| Paget
disease and sensorineural hearing loss associated with spiral
ligament degeneration. Otol Neurotol.2005 May;26(3):387-91.
HYPOTHESIS:
Previously unreported cystic degeneration of the spiral ligament in
cases of Paget disease. BACKGROUND: About 70% of cases of Paget
disease involve the skull, with hearing affected in approximately
50% of these. The hearing impairment may be sensorineural, mixed,
or, rarely, only conductive. The etiology and pathogenesis of the
hearing loss are not yet understood, and reports in the literature
are inconsistent regarding the pathologic changes responsible for
sensorineural hearing loss. Of six pairs of temporal bones from
patients with Paget disease in the temporal bone collection of a
research institution, two pairs have abnormalities not previously
associated with sensorineural hearing loss or Paget disease. We
report the histopathologic findings in these temporal bones.
METHODS: The temporal bones were fixed in formalin, decalcified in
ethylenediaminetetraacetic acid, embedded in celloidin, and
sectioned in the horizontal plane at a thickness of 20 microm. Every
10th section was stained with hematoxylin-eosin and mounted on glass
slides. The sections were examined by light microscopy. RESULTS:
Cystic degeneration of the spiral ligament, primarily in the basal
segment, was found in both cases. Endolymphatic hydrops and a small
endolymphatic sac with calcification of the perisaccular tissue were
found in one case. CONCLUSIONS: Cystic degeneration of the spiral
ligament has not been previously reported and may be unique to Paget
disease. This is consistent with recent literature showing a
previously unsuspected role of the spiral ligament in sensorineural
hearing loss.
The mechanism of
hearing loss in Paget's disease of bone. Laryngoscope. 2004
Apr;114(4):598-606.
OBJECTIVES/HYPOTHESIS:
The mechanism of hearing loss (HL) in Paget's disease of bone was
investigated. The present study was a systematic, prospective,
controlled set of clinical investigations to test the hypothesis
that there is a general underlying mechanism of HL in Paget's
disease of bone and to gain additional insights into the auditory
and otologic dynamics of this disease. Specific questions were 1)
whether the mechanism is cochlear or retrocochlear and 2) whether
the bone mineral density of the cochlear capsule is related to
hearing levels. STUDY DESIGN: Several double-blinded,
cross-sectional, prospective, correlational studies were conducted
in a population of elderly human subjects with skull involvement
with Paget's disease versus a control population of elderly subjects
free of Paget's disease. Demographic and clinical data were
recorded. Longitudinal observations were made in subjects under
treatment. METHODS: Subjects were recruited from a Paget's disease
clinic. Pure-tone auditory thresholds, word recognition, and
auditory brainstem responses (ABRs) were recorded. The dimensions of
the internal auditory canals were measured using computed
tomographic (CT) images and digital image analysis. The precision,
accuracy, and temporal stability of methods to measure the bone
mineral density of the cochlear capsule and an adjacent area of
nonotic capsule bone were validated and applied. Correlations were
sought between hearing levels and cochlear capsule bone mineral
density. RESULTS: ABRs were recorded in 64 ears with
radiographically confirmed Paget's disease involving the skull.
Responses were absent in eight ears, all of which had elevated high
pure-tone thresholds. ABRs were interpreted as normal in 56 ears;
none were abnormal. The mid-length diameter and minimum diameter of
the internal auditory canal of 68 temporal bones from subjects with
Paget's disease were found to have no statistically significant
relationship to hearing thresholds. The Pearson product-moment
correlation coefficients (age- and sex-adjusted) in the group with
Paget's disease involving the temporal bone were -0.63 for left ears
and -0.73 for right ears for high-frequency air conduction pure-tone
thresholds (mean of 1, 2, and 4 kHz) versus cochlear capsule
density. Correlation coefficients (age- and sex-adjusted) between
cochlear capsule density and air-bone gap (mean at 0.5 and 1 kHz)
for the affected group were -0.67 for left ears and -0.63 for right
ears. All correlations between hearing thresholds and cochlear
capsule density in pagetic subjects were significant at P <.001.
The regressions were consistent throughout the ranges of hearing
level. There were no significant correlations between cochlear
capsule mean density and hearing level in the volunteer subjects.
CONCLUSIONS: The evidence supports the existence of a general,
underlying, cochlear mechanism of pagetic HL that is closely related
to loss of bone mineral density in the cochlear capsule. This
mechanism accounts well for both the high-frequency sensorineural HL
and the air-bone gap. Early identification, radiographic diagnosis
of temporal bone involvement, and vigorous treatment with
third-generation bisphosponates are important to limit the
development and progression of pagetic HL.
Paget's disease
and fibrous dysplasia.Otolaryngol Clin North Am.2003
Aug;36(4):707-32.
Paget's
disease and fibrous dysplasia are benign disorders that can involve
the temporal bone and skull base. They commonly lead to
otolaryngologic symptoms such as impingement of cranial nerves or
the orbit or blockage of the external auditory canal or paranasal
sinuses, although they can often be a challenge to diagnose because
of their insidious presentation. Their benign nature and common
presentation within the difficult-to-access confines of the skull
base should lead the clinician to exercise caution in their
treatment, reserving surgical intervention for either diagnosis or
the relief of symptoms. As a better understanding of the etiology of
these conditions develops and new pharmacotherapeutic agents are
tested, it is likely that physicians will be able to turn to medical
rather than surgical techniques to treat these disorders.
A case of Paget's
disease with progressive sensorineural hearing loss involved in the
bilateral bony labyrinths.Nippon Jibiinkoka Gakkai Kaiho.1996
Jun;99(6):910-7.
Paget's
disease of the bone is a chronic, progressive disease of unknown
etiology characterized by abnormal bony resorption and deposition.
It is a common skeletal disease in Europe and North America, while
in Japan it is very rare. Paget's disease of the temporal bone has
been reported to cause hearing loss frequently. We report a
50-year-old woman with Paget's disease who had progressive bilateral
sensorineural hearing loss and right vestibular dysfunction. CT and
3 Dimensional CT (3D-CT) images demonstrated resorption of the
entire bony labyrinth and its surroundings on both sides and that
the temporal bone elsewhere remained intact. Bone scans revealed
disease symmetrically in the bilateral otic capsules. Previous
studies indicated that the bone changes in Paget's disease in the
petrous pyramid begin in areas best supplied with marrow tissue and
that the otic capsule is relatively spared until advanced changes
are present in the remainder of the petrous pyramid. But, this
patient mainly had foci in the bilateral otic capsules and the
pattern was similar to cochlear otosclerosis. 3D-CT was useful for
differentiation of Paget's disease and cochlear otosclerosis. The
pattern of the affected areas indicated that this is a very rare
situation even in the reports of Europe and North America, where the
disease is rather common.
Hearing loss in
Paget's disease of bone: the relationship between pure-tone
thresholds and mineral density of the cochlear capsule. Hear
Res.1995 Mar;83(1-2):114-20.
We have
developed a unique method of quantitative computed tomography (QCT)
that enables measurement of the density of the cochlear capsule in
vivo. We performed pure-tone audiometry and QCT on 67 ears from 35
subjects with radiographically confirmed Paget's disease of the
skull and on 40 ears from twenty volunteer subjects. The Pearson
product-moment correlation coefficients (age- and sex-adjusted) in
the group affected by Paget's disease were -0.63 for left ears and
-0.73 for right ears for high-frequency air conduction pure-tone
thresholds (mean of 1, 2, and 4 kHz) versus cochlear capsule
density. Correlation coefficients (age- and sex-adjusted) between
cochlear capsule density and air-bone gap (mean at 0.5 and 1 kHz)
for the affected group were -0.67 for left ears and -0.63 for right
ears. All correlations between hearing thresholds and cochlear
capsule density in pagetic subjects were significant at p <
0.001. The regressions were consistent throughout the ranges of
hearing level. There were no significant correlations between
cochlear capsule mean density and hearing level in the volunteer
subjects. These findings demonstrate the feasibility of precise and
accurate density measurements in the temporal bone in vivo and
support the use of the mean cochlear capsule density as a marker of
disease effect. Alteration of cochlear capsule bone density may be
related to the mechanisms of hearing loss in Paget's disease of
bone.
In search of
pathologic correlates for hearing loss and vertigo in Paget's
disease. A clinical and histopathologic study of 26 temporal bones.
Ann Otol Rhinol Laryngol Suppl.1990 Mar;145:1-16.
Mixed
sensorineural and conductive hearing loss is a common clinical
manifestation of Paget's disease of the temporal bone, and while
there are numerous clinical and pathologic reports on the condition,
none have identified a consistent pathologic explanation for the
hearing loss. We performed histologic studies on 26 temporal bones
exhibiting Paget's disease from 16 persons, of whom 7 had
audiometric testing performed. Contrary to common opinion, the
conductive hearing loss is not caused by ossicular fixation; in
fact, no cause could be found in the seven ears with documented
conductive hearing losses. While the sensorineural hearing losses
were greater than normal for age, we could not identify cochlear
disorders that could be attributed to Paget's disease. It is
concluded that the hearing losses in Paget's disease are caused by
changes in bone density, mass, and form that serve to dampen the
finely tuned motion mechanics of the middle and inner ears.
Pathology of the
otic capsule. J Laryngol Otol.1990 Feb;104(2):83-90.
The otic
capsule is unique in retaining calcified cartilage, known as globuli
interossei, throughout life and shows changes consequently, which
are peculiar to it. In Paget's disease of bone, the otic capsule
appears resistant to involvement and this occurs with extensive
disease at a late stage. In contrast, otosclerosis is a new bone
formation of unknown cause that is limited to the otic capsule. In
osteogenesis imperfecta, the poor formation of collagen leads to
abnormally thin bony trabeculae with a poorly formed otic capsule.
In osteopetrosis, the otic capsule is greatly expanded by increased
globuli interossei, as a result of defective osteoclast function.
When fractured the middle layer of the otic capsule does not form
callus, but heals by fibrosis.
Stabilization of
hearing loss in Paget's disease with calcitonin and etidronate. Arch
Otolaryngol Head Neck Surg.1988 Aug;114(8):891-4.
Paget's
disease is a common skeletal disease characterized by abnormal bony
resorption and deposition. Although the disease principally affects
the axial skeleton, skull and temporal bone involvement occur.
Affected individuals have significantly different rates of
progression of hearing loss than normal persons. We describe two
patients in whom this progressive hearing loss was halted and even
reversed. These patients were treated with calcitonin and etidronate
disodium and have been followed up for five and 3 1/2 years,
respectively. Computed tomographic scans demonstrating extent of
disease are included along with our treatment regimen and alkaline
phosphatase response curves.
Hearing disorders
in Paget's disease.
Laryngol Rhinol Otol (Stuttg). 1986 Apr;65(4):213-7.
The
audiometric data of 41 patients with Paget's disease of the bone
show that hearing loss exceeds the age-related presbycusis in
patients with deformities of the temporal bone as demonstrated by
X-ray (27%). The usual type of hearing loss is a sensorineural one.
80% of these patients showed hair cell damage, 32% of them a
retrocochlear lesion, depending on the degree of deformity of the
temporal bone. Conductive hearing loss of the otosclerosis type was
found in only 20%. The Paget-related hearing loss can be regarded as
a consequence of the deformation occurring both at the bone
surrounding the cochlea and at the internal auditory canal.
Paget's disease
and the temporal bone-- a clinical and histopathological review of
six temporal bones. J Otolaryngol.1985 Feb;14(1):20-9.
Paget's
disease of bone occurs more commonly in the elderly and has been
reported to involve the temporal bone in 30% of those afflicted. The
clinical and histopathological features of six temporal bones from
three patients with this disease are reported and the relevant
literature reviewed. The effect of Paget's disease on the middle ear
structures was more variable than its extension into the otic
capsule. Pagetic involvement of the otic capsule was observed in
five temporal bones. One patient had bilateral asymptomatic
neurofibromas in the eighth cranial nerve. the potential mechanisms
responsible for the conductive deafness, the sensorineural deafness
and vestibular dysfunction associated with Paget's disease are
discussed.
Temporal bone
histopathology of Paget's disease with sensorineural hearing loss
and narrowing of the internal auditory canal.Laryngoscope.1977
Oct;87(10 Pt 1):1753-9.
A temporal
bone report is presented of a patient with progressive sensorineural
hearing loss and advanced Paget's disease involving the skull. The
histopathology reveals Pagetic bone invading the internal auditory
canal and compressing the cochlear division of the VIIIth cranial
nerve resulting in severe neural degeneration. There are no other
histopathologic abnormalities present in the cochlea to explain the
hearing loss. A low-frequency conductive hearing loss was also
present, but no histopathologic correlate could be identified.
Tomography of
petrous bone in deafness associated with Paget's disease. Afr J Med
Med Sci.1977 Sep;6(3):115-8.
Two cases of
Paget's disease presenting with deafness are described. Tomography
of the petrous bones showed, in both cases, an upward tilt of the
long axes of the bones including their auditory canals, generalized
sclerosis of the petrous pyramids and enlargement of the ossicles.
In one case, there was narrowing of the internal auditory canal.
Review of the literature revealed three radiological phases in
Paget's disease of the temporal bone:--(i) early changes consisting
mainly of demineralisation; (ii) an intermediate phase consisting of
diminishing visibility of the semicircular canals and cochlea; and
(iii) a late phase consisting of complete blotting out of the
structures of the optic capsule and replacement of the ossicles by
Pagetic bone. Our two cases fall within the last phase.
Paget's disease
and sensori-neural deafness: temporal bone histopathology of Paget's
disease.Laryngoscope.1976 Jul;86(7):1029-42.
Four cases
with Paget's disease of the temporal bone are presented to
illustrate the pathogenesis of the associated deafness. One case
illustrates the combination of severe deafness due to bilateral
otosclerosis with probably asymptomatic bilateral Paget's disease.
One case with advanced Paget's disease presents features to explain
early stages of sensori-neural deafness before actual cellular
invasion of the inner ear. One case of profound deafness due to
Paget's disease presents a different stage of cellular invasion of
the inner ear by the disease on each side. One case illustrates
invasion of the internal auditory meatus by Paget's disease with
infiltration of the acoustic division of the nerve and profound
deafness.
Deafness and
Paget's disease. Rev Rhum Mal Osteoartic.1975 Oct;42 (10):595-9.
Cases of
deafness due to Paget's disease can be separated into two types :
(1) deafness of a mainly mixed type in which progressive aggravation
occurs particularly in the inner ear, and (2) perceptional deafness
which progresses without involvement of the transmission apparatus.
Among 35 hospitalized patients with Paget's disease, 21 of whom
suffered cranial involvement, 18 cases of deafness related to the
bone disease were discovered by means of systematic examinations.
The deafness was of the mixed type in 11 cases and of the
perceptional type in the other 7. Of the 11 patients with the mixed
type of deafness with an ankylosis syndrome of the ossicles, 3 were
operated upon : one of them underwent an operation to mobilize the
stapes, and the two others underwent total stapedectomy followed by
venous interposition and the positioning of a Teflon piston. The
long-term results were frankly disappointing and did not suggest
that these attempts at surgical treatment should be followed up.
Calcitonin was employed in 9 patients (6 with mixed deafness and 3
with perception type deafness), with comparative audiograms in 5
cases, and was also not very effective : this lack of effect was a
result of the long duration of the Paget's disease and of the
deafness in the patients treated. The value of hormonal treatment in
cases of deafness associated with Paget's disease will probably be
in the prevention of this complication.
|
