1. Healthcare

European Annals of Otorhinolaryngology, Head and Neck diseases 131 (2014) 265–266
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What is your diagnosis?
Sudden bilateral hearing loss revealing polyarteritis nodosa
F. Rubin , N. Tran Khai Hoan , P. Bonfils ∗
Département d’ORL et chirurgie cervico-faciale, hôpital Européen Georges-Pompidou, faculté de médecine Paris Descartes, université Paris V, 20, rue
Leblanc, 75015 Paris, France
1. Description
Ms L., 56 years old, consulted for an episode of fever (39.5 ◦ C)
associated with asthenia, dry cough, tendon pain and sudden
bilateral hearing loss. She was admitted to the internal medicine
department in view of the persistent fever, severe asthenia,
lower-limb pain-affecting gait, diffuse myalgia and night sweats.
Weight-loss was moderate (2 kg). There was no infectious syndrome or headache. Clinical examination was normal. Initial
biological assessment found biological inflammatory syndrome
(leucocytes 9000/mm3 , CRP 143 mg/L, and slight microcytic anemia at 10.5 g/dL), without kidney failure. Otoscopy found bilateral
serous otitis. Audiography (Fig. 1) found severe bilateral mixed
hearing loss, with mean bone conduction deficit of 53 dBHL
and mean Rinne 14 dBHL (BIAP). Temporal bone CT scan found
moderate middle-ear effusion. Brain and internal auditory canal
MRI found no tumors or signs of central nervous involvement.
Ophthalmologic examination ruled out Cogan’s syndrome. The
nasal cavities and nasopharynx were normal. Lumbar puncture
and microbiology culture were negative. Paracentesis of the
middle-ear sample found hematic inflammatory liquid with 120
elements/mm3 , 1800 erythrocytes/mm3 , 70 neutrophils/mm3 and
10 lymphocytes/mm3 , with negative culture.
2. Question 1
In sudden-onset bilateral hearing loss, what differential diagnosis is to be ruled out in priority?
3. Question 2
What diagnosis would you suggest?
Fig. 1. Pure-tone audiogram.
∗ Corresponding author.
E-mail address: pierre.bonfi[email protected] (P. Bonfils).
http://dx.doi.org/10.1016/j.anorl.2014.03.003
1879-7296/© 2014 Elsevier Masson SAS. All rights reserved.
What is your diagnosis?
266
F. Rubin et al. / European Annals of Otorhinolaryngology, Head and Neck diseases 131 (2014) 265–266
4. Reply 1
Stroke is to be ruled out urgently [1].
Disclosure of interest
The authors declare that they have no conflicts of interest concerning this article.
5. Reply 2
References
Presence of general signs suggested vascularitis. Temporal
artery biopsy revealed lymphocytic arteritis. Polyarteritis nodosa
(PAN) was diagnosed on American College of Rheumatology criteria: myalgia, neuropathy (sudden hearing loss with perceptual
component) medium caliber arterial abnormalities. Corticotherapy (1 mg/kg/day) was initiated. All symptoms except hearing loss
improved within a week. Audiometry at 3 months found bilateral
24 dBHL hearing loss. Only 3 cases of perceptual hearing loss and
4 of bilateral mixed hearing loss have previously been reported in
association with PAN [2–5], with involvement of the arteries of the
ear.
[1] Shibata K, Matsui K, Ito H, et al. Bilateral intracranial vertebral artery dissection presenting as sudden bilateral hearing loss. Clin Neurol Neurosurg
2012;114:1266–9.
[2] Vathenen AS, Skinner DW, Shale DJ. Treatment response with bilateral
mixed deafness and facial palsy in polyarteritis nodosa. Am J Med 1988;84:
1081–2.
[3] Joglekar S, Deroee AF, Morita N, et al. Polyarteritis nodosa: a human temporal
bone study. Am J Otolaryngol 2010;31:221–5.
[4] Adkins WY, Ward PH. Temporal bone showing polyarteritis nodosa, otosclerosis,
and occult neuroma. Laryngoscope 1986;96:645–52.
[5] Rowe-Jones JM, Macallan DC, Sorooshian M. Polyarteritis nodosa presenting as
bilateral sudden onset cochleo-vestibular failure in a young woman. J Laryngol
Otol 1990;104:562–4.