An unusual cause of hearing loss in an 81-year-old man

An 81-year-old man presented to accident and emergency with a recent history of rapidly progressive bilateral hearing loss. Further questioning revealed recent sinusitis, fevers and general malaise. Initial bloods showed raised inflammatory markers, raised creatinine, low albumin and urine dip was positive for blood and protein. Chest x-ray showed faint bilateral mid-zone infiltrates. He was treated with intravenous methylprednisolone (500 mg×3) for presumed Wegener’s granulomatosis which was later confirmed by c-anti-neutrophil cytoplasmic antibodies testing (proteinase-3 positive) and supportive findings on CT chest and sinuses. He was discharged after 10 days in hospital on oral prednisolone and cyclophosphamide. He is continuing treatment and seen regularly in outpatient follow-up. Unfortunately his hearing has not fully recovered and he is awaiting a hearing aid.

[1]  C. Kallenberg Pathogenesis of ANCA-associated vasculitides , 2011, Annals of the rheumatic diseases.

[2]  D. Scott,et al.  Prevalence and incidence of Wegener's granulomatosis in the UK general practice research database. , 2009, Arthritis and rheumatism.

[3]  Li Chen,et al.  [Otologic manifestations of Wegener's granulomatosis]. , 2007, Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgery.

[4]  K. Chakravarty,et al.  BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis. , 2007, Rheumatology.

[5]  P. Langevitz,et al.  Hearing Loss as the Presenting Feature of Systemic Vasculitis , 2007, Annals of the New York Academy of Sciences.

[6]  M. Rabbani,et al.  Unusual Presentations of Wegener's granulomatosis: Pitfalls in Early Diagnosis. , 2003, Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia.

[7]  Hiroyuki Ozawa,et al.  Sensorineural hearing loss from suspected Wegener's granulomatosis; report of 2 cases , 2003 .

[8]  Y. Furuta,et al.  Otologic Manifestations of Wegener's Granulomatosis , 2002, The Laryngoscope.

[9]  J. Feehally,et al.  Age-Related Variations in Presentation and Outcome in Wegener's Granulomatosis , 1997, Journal of the Royal College of Physicians of London.

[10]  M. Cotch,et al.  The epidemiology of Wegener's granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. , 1996, Arthritis and rheumatism.

[11]  B. Haynes,et al.  Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. , 1983, Annals of internal medicine.

[12]  H. Schuknecht,et al.  Pathology of Presbycusis , 1969 .