-power quantities, a change in ''ampl ...
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Sensorineural Hearing Impairment
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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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 ...
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Auditory Processing Disorder
Auditory processing disorder (APD), rarely known as King-Kopetzky syndrome or auditory disability with normal hearing (ADN), is a neurodevelopmental disorder affecting the way the brain processes auditory information. Individuals with APD usually have normal structure and function of the outer, middle, and inner ear (peripheral hearing). However, they cannot process the information they hear in the same way as others do, which leads to difficulties in recognizing and interpreting sounds, especially the sounds composing speech. It is thought that these difficulties arise from dysfunction in the central nervous system. It is highly prevalent in individuals with other neurodevelopmental disorders, such as Attention Deficit Hyperactivity Disorder, Autism Spectrum Disorders, Dyslexia, and Sensory Processing Disorder. The American Academy of Audiology notes that APD is diagnosed by difficulties in one or more auditory processes known to reflect the function of the central auditory nervo ...
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Auditory Neuropathy Spectrum Disorder
Auditory neuropathy spectrum disorder (ANSD) is a specific form of hearing loss defined by the presence of normal or near-normal otoacoustic emissions (OAEs) but the absence of normal middle ear reflexes and severely abnormal or completely absent auditory brainstem response (ABRs). Individuals presenting with this recently recognised hearing loss appear to display sporadic windows of hearing and not. Very few (1 in 14) will go on to develop normal speech and language but with poor speech perception in background noise and in others, no speech perception and therefore language development is possible. The condition was originally termed auditory neuropathy (AN) and in 2001 as Auditory Neuropathy / Auditory Dys-synchrony (AN/AD) (to include those cases where no true neuropathy was apparent). In 2008 at a meeting convened at Lake Como in Italy (Guidelines Development Conference on the Identification and Management of Infants with Auditory Neuropathy, International Newborn Hearing Sc ...
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Audiologist
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 diagnos ...
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Cochlear Implants
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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Hearing Aids
A hearing aid is a device designed to improve hearing by making sound audible to a person with hearing loss. Hearing aids are classified as medical devices in most countries, and regulated by the respective regulations. Small audio amplifiers such as personal sound amplification products (PSAPs) or other plain sound reinforcing systems cannot be sold as "hearing aids". Early devices, such as ear trumpets or ear horns, were passive amplification cones designed to gather sound energy and direct it into the ear canal. Modern devices are computerised electroacoustic systems that transform environmental sound to make it audible, according to audiometrical and cognitive rules. Modern devices also utilize sophisticated digital signal processing to try and improve speech intelligibility and comfort for the user. Such signal processing includes feedback management, wide dynamic range compression, directionality, frequency lowering, and noise reduction. Modern hearing aids require confi ...
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Friedreich's Ataxia
Friedreich's ataxia (FRDA or FA) is an autosomal-recessive genetic disease that causes difficulty walking, a loss of sensation in the arms and legs, and impaired speech that worsens over time. Symptoms generally start between 5 and 20 years of age. Many develop hypertrophic cardiomyopathy and require a mobility aid such as a cane, walker, or wheelchair in their teens. As the disease progresses, some affected people lose their sight and hearing. Other complications may include scoliosis and diabetes mellitus. The condition is caused by mutations in the ''FXN'' gene on chromosome 9, which makes a protein called frataxin. In FRDA, cells produce less frataxin. Degeneration of nerve tissue in the spinal cord causes the ataxia; particularly affected are the sensory neurons essential for directing muscle movement of the arms and legs through connections with the cerebellum. The spinal cord becomes thinner, and nerve cells lose some myelin sheath. No effective treatment is kn ...
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Electrocochleography
Electrocochleography (abbreviated ECochG or ECOG) is a technique of recording electrical potentials generated in the inner ear and auditory nerve in response to sound stimulation, using an electrode placed in the ear canal or tympanic membrane. The test is performed by an otologist or audiologist with specialized training, and is used for detection of elevated inner ear pressure (endolymphatic hydrops) or for the testing and monitoring of inner ear and auditory nerve function during surgery. Clinical applications The most common clinical applications of electrocochleography include: * Objective identification and monitoring of Ménière's disease and endolymphatic hydrops (EH) * Intraoperative monitoring of auditory system function during surgery on the brainstem or cerebellum * Enhancement of Wave I of the auditory brainstem response, particularly in patients who are hard of hearing * Diagnosis of auditory neuropathy Cochlear physiology The basilar membrane and the hair cell ...
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