Nonsyndromic Hearing Loss

The ear is an extraordinary human organ that many people take for granted until it doesn’t function. It is the only device that allows the human to hear sounds in their environment. The ear is made up of many parts that distinguish various sounds through different means. The ear anatomy and physiology along with how sound waves are transmitted into meaningful sounds will help one understand how hearing loss occurs.

Conductive hearing loss is from one or more deformity with the outer, inner, or middle ear which impacts sound from transferring to the nerves 5. Conductive hearing loss is often corrected with surgery, drugs, or hearing aids. Sensorineural hearing loss affects the cochlea, which transfers the sounds vibrations to the nerves 5. Although hearing aids are typically used for conductive hearing loss, a profoundly deaf sensorineural patient needs to complete a 6-month trial with them before they are considered ineffective and referred on for further testing for cochlear implant candidacy 5. Sensorineural hearing loss is primarily due to a malfunctioning cochlea so doctors run a hearing test to see how many decibels can be picked up by the cochlea. For pediatric patients to qualify for implants they need to be considered profoundly deaf in both ears. The tone threshold for mild hearing loss is between 21 and 40 dBHL (decibels Hearing Level) and profound hearing loss is greater than 90 dBHL 5. Typically, a pediatric patient looking for cochlear implants will go through a battery of speech-recognition testing while simultaneously undergoing the audiological

Otosclerosis is a disorder that is inherited that can cause loss of hearing because of lack

As an undergraduate, I performed research on deafness, investigating hearing regeneration and unique auditory phenotypes. Based on my work, I was selected to be a Post-Baccalaureate Fellow at the National Institute on Deafness and Other Communication Disorders within the National Institutes of Health. This is a two-year program (07/14-07/16). I lead three projects focused on novel clinical therapies to prevent hearing loss from ototoxic drugs—drugs that cause hearing loss in hundreds of thousands of patients. There exists the need for therapies that protect the inner ear from ototoxic drugs without altering their therapeutic effects, and the focus of my research has been to help develop novel therapies.

Cochlear implants, the surgical device which the media has frequently offered as the miracle cure for deafness, has been utilized as a treatment for hearing loss since the early 1960s (Paludnevicience & Harris, 2011, p.100). In patients with a hearing loss due to damage of hair cells in the inner ear, cochlear prostheses are able to provide some degree of hearing restoration by bypassing these structures and transmitting sounds directly to the auditory nerve (Paludneviciene & Harris, 2011, p. 100). With its start in the mid twentieth century, the technological resources available to the creators of cochlear implants limited their ability to provide patients a natural hearing experience. Though they initially provided only limited gains to deaf

Given that there is no cure for sensorineural hearing loss, the goal of AR is not to fix the hearing impairment or to restore perfect hearing. As such, AR does not fit well within a traditional medical model of health, which focuses on the restoration of physical function. AR fits better within the WHO (2001) ICF model, a biopsychosocial classification of health (Gagne, Jennings & Southall, 2009). The WHO ICF is particularly well suited to chronic conditions, such as hearing loss, which involve “impaired body structures or functions that cannot return to normalcy” (Gagne, & Jennings, 2011, The ICF: A Conceptual Framework, para. 1). This model allows for consideration of factors beyond the impairment of body structures

More than 250 million people in the world are born deaf, the hearing loss is generally caused by damage to sensory receptors (hair cells) and nerves associated with them (5). The basic function of hair cells (cells Mkanvsnsvry) within the cochlea of ​​the inner ear, with that mechanical vibrations of sound into electrical signals, then these messages reach the brain through the auditory nerve. When the hair cells are damaged, a person with hearing loss or deafness. It may also fix the cause, these cells have been rebuilt and to continue their activities. But in most cases this is not possible and will be a permanent hearing

When outer hair cells are damaged, compression of cochlea is diminished. The Basilar membrane is broadly tuned and becomes linear in case of total damage to OHC. Because of which, there is a less gain or no gain for low intensity levels. Also, the threshold level elevates from normal threshold level and reduces the dynamic range. In a condition called loudness recruitment, the loudness growth near the threshold is same as normal or other hearing impaired, but abnormally high at the elevated thresholds (Buus & Florentine,2002). This loudness growth observed in hearing impaired is very faster than in normal hearing. This is predominantly seen in permanent sensorineural hearing loss (Oxenham and Plack, 1997 & Nelson et al.2001). The overall effects of damaged outer hair cells are reduced

Hearing Loss is characterized as the diminished or inability to process sound-related data. An imperfection at hearing level in a living organism can prompt to hearing loss. Speech is the essential method of correspondence between people. Thus the capacity to hear and convey makes the ear most imperative for an individual’s development. Hence hearing and communicating plays an important role in an intellectual’s growth. The non-syndromic hearing loss is not related with any signs or side effects as syndromic hearing loss which is a dangerous condition. An online survey was done for qualitative and quantitative data analysis. There are more than 4000 new born babies with a hearing malfunction every year out of which the greater part has an or

As reported by Watkin and Balwin(2012), the frequency ofUnilateral hearing loss in newborns is estimated to be (0.5/1000 newborns), and the incidence increases with age. (Shargorodsky et al.,(2010)). Padraig T Kitterick, Gerard M O’Donoghueetal., (2014) estimated the incidence of SSD in adults to be 12 to 27 in 1,00,000 who exhibit a sudden or idiopathic onset.

As the textbook describes, hearing loss and impairments are described in terms of loudness (decibels) and pitch (frequency or hertz). Because there is a correlation between general knowledge and the degree of hearing loss, it is imperative that the teacher understand the amount of hearing loss for a student diagnosed with a hearing impairment. The hearing loss may range from slight to profound, depending on the decibel level needed for the individual to hear sounds at different frequencies. Hearing loss can also characterized by where the problem exist in the anatomical system. If the location of the problem is within the outer or middle ear it is a conductive loss. If the problems exist in the inner ear and auditory

The connexin26 protein subunit is 226 amino acids long and has a molecular weight of 26215 Da. Its quaternary structure is the connexion hemichannel, a hexamer composed of six connexin subunits. Normal expression of connexin proteins such as CX26 and CX43 in normal mammary epithelial is essential for the formation of gap junctions (Lee et al. (1992) and mutations in the conserved genes can result in a variety of conditions . Variants of the GJB2 gene coding for connexin26 in particular have been correlated with a number of phenotypes as shown in the table 1 in appendix. One of the most noted phenotype correlated with CX26 mutations is autosomal recessive neurosensory deafness. Some mutations in connexin26 subunit of gap junction hemichannels result in the inability to from functional gap junction channels or the formation functional channels an impaired potassium recirculation in the inner ear. Potassium cations are the major charge carriers in the sensory transduction in hearing, which is why mutations causing an abnormal potassium transport result in deafness. Besides GJB2, mutations in GJB3 (connexin 31) and GJB6 (connexin 30) gap junction protein subunits as well as mutations in potassium channel proteins such as KCNQ4 also results in deafness. (Wangemann P. ,

Congenital hearing loss is described as hearing loss that exists at birth. Factors responsible for this condition include those present during pregnancy (such as hereditary factors), as well as factors present after pregnancy. An inherited congenital hearing loss could be conductive, sensorineural, or even a combination of both. The amount or progression of this type of hearing loss varies according to each individual’s case. According to Richard Smith, congenital hearing loss is “syndromic (associated with malformations of the external ear or other organs or with medical problems involving other organ systems) or nonsyndromic (no associated visible abnormalities of the external ear or any related medical problems)…” Over 400 genetic

While some people may not think much about their ears, it is a gift that many take for granted. Unfortunately, hearing loss is a very common health problem in the United States, and the number of people affected with hearing loss has been rising throughout the years. Hearing loss is a serious issue, even though it’s not often thought about, your ears play a huge role in your relationships with others and adds to the quality of your life. While hearing loss does happen with aging, with modern life thrown into the mix, all bets are off. Nowadays, there are other factors that can negatively affect one’s ability to hear, and they are outside the realm of aging, genetics, and illness.

Aging is the single factor that is most responsible for irreversible hearing loss due to weakening of the tympanic membrane, disordered