10.00 Multiple Body Systems

A. Down syndrome (except for mosaic Down syndrome (see 10.00C) established by clinical findings, including the characteristic physical features, and laboratory evidence is considered to meet the requirement of listing 10.06, commencing at birth.

B. Documentation must include confirmation of a positive diagnosis by a clinical description of the usual abnormal physical findings associated with the condition and definitive laboratory tests, including chromosomal analysis. Medical evidence that is persuasive that a positive diagnosis has been confirmed by appropriate laboratory testing, at some time prior to evaluation, is acceptable in lieu of a copy of the actual laboratory report.

C. Other chromosomal abnormalities, e.g. mosaic Down syndrome, fragile X syndrome, phenylketonuria, and fetal alcohol syndrome, produce a pattern of multiple impairments but manifest in a wide range of impairment severity. Therefore, the effects of these impairments should be evaluated under the affected body system.

10.01 Category of Impairments, Multiple Body Systems

10.06 Down syndrome (excluding mosaic Down syndrome) established by clinical and laboratory findings, as described in 10.00B. Consider the individual disabled from birth.

A. Disorders of Vision

1. Causes of impairment. Diseases or injury of the eyes may produce loss of visual acuity or loss of the peripheral field. Loss of visual acuity results in inability of an individual to move about freely. The extent of impairment of sight should be determined by visual acuity and peripheral field testing.

2. Visual acuity. Loss of visual acuity may result in impaired distant vision or near vision, or both. However, for you to meet the level of severity described in 2.02 and 2.04, only the remaining visual acuity for distance of the better eye with best correction based on the Snellen test chart measurement may be used. Correction obtained by special visual aids (e.g., contact lenses) will be considered if the individual has the ability to wear such aids.

3. Field of vision. Impairment of peripheral vision may result if there is contraction of the visual fields. THe Contraction may be either symmetrical or irregular. The extent of the remaining peripheral visual field will be determined by usual perimetric methods at a distance of 330 mm. under illumination of not less than 7-foot candles. For the phakic eye (the eye with a lens), a 3 mm. white disc target will be used, and for the aphaki eye (the eye without the lens), a 6 mm. white disc target will be used. In neither instance should corrective spectacle lenses be worn during the examination but if they have been used, this fact must be stated.

Measurements obtained on comparable perimetric devices may be used; this does not include the use of tangent screen measurements. For measurements obtained using the GOldmann perimeter, the object size designation III and the illumination designation 4 should be used for the phakie eye, and the object size designation IV and illumination designation 4 for the aphakic eye.

Field measurements must be accompanied by notated field charts, a description of the type and size of the target and the test distance. Tangent screen visual fields are not acceptable as a measurement of peripheral field loss.

Where the loss is predominantly in the lower visual fields, a system such as the weighted grid scale for perimetric fields described by B. Esterman (see Grid for Scoring Visual Fields, II. Perimeter, Archives of Ophthalmology, 79:400, 1968) may be used for determing whether the visual field loss is comparable to that described in table 2.

4. Muscle function. Paralysis of the third cranial nerve producing ptosis, paralysis of accommodation, and dilation and immobility of the pupil may cause significant visual impairment. When all the muscle of the eye are paralyzed including the iris and ciliary body (total ophthalmoplegia), the condition is considered a severe impairment provided it is bilateral. A finding of severe impairment hased primarily on impaired muscle function must be supported by a report of an actual measurement of ocular motility.

5. Visual efficiency. Loss of visual efficiency may be caused by disease or injury resulting in reduction of visual acuity or visual field. The visual field. THe visual efficiency of one eye is the product of the percentage of visual acuity efficiency and the percentage of visual field efficiency. (See tables no. 1 and 2, following 2.09)

6. Special situations. Aphakia represents a visual handicap in addition to the loss of visiual acuity. The term monocular aphakia would apply to an individual who has has the lens removed from one eye, and who still retains the lens in his other eye, or to an individual who has only one eye which aphakie. The term binocular aphakia would apply to an individual who has had both lenses removed. In cases of binocular aphakia, the efficiency of the better eye will be accepted as 75 percent of its value. In cases of monocular aphakia, where the better eye is aphakie, the visual acuity in the poorer eye can be corrected only to 20/200, or less, the visual efficiency of the better eye will be accepted as 50 percent of its value.)

Ocular symptoms of systemic disease may or may not produce a disabling visual impairment. These mainfestations should be evaluated as part of the underlying disease entity by reference to the particular body system involved.

7. Statutory blindness. The term "statutory blindness" refers to the degree of visual impairment which defines the term "blindness" in the Social Security Act. Both 2.02 and 2.03 A and B denote statutory blindness.

B. Otolaryngology

1. Hearing impairment. Hearing ability should be evaluated interms of the person's ability to hear and distinguish speech.

Loss of hearing can be quantitatively determined by an audiometer which meets the standards of the American National Standards Institute (ANSI) for air and bone conducted stimuli (i.e., ANSI S 3.6-1969 and ANSI S 3.13-1972, or subsequent comparable revisions) and performing all hearing measurements in an environment which meets the ANSI standard for maximal permissible background sound (ANSI S 3.1-1977).

Speech discrimination should be determined using a standardized measure of speech discrimination in quiet at a test presentation level sufficient to ascertain maximum discrimination ability. The speech discrimination measure (test) used, and the level at which testing was done, must reported.

Hearing test should be preceded by an otolaryngologic examination and should be performed by or under the supervision of an otolaryngologist or audiologist qualified to perform such tests.

In order to establish an independent medical judgment as to the level of impairment in a claimant alleging deafness, the following examinations should be reported: Otolaryngologic examination, pure tone air bone audiometry, speech recption threshold (SRT), and speech discrimination testing. A copy of reports of medical examination and audiologic evaluations must be submitted.

Cases of alleged "deaf mutism" should be documented by a hearing evaluation. Records obtained from a speech and hearing rehabilitation center or a special school for the deaf may be acceptable, but if these reports are not available, or are found to be inadequate, a current hearing evaluation should be submitted as outlined in the preceding paragraph.

2. Vertigo associated with disturbances of labyrinthine-vestibular function, inculding Meniere's diesease. These disturbance of balance are characterized by an hallucination of motion or loss of position sense and a sensation of dizziness which may be constant or may occur in paroxysmal attacks. Nausea, vomiting, ataxia, and incapactitation are frequently observed, particularly during the acute attack. It is important to differentiate the report of rotaary vertigo from that of "dizziness" which is describled as lightheadedness, unsteadiness, confusion, or syncope.

Meniere's disease is characterized by paroxysmal attacks of vertigo, tinnitus, and fluctuating hearing loss. Remissions are unpredictable and irregular, but may be long lasting; hence, the severity of impairment is best determined after prolonged observation and seriala reexaminations.

The diagnosis of a vestibular disorder requires a comprehensive neuro-otolaryngologic examination with a detailed description of the vertiginous episodes, including notation of frequency, severity, and duration of the attacks. Pure tone and speech audiometry with the appropriate special examinations, such as Bekesy audiometry, are necessary. Vestibular functions is assessed by positional and calorie testing, preferably by electronystagmography. When polytomograms, contrast radiography, or other special tests have been performed, copies of the reports of these tests should be obtained in addition to appropriate medically acceptable imaging reports of the skull and temporal bone. Medically acceptable imaging includes, but is not limited to, x-ray imaging, computerized asial tomography (CAT scan) or magnetic resonance imaging (MRI), with or without contrast material, myelography, and radionuclear bonee scans. "Appropriate" means that the technique used is the proper one to support the evaluation and diagnosis of the impairment.

3. Loss of speech. In evaluating the loss of speech, the ability to produce speech by any means includes the use of mechanical or electronic devices that improve voice or articulation. Impairments of speech may also be evaluated under the body system for the underlying disorder, such as neurological disorders, 11.00ff.

2.1 Category of Impairments, Special Senses and Speech

2.02 Impairment of visual acuity. Remaining vision in the better eye after best correction is 20/200 or less.

2.03 Contraction of peripheral visual fields in the better eye.

A. To 10 or less from the point of fixation; or

B. So the widest diameter subtends an angle no greater then 20; or

C. To 20 percent or less visual field efficiency.

2.04 Loss of visual efficiency. The visual efficiency of the better eye after best correction is 20 percent or less. (The percent of remaining visual efficiency is equal to the product of the percent of remaining visual acuity efficiency and the percent of remaining visual field efficiency.)

2.05 (Reserved)

2.06 Total bilateral ophthalmoplegia.

2.07 Disturbance of labyrinthine-vestibular function (including Meniere's disease), characterized by a history of frequent attacks of balance disturbance, tinnitus, and progressive loss of hearing. With both A and B.

A. Disturbed function of vestibular labyrinth demonstrated by calorie or other vestibular tests; and

B. Hearing loss established by audiometry.

2.08 Hearing impairments (hearing not restorable by a hearing aid) manifested by:

A. Average hearing threshold sensitivity for air conduction of 90 decibels or greater and for bone conduction to corresponding maximal levels, in the better ear, determined by the simple average of hearing threshold levels at 500, 1000 and 2000 hz. (see 2.0B1); or

B. Speech discrimination scores of 40 percent or less in the better ear;

209 Loss of speech due to any cause, with inability to produce by any means speech that can be heard, understood, or sustained.

Visual acuity efficiency is also easily determined by referring to tables that assign percentages to different central visual acuities, as follows:

This table assumes that you have either your natural lenses in both eyes, or artificial lenses in your eyes (intraocular lenses surgically placed). It is not relevant whether or not you have corrective lenses in the form of glasses or contact lenses, because the table refers to your best-corrected acuity by an eye doctor’s special equipment and not how well you see with your own glasses or contacts. It is easy to see that if you have 20/20 visual acuity in an eye, then that eye has a 100% visual acuity efficiency.

On the other hand, a 20/100 acuity has a visual acuity efficiency of only 50%. Your visual acuity efficiency percent will be even lower if you suffer from some types of aphakia, but this involves more complicated rules that are not provided in the above table. Visual efficiency in each eye is obtained by applying the formula visual efficiency = visual field efficiency x visual acuity efficiency. Example : Your visual field efficiency in the better eye is 40% as revealed by peripheral visual field testing with a Goldmann perimeter. Also, your best-corrected visual acuity in that eye is 20/100, which translates to a visual acuity efficiency of 50%. Your visual efficiency is 20%—the visual field efficiency (40%) times the visual acuity efficiency (50%). Note that one eye might be “better” in visual acuity, while the other eye might be “better” in the amount of peripheral visual field remaining. The listing applies to the eye that has the better visual efficiency.

1.00  Musculoskeletal System 2.00  Special Senses and Speech 3.00  Respiratory System 4.00  Cardiovascular System 5.00  Digestive System

6.00  Genitourinary System 7.00  Hematological Disorders 8.00  Skin Disorders 9.00  Endocrine System 10.00  Impairments that Affect Multiple Body Systems

11.00  Neurological 12.00  Mental Disorders 13.00  Malignant Neoplastic Disease 14.00  Immune System