Wegener's granulomatosis with otological and nervous system involvement

Wegener's granulomatosis usually presents to the otolaryngologist with nasal manifestations. Complications including otologic and central nervous system symptoms occur in 20-40 per cent of cases. Two such cases presented to our clinic with nasal manifestations initially, one subsequently developing otologic sequelae and one progressive sensory motor neuropathy. These patients were followed to determine the reversibility of the otologic and sensory motor neuropathy while on therapy. The otologic changes remain and this patient was required to use a hearing aid. The sensory motor neuropathy improved rapidly so that the other patient's ability to walk soon became normal but sensory deprivation continues below the ankle bilaterally. The literature concurs with findings that involvement of the central nervous system and special sense organs such as the cochlea have limited reversibility once they occur, and early detection, therapy, and prevention of such complications are most important.