The physician’s perspective. Most of us go into surgery or medicine because of a deep-seated desire to heal the sick and relieve suffering. We have trained for countless years and have worked tirelessly to perfect our craft. I say this as an introduction to an unfortunate, yet understandable by-product. We hate failure!
At the beginning of the 20th century, the mortality rate from surgery for an acoustic neuroma was greater than 50%. Tumors were diagnosed when quite large and the concept of preserving facial nerves or hearing was non-existent and the primary goal was preservation of life.
Needless to say our diagnostic and treatment tools have come a long way. Now, preservation of life is commonplace as is preservation of facial nerve function. When we see a good result either from radiation or microsurgery we are so delighted. We feel like we have won this battle and all of our training and experience has been put to good use.
We move on to our next challenging case. In doing so, we are missing something very significant.
Taking care of patients with Acoustic Neuroma (AN) is a long and involved process and the most rewarding part of my practice. It begins with a letter, an email, or an appointment in the office. The visit and subsequent conversations are centered on education, helping patients understand the disease and the options for care. After decisions are made, the next phase is treatment.
The patient’s perspective. After managing many hundreds of patients with AN, I have come to understand another perspective: the patient’s. Many of you are happy with your outcomes and anticipate that a great result means a rapid return to “normalcy”. Well I think we would all agree that this is really a new “normal”. The new normal, a condition potentially fraught with dizziness, hearing loss, tinnitus, headaches, fatigue, depression and many other symptoms too numerous to mention. Many patients don’t want to be a bother to their doctor or simply don’t want to seem ungrateful but the bottom line is many of you still need our help.
What needs to change? The doctor/patient relationship needs to continue long after the treatment. The aftermath of treatment needs to be addressed, cared for and followed up. In short, the care continues long after the treatment. When looking at the ANA surveys and talking with hundreds of patients, what seems to stand out as the issues are hearing loss, tinnitus and imbalance.
Let’s begin with hearing loss. First, a basic fact has to be accepted; ANs are tumors of the eighth cranial nerve, or the cochleovestibular nerve, which is the nerve for hearing and balance. Overwhelmingly, the first symptom is hearing loss. Over a century of clinical research, including a rich modern reference base, reveals that regardless of whether you “wait-and-watch,” operate or radiate, the vast majority of patients must live with single-sided hearing loss. That is a fact that must be accepted and dealt with.
Hearing is a bilateral process. Both ears are required for two very simple functions: localizing sound and hearing in noisy environments. Both of these functions are permanently impacted. Now, we must address the “new normal”.
The options include CROS (Contralateral Routing of Signal) – for single-sided hearing loss – or BiCROS (used when the remaining ear also is impaired) hearing aids, conventional deep-fitting hearing aids and bone-integrated hearing devices that capitalize on the ability to transmit sound very well through the normal skull.
The bone conducting devices can be either surgically placed or, utilizing the tooth, can be fashioned by a dentist. These devices allow you to hear sound from your hearing impaired side through your skull and in your “good” ear. They do not restore sound in the affected ear. These devices do very little to help hearing in noisy environments and do nothing for sound localization. Those two functions cannot be restored and must be accepted as the “new normal”.
What does the future hold? In most cases, the hearing nerve is damaged by the tumor whether you are observing or treating. In some instances, the auditory nerve may still be intact and could possibly be useful. Although not currently available in the US, cochlear implants for single-sided deafness may provide some additional benefits for patients that are candidates. All of these things should be discussed with your AN treatment team.
Disequilibrium, or dizziness, is a symptom that, like hearing loss, results from damage to the balance portion of the eighth nerve whether the AN is treated or observed. Dizziness is a “new normal”.
The dizziness can improve after a surgery; not immediately, but, over time. The brain can ultimately compensate for the complete loss of balance function in one ear better than a partial loss. Despite all great intentions, some degree of dizziness is to be expected and managed. For some patients, dizziness resolves completely. For others, dizziness is worse when fatigued. Rarely, dizziness is debilitating.
Although there are limited treatment options, for dizziness, one thing stands out. The harder you work at rehabilitating your balance function, the more likely you are to have a successful recovery.
Successful recovery is largely influenced by vestibular rehabilitation. In order for us to maintain balance we must have three systems intact. First, our vision is key. Moderate or severe visual loss can impact your ability to recover. Second, proprioception, your ability to know where your limbs are in space, is critical to normal balance.
People with diabetes and some vitamin deficiencies, B12 (rare), can have impaired proprioception. Lastly, our inner ears are critical to this process. Vestibular physical therapy is designed to retrain the central balance centers in the brain by exercising the components of this vital triad that are functioning well. The parts of the balance systems that are working well can be strengthened in such a way that they can compensate for the missing parts. Physical therapy should be addressed throughout the treatment process and long after immediate care.
What does the future hold here? Devices are under development that can electronically stimulate the balance centers. These devices may one day provide help for our patients suffering from disequilibrium.
Tinnitus, or head noise, is an unfortunate by-product of hearing loss. Because virtually every AN patient experiences some hearing loss, virtually every AN patient experiences tinnitus.
Tinnitus is a poorly understood phenomenon. What is clear is that it originates in the ear, but over time the response persists and exists in the higher centers of the brain. How do we know this? First, any attempts to correct this symptom by cutting the hearing portion of the eighth nerve only lead to persistence of the tinnitus, or in some cases, worsening. Modern brain imaging such as positron emission tomography (PET) scanning and functional magnetic resonance imaging (MRI) scanning clearly shows that tinnitus moves from the ear and ultimately resides in the brain.
At present, there is very little available that can “stop” tinnitus. Our ultimate goal is to interrupt using medicines the neural pathways that cause tinnitus. As of yet, there are no available medical options.
Many of the current treatment strategies take advantage of the fact that tinnitus is only a problem when it reaches awareness. As most tinnitus suffers agree, when engaged in something fully, tinnitus does disappear. With this in mind, there are a number of devices available that attempt to connect tinnitus to a more pleasant sound or musical stimulus thereby taking away some of the negative associations. The use of these devices can be coupled with cognitive therapy. Several important studies have shown that these two modalities act synergistically to relieve some of the difficulties surrounding tinnitus.
Devices and in some cases simple hearing aids can be very helpful, but each require that the affected ear has some retained hearing. Single-sided deafness presents greater challenges. Some patients find alternative medicine practices such as acupuncture and meditation to be helpful. The future of tinnitus care likely rests on the pharmaceutical discovery. Many centers around the world are hard at work on this.
Summary. Providing the right care for patients with AN requires a comprehensive and dedicated program. Staff with expertise in treatment and a commitment to care before, during and long after the process is essential to maintaining a good post-care quality of life. What is clear from my experience of caring for many hundreds of patients and being deeply involved in the Acoustic Neuroma Association is that we as surgeons and/or radiosurgeons must stay connected with each patient from the beginning and long after treatment has been provided. The presence of a supportive and knowledgeable team is critical to long-term success.