My son has had his first serious attack of relapsing polychondritis. He has been in the hospital taking 600mg of steroids for three days and still has only partial hearing in one ear. Any chance he will still regain his hearing? His nose has collapsed from the cartilage damage. Can this be repaired by a plastic surgeon?

T.D.

Relapsing polychondritis (RP) is an unusual disease characterized by inflammation of cartilage in many parts of the body. The external ear and nasal septum are the most commonly affected areas. The voice box and windpipe may also become inflamed, and involvement of these structures can lead to lethal airway complications. Inflammation of the ribs and joints can lead to serious disability.

The nasal septum is a part-bony, part-cartilaginous partition that separates the two nasal cavities. If it is destroyed by infection, inflammation or trauma, then the nose loses a great deal of its intrinsic structural support. The nasal collapse which results is both cosmetically disfiguring and disabling, as nasal airway obstruction is usually severe.

It is indeed possible to "rebuild" a collapsed nose. Small, rectangular pieces of bone can be taken from the skull to create new structural support for the nose. Some plastic surgeons and some ear, nose and throat surgeons are competent to perform such an operation. Your son should carefully question a prospective surgeon regarding his or her experience with this operation.

With regard to hearing, RP can cause conductive hearing loss due to inflammation of the external auditory meatus (the opening to the ear canal) or inflammation of the canal itself. Conductive hearing loss occurs when sound energy is blocked in its passage from the external ear to the inner ear. This form of hearing loss should be reversible. Depending the severity of the problem, surgery may or may not be necessary.

Oddly enough, RP may also cause deafness due to inner-ear involvement. I say "oddly enough," because the inner ear is encased in bone, not cartilage. Nevertheless, RP may at times affect noncartilaginous tissues such as this or even the eye or heart.

If your son's hearing loss is due to inner-ear involvement, there is little that can be done to help him, short of hearing aids. The nature of his hearing loss (conductive and/or inner-ear related) can be determined with an audiogram.

Although I have stated that his nasal and hearing problems might both be amenable to surgical correction, I should add a strong word of warning. Surgery in patients with relapsing polychondritis is notoriously treacherous. Injury -- even mild injury -- to cartilage-bearing structures can provoke an attack, leading to further complications. For example, in order to undergo a nasal reconstruction, your son may have to have general anesthesia. This could require endotracheal intubation (placement of a tube into the windpipe, to ventilate your son's lungs during the operation.) This, in turn, could lead to an attack of RP affecting the windpipe -- possibly with deadly results. Thus, before undergoing any operation to correct the effects of RP, your son must have a very careful discussion of RP-specific risks with the surgeon AND the anesthesiologist.