Anemia in the pediatric patient
Lisa Jarvis: A Cure For Some Kinds Of Hearing Loss? Gene Therapies Are ...
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Sensorineural Hearing Loss (SNHL) - News-Medical.net
Sensorineural hearing loss (SNHL) or nerve deafness can be defined as a type of hearing impairment that is caused due to dysfunction of the sensory receptors present in the inner ear. This condition causes a defect in the cochlea and/or the auditory section of the vestibulocochlear nerve, thus creating difficulty in the perception of sound.
SNHL can be either unilateral or bilateral. Although it is an irreversible condition, medical science has proposed certain beneficial hearing aids/implant techniques to support such patients.
Sensorineural Hearing Loss Occurs in the CochleaMED-ELPlay
Causes and ClassificationThere are numerous causes for this condition. Depending upon these causes, SNHL can be grouped into three categories:
This is the type of nerve deafness that is present from birth, and can be genetic or non-genetic. Genetic causes may be delayed in action and affect only the hearing. This is also called "Familial Progressive Sensorineural Hearing Loss."
Non-genetic causes develop during the prenatal and perinatal stages of the baby. Maternal rubella, German measles are examples which were known to cause congenital hearing loss before the development of a proper vaccine.
Acquired Hearing LossThis can be categorized as the hearing loss that is caused after birth due to several reasons such as infections, inflammations, toxic drugs and threatening medical conditions, traumas, exposure to loud noise, age-related factors, tumors, injuries, and sudden accidents.
Presbycusis is a type of SNHL that is caused due to age-related factors. It commonly affects both ears and it takes a long time for the disease to affect a person. More than one-third of the elderly is affected by this hearing loss.
SNHL that is caused by exposure to loud noises can be divided into two types—Acoustic Trauma and Noise-Induced Hearing Loss (NIHL). Acoustic trauma is the hearing loss caused by the sudden brief exposure to loud noise, while NIHL is the hearing loss that is caused by chronic exposure of noisy occupations. The former leads to permanent damage to hearing while the latter is preventable.
Acquired factors can also cause "Sudden Sensorineural Hearing Loss" that develops over a period of hours or a few days. This may be either partial or complete.
"Immune-Mediated Sensorineural Hearing Loss" that is caused due to autoimmune disorders is medically treatable.
Auditory NeuropathyThis is a type of SNHL that can be congenital or acquired. This can be differentiated from other types of hearing loss by the combination of normal OAE (Otoacoustic Emissions) results and abnormal ABR (Auditory Brainstem Responses) responses. Abnormal auditory functions are considered as the reasons for this condition.
Signs and SymptomsThe symptoms exhibited by people who are affected by SNHL are as follows:
The first step in the examination of a patient involves the gathering of case histories related to the patient. This step involves collecting of details such as follows:
ENT specialists use many techniques for the diagnosis of this condition. There are two main techniques used for this purpose:
Other methods such as differential testing, audiometry, tympanometry are also used for the diagnosis of SNHL.
Rehabilitation MethodsDifferent rehabilitation methods help the patient to overcome the difficulties they face due to hearing loss. To support them, various hearing aids are provided in the initial stages. These hearing aids can help them in mild to moderate SHLS for both the ears. Hearing aids amplify the sound, but cannot make it clearer. Apart from this, telephone amplifiers, as well as other assistive devices, can be useful.
People with intense hearing loss, who do not benefit from using hearing aids, can opt for implant devices. The cochlear implant device is one of such implant devices. It bypasses the damaged structure of the cochlea and stimulates the function of the auditory nerve.
For patients with neurofibromatosis type 2, auditory brainstem implants are mostly used.
Patients are also trained in sign language and speech reading techniques to support their understanding level.
Reviewed by Afsaneh Khetrapal BSc (Hons)
Further ReadingIPS Cell-derived Inner Ear Cells May Improve Congenital Hearing Loss
A Japanese research group has successfully grafted human iPS cell-derived inner ear cells that express human-derived proteins into the inner ears of embryonic mice. Hereditary hearing loss accounts for about half of all congenital hearing loss cases, and this work is a major contribution toward research that targets the embryonic inner ear.
With an incidence frequency of one per 500 to 1,000 newborns, congenital hearing loss is the most frequently occurring congenital disease, and approximately half of all congenital hearing loss cases are hereditary. Treatment for this kind of hearing loss includes cochlear implants and hearing aids, but there is no fundamental therapy.
In human hereditary hearing loss, deafness has already occurred by birth. It is therefore considered that treatment during the fetal stage would be most effective. The GJB6 gene encodes the CONNEXIN 30 protein, which is essential in hearing development, and a deficiency of the gene results in the second most common disease among cases of hereditary hearing loss that have no other disease in the inner ear.
In previous work, Dr. Ryosei Minoda, head of the Department of Otolaryngology at Kumamoto General Hospital, reported that hearing loss was restored by gene therapy to the inner ear of Connexin 30 deficient fetal mice. The current research team, which includes Dr. Minoda and Dr. Hiroki Takeda of Kumamoto University, and several researchers from Keio University, have successfully grafted human iPS-derived cells into the inner ear of embryonic mice, a feat with a high level of technical difficulty.
First, the research team succeeded in efficiently inducing inner ear cells expressing inner ear specific proteins, such as CONNEXIN 26, CONNEXIN 30, and PENDRIN, from human iPS cells. They then transplanted progenitors of the inner ear cells into the inner ear of embryonic normal and Connexin 30 knockout mice using glass tubes with optimized tip sizes. The transplanted cells grafted to various sites throughout the inner ear in both groups. However, the Connexin 30 knockout mice had more grafted cells than the normal mice, and some of the grafted cells were found to express CONNEXIN 30.
The fact that Connexin 30 knockout mice had a higher number of grafted cells than normal mice, and that some of the grafted cells expressed CONNEXIN 30 is a very important finding when considering cell transplantation as a treatment for hereditary hearing loss caused by CONNEXIN deficiency. Cell transplantation can compensate for missing CONNEXIN and may improve hearing loss by adding properly functioning CONNEXIN proteins. Future work will attempt to increase the number of grafted cells and improve hearing ability. The prevention of hearing loss is an exciting prospect.
Furthermore, this study revealed that cells derived from humans can be grafted into the heterozygous inner ear of mouse embryos. This adds the possibility of in vivo experiments on therapeutic effects using human-derived cells in the mouse embryo. It is thought that this line of research can greatly contribute to the development of a fundamental treatment for hereditary hearing loss and inner ear development research.
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