A Web Resource and Blog For Parents of Children With Auditory Neuropathy Spectrum Disorder (ANSD)
ABR – Auditory brainstem response audiometry is a screening test to monitor for hearing loss or deafness, especially notable for its use with newborn infants. It is a method employed to assess the functions of the ears, cranial nerves, and various brain functions of the lower part of the auditory system, prior to the child developing to the point of describing a possible hearing problem.
ANSD – Auditory Neuropathy Spectrum Disorder. Originally referred to as Auditory Neuropathy (AN) and later Auditory Neuropathy/Auditory Dyssynchrony (AN/AD)
CAEP – Cortical Auditory Evoked Potentials are brain waves representing higher-level activity in auditory areas of the brain, measured using sensors placed on the head. CAEPs can be used to assess the cognitive processing involved in the identification and discrimination of speech sounds. CAEPs can be reliably recorded in infants and therefore are a useful tool for objectively evaluating CI benefit in this population.
CI – a Cochlear Implant is a surgically implanted electronic device that provides a sense of sound to a person who is profoundly deaf or severely hard of hearing. The cochlear implant is often referred to as a bionic ear. Unlike hearing aids, the cochlear implant does not amplify sound, but works by directly stimulating any functioning auditory nerves inside the cochlea with electric field stimulated through an electric impulse. External components of the cochlear implant include a microphone, speech processor and an RF transmitter. Similarly an RF receiver is implanted beneath the skull’s skin. The transmitter has a piece of magnet by which it attaches to another magnet placed beside the receiver. When the receiver gets a signal, it will be transmitted to the implanted electrodes in the cochlea. The speech processor allows an individual to adjust the sound level of sensitivity.
CMV – CMV is an abbreviation for cytomegalovirus. If a mother gets a CMV infection for the first time in her life while pregnant, it can be passed to the fetus during birth. Most pregnant women will not have any symptoms, and those that do have symptoms experience something similar to mononucleosis. Fortunately, according to the Centers for Disease Control and Prevention (CDC), only a small percentage (1% to 4%) of uninfected pregnant women will become infected with CMV. At birth, the newborn may not have any symptoms. If it does, the symptoms usually include jaundice or an enlarged liver or spleen. As the baby grows, the damage caused by the CMV becomes evident — mental and physical disabilities (including hearing loss) appear.
DFNB59 – The DFNB59 gene provides instructions for making a protein called pejvakin. This protein is present in the nerves leading from the inner ear to the brain (auditory nerves). It is also found in the part of the brain that is connected to the spinal cord (the brainstem). Although the exact function of pejvakin is uncertain, it appears to be essential for normal hearing. It is the second most common genetic cause of ANSD.
OAE – An otoacoustic emission (OAE) is a sound which is generated from within the inner ear. Having been predicted by Thomas Gold in 1948, their existence was first demonstrated experimentally by David Kemp in 1978 and they have since been shown to arise by a number of different cellular mechanisms within the inner ear. Numerous studies have shown that OAEs disappear after the inner ear has been damaged, so OAEs are often used in the laboratory and the clinic as a measure of inner ear health.
OTOF – The OTOF gene provides instructions for making a protein called otoferlin. This protein is present in the brain and the cochlea, which is a snail-shaped structure in the inner ear that helps process sound. Although the exact function of otoferlin is uncertain, it appears to be essential for normal hearing. Researchers believe that otoferlin may play a role in releasing chemical signals (neurotransmitters) from nerve cells that are involved in hearing. A mutation in the OTOF gene is the largest single genetic cause of ANSD.
PLS – A test of your child’s expressive and receptive language skill measured against the normal distribution. More information here
Stapedial Reflex – is an involuntary muscle contraction that occurs in the middle ear of mammals in response to high-intensity sound stimuli.
VRA – Visual Reinforcement Audiometry is a behavioral audiometric test obtained in a sound-treated room. The child is seated on a parent’s lap or in a chair between two calibrated loudspeakers, or using earphones. When a sound such as a tone at a specific frequency, speech, or music is presented, the infant’s eye-shift or head-turn response toward the sound source is rewarded by activation of a lighted mechanical toy mounted near the loudspeaker. The child’s attention then is distracted back to the midline so that additional sounds can be presented