Dr
JR Newton, SHO in Otolaryngology
Dr
K D Morley, Consultant in
Rheumatology
Mr
S S M Hussain, Consultant
Otolaryngologist
Ninewells
Hospital, Dundee.
SMJ 2003 49(1): 66-68
Address
for correspondence: jnewton59@hotmail.com
Abstract:
Hearing
loss in a young adult can be devastating and prompt diagnosis and treatment of
the cause are vital. This case report is of a young musician presenting to the
ENT surgeons with deafness and a lower motor neurone facial nerve palsy which
was given a presumptive diagnosis of infective pathology but subsequent
investigations indicated this was an uncommon presentation of Wegener’s
Granulomatosis. Treatment involved systemic Cyclophosphamide followed by
steroids and Azathioprine. Two years after commencing therapy the disease
remains under control but the patient is left with bilateral hearing aids and a
grade 3 facial nerve palsy. Diagnosis and treatment of this multi-system disease
are discussed.
Key
words: Ear, facial palsy, Wegener’s Granulomatosis, Hearing loss,
sensorineural
Introduction:
Hearing
loss in a young adult can be devastating, particularly when the patient is a
music student and the onset of deafness is relatively sudden. Prompt diagnosis
and treatment of the cause are of the utmost importance. Prevalence of deafness
in this young age group has been reported to be as high as 5% 1,2. The most
frequent aetiologies for conductive hearing loss include
impacted wax, infection and effusion, 3 . Sensorineural deafness is
usually idiopathic, 4 with infections and ototoxic drugs 5 accounting for a
small number of cases.
The following report illustrates a case which initially
suggested an infective pathology, but subsequent
clinical manifestations indicated a more significant and potentially fatal
diagnosis.
Case
Report:
A
17 year old music student presented to the Accident and Emergency
Dept. with a one month history of increasing right sided ear ache,
discharge and deafness . He was referred to the ENT department and an inflamed
tympanic membrane with mucoid
discharge in the canal was noted. No obvious herpetic lesions were seen at this
stage. A mild right sided lower motor neurone facial nerve palsy was seen[ House
Brackman grade 2 6] and mixed deafness elicited on tuning fork tests. A
presumptive diagnosis of herpetic infection was made and the patient commenced
on acyclovir with local aural treatment and analgesia. He returned the following
day for pure tone audiometry and a mixed deafness confirmed. He was noted to be
pyrexial, Co-amoxiclav was added and the patient asked to return in one week.
On
review, the right facial nerve palsy had progressed to House Brackman grade 4 6.
The deafness had become bilateral; profound mixed on the right and moderate
conductive loss on the left. There was clear evidence of middle ear disease on
both sides with the right tympanic membrane bulging and erythematous.
The patient was admitted for investigation. Full blood count,
urea and electrolytes, glucose, liver function, viral titres including those for
herpetic infection, and chest x-ray were all normal. The Plasma viscosity was elevated at 1.95 cp, and urinalysis
was positive for protein. An urgent CT Scan revealed complete opacification of
both middle ear clefts. [Figure 1]
A
right tympanotomy was done and the mastoid explored. Extensive granulation
tissue was found but the ossicular chain was intact. There was no pus. Biopsies
were taken from several sites of the granulation tissue.
Whilst
waiting the biopsy results, cANCA immunology was reported as strongly positive,
along with positive anti- PR-3, indicating a likely diagnosis of primary
vasculitis, including Wegener’s Granulomatosis[WG].
Histology then showed a necrotizing vasculitis with a few multi-nucleated
giant cells. In association with the clinical picture, a diagnosis of
Wegener’s Granulomatosis was made.
An
urgent rheumatology opinion was sought and after sperm storage, the patient was
commenced on cyclophosphamide treatment. This involved six separate intravenous
pulses, at fortnightly intervals. Oral daily prednisolone was commenced
concurrently.
At
3 months follow up, the clinical situation had improved, and the PV was within
normal limits. Respiratory and renal function remained unaffected. The patient
was transferred on to maintenance azathioprine and low dose steroid.
Two
years after commencing therapy, his disease remains under control. The hearing
loss is at 40 dB level, and the patient copes well with bilateral hearing aids.
The facial nerve palsy is at House Brackmann Grade 3, and neurophysiological
evidence for further improvement is guarded. There is no systemic disease
involvement, and the blood parameters within normal limits. The cANCA test is
normal. The patient is a musician by trade, and continues as a percussionist
with his orchestra. Two months ago he did his first recording and presented us
with a copy.
Discussion:
This
multi-system disease was first described by Klinger in 1932 7, then subsequently
in 1936 by Friedrick Wegener. 8It is associated with either respiratory or renal
manifestations, but up to a third may present with locoregional processes 9.
Histologically the disease is characterized by granuloma formation and a
necrotizing vasculitis. Incidence of the condition may be rising 10, peaks at
age 40, 11 and shows equal sex
distribution. Cyclophosphamide and prednisolone improve remission rates up to
85% in some series. 12,13.
The
ear may be involved in WG in a number of ways. The commonest presentation is
otitis media 14with effusion,caused by nasopharyngeal inflammation leading to
Eustachian tube dysfunction. These patients often undergo failed medical
treatment for serous otitis media, and tympanostomy tube placement.
Acute
otitis media is a second common presentation; this is caused by the disease
process within the middle ear and mastoid cavity leading to granulomatous
destruction and dissemination 14.
The
aetiology of sensorineural hearing loss in patients with WG remains unknown;
positive immunological findings suggest a possible immunological process within
the cochlea. Other theories about the pathological mechanism include vasculitis
within the cochlear vessels, or granulomatous involvement of the auditory nerve.
Facial
nerve involvement in WG is very uncommon; the VII nerve may be compressed in the
temporal bone, a necrotizing vasculitis of the nerve’s microvasculature or by
neuritis from granulomatous tissue in the middle ear.
Diagnosis
at an early stage in locoregional WG is vital since progression to the classical
generalized form of the disease is common; if this occurs, and remains
untreated, mean survival is 5 months 12,15. Clinical suspicion is therefore
important in any patient with middle ear disease which is unresponsive to
conventional medical therapies. Antineutrophil Cytoplasmic Antibody [ANCA] is
typically positive. This test uses an immunofluorescence staining technique, and
was first linked with WG in 1985 16. Subsequently, ANCA has also been
demonstrated in Polyarteritis nodosa and Churg Strauss vasculitis. The antigen
which correlates with cytoplasmic ANCA is proteinase-3 [PR3].
Rheumatology literature suggests PR3-ANCA is 90% sensitive in active Wegener’s
and 40% when the disease is in remission17. Specificity for PR-3 ANCA is also
90%. The role of serial PR3 ANCA in assessment of disease activity is
controversial17. Its ability to predict relapse or assess disease is limited.
The PV may correlate better with disease activity.
Biopsies from the middle ear can frequently be unsuccessful in
obtaining the definitive diagnosis, as demonstrated in this case.
Treatment
of the condition is best achieved using a multi-disciplinary approach. In this
case, early contact was made between the ENT surgeon and the Rheumatologist. A
combination of cyclophosphamide and prednisolone, administered in cyclical
intravenous pulses not only suppresses systemic disease, but also improves the
otological manifestations 18. A Mastoid exploration was carried out in this case
because of the worsening clinical picture but a hazardous decompression of the
Facial nerve was avoided.
Renal
disease and to lesser extent lung manifestations may be fatal in 80% of patients
within a year if untreated. Further disabling sequellae include profound
sensorineural deafness, facial nerve paralysis,or extension of the inflammatory
process into the skull base leading
to multiple cranial neuropathies. For these reasons, the clinician must be
highly vigilant to the presentation of unresolving middle ear disease.
Conclusion:
The
case described is that of deafness in a young man and the diagnosis was shown to
be Wegener’s Granulomatosis involving the middle ear. Treatment with
cyclophosphamide and systemic steroid was commenced and induced a prolonged
remission. Unresolving middle ear disease should be investigated promptly to
exclude more serious systemic disease such as Wegener’s Granulomatosis, and
once diagnosis has been made, treatment should be commenced immediately.
References:
1
National Centre for Health Statistics. Current estimates from the national
health interview survey 1990 Series 10.
2
Hearing Status of French Youth from 18-24 in 1997; Rev Epidemiologie Sante
Publique . 2000;48:227-37
3
Nadol J. Hearing loss Review article. New Eng J of Med 1993;329:1092-1102
4
Ogawa.H. Nippon Jibiinkoka Gakkai Kaiho 2001;104:1034-43
5
Diseases of the Ear. Sixth Edition. Chapter 36; Acquired Sensorineural Hearing
Loss. Ludman H and Jones N. 1998.
6
JW and Brackmann DE. Facial nerve grading system. Otolaryngology- Head and Neck
surgery 1985;93:146-7
7
Klinger H. Grenzformen der Periarteritis Nodosa. Z Pathol 1932;42:455-80
8
Wegener F. Uber generalisierte septische. Gefasserkrankungen Verh Dtsch pathol
Ges 1936;29:202-12
9
Gross et al. Antineutrophil cytoplasmic autoantibody-associated diseases: a
rheumatologists perspective. American J Kidney Disease 1991;18:175-9
10
Carruthers DM et al Wegener’s Granuolmatosis; increased incidence or increased
diagnosis. Br J of Rheum 1996 ;35:142-5
11
Dagum P and Robertson JB. Otologic Wegener’s Granulomatosis with Facial Nerve
Palsy. Ann Otol Rhinol Laryngol;1998;107:555-8
12
Fauci A.S.,Wolff S.M.Wegener’s Granulomatosis: studies in eighteen patients
and a review of the literature. Medicine [Baltimore] 1973;52:535-62
13
Illum P., Thorling K. Wegener’s granulomatosis. Longterm results of treatment.
Ann Otol Rhinol Laryngol 1981;90:231-5
14
Bradley P.J. Wegener’s Granulomatosis of the ear. J Laryngol Otol
1983;97:623-61
15
Gross WL. Systemic necrotizing vasculitis. Bailliere’s Clinical Rheumatology
11, No 2, 1997
16
van der Woude FJ et al. Autoantibodies against neutrophils and monocytes:tool
for diagnosis and marker of disease activity in Wegener’s granulomatosis.1985
Lancet 1;806
17
Mohan N. and Kerr G. Best Practice and Research Clin Rheum. 2001 Vol 15:203-23
18 Macias J.D. et al. Early diagnosis of otological Wegener’s granulomatosis using the serological marker c-ANCA. Ann Otol.Rhinol.Laryngol. 1993;102:337-41