Deafness in Congenital Syphilis

COLLIN S. KARMODY, MD, AND HAROLD F. SCHUKNECHT, MD, BOSTON

RECENT
ECENT reports from most parts of the
world show a definite progressive increase in
the incidence of early syphilitic infection.1
Moore et al 2 state that there were 124,000
cases of syphilis reported in the USA in
1962 and also conclude that this figure
represents but \m=1/5\of the total number. The
most disturbing factor in the recent upsurge
is the large number of young people involved
with the possibility of transmission of the
disease to their progeny. In 1962 there was
more than a 100% increase in reported
congenital syphilis compared to 1957,3 and
the upward trend continues. In 1963, a total
of 4,140 cases were reported in the USA
compared to 4,085 for 1962.4
We are concerned in this paper with a
clinical and pathological study of the deaf-
ness which is such a common symptom of
congenital luetics and present our findings
of a review of 123 cases of congenital
syphilis seen at the Massachusetts General
Hospital between the years 1942 to 1964.
The hearing loss of the congenital luetic has
been the subject of previous studies by
Mayer and Fraser,5 Alexander,6 Lund,6
Perlman and Leek,T etc. Its incidence varies
from author to author, having been given
as 25% and 33% in separate studies by
Alexander and by Lund, respectively.6 In
our series, 47 cases were found to have hear¬
ing problems, ie, 38% of the total. This
figure is probably too low, as many of these
cases were seen only once in childhood, and
(Se Table 1). The predominance in women
has previously been discussed by Perlman
and Leek and seems to be quite definitely
established by our study in a larger series.
Auditory Manifestations.\eris-Characteris¬
tically
the pattern of deafness in congenital
luetics shows considerable variation both in
time of onset, type of hearing loss, and
rapidity of progression. Typically it is
sensorineural in type with a flat audiometric
curve, with an added conductive element if
the middle ear is involved in the luetic proc-
ess. Hitherto, emphasis has been laid on
its presentation in adulthood; however, in
our material, 15 cases (ie, 37% of those
with hearing problems) developed their
symptoms in early childhood, while in 22
instances (ie, 51%), symptoms began be-
tween 25 to 35 years of age (See Table 2).
Our study shows marked differences in
the symptomology for the two age groups.
TABLE 1.\p=m-\SexDistribution of 47 Cases
With Hearing Problems
No. Cases No. With Deafness Females Males
123 47 30 17
TABLE 2.\p=m-\Ageof Onset of Deafness
in 42 Cases of Congenital Syphilis
Out of a Total of 120 Cases
Age of Onset of Deafness No. Cases

no follow-up examinations were made. Childhood (1-10 yr) 15

Thirty (64%) of our 47 cases were women 25-35 yr
Over 35 yr
22
5
Submitted for publication Aug 12, 1965. Unknown 5
From the Massachusetts Eye and Ear Infirmary, Harvard
Medical School, and Massachusetts General Hospital. Total 47
Reprint requests to 243 Charles St, Boston, Mass 02114
(Dr. Schuknecht).\

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 125 250 500
-

9-30-60
AGE 46 F

Fig 1.—Audiogram of a woman aged 46 years, Fig 3.—Audiogram of a 26-year-old-woman di¬

a knowncongenital syphilitic. At 34 years of age she agnosed as a congenital syphilitic at age 6 years. At
developed tinnitus and depressed hearing in the right age 25 years she complained of bilateral tinnitus,
ear together with episodic vertigo. These attacks
have persisted to the present time. hearing loss, and recurrent attacks of vertigo.
Hinton test was positive.
Onset of deafness in childhood is usually
very sudden, bilateral, symmetrical, pro¬ 125 250 500
found, and unaccompanied by marked vestib¬
ular symptoms as far as could be determined
-
from the parent's history. By contrast, in
©D.
the adult group where symptoms also begin
abruptly, the result is a partial asymmetric,
flat, sensorineural-type hearing loss (Fig 1) ". \
which often fluctuates (Fig 2) but pro¬
gresses at a rate which is variable in the - -65

same patient and from patient to patient. AGE 39 F

Most times it is accompanied by episodic

vertigo and tinnitus.
In the milder case the auditory deficit may Fig 4.—Audiogram of a woman. At age 24 years
she experienced the sudden onset of vertigo and
be more marked for the low frequencies hearing loss on both sides. Examination revealed a
(Fig 3)—a finding commonly found in dry central perforation on the left side. Response to
Meniere's disease. Alternatively, there may caloric tests was diminished. The Hinton test was
known to be positive since age 12 years.
be a greater loss for the higher frequencies
(Fig 4). The sensorineural loss is frequent¬
ly accompanied by poor speech discrimina¬
tion and loudness recruitment which is 125 250 500 IK 2K 4K 8 6

indicative of a cochlear lesion (Fig 13). -0

42% -10
43yrs Discrim. R
_2K_ L 92% -20
ffi ?fo_§<JS_ 5-26-65
Colories R 56 sec -30
L No Response
-40

./-'
\
50
-

-60
-70
-80
O O* -90
100
o I7yrs 9-21-54 .

11-15-54
-

11-19-54
Fig 2.—Audiogram of a girl, aged 17 years, di¬
agnosed as a congenital syphilitic in childhood.
Audiograms show the marked fluctuations in audi¬
tory thresholds over a two-month period.
Fig 5.—Audiogram of a man known to be a con¬
genital luetic since age 10 years. At age 30 years
he noticed the onset of bilateral throbbing tinnitus,
depressed hearing on both sides, and recurrent at¬
tacks of incapacitating vertigo. Notice the absence
of response to ice water stimulation on the left side
—a finding more typical of an acoustic neuroma.

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 Fig 6.—Photomicrograph of the temporal bone of a 70-year-old congenital luetic woman. She was deaf
for many years prior to death. There is extensive erosion of the optic capsule and the margins of the inter¬
nal auditory meatus. Spaces in the optic capsule are filled with either a highly cellular marrow-like tissue or
loose connective tissue which is infiltrated by round cells (Printed with permission of Prof Luzius Ruedi,
University of Zurich).
The vertigo experienced by the patients of both congenital and acquired lesions are
in ourseries was most often sudden in onset, also similar. Mononuclear leukocytic infiltra¬
episodic, and accompanied by nausea, vomit¬ tion and obliterative endarteritis are the
ing, and an unsteady gait. Caloric responses, pathological entities common to all syphilitic
like the hearing thresholds, seemed to fluctu¬ lesions, whatever the organ affected. With
ate considerably, but on the whole were the milder type of reaction there is infiltra¬
diminished and may even have been absent tion by connective tissue leading to an in¬
(Fig 5). A diagnostic test frequently con¬ flammatory fibrosis. However, in the more
sidered to be pathognomonic of congenital severe reaction a gumma results—character-

syphilis is the presence of a positive fistula

test in the absence of an obvious fistula 125
—
250
500
—
IK
-1-1-1-1-1- -
2K 4K 8 6

Hennebert's sign). In this test the nys¬ -

10-29-53 -10
tagmic response is most marked on the AGE 39 -20
application of negative pressure.7 We have NO RESPONSE TO AC-AD -30
confirmed this observation many times on -40

our patients. CX. -50

Pathology.-—The manifestations of early c-\

\
\
X
-leo
-70
congenital syphilis roughly correspond to 80
those of the secondary stage of the acquired -

-90
infection, while late manifestations are sim¬ -100
ilar to those of the tertiary stage of acquired
Fig 7.—Audiogram of case 1. There was no re¬
syphilis. The basic histopathological changes sponse to air conduction on the right side.

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 Fig 8.—High power view of a focus of bony erosion. Round cells and plasma cells are clearly visible. A
giant cell is just discernible in the upper left central part of the field.
Fig 9.—Photomicrograph of the posterior semicircular canal of case 1. An area of résorption has
evolved down to the endosteum. Dense fibrous connective tissue lines the canal while delicate strands of con¬
nective tissue cross the perilymphatic space.

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 ized by central necrosis with surrounding gives rise to the formation of peg-shaped,
lymphocytic infiltration and vascular occlu¬ notched incisors—Hutchinson's teeth. In¬
sion. Gummata vary greatly in size, ranging volvement of epiphyseal cartilages causes a
from microscopic proportions to several generalized reduction in stature. Interstitial
centimeters in diameter. Every tissue type keratitis, the commonest manifestation of
and organ of the body can be affected to a congenital syphilis, is also caused by leuko-
greater or lesser degree. Bone, cartilage, cytic infiltration of the cornea. Similar
liver, kidney, lungs, brain, meninges, spinal changes are found in the meninges in menin-
cord, skin, cornea, choroid may all be in¬ govascular syphilis.
volved. Essentially the lesion of bone is an The otitic symptoms of the congenital
osteitis in the early stages with gummata syphilitic are caused by pathological change

Fig 10.—Photomicrograph of case 1. The internal auditory meatus is lined by thickened dura and con¬
tains herniated cerebellum. There is gross dilatation of the cochlear duct. Rosenthal's canal contains very
few ganglion cells.

occurring more frequently in the more ad¬
vanced conditions.
Clinical signs and symptoms which are
considered to be typical of congenital lues
depend upon the predilection for patho¬
logical change in certain organs. For ex¬
ample, perichondritis and periostitis of the
nasal framework causes rhinitis (snuffles)
of infants. Periostitis of the cranial bones
results in bossing of the skull—the hot cross
bun skull. Periostitis of the tibia leads to
sabre shins. Damage to odontogenous tissue
in the membranous labyrinth which are
secondary to lesions of the bony otic capsule.
The primary pathology of the otic capsule
has been previously described by Mayer and
Fraser,5 Nager,8 and Goodhill.9 Mayer and
Fraser divided the histopathology into three
entities:
(1) gummatous osteomyelitis; (2)
gummatous periostitis; (3) nongummatous
osteitis. The basic underlying histological
feature of each is the same as described
above for all tissue types, there being round
cell infiltration and endarteritis leading to

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 varying degrees of tissue destruction. Patho¬ improvement. At this time examination of the ears
revealed normal tympanic membranes, profound
logical changes may involve the periosteum, deafness on the right, and severe hearing loss on
periosteal bone, enchondral bone, and en¬
the left (Fig 7). The liver and spleen were en¬
dosteum individually or collectively (Fig 6). larged. The Hinton test was
positive in one in four
Fig 6 shows the extent of possible bony dilution. There is no record of vestibular tests or
pathology. In this specimen there is exten¬ the fistula test having been performed. Two further
sive erosion of the otic capsule around the courses of penicillin were given at age 44 years.
semicircular canals, over the cochlea, and However, he died four later at 48
years of years
age from massive hematemesis secondary to con¬
even around the internal auditory meatus. genital luetic cirrhosis of the liver. The right tem¬
The defects are filled with fibrous connective poral bone was obtained for study.
tissue which is heavily infiltrated by round Histopathology.—There is patchy résorption of
cells of the chronic inflammatory type sim¬ all three layers—periosteal, enchondral, and endos¬
teal—of the otic capsule. These areas of résorption
ilar to those seen in Fig 8. In addition, in
are filled with a loose connective tissue which is
this temporal bone there was fusion of the infiltrated by round cells, plasma cells, and a few
head of the malleus and body of the incus. giant cells of the foreign-body type (Fig 8). Bony
Mayer and Fraser were able to demonstrate erosion extends down to the endosteum of the pos¬
terior and horizontal semicircular canals (Fig 9). A
foreign-body type giant cells in their ma¬ layer of dense connective tissue lines the endosteal
terial which were associated with areas of surfaces of these canals which have been made ir¬
active bone résorption. Bone changes in regular by small foci of new bone. In one area a
the otic capsule show a predilection for in¬ larger focus of new bone almost completely occludes
volvement around the semicircular canals the lumen of the horizontal canal. Delicate strands
with lesser involvement of bone over the of connective tissue containing small blood vessels
cross the perilymphatic spaces of the semicircular
cochlea. Although Fig 6 does not demon¬ canals at irregular intervals.
strate this point in outstanding fashion it There are wide dehiscences in the membranous
has been well documented in previously walls at the ampullary ends of the posterior and
reported cases (Nager) and is well shown horizontal canals. The edges of these ruptures have
adhered to the endosteum with the formation of
in our own case (Fig 9). Where the en¬
persistent fistulae between endolymphatic and peri¬
dosteum is involved particularly in the pars lymphatic spaces. Elsewhere the membranous semi¬
superior, proliferative fibrotic reaction leads circular canals ar collapsed.
to replacement of the endosteum by a thick The entire endolymphatic system is lined by an
layer of fibrous connective tissue and to eosinophilic deposit. The cochlear duct, saccule, and
utricle are grossly dilated with displacement of
tissue infiltration of the perilymphatic Reissner's membrane into the scala vestibuli. The
spaces (Fig 9). The changes in the mem¬ distended saccule makes contact with the vestibular
branous labyrinth are, characteristically, surface of the stapes footplate and probably the
cochlear hydrops and later degeneration of cochlear duct has herniated into the vestibule. As
a result the vestibule is traversed in a haphazard
the sensory endorgans of both the auditory
arrangement by the delicate endothelial walls of
and vestibular systems (Fig 6 and 10). the membranous labyrinth (Fig 10).
An interesting finding is gross thickening of the
of Cases connective tissue surrounding the endolymphatic
Report duct with resulting narrowing of the lumen of the
Case 1.—This man died at age 48 in 1962. His duct and of the saccus endolymphaticus.
mother had been treated for syphilis which was di¬ The Organ of Corti and the vestibular sense or¬
agnosed when he was a few years old. gans are severely atrophied. Corti's organ is repre¬
History.-—At the age of 16 he developed inter¬ sented throughout by small mounds of cells having
stitial keratitis resulting in partial blindness. At no resemblance to the normal structures. No nerve
18 years of age he began to lose the hearing in both fibres are identifiable in the osseous spiral laminae,
ears with rapid progression to complete loss on the but a few isolated ganglion cells remain (Fig 10).
right and severe but incomplete loss on the left. He The maculae of both utricle and saccule have de¬
had used an air conduction hearing aid on the left generated into clumps of tissue with loss of histo¬
since 20 years of age. He denied ever experiencing logical detail. The cristae of all the semicircular
vertigo. At 32 years of age penicillin was adminis¬ canals are atrophie. Their cupulae are distorted, and
tered parenterally for a period of seven days ; de¬ no hair cells can be identified. There is
atrophy of
tails of therapy are unknown. When 36 years old the stria vascularis in all turns of the cochlea.
he developed bilateral lower limb paresis, for which The cochlear and vestibular nerves are severely
a lower dorsal laminectomy was performed without degenerated and are represented by only a few

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 strands. This contrasts sharply with the well pre¬ Table 3.—Data on Therapeutic Procedures
served facial nerve. Thickened dura lines the in¬
ternal auditory meatus which contains a herniation No. Cases Previously Treated for
of cerebellar tissue (Fig 10). Cases Treated Unknown Hearing Loss
The malleus is thickened by bony hyperplasia
34
while the body and long process of the incus presents
a spongy appearance because of the presence of
numerous connective tissue-filled spaces surrounding
and it is likely that the majority of these received
thin bony trabeculae. The round window niche, no form of therapy prior to the beginning of oto¬
stapes footplate, and the annular ligament appear logical symptoms.
normal. On the posterosuperior aspect of the bony
Assessment of the efficacy of various therapeutic
tympanic annulus is an area of bony change charac¬ measures used for the deafness of congenital syphi¬
terized by the presence of spicules of undifferenti¬
lis is difficult because of the extreme variability in
ated bone surrounded by dense fibrous tissue. This
pattern and fluctuation in thresholds which occur
lesion probably represents a focus of healed luetic
osteitis. A similar focus is present around the spontaneously. Treatment of the deafness, per se,
had in the past been based mainly upon the adminis¬
canalis singularis.
tration of the standard antiluetic drugs. These
Therapy therapeutic regimes with arsenicals, bismuth, peni¬
cillin, etc, have little effect upon the onset, pattern,
There have been many changes and advances in or course of the hearing loss in the congenital
the therapy of syphilis since the disease was first disease. The more recent use of steroids in combi¬
described in the early 16th century. Congenital lues nation with penicillin seems to offer some hope in
was identified a century later, and, until the turn
slowing the relentless progression of this type of
of this century, its therapy was no more successful
than that for the acquired disease. Table 4.—Types of Treatment Used
Mercury and the iodides, or their combination, Before the Onset of Hearing Loss
formed the mainstay of treatment for three cen¬
turies. Mercury was used in every possible manner Therapy No.
—orally, as a salve, plaster, inhalant, and more
latterly by injection. However, on the whole, these Arsphenamine & bismuth
Mercurials & bismuth
16
1
medications were singularly unsuccessful in either
Malaria 2
eliminating or even attenuating this dreaded infec¬ Penicillin 5
tion. At the beginning of the 20th century Ehrlich Details of therapy unknown 8
introduced salvarsan (cmpd 606). Although an
effective therapy was at last available, severe tissue
necrosis at injection sites limited its usefulness. deafness. Table 5 gives the results of treatment of
Bismuth compounds were introduced in the early the hearing loss in 15 of our cases.
1920's, and soon the standard treatment of all forms In this series "improvement" denotes temporary
of luetic infection was a combination of arsenicals improvement as none of these cases maintained their
and bismuth. gain. Morton,10 Perlman, and Leek' have reported
on the effect of corticotropin (ACTH) and corti¬
Malaria therapy was first used successfully in
1917 but was reserved for cases which were resist¬ sone on their patients. Morton treated four cases

ant to other forms of treatment. As such, it was with prednisolone but obtained no improvement. He
sometimes used in the congenital syphilitic for the concluded that cortisone therapy should be tried in
therapy of interstitial keratitis and continued to be the early stages. Perlman and Leek gave combined
used until the late 1940's. In 1943, the introduction corticotropin and penicillin therapy to five patients
of penicillin as a successful therapeutic weapon and found subjective and objective improvement in
against the treponema marked the beginning of a three. They came to the conclusion that penicillin
new era in the control of all forms of luetic infec¬ and steroid therapy in the early stages may be of
tion. However, it soon became obvious that even help. More recently, Hahn et alu have reported
penicillin in massive doses seemed ineffective in their results on the therapy for 19 congenital luetics
preventing the progression of established deafness with sensorineural deafness of duration ranging
in the congenital luetic. Further, our series illus¬
trates that adequate and timely therapy of the Table 5.—Data on Results of Therapy
infected child by various treponemicidal agents, eg,
arsphenamines, bismuth, malaria, and penicillin does No. Temporary Not
not seem to prevent the onset of luetic labyrinthitis. Therapy Treated Improvement Improved
Tables 3 and 4 summarize the information about Arsenicals & bismuth 2 1 1
therapeutic procedures available in our material. Penicillin 9 3 6
They demonstrate that of the 47 cases with hearing Penicillin & cortisone 3 2 1
Unknown 10
problems, 34 were treated previous to the onset of 1
deafness. Most of the 13 unknown were children,

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 m aso_500_ _2K_
O I7yrs 11-30-54
1-4-55
Fig 11.—Audiogram of case 2, showing the
changes in thresholds before and after therapy with
penicillin and cortisone for ten days.
from seven months to 40 years. Their dosage scheme
was prednisone 30 mg daily for one week, then 20
mg daily with reduction by 2.5 mg daily if progress
was satisfactory. Seventeen of their patients could
be assessed for speech discrimination during and
following therapy. Of these, 11 showed improve¬
ment, two showed no change, and in one the speech
discrimination was made worse. Improvement in dis¬
crimination ranged from between 92% and 20%.
Their opinion was that steroids were useful in these
cases. Three of our patients were treated with
penicillin and steroids. One of these obtained no
improvement whatsoever, and therapy was discon¬
tinued. The following is a report of the second pa¬
tient placed on this regime.
Case 2.—This patient, the daughter of
syphilitic, was first seen on Nov 26, 1954,
years. In early infancy a diagnosis of congenital
syphilis had been made, and she was treated with
numerous injections of an unknown drug. When 7
years old she developed bilateral interstitial keratitis
resulting in grossly scarred corneae. At the age of 9
years she began to experience episodes of vertigo,
nausea, vomiting, and tinnitus. Beginning with these
attacks she noticed progressive but fluctuating bi¬
lateral hearing loss. In 1952, at 15 years of age, a
right corneal transplant was successfully performed.
Simultaneous with this procedure a course
!5_250_
8-1-62
Oiscrim.
25yrs R 58% 8-1-62 —
L 32% 10-10-62
Fig 12.—Audiogram of case 2, at age 25 years,
taken before and during her fifth course of steroids.
Notice the change in pretreatment thresholds com¬
pared with Fig 11.
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cillin and cortisone was given for ten days. There
was subjective improvement in hearing with this
therapy.
When first seen in the ENT Department, exami¬
nation revealed normal nose, throat, palate, and
tympanic membranes. Ice water calorics gave the
following responses : right : 1 minute 5 seconds ;
left : 1 minute 25 seconds. Audiometry revealed a
flat sensorineural hearing loss averaging 40 db for
the speech frequencies on each side. The Hinton
·
test was positive to one in four dilution. Because of
her past history a course of penicillin, 600,000 units
daily, and cortisone, 100 mg t.i.d. was given for
ten days. Pure tone audiograms taken before and
after treatement, as shown in Fig 11, demonstrate
the temporay improvement in thresholds which was
obtained. Six months later in March 1955, after
a left corneal transplant she received her third
course of penicillin and cortisone over a ten-day
period. The exact dosage scheme is not known, but
once again there was a temporary gain in auditory
thresholds. For the next two years she complained
of gross fluctuations in auditory acuity and at the
age of 20 in 1957 a fourth course of steroids over a
period of seven days, once again, produced only
temporary improvement in symptoms. In 1960, at
the age of 23 years she began to use a hearing aid
on the right side. However, it was decided in 1962
to try the effect of prolonged steroid therapy. Her
Hinton test at this time was still positive to one in
four dilution. On Oct 10, 1962, prednisone 10 mg
q.i.d. was prescribed, producing depression of pure
a known tone thresholds after seven days (Fig 12). She con¬
at age 17 tinued to take prednisone 5 mg q.i.d. until March 21,
1963 when dosage was increased because of further
hearing loss at the maintenance level of therapy.
Dosage was gradually increased to 30 mg q.i.d. and
again her hearing improved with improvement in
discrimination on the right side (Fig 13). For the
first time the fistula test was reported as being posi¬
tive. High doses were maintained for two weeks
when withdrawal was necessitated by the develop¬
ment of moon face, etc. Table 6 summarizes the
therapy and progress of this patient.
of peni-
Comment
In congenital syphilis there is résorption
of the otic capsule by a highly cellular,
vascular, and chronic inflammatory process,
resulting in labyrinthitis characterized by
endolymphatic hydrops and degeneration of
the membranous labyrinth with its sense
organs. The degree of involvement is vari¬
able but, typically, the course is progressive.
It is almost always bilateral and nearly
always more severe in early onset. The
audiometrie pattern is typically that of a flat
sensorineural loss with loudness recruitment,
and often the hearing levels fluctuate con-
 125 25Q_500_
O 26yrs 3-21-63
4-3-63-L
.O.
".· ·.0k-~X-X**"·*
¡Í.X
4-3-63
Discrim
R 100%
L 36% o ruptures which are evident in the distended
Fig 13.—Audiogram of case 2 during therapy
with steroids. Black continuous lines indicate levels
to which thresholds had regressed on maintenance
doses of steroids. Note improvement in discrimina¬
tion scores on the right side.
siderably. Most patients have associated
vestibular symptoms with episodic vertigo
and give diminished responses to caloric
tests. In fact, the otologie symptomatology
of congenital lues is virtually indistinguish¬
able from that of Meniere's disease.
Symptoms of spontaneous episodic ver¬
tigo, fluctuating flat sensorineural type hear¬
ing loss with loudness recruitment, tinnitus,
and diminished caloric responses are typical
of two disease entities—congenital lues and
Meniere's disease. The common pathological
correlate of both is endolymphatic hydrops.
That inflammatory disorders can cause endo¬
lymphatic hydrops is evident in serous and
suppurative labyrinthitis. However, although
recovery from these conditions results in
persistent hydrops, it is not accompanied by
episodic vertigo. Nor does study of these
cases reveal the presence of ruptures in the
walls of the membranous labyrinth; rather
there exists a state of hydrostatic equilib¬
- rium.
The sudden disturbances of the auditory
and vestibular systems so characteristic of
congenital lues and Meniere's disease are,
presumably, functional manifestations of
progressive increase in endolymphatic pres¬
sure. It has been proposed12·13 that the
membranous labyrinth in Meniere's disease
are related to the attacks of vertigo and
hearing loss. It is probable that the forma¬
tion of these defects which are seen in the
walls of the saccule, utricle, and semicircular
canals, coincide with the onset of auditory
and vestibular disturbances. Successive heal¬
ing of the breech, redistension, and rerup¬
ture result in the discrete attacks which are
so typical of these conditions. The dis¬
continuity of the walls of the greatly
distended membranous labyrinth in the con¬
genital luetic with hearing problems further
supports this idea.
There are probably different etiologies for
the endolymphatic hydrops of congenital
lues and for that of Meniere's disease. In
the latter there is no evidence of an in¬
flammatory process of any type in any part
of the inner ear. In addition congenital lues
is almost always a bilateral disease, while
Meniere's is a unilateral condition in 90%
of cases. Also Meniere's disease of child¬
hood is a rare and dubious entity. Neverthe¬
less, these two conditions are strikingly
similar in symptomatology and histopathol-

Table 6.—Data on Therapy and Progress of Case 2

Pretreatment Posttherapy
Levels, db Levels, db
Date of Details of Duration
Therapy Therapy of Therapy AD AS Hinton

March 1953 Penicillin & cortisone 10 Days Improved Improved Positive

Mov 1954 Penicillin, 600,000 units & 12 Days 35 27 S Positive
cortisone, 100 mg t.i.d. 1:4
Penicillin & cortisone 10 Days Improved Improved Positive
1:8
Prednisone, 5 mg t.i.d. 7 Days 70 57 60 47 Positive
1:2
Penicillin, 600,000 units & 11 Days 55 45 55 15 Positive
prednisone, 5 mg t.i.d. 1:2
Prednisone 6 Mo 60 60 33 55
Dis. 58 % Dis. 32 % Dis. 100% Dis. 36%
40 55 Positive
Dis. 88 % Dis. 44 % 1:2

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 ogy and in practice may be indistinguishable
one from the other.
The characteristic diagnostic positive
fistula test of the congenital luetic probably
is due to the presence of the wide compressi¬
ble marrow and fibrous-tissue filled spaces
around the semicircular canals.
Summary
The case histories of 123 congenital
syphilitics were studied. A total of 47 (38%)
developed hearing problems. Of these, one
third were children.
The otological symptoms of late congenital vertigo.
REFERENCES
1. Proceedings of World Forum on Syphilis and
Other Treponematoses, Atlanta: US Communicable
Disease Center, Sept 4-8, 1962.
2. Moore, M.B., Jr.: The Epidemiology of
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lues are virtually indistinguishable from
those of Meniere's disease. Pathology of
the deafness in the congenital luetic is based
upon primary osteitis of the otic capsule
with secondary endolymphatic hydrops. Epi¬
sodic vertigo indicates progressive dilatation
of the endolymphatic system with repeated
ruptures.
Steroid therapy appears to create tempo¬
rary improvement in the auditory thresholds
of the congenital syphilitic. Because of the
recent rise in the incidence of syphilitic in¬
fection, congenital syphilis must, once more,
be included in the differential diagnosis of
8. Nager, F.R.: Die Lues hereditaria tarda des
Innerohreseine Folge chronischer Osteomyelitis
des Felsenbiens, Pract Otorhinolaryng 17:1-22,
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12. Schuknecht, H.F.: Meniere's Disease: A Cor-
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13. Schuknecht, H.F.; Benitez, J.T.; and Beek-
huis, J.: Further Observations on the Pathology of
Meniere's Disease, Ann Otol 71:1039-1053, 1962.

NUGGETS OF
LASTING VALUE

A pliable plastic tube described by Montgomery provides excellent palliation for patients with
incurable cancer of the mid and lower pharynx. The tube can be left in place for many months,
and it permits the patient to be fed by the normal oral route and be greatly helped both psycho¬
logically and socially. Minimal maintenance is required. Using local anesthesia, the tube is
inserted through the mouth and its lower end placed in the esophagus, bridging the area of the
tumor.—May, M. ; Middleton, P. ; and Gildersleeve, G. : The Montgomery Tube to Palliate
Hypopharyngeal Cancer, Ann Otol 74:63-68 (March) 1965.

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