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This type of hearing loss refers to a malfunction in the cochlea (sensory) and/or in the nerve (neural) to the brain.
There are many causes of sensorineural hearing loss. In children, genetic causes are common. Sometimes infectious diseases, mechanical trauma or other conditions can create this type of hearing loss.
Most states now have universal hearing screening for newborns. This type of hearing test screens to check the ears of normal babies. The test was first performed on babies who had been critically ill and had graduated from Neonatal Intensive Care Units where there is a high incidence of sensorineural hearing loss. Now, hearing loss can be detected in many babies near the time of their birth. It is usually necessary to follow up with more advanced hearing testing if there is a failure measured by screening.
In some children, hearing remains stable. In other children, the loss progresses. For example, a condition known as "the enlarged vestibular aqueduct syndrome" progressively fluctuates and worsens in many patients. There are genetic conditions involved with progressive sensorineural hearing loss. Fortunately, many children with sensorineural hearing loss do have stable hearing that does not fluctuate.
Hearing aids may be necessary for this type of hearing loss, depending on whether one or both ears are involved, as well as on the degree of hearing loss.
On occasion, we can try medications such as steroids with some success. There is no "simple operation" for sensorineural hearing loss although there are certain circumstances that warrant an operation, for example, to explore the ear for an inner ear fluid leak. For patients who have so little hearing that they cannot respond to hearing aids, there is an operative procedure called a cochlear implant. The implant procedure, however, is reserved for the very few patients who have a profound sensorineural loss in both ears.
X-rays are frequently obtained to look for an inner ear malformation.
In cases where the hearing loss could be familial, lab work can be obtained. It is a long process and not all families can be selected. However, we have had success in locating the gene in select families.
There is another condition that involves a gene for a protein called connexin 26. There are times when we draw blood if this condition is suspected. The results, however, are not always clear at this time.
Quite possibly. Besides hearing aids, in a classroom setting, sometimes an instrument called an FM trainer is used that helps to broadcast sound from the teacher. Other classroom maneuvers can be adopted as necessary. There are times when being educated in a mainstream school is appropriate for the child but other children may need to go to a school that specializes in education for the hearing impaired.