Sensorineural hearing loss in a patient with ankylosing spondylitis.

Abstract

The publication of a case report by Sang et al. in which immune-mediated sensorineural hearing loss was observed in a patient with ankylosing spondylitis (AS) prompted us to report this case [1]. We wish to describe a patient with AS, sensorineural hearing loss, and minimal aortic insufficiency whose mother and brother are also human leukocyte antigen (HLA)-B27-positive AS patients. The 22-year-old male was first referred to our department in March 2003 with lower back pain, prolonged morning stiffness, pain in the right hip, synovitis in the left knee, and bilateral hearing loss. He had had lower back pain and stiffness for 4 years. On physical and neurological examination, the patient showed pain and limitation of lumbar spine movement and loss of lumbar lordosis. He had had right hip pain for 3 weeks and synovitis in the left knee for 2 weeks. Sacroiliac stress tests were positive bilaterally. On testing, the chest wall expansion was 3.5 cm. Modified Schober’s test was 4 cm in range, finger-to-ground distance was 10 cm, and occiput-to-wall distance was 0 cm. Results of the laboratory investigations showed haemoglobin 13.0 g/dl, white cell count 17.1·103/ll, predominantly neutrophils (83.4%), increased erythrocyte sedimentation rate of 58 mm/h (Westergren), normal serum chemistry, and normal urine analysis. Immunology screening showed a positive result for Creactive protein of 15.3 mg/dl (normal 0.00–0.800) and negative tests for rheumatoid factor, antinuclear antibodies, and antibodies against double-stranded DNA. Human leukocyte antigen typing was positive for B27. The immunoglobulin G level was increased to 1740 mg/dl (normal 751–1560). Immunoglobulin A and M levels were 231 mg/dl (normal 82–453) and 146 mg/ dl (normal 46–304), respectively. Synovial fluid analysis of the left knee was normal. Pulmonary function tests and arterial blood gas analysis were also found to be normal. Anterior radiography of the pelvis and computed tomography of the sacroiliac joints showed bilateral sacroiliitis. Magnetic resonance imaging of the lumbar vertebrae was normal. The diagnosis of AS was made according to New York criteria [2], and the patient was given sulphasalazine at a dosage of 2 g/day. The patient had additionally suffered from hearing loss for about 6 months. He did not indicate any other previous disease that could affect hearing. In clinical evaluation, the outer ear, tympanic membrane, and middle ear were found to be normal. Pure tone audiometry results revealed bilateral sensorineural hearing loss in the speech frequencies measuring 80 dB in the left ear and 100 dB in the right ear. Magnetic resonance imaging of the temporal bone was normal. Sensorineural hearing loss is a rare complication of ankylosing spondylitis and described in only three reports as an accompanying symptom of AS. This situation prompted us to report this interesting case. Conductive hearing loss in association with AS has been discussed previously. In that report, Magaro suggested the possible occurrence of conductive hearing loss due to arthritic involvement of the ossicular joints in patients with AS [3]. In contrast to this finding, De Miguel suggested in a letter that middle ear involvement did not occur in patients with AS, and he pointed out that loss of sensorineural hearing was found more frequently in patients than in a control population, Rheumatol Int (2004) 24: 252–253 DOI 10.1007/s00296-003-0425-x