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Deafness and Retinitis Pigmentosa
N O T E S , CASES, I N S T R U M E N T S DEAFNESS AND R E T I N I T I S PIGMENTOSA GUERDAN HARDY, Saint Louis
M.D.
In August, 1938, a boy of 11 years was brought to me because of poor hear ing. It had been observed that he was having difficulty in hearing his teacher at school. No note had been made at the time of any visual disturbance. There was no history that would suggest a pre ceding illness, infection, or injury as a cause of the deafness. Physical examination was essentially negative except for the fundi. Following is the report from Dr. Alan Calhoun: "Right fundus. Slight pallor of the disc; no lamina cribrosa showing; margins dis tinct. The surrounding retina shows an almost complete loss of retinal pigment. There is no sclerosis of the choroidal vessels. The arteries are slightly attenu ated. About midway to the periphery the retina is dotted with fine pigment, giving AUDIOORAM o r C . —20
N«
AGE
11
it a stippled appearance. The fundus does not resemble a 'salt-and-pepper fundus.' Farther toward the periphery are several beginning bone-corpuscle pigment de posits along the vessels. Here also are numerous yellow-white dots that seem to be situated deep in the outer retina or choroid. No areas of typical inflamma tory pigment are noted. This strongly resembles an early case of retinosa pigmentosa. The fundus of the left eye is quite similar. The visual fields show con centric contraction of about 10 degrees but no ring scotoma." Refraction under cycloplegia: O.D. - 1 . 0 0 D. sph. o +1.25 D. cyl. ax. 90°, vision 15/15; O.S. - 0 . 5 0 D. sph. O + 1.00 D. cyl. ax. 90°, vision 15/15. The hearing was found to be dimin ished considerably: Right ear, whispered voice (loud) heard at 2 inches; spoken voice heard at 3 feet; Left ear, whispered voice heard at 2 inches; spoken voice heard at 2 feet. The audiogram is particularly interest__10
PATE 8-16-38
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NOTES, CASES, INSTRUMENTS
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ing since it shows a progressive and steep loss as one passes from t h e low tones, through t h e middle, to t h e high tones. T h e second audiogram shows the hear ing loss of a woman, aged 39 years, w h o also h a s retinosa pigmentosa. T h i s diag nosis was made by D r . W m . F . H a r d y , Dr. J . A. Flury, and by the late D r . E . Fuchs. Again one notes the progressive
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loss as t h e higher tones a r e approached until the highest a r e not heard at all. T h e third audiogram shows t h e tone loss of a woman, aged 37 years, a sister of the previously mentioned patient. She likewise h a s retinosa pigmentosa. T o t h e best of my knowledge there have been no published audiograms of similar cases a n d for this reason this
NOTES, CASES, INSTRUMENTS
chart is presented. Textbooks on the eye and on the ear merely mention the oc currence of an associated deafness. Humboldt Building.
A N E W ULTRAVIOLET LAMP FOR CATARACT SURGERY ELIOTT B. HAGUE,
M.D.
Buffalo, New York The spectrum visible to the human eye extends approximately from 7,000 ang strom units in the red to 4,000 in the violet. The ultraviolet spectrum covers a
317
than was previously possible, for use in cataract surgery. This lamp has been en thusiastically received by certain experi enced operators. Care was taken in the design of the Hildreth instrument to achieve maximum results, optically, with the source of ultraviolet illumination then available. In 1938 I suggested to Hildreth the possibility of utilizing a relatively new source of ultraviolet light, a new type of mercury-vapor lamp. Since that time I have worked out a method of ultraviolet illumination of the anterior segment and the lens, using a mercury-vapor lamp and
Fig. 1 (Hague). New ultraviolet lamp for cataract surgery.
range comparably extensive. It has been conclusively shown that only the short-wave ultraviolet is biologically harmful. Well separated from the abiotic wave lengths, and just beyond the visible spectrum, there is a relatively narrow band of ultraviolet light which, if directed into the eye, will cause the lens to fluoresce. In 1936 Hildreth devised a source of ultraviolet illumination constructed to visualize lenticular detail more distinctly
filter chosen for transmission character istics, availability, safety, and adaptability to manipulation. This lamp, which I have developed in cooperation with the Scientific Re search Departments of the American Op tical and Spencer Lens Companies, will soon be available for general use. Basically it consists of a new mercuryvapor bulb manufactured by the General Electric Company and a filter selected by interpolating data concerning available
M.D.
In August, 1938, a boy of 11 years was brought to me because of poor hear ing. It had been observed that he was having difficulty in hearing his teacher at school. No note had been made at the time of any visual disturbance. There was no history that would suggest a pre ceding illness, infection, or injury as a cause of the deafness. Physical examination was essentially negative except for the fundi. Following is the report from Dr. Alan Calhoun: "Right fundus. Slight pallor of the disc; no lamina cribrosa showing; margins dis tinct. The surrounding retina shows an almost complete loss of retinal pigment. There is no sclerosis of the choroidal vessels. The arteries are slightly attenu ated. About midway to the periphery the retina is dotted with fine pigment, giving AUDIOORAM o r C . —20
N«
AGE
11
it a stippled appearance. The fundus does not resemble a 'salt-and-pepper fundus.' Farther toward the periphery are several beginning bone-corpuscle pigment de posits along the vessels. Here also are numerous yellow-white dots that seem to be situated deep in the outer retina or choroid. No areas of typical inflamma tory pigment are noted. This strongly resembles an early case of retinosa pigmentosa. The fundus of the left eye is quite similar. The visual fields show con centric contraction of about 10 degrees but no ring scotoma." Refraction under cycloplegia: O.D. - 1 . 0 0 D. sph. o +1.25 D. cyl. ax. 90°, vision 15/15; O.S. - 0 . 5 0 D. sph. O + 1.00 D. cyl. ax. 90°, vision 15/15. The hearing was found to be dimin ished considerably: Right ear, whispered voice (loud) heard at 2 inches; spoken voice heard at 3 feet; Left ear, whispered voice heard at 2 inches; spoken voice heard at 2 feet. The audiogram is particularly interest__10
PATE 8-16-38
yrs.
_N0
—io *■
o
;
\
>o 20 30
SO 90 lOO
i
bt
V ^ f--
\
BO TO
< s ■
\
-JO CO
\,
i^ ^
i i --J
\ 0 = U ; h t Ei ,r. RI rai ; p j s : :TI VE
Bo i e
CO ndi ICt l o i
,
L
X
^ ^ \.
>K
\
\ n ar
1
\
!
1
/
rec17
TOTAL LOSS OF SERVICEABLE HEARING
PITCH
/
k
,'
d« CT^»ai ted
/ >, / ^
''
,- s " 16384 C
32 C,
315
NOTES, CASES, INSTRUMENTS
316
..AGE_32_yra..
AUDIOGRAM OF.Mifl.fl...E. -20
DATe„3rlI=39Jt....
—IO IMAL.
— 1
\
'O 20 m
:
30
s
1 40 0
B
*~>i
\
O
1
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i
70
OS
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1
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2 "°H< a r 120
tot mi >g
tar
\K
^ ne c<
/ ud l o 11*
,' ," 1024 C
c,
M.
>'
# / v
32
AUDIOGRAM OF Ml a s
B.
>' ,'
TOTAL LOSS OF SERVICEABLE HEARING
130
1
\
2100
B I
1
\
VN \
\
NN i I IT :vi • >os S so TT. 1
t
AGE
37
yrs.
PATE
4096 C
2048
S192
3-31-39
16384
-.10
32768
-No.
—20
► 0
\
10
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2 0
*<
N
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Sy'•
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,
^ \
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V
\ 0 •RJ 100
R I JN1
Eh
t, 1
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\
ar
■V
; vOS! :TI VK V
110
n r»t.l I1TI IT no Mil vr rm i * QS. i n TOTAL LOSS OF SERVICEABLE HEARING
rjn
PITCH
32 C,
64 C
128 c
258
ing since it shows a progressive and steep loss as one passes from t h e low tones, through t h e middle, to t h e high tones. T h e second audiogram shows the hear ing loss of a woman, aged 39 years, w h o also h a s retinosa pigmentosa. T h i s diag nosis was made by D r . W m . F . H a r d y , Dr. J . A. Flury, and by the late D r . E . Fuchs. Again one notes the progressive
1024
//
,
( •u
r.t, Im 1 ,
512
k
^ >
—' >—
,' 2048 C
/ - ■ "
4096
8192 c'
162184 c'
32768
loss as t h e higher tones a r e approached until the highest a r e not heard at all. T h e third audiogram shows t h e tone loss of a woman, aged 37 years, a sister of the previously mentioned patient. She likewise h a s retinosa pigmentosa. T o t h e best of my knowledge there have been no published audiograms of similar cases a n d for this reason this
NOTES, CASES, INSTRUMENTS
chart is presented. Textbooks on the eye and on the ear merely mention the oc currence of an associated deafness. Humboldt Building.
A N E W ULTRAVIOLET LAMP FOR CATARACT SURGERY ELIOTT B. HAGUE,
M.D.
Buffalo, New York The spectrum visible to the human eye extends approximately from 7,000 ang strom units in the red to 4,000 in the violet. The ultraviolet spectrum covers a
317
than was previously possible, for use in cataract surgery. This lamp has been en thusiastically received by certain experi enced operators. Care was taken in the design of the Hildreth instrument to achieve maximum results, optically, with the source of ultraviolet illumination then available. In 1938 I suggested to Hildreth the possibility of utilizing a relatively new source of ultraviolet light, a new type of mercury-vapor lamp. Since that time I have worked out a method of ultraviolet illumination of the anterior segment and the lens, using a mercury-vapor lamp and
Fig. 1 (Hague). New ultraviolet lamp for cataract surgery.
range comparably extensive. It has been conclusively shown that only the short-wave ultraviolet is biologically harmful. Well separated from the abiotic wave lengths, and just beyond the visible spectrum, there is a relatively narrow band of ultraviolet light which, if directed into the eye, will cause the lens to fluoresce. In 1936 Hildreth devised a source of ultraviolet illumination constructed to visualize lenticular detail more distinctly
filter chosen for transmission character istics, availability, safety, and adaptability to manipulation. This lamp, which I have developed in cooperation with the Scientific Re search Departments of the American Op tical and Spencer Lens Companies, will soon be available for general use. Basically it consists of a new mercuryvapor bulb manufactured by the General Electric Company and a filter selected by interpolating data concerning available