Download PERIOTIC DEAFNESS. A FEW months ago a colleague, whose

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296
PERIOTIC DEAFNESS.
By
C. A. HUTCHINSON,
Royal Army Medical Corps.
MAJOR
A FEW months ago a colleague, whose opinion in most things medical I
value very highly, asked me to see a case of " otosclerosis," and added that he
had had several cases of this complaint. In due course the patient arrived
and turned out to be a man on the wrong side of 60.
Further inquiry since has elicited the fact that many medical men make
a similar error in diagnosis, and it is in the hope of helping to elucidate this
point that I am venturing on this article.
Now periotic deafness is of two types : (1) Due to conditions starting in the mucous membrane of the middle
ear-It Membranous periotic deafness."
(2) Due to conditions starting in the bone of the periotic capsule" Otosclerosis."
(1) MEMBRANOUS PERIOTIC DEAFNESS.
This condition is usually transitory and recovers spontaneously. Its
earliest stages are seen in acute and subacute otitis media, the result of a
common cold.
There is no particular age-incidence, nor does it tend to predominate
in one sex more than in the other, except in so far as that adult males
having in the past, at all events, followed more outdoor occupations and
having therefore been .more subject to colds, there has been a predominance
among males.
Pathology.
There is a subacute inflammation of the mucosa of the middle ear,
especially in the region of the oval and round windows; once a fibrotic
state has been initiated, no treatment will remove the excess of fibrous
tissue already formed, or prevent it from subsequently contracting.
In certain cases it is the result of acute otitis media and persists until
myringotomy has been performed and for a while afterwards, the duration
of such persistence depending on: (a) Duration of the otitis media before
myringotomy was performed; (b) the activity and nature of the inflamma.
tory process.
In very few cases is the bearing damaged after acute otitis media, but
when it is the deafness is so severe in degree as to interfere materially with
the earning of a livelihood.
Now, whereas in acute otitis media the eustachian tube is blocked, in
subacute otitis media it is intermittently patent; moreover in the latter
condition the inflammatory process is of low grade as the micro· organisms
are of low virulence and the pus can drain away down the patent eustachian
tube; if such drainage is efficient, then recovery occurs, but should it be
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O. A. Hutchinson
297,
inefficient the state of inflammation may be long maintained and there is
a very gradual advance in the degree of deafness.
Subjective Symptoms.
Deajness.-There are three different ways in which the hearing may be
affected in membranous periotic deafness : (1) The mucosa round the oval window is affected, but that in the region
of the round window escapes. There is considerable loss of hearing for the
lower tones; the hearing for higher tones is comparatively unaffected;
Rinne is negative (i.e. B.O. is greater than A.O.) for the lower tones.
(2) The mucosa in the region of the round window is affected, but that
round the oval window escapes. The lower tones are heard moderately
well; the hearing for the highest tones is lost.
(3) The mucosa in the region of both windows is affected. There is
good, or at least fair, hearing for the middle tones; there is also progressive
loss of auditory acuity for the tones towards either end of the register.
Paracusis.-In about half the cases there is well marked paracusis, but
in 99 per cent of these it is " false paracusis."
There are three conditions of altered hearing which dosely simulate
periotic deafness and which have to be excluded in making a diagnosis.
In all three the degree of deafness is high, but the hearing is quite suddenly
restored almost, or quite, to normal.
(a) In the case of audit01'y fatigue, to which ears with this kind of
deafness are very prone, temporary recovery occurs.
(b) Where the condition is apparently due to an altered cochlear blood
supply the inhalation of amyl nitrite, or listening to a loud buzzing noise
(which congests the tympanic blood-vessels) will temporary restore the
hearing.
'
(c) Where there is an overstretched tensor tympani: here the clinical
symptom is deafness for conversation, coming on.in waves, while there is
good hearing for simple tones (so-called" curtain deafness "). Temporary
recovery of hearing can be secured by the use of artificial wax.
Prognosis.
If periotic deafness is present and the patient has never had a perforation
the eustachian contents should be aspirated and subjected to microscopical
examination.
If the contents contain no micro-organisms, or such micro-organisms as
tbere are disappear in one and a half hours on standing, the case is recovering
spontaneously.
If desquamated cells are present, Jeucocytes are sparse, and the mucus
is thin, the deafness is apt to be more severe than in the above type of case.
Treatment.
The treatment of both types of periotic deafness will be considered
together.
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Periotic Deafness
(2)
BONY TYPE OF OTOSCLEROSIS.
This is a condition affecting 0'5 per cent of patIents.
It does not necessarily produce deafness; only doing so when either
(a) the channels of the cochlea, (b) the auditory nerve fibres, or (c) the
membranes of the round and oval windows are encroached upon. It is
then characterized by chronic progressive deafness; there are no ordinary
symptoms of catarrh.
Pathology.
There are three distinct stages in its development :Stage ]. Periosteal· congestion of the inner tympanic wall, causmg
trophic changes in the bone.
Stage '2. Osteoporosis: (a) Most commonly of the promontory in the
region of the anterior margin of' the oval window, where there is an
anastomosis between the vessels of the middle ear and those of the labyrinth
capsule; (b) of the bone of the vestibule; (c) of the cochlear walls; (d) of
the bony semicircular canals. Along with the osteoporosis there is osteosclerosis and degeneration of the organ of Corti.
Stage 3. Bony ove1"g1"Owth, resulting in ankylosis of the stapes.
Many theories have been advanced as to the retiology of this interesting
condition, of which the principal are : (1) That it is due to a chronic, locally infective, inflammatory process
starting in the mucoperiosteum of the middle ear.
('2) 'fhat it is due to a hereditary developmental anomaly in the postembryonic growth of the labyrintb capsule, the primary fault being an
inherent defect in the'living cells of the organ of hearing.
(3) That it is part of a general toxic condition; the bony changes being
similar to those found in.osteitis deformans, rickets and osteomalacia.
(4) That it is due to trophic changes in the eighth nerve, associated
with parathyroid hypofunction. (1'here is diminished blood calcium in
otosclerosis.)
(5) That it follows diminished local arterial supply.
(6) That it is associated with anremia.
(7) That it follows otitis media.
(8) That it is of the nature of a neoplasm.
-(9) That it is a familial or congenital condition (Hammerschlag's type,
and Van del' Hoewe's syndrome).
(10) That it is in some way associated with the sex functions, and
alterations in the!:le; for it occurs most frequently at puberty, during
pregnancy and the menopause (some authorities, however, dispute this,
and state that it occurs most frequently between the ages cif 20 and 30) ;
while a series of exacerbations are associated with any causes making a
severe call on the vital forces.
(11) That it is a gouty manifestation, it being often associated with this
condition.
(12) That it is associated with other mesenchymous tissue abnormalities
(e.g. fragilitas ossium, blue.sclerotics, etc.).
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A. Hutchinson
299
None of the above theories, however, seems to cover all the facts and
the most attractive explanation to date is that of Eckert Moebius, which is
that there are the following factors: (a) A constitutional factor-inherent
endocrine disturbances affecting the mesenchyme; (b) biologically feeble
osseous tissue-in the region of the anastomosis between the vessels of the
middle ear and those of the labyrinth capsule; (c) metabolic disturbancesacting as exciting factors.
Proven facts regarding it are: (a) That it has a definite racial predilection for Jews; (b) that it is especially prevalent in females; (c) that
in certain cases it certainly is congenital and familial; (d) that fragilitas
ossium is prevalent in families showing the familial tendency to otosclerosis; (e) that a high proportion of cases are congenital in nature, but
delayed in onset.
Subjective Symptoms.
Tinnitus.- With a family history of early progressive deafness, which
does not respond to treatment, tinnitus is at first intermittent; it may
become continuous and very intense. The rapidity of progress of the
condition varies with the intensity of the tinnitus.
Whereas in dry catarrh the tinnitus is usually referred to the ear
affected, in otosclerosis the tinnitus is as a rule referred to the head.
Progressive Deafness.-Very little affected by climatic changes; it is
worse after fatigue. At first it is unilateral, and often the first sign is
inability to hear low general conversation.
Vertigo.-This is rarely found.
Paracusis.-Often marked, but" false" in character.
Objective Signs.
(1) Inspection of the tympanic membrane shows nothing; or it may be
found that the membrane transmits the pinkish colour of the promontory.
This, when found, is pathognomonic.
(2) The external auditory meatus is glazed and dry.
(3) The eustachian tube is often widely patent.
Note.-It must be borne in mind tpat evidence of eustachian obstruction
or the presence of chronic suppurative otitis media does not negative a
diagnosis of otosclerosis.
Types of altered Hearing found in Bony Otosclerosis.
These conform to those found in membranous periotic deafness (q.v.).
It is therefore unnecessary to repeat them in detail, and it will suffice
to point out :Type I, some 1 per cent of cases.-This is due to altered movements of
the stapes. It produces deafness very similar in type to obstructive
deafness, but eventually of far greater degree.
Type II, some 2 per cent ot cases. -This is found when the region of
the round window is involved. It is characterized in some cases by a sudden
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Periotic Deafness
drop in hearing for the highest tones (this constitutes the "Manasse type").
Here it is the first turn of the cochlea which is affected, and the altered
bone presses on the nerve filaments between Corti's organ and the spiral
ganglion.
Type Ill, some 97 per cent of cases.-This is characterized by: Negative
Rinne for the lower tones; positive Rinne for tones about the middle of the
register; neutral Rinne for two tones-one in the neighbourhood of 64,
and the other between 256 and 1,024. Lastly, the pitch at which the
lower Rinne is obtained rises as the deafness increases.
Deafness in bony type of otosclerosis tends to be of " uncompensated
type." Now many cases of "uncompensated deafness" do not hear
because the patient does not listen, but in clinically recognizable
otosclerosis there is a definite reason for the lack of compensation, which
is that the ends of words are not cut off sharply by the ear in hearing, i.e.
"Abklingen Deafness ", and speech is only audible to these patients when
its rate is very slow, i.e., some one or two syllables per second.
The diagnosis of osseous otosclerosis cannot be made with certainty
during life, but is mainly a matter of conjecture based on the presence
of profound middle ear deafness, not responding to any form of treatment,
without there being any signs of middle ear disease.
Prognosis.
This is definitely bad.
The patient should be told that the condition is not serious so far as
danger to life is concerned; that the progress of the condition is very
slow, and that she (for the patient is usually a female) has not got
"brain disease "-for with some patients fear of this amounts almost to
an obsession.
Treatment.
Beware of confusing the membranous and osseOllS types in the early
stages. The membranous type is eminently curable when taken early,
and it is therefore best to regard all cases as being membranous in type
and to treat them as such, until the utter hopelessness of the case shows
it to belong to the bony type.
It must be clearly realized that local treatment is useless, or may be
actually harmful. It may, however, be worth while trying eustachian
. aspiration at weekly or more frequent intervals, and when pus is no
longer present spraying the eustachian tube with liquid paraffin on one
or two occasions.
The general health should be maintained, and anremia should be
energetically treated with iron and arsenic. This is most important.
The iodides and phosphorus have their advocates.
Any local" septic focus in the nose, nasopharynx, etc., should be thoroughly
attended to.
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O. A. Hutchinson
Some cases show a definite mild acidosis, and for such the judicious
use of sodium bicarbonate is recommended.
Autogenous vaccines from the nasopharynx have been suggested, but
their use is of very doubtful value.
Galvanism may be tried, but it acts (if at all) mainly by suggestion.
Endocrine therapy may be given a trial as follows: Pituitary extract
one half grain daily by the mouth for a month. Then wait a month
and repeat.
Adrenal extract has also been tried.
A line of treatment which has found favour with some authorities is
to give:Zinc phosphide
-Lecithin
Calcium glycerophosphate
grain
4 grains
4
i'l)
One cachet of the above thrice daily for three months. Then interrupt
tbis treatment for one month; during the interval give four injections
of Martindale's ampoules of pituitary-one cubic centimetre at a time.
This full course to be repeated three times.
As regards the hearing, try and establish" compensation," and re-train
the patient to hear.
The tinnitus and vertigo should be treated by first freeing the eustachian
tube from infection and removing the primary infective focus; then inflate
via the eustachian tube with the external auditory meat us filled with
artificial wax. This must not be left in for more than seven days.
A good formula for artificial wax is :IJ;
Spermaceti ..
Cera tlava ..
Lanolin
1 dram
1
"
1 fluid ounce
the right consistency being arrived at by varying the proportions of the
ingredients.
If all the above methods have failed the patient should be taught to
lip-read, and, if there be paracusis, given an electrical aid to hearing-once
the bearing has become stabilized. If there be no paracusis, give the shell
type of instrument.
Lastly, there rf'mains Sourdille's operative method. This has been
performed by him in over 150 cases with some fair prospect of success,
but the risk of entirely destroying the hearing has to be taken. The
operation consists in the construction of a new secondary tympanic membrane in the region of the external semicircular canal from a flap of
tympanic membrane, and is carried out in three stages as follows :Stages 1 and 2.-'l'hese are performed under general anresthesia, and
consist of: (a) Construction of a flap of membrana tympani, after
performance of a modified radical mastoid operation. (b) Sliding along of
the flap as a graft to a position over the external Remicircular canal. The
cavity is allowed to epithelialize completely after each stage.
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Periotic Deafness
Stage 3.-Under local anresthesia the graft is raised and the external
semicircular canal gradually chiselled through, till suddenly the patient
remarks that he can hear. The graft is then replaced and the wound
allowed to heal.
SUMMARY.
(1) There are two distinct types of periotic deafness: . That due to
changes in the mucous membrane and that due to changes in the bone.
(2) The prognosis of the two is very different, the membranous type
being eminently curable in the early stages, while the bony type is
definitely hopeless.
(3) It is best to regard all cases as membranous and to treat them
energetically as such, until failure of response to treatment after prolonged
trial shows that it is useless to persevere further, as the osseous type must
be the one prevailing.
(4) When attention to any local focus of sepsis has been paid and the
various medical lines of treatment suggested have been all tried out to no
purpose, the patient should be taught to lip-read and fitted with the
appropriate aid to hearing. Finally, it may be worth while giving
Sourdille's operation a trial.
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Periotic Deafness
C. A. Hutchinson
J R Army Med Corps 1934 63: 296-302
doi: 10.1136/jramc-63-05-02
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