To investigate Stephen’s ear spasms, I ordered a complete hearing test. Any neurological process involving two or more cranial nerves almost always points the finger of diagnostic suspicion to the brain stem, which is the anatomical takeoff point of the cranial nerves. So I also ordered an MRI of my patient’s brain, with and without contrast enhancement, to get a good look at that area.
The tests showed that Stephen’s hearing was completely normal, but the acoustic reflexes could not be tested because of the ongoing spasms in each ear. The MRI of his brain was entirely normal as well. At this point, my most likely diagnosis was myoclonus (muscle spasm) of either or both the stapedius and tensor tympani muscles. The painful eardrum spasms were the result of the contractions of one or both of the middle ear muscles.
All physicians are trained to begin by thinking broadly and then apply reasonable medical and surgical measures to the most likely cause of the clinical problem. Physicians are also trained to treat medically first and to reserve surgery for cases that fail medical management. Because Stephen had already been treated for Lyme with intravenous antibiotics, I suggested a consultation with a neurologist and perhaps a short trial of common medications used for either seizure or peripheral neuropathy, such as tegretol or gabapentin. Tegretol decreases the spread of seizure; it is also used to treat bipolar disorder and trigeminal neuralgia, a condition causing severe facial pain. Gabapentin is a molecule related to gamma-aminobutyric acid, or GABA, a common neurotransmitter. It has well-studied analgesic and anticonvulsant effects, although its exact mechanism of action is unknown. Gabapentin is used for a variety of neurological disorders, including spasm and extremity pain.
Stephen began with a short course of tegretol, prescribed by his neurologist. It did not lessen the symptoms or block the spasms, so I started Stephen on gabapentin. After a few weeks, he reported that it had no beneficial effect either.
Understandably anxious, Stephen had sought a few other consultations, with conflicting recommendations. I suggested he see a colleague of mine in Boston who had a great deal of experience with eustachian tube dysfunction. The Boston doctor made a small opening in Stephen’s left eardrum and inserted a microscopic telescope attached to a video camera to visualize the middle ear muscles. All of this was done under topical anesthesia while Stephen was awake. The results matched my diagnosis but added an important new piece of information: The offending muscle was the tensor tympani muscle, not the stapedius.
Now that we had identified the culprit, how to treat it? There were two possibilities: injecting a neuromuscular-blocking medication, such as botulinum toxin (Botox), into the tensor tympani muscle in the middle ear, or cutting the muscle surgically. The former would be a temporary trial that would wear off in about 6 to 12 weeks. The second option would be permanent. There were potential risks associated with either choice. First, either temporarily or permanently blocking the muscle might not solve the problem. Second, loud sounds coming toward the treated ear might seem even louder than before. Stephen considered his options and, after speaking with his parents, decided to have the muscle cut. He had been suffering with the spasms for so long that he did not want to try a temporary solution.
I started with the left ear because it was the more bothersome. The surgery was all done through the outer ear channel; it involved making small incisions around the eardrum so it could be turned aside like the page of a book. The eardrum was then dissected off the hammer bone to allow me to see the tensor tympani muscle as it approaches the hammer bone at its narrow neck. The nerve to Stephen’s face runs in a bony channel near the tensor tympani muscle. We monitored his face with a electromyography system, which allowed me to electrically stimulate the facial nerve and make sure it was not being injured by the surgery. I then found and divided the tensor tympani muscle, first partially with a small knife and then completely with a scalpel-like laser. I placed a small amount of packing under the eardrum, turned it back into its usual place, put some packing material over the eardrum, and bandaged the ear. Stephen was awakened and transferred to the recovery room.
About an hour later, my patient was fully awake and ready to go home. I went to see him in the patient recovery area and he exclaimed, “It’s gone! I think it’s really gone!” His parents were as overjoyed as he was. It appeared that his long nightmare might finally be ending. Fast-forward several weeks: The surgery did in fact abolish the spasm in Stephen’s left ear. His hearing remained normal, and the spasms around his left eye subsided.
About six weeks after the left side, I operated on the right ear in a similar fashion. This side also healed, and the spasms went away with no significant side effects. Today Stephen has resumed his university studies and is getting his life back on track.
I still had not addressed Stephen’s original question: Was Lyme disease responsible for his ear agonies? I would say yes. The disease certainly has many far-reaching neurological symptoms. As Sir William Osler, regarded by many as the father of modern medicine, said many years ago, “He who knows syphilis knows medicine.” Of course, syphilis was not Stephen’s problem, but the cause of Lyme disease, Borrelia burgdorferi, is in the same family as the causative agent of syphilis, Treponema pallidum. They are both spirochetes, bacteria that cause an insidious range of health issues.
For me, Stephen’s case reinforced the notion that the medical history, as detailed by the patient himself or herself, is the most important part of any medical or surgical encounter. Patients who have difficult medical conditions are usually good historians: They live with the problem all day, every day, and can reflect upon its origins and its clinical course. The patient almost always provides all of the diagnostic clues to the solution, guiding the physician in where to look and how to treat.
Christopher Linstrom is an otologist/neurotologist and professor of otolaryngology at the New York Eye and Ear Infirmary. The cases described in Vital Signs are real, but names and certain details have been changed.