If you have a hearing impairment, Social Security disability benefits may be available. To determine whether you are disabled by a hearing impairment, the Social Security Administration first considers whether your hearing impairment is severe enough to meet or equal a listing at Step 3 of the Sequential Evaluation Process. See Winning Social Security Disability Benefits for a Hearing Impairment by Meeting a Listing.
If your hearing impairment is not severe enough to equal or meet a listing, the Social Security Administration must assess your residual functional capacity (RFC) (the work you can still do, despite your hearing impairment), to determine whether you qualify for benefits at Step 4 and Step 5 of the Sequential Evaluation Process. See Residual Functional Capacity Assessment for a Hearing Impairment.
The Structure of the Ear
Our hearing apparatus consists of several structures (see Figure 1 below):
Causes of Hearing Loss
Hearing loss can be caused by a variety of different things. Congenital defects anywhere in the hearing apparatus or brain can result in hearing loss. Infections and other diseases account for other cases. Allergies that cause fluid in the middle-ear can also result in hearing loss, if allowed to persist. Other causes of hearing loss are drugs, trauma, immune diseases, cancers, circulatory, genetic and degenerative disorders.
Most hearing loss results from problems with the cochlea or auditory (acoustic) nerve. This is called sensorineural. Hearing loss due to damage to areas of the brain cerebral cortex used in hearing is called central hearing loss. Hearing loss due to damage to the bones of the middle ear is called a conductive hearing loss. Mixed hearing loss means there is a combination of sensorineural and conductive hearing losses.
The most common cause of a combination of deafness and blindness, accounting for about half of the deaf-blind cases in the U.S. is a recessive genetic disorder called Usher syndrome.
There are three types of Usher syndrome:
Many adults with any type of Usher syndrome will qualify for disability, based on hearing or vision impairment. It is important that the Social Security Administration know the diagnosis in children and teenagers, since progressive severity is to be expected rather than stability or improvement.
Waardenburg syndrome is a genetic disorder resulting in deafness, and one defective gene from either parent is enough to produce the disorder.
There are at least four types of Waardenburg syndrome, with Types 1 and 2 being the most common. In Type 1 Waardenburg syndrome, there is a mutated gene that controls development of part of the face and inner ear. In Type 2 Waardenburg syndrome there is also a mutated gene that is related to development of ear structures and hearing. About 20% of Type 1 and 50% of Type 2 Waardenburg syndromes have hearing deficits to some degree.
An interesting fact about Waardenburg syndrome is that there may be other unusual features. For example, due to possible problems with pigmentation, there can be oddly colored patches of skin or hair (like a white forelock of hair or white patch of skin), and eyes of differing color. A low frontal hairline and eyebrows that grow together are other possible features, or the root of the nose may be widened. Hearing loss may be moderate to profound, and does not correlate with pigmentary or facial peculiarities.
Testing of Hearing
Hearing testing is done by audiometry, and is usually performed by audiologists.
Hearing is tested at several different frequencies. The ones that are important to the Social Security Administration are 500, 1000, 2000, and 3000 Hertz [Hz]. The intensity of sound is measured in decibels (dB), and the decibel level at which a sound of a particular frequency can be heard is the pure tone threshold.
Ability to hear sound of 0–25 dB is normal. Normal conversation takes place in about the 45–60 dB range.
People hear by sounds conducted both through the air and sound conducted through bones in the ear and skull. Audiometry tests both types of hearing. Hearing through air is air conduction and through bone is bone conduction.
Audiometry usually includes a test of how well you can understand words, and is called speech discrimination. Speech discrimination, as determined by speech audiometry, is the percentage of test words correctly identified when spoken from standardized and pre-recorded lists. A normal person will achieve nearly 100% correct identification. This test can be used for adults and older children. However, speech discrimination should not be confused with the speech recognition threshold (SRT), which involves a measure of the lowest decibel intensity at which test words can be heard 50% of the time. SRT is a measure of loudness and does not imply ability to understand speech. Speech discrimination is used to determine how well a patient can understand what he hears. The SRT should reasonably correlate with the pure tone average (PTA) for the 500, 1000, and 2000 Hz frequencies, and can thus serve as a check on the validity of the test. The pure tone average is by standard acceptance the sum of the decibel levels necessary to hear 500, 1000, and 2000 Hz, divided by 3. When the person being tested does not cooperate with the testing procedures, such as in malingering, there will be a substantial discrepancy between the SRT and PTA.