Abstract
We report on a patient with Lyme disease who presented with chronic bilateral otitis media and cranial neuropathy with rapid progresive hearing loss. After ceftriaxone and high-dose intravenous immunglobulins, the disease was controlled only with methylprednisolone and cyclophosphamide. The relationship between Lyme disease and granulomatous vasculitis is discussed. Lyme disease should be kept in mind in the differential diagnosis of various otolaryngological and neurological presentations.
1
Introduction
Otolaryngological manifestations of Lyme borreliosis (LB) may be present in up to 75% of patients, in all disease stages . Sensorineural hearing loss in LB seems to be due to cranial neuropathies affecting the vestibulocochlear nerve, more often in its cochlear division. Several different rates of hearing loss (1.5%–44%) have been reported . However, granulomatous otitis is not classically described in conjunction with Lyme disease.
Granulomatosis with polyangiitis (GPA) is a small-vessel necrotizing systemic vasculitis with oto-laryngological, pulmonary and/or renal involvement . Common presenting symptoms include headache, sinusitis, rhinorrhea, otitis media, fever and arthralgia . Although secondary vasculitis was reported in LB, there are no reports, to our knowledge, of granulomatous vasculitis involving the ear with GPA features, in this setting.
2
Case report
A 60-year-old diabetic male patient presented with an 8-week history of headache, otalgia, draining and diminished hearing in his right ear. Otoscopy revealed a perforated tympanic membrane and a polypoidal granular mass arising from the middle ear ( Fig. 1 ). Audiometry confirmed a mixed, mainly conductive, hearing loss in the right ear. Temporal bone CT-scan showed blurring of the tympanic cavity and of the mastoid cells. Skull MRI revealed a hyperintensity signal in T2 on the right mastoid bone. Biopsies for presumed carcinoma of the middle ear were negative. Instead, chronic granulating otitis media was diagnosed.
Since there was no clinical response to conservative therapy, he underwent a tympanomastoidectomy canal wall up procedure. Severe granulation tissue filling the epitympanum, aperture of the mastoid antrum and mastoid cavity was found during surgery. The histopathological exam from the ear revealed granulomatous inflammation with areas of necrosis ( Fig. 2 ). Due to the diabetes mellitus and to endemic area, the first etiology to be excluded was tuberculosis; the chest radiology, quantiferron test and the Ziehl-Nielsen coloration were negative, as well as the bacteriological and fungal examination. The ANCA antibodies, searched for in order to rule out a GPA, were absent. The patient was discharged with antibiotic and non-steroidal antiinflammatory therapy.
Two months later the patient was admitted to the Neurological Department with a right-sided facial drop, along with severe headaches, diplopia and blurred vision, bilateral hearing loss, speaking and liquid swallowing difficulties ( Fig. 3 ). A polyneuropathy of cranial nerves most probable of inflamatory origin (Bannwarth syndrome) was diagnosed. Audiometry showed a mixed bilateral medium hearing loss. The laboratory showed elevated ESR (80 mm/h), leukocytosis (11,800 WBC with left shift) and anemia (Hb 8.2 g/dl). He had normal renal function with no signs of glomerular involvement. The hepatitis B and C viruses, HIV, ANCA p and c antibodies, antinuclear antibodies, rheumatoid factor, anticardiolipin antibodies, lupus anticoagulant, C3 and C4 complement fractions, acetylcholine receptor antibody, angiotensin converting enzyme, vitamin B12, and folic acid level test results were normal. The ELISA anti Borrelia burgdorferi IgG antibodies were positive (147.2 RU/ml, EUROIMMUN kit, normal value < 16 RU/ml), with no IgM detectable. Western-Blot confirmed the IgG positivity for Borrelia garini i (p18, p39, Osp-C, p58), with negative IgM.
Results of cerebrospinal fluid testing showed pleiocytosis with lymphocytosis (> 85% lymphocytes), with negative cultures. The CSF albumin was 657 mg/ml while in serum was 38.6. The CSF/serum albumin report (QAlb) was 17. The CSF/serum report for IgG (QIgG) was 19.9, the same report for IgM (QigM) was 7.8, and 1.17 for IgA respectively, revealing according to Reiber’s diagram an increased intratechal synthesis of IgG and IgM (Vrethem). The specific Borrelia CSF index (EUROIMUN) was 1,7. With the diagnosis of Lyme neuroborreliosis, ceftriaxone (2 g once daily intravenously) was prescribed for 4 weeks, along with a tapering course of prednisone. Despite partial neurological improvement, hearing loss showed no alleviation. One month later the patient was admitted to the hospital having general weakness, arthralgias, myalgias, difficulties in walking and sudden bilateral hearing loss. Audiometry showed a mixed bilateral severe hearing loss. The Borrelia IgG titer was still elevated (160 RU/ml), with negative IgM. Computed tomography (CT) of the sinuses revealed inflammation in the both tympanic chambers, epitympanic recess and mastoid air cells. A vasculitis (GPA) possibly triggered by the chronic Borrelia infection was suspected. The repeated ANCA testing was again negative. Antimicrobial therapy was repeated and a course of high-dose intravenous immunoglobulins (0.4 g/kg for 4 days) was not followed by any improvement. After 3 weeks, pulsed methylprednisolone (totally 2 g) and then cyclophosphamide (15 mg/kg), a total of 6 pulses, with oral corticotherapy and co-trimoxazole between pulses, resulted in marked improvement of clinical condition. After 6 months the patient suddenly died because of acute myocardial infarction. Autopsy was refused by the family.
2
Case report
A 60-year-old diabetic male patient presented with an 8-week history of headache, otalgia, draining and diminished hearing in his right ear. Otoscopy revealed a perforated tympanic membrane and a polypoidal granular mass arising from the middle ear ( Fig. 1 ). Audiometry confirmed a mixed, mainly conductive, hearing loss in the right ear. Temporal bone CT-scan showed blurring of the tympanic cavity and of the mastoid cells. Skull MRI revealed a hyperintensity signal in T2 on the right mastoid bone. Biopsies for presumed carcinoma of the middle ear were negative. Instead, chronic granulating otitis media was diagnosed.
