A Case for Multidisciplinary Management of Paragangliomas
A middle-aged man presented with dizziness almost 15 years after he had undergone resection of a glomus jugulare tumor. MRI revealed a massive recurrence of the tumor, along with incidental findings of a synchronous intracanalicular vestibular schwannoma and a large cholesteatoma that was located in his previously closed ear canal (Figure 1). The patient’s dizziness was the result of the cholesteatoma invasion into the lateral semicircular canal. His cranial nerves other than the auditory nerve were intact.
A Role for the Geneticist
For many years, paragangliomas were regarded as the “10 percent tumor” — 10 percent familial, 10 percent malignant and 10 percent extra-adrenal. However, we have since learned that the familial component is greater than 10 percent. The discovery of the succinate dehydrogenase genes (SDHB, SDHC and SDHD) led to the revelation that at least 30 percent of patients with a paraganglioma and no other known risk factors (i.e., no family history, a single tumor) harbor a genetic mutation that increases their risk for these tumors and other neoplasia.
Since such a large percentage of these patients have an underlying hereditary predisposition syndrome, we in the Head & Neck Institute and the Genomic Medicine Institute now recommend referral for a genetics consultation for all patients with a paraganglioma, including those with an apparently sporadic tumor. Not only is genetic counseling and testing critical for guiding treatment and surveillance of the patient, it has the potential to benefit their gene-positive relatives as well.
A Role for Radiation Therapy
An attractive alternative to or adjunct to surgical resection of glomus jugulare tumors is gamma knife radiosurgery. It allows for the delivery of highly conformed low-dose radiation to the tumor in a single administration. This type of delivery minimizes the amount of radiation that is directed to critical structures. According to the literature, control rates have been excellent; one meta-analysis showed that they exceeded 95 percent.1
Yet despite the excellent control rates, long-term expectations following therapy must be taken into consideration. Judicious surgical resection will still be a viable option for younger patients who have a hereditary syndrome that predisposes them to multifocal, recurrent or histologically aggressive tumors.
The Multidisciplinary Approach
The specialists at Cleveland Clinic believe in a balanced approach that includes surgery, observation and radiation. With the goal of maximizing functional outcomes, we advocate customized management. To our way of thinking, there is no single “right way” to treat all patients. For example, we might offer some patients a partial tumor resection with the aim of reducing symptoms and improving function.
Case Study: Epilogue
After further workup of our patient, we developed a comprehensive surgical plan. With the collaboration of specialists in neurotology, head and neck oncology, neurosurgery and facial plastic/reconstructive surgery, we devised a plan for a single-stage resection and reconstruction surgery. The plan was to resect the bulk of the disease, protect cranial nerve function and allow for any residual tumor to be treated with radiosurgery. Unfortunately, preoperative embolization caused a functional deficit in cranial nerves IX through XII, likely secondary to swelling of the tumor. Nevertheless, surgery was considered successful overall; more than 90 percent of the tumor was removed, the integrity of the facial nerve was preserved, the schwannoma and cholesteatoma were removed and an extensive area of involved dura was resected. Free-flap reconstruction allowed for containment of the cerebrospinal fluid. While the patient exhibited a complete facial paresis during the immediate postoperative period, his lower cranial nerve function was unchanged from its preoperative status.
The patient was able to take a fully oral diet (no tube feeds were required), and he was discharged home. He returned to work two months after surgery. At follow-up eight months postoperatively, he exhibited a near-total return of function in each of his lower cranial nerves, and examination of his facial nerve function revealed an improvement to House-Brackmann grade III. His residual tumor burden was indiscernible on MRI (Figure 2). Radiosurgery will be offered if the tumor remnant begins to grow. The results of his genetic studies are still pending.
1. Guss ZD, Batra S, Limb CJ, et al. Radiosurgery of glomus jugulare tumors: a meta-analysis. Int J Radiat Oncol Biol Phys. 2011;81(4):e497-502.
Dr. Woodson is a staff physician in the Head & Neck Institute and the Gamma Knife Center. She can be reached at 216.444.6696 or firstname.lastname@example.org.
Ms. Moline is a certified genetic counselor-coordinator with Cleveland Clinic’s Genomic Medicine Institute and Taussig Cancer Institute. She can be reached at 216.445.6798 or email@example.com.
Dr. Eng is Chair of the Genomic Medicine Institute and a staff physician in the Taussig Cancer Institute. She is also an American Cancer Society Clinical Research Professor and Hardis Endowed Chair in Cancer Genomic Medicine. She can be reached at 216.444.3440 or firstname.lastname@example.org.
Dr. Lorenz is a staff physician in the Head & Neck Institute and the Taussig Cancer Institute. He can be reached at 216.444.3006 or email@example.com.
Figure 1. Axial (A) and coronal (B) MRIs show the massive recurrent left glomus jugulare tumor (arrows) involving the petrous apex and the posterior fossa dura in the previously operated ear. During the patient’s previous surgery, the facial nerve was rerouted at the geniculate ganglion, and the ear canal was oversewn. Not shown are a labyrinthine fistula from the epidermoid cholesteatoma lateral to the glomus tumor and a synchronous intracanalicular vestibular schwannoma in the same ear.
Figure 2. Postoperative MRI confirms the near-total resection of the glomus jugulare tumor and the complete removal of the vestibular schwannoma and cholesteatoma. The tumor remnant (circle) in the petrous apex was left in order to preserve the pars nervosa. An anterolateral free-tissue transfer from the thigh was used to reconstruct the dura and soft tissue.
HNI Partners with Asthma Center To Treat Patients with Laryngeal Hyper-responsiveness
In an arrangement that is not often seen in a large medical institution, clinicians in the Voice Center at the Head & Neck Institute are working closely with those in the Respiratory Institute’s Asthma Center to optimize care for patients with laryngeal hyper-responsiveness (LHR). This is an important benefit for patients because the impact that LHR can have on daily life is substantial. Indeed, some patients experience relentless symptoms that can persist for years, even decades, and significant disability is seen in some cases. Frequent hospitalizations are common.
A Difficult Condition to Manage
LHR is a challenging condition because its constellation of symptoms can defy easy diagnosis. These symptoms can include episodes of severe shortness of breath, chronic refractory cough, a prominent sensation of chest and throat constriction, dramatic stridor and hypersensitivity to fumes and strong smells. The collaboration between the Voice Center and the Asthma Center is a natural progression to multidisciplinary care since LHR patients have been referred to both centers for evaluation.
In many cases, patients who are referred to either the Voice Center or Asthma Center have been previously misdiagnosed with asthma or recurrent anaphylaxis. In fact, the average time it takes to accurately diagnose LHR has been reported to be 4.5 years. Among the reasons for this long delay are that (1) the underlying pathophysiology can be difficult to understand and (2) there has been a lack of unifying terminology. Through the collaboration of our two centers, we hope to significantly shorten the time from symptom onset to diagnosis to effective treatment.
The “Umbrella Construct”
A key component to diagnosing LHR is to think outside the usual algorithms when a patient’s signs and symptoms do not easily fit a traditional diagnosis. LHR encompasses several entities, including irritable larynx syndrome, chronic refractory cough and paradoxical vocal fold dysfunction; this combination of factors is known as the “umbrella construct.” A growing body of literature supports the idea that the aberrant laryngeal activity in these conditions is caused by peripheral sensory and motor neuropathies. Research also suggests that the CNS may play a key role, as we have seen encouraging reports of progress with various neuromodulators, such as gabapentin, amitriptyline, tramadol and pregabalin.
Once a patient has been accurately diagnosed with LHR, our treatment regimen goes beyond drug therapy to include behavioral therapy. As an essential part of our treatment protocol, our expert speech-language pathologists provide respiratory and laryngeal control therapy.
A Model for Others
The degree of cooperation between the Voice Center and the Asthma Center is becoming more common in major healthcare facilities, and it can become a part of community practice as well. Such a collaboration offers the promise of many benefits:
- Streamlines management recommendations.
- Lowers healthcare costs by (1) avoiding duplication of tests and (2) reducing the number of patient visits.
- Improves the timing of diagnosis.
- Shortens the time necessary to achieve successful treatment.
- Improves outcomes.
- Improves patient satisfaction.
- Results in better patient care.
We believe that voice specialists at all levels should make an effort to work with their colleagues in pulmonary and allergy medicine and develop their own alliances.
Dr. Milstein is Director of the Voice Center. He can be reached at 216.444.8677 or firstname.lastname@example.org.
Dr. Hicks is Section Head of Speech-Language Pathology. He can be reached at 216.444.6691 or email@example.com.
Audiology Clinic Is Making Some Noise with a More Realistic Background Sound Replication System
By Sarah A. Sydlowski, AuD, PhD
For most individuals with hearing loss, listening situations that are complicated by background noise and reverberation are common and challenging. But despite the ubiquity of noise in our daily lives, most hearing evaluations are conducted in a quiet environment. Many patients with hearing devices, such as hearing aids and cochlear implants, experience their greatest frustrations in noisy environments. These patients may score well on tests conducted in quiet while wearing their hearing device, but they struggle to understand speech in the noisy environments outside the sound booth. Thus, objective measures designed to evaluate the ability of a patient using hearing devices to understand speech may not accurately reflect the patient’s true hearing ability in their typical listening environments.
Even in those cases when noise is applied during testing, it is typically presented through a single audio speaker that has been placed in front of or behind the listener. Such noise can include narrowband or speech-shaped noise, as well as multi-talker babble. With this noise in the background, the speech of a single talker who is situated directly in front of the patient being tested is used to measure word and sentence comprehension.
Most Simulations Are Not Realistic
Despite our best efforts to replicate a real-world environment with these sound stimuli, most patients report that these simulations fall short of realistic. The reason that current speech-in-noise tests sound disappointingly contrived is that they fail to address four fundamental components of real-world listening in noise:
- Noise arises from many directions at one time.
- The level and spectrum of interfering noise fluctuate, unlike noise that is commonly used in a laboratory environment (e.g., speech-shaped noise).
- Real-world noise signals are partially correlated, as opposed to the two typical options provided in a sound booth (i.e., either correlated or uncorrelated).
- Evaluations are conducted in sound-treated rooms where the levels of reverberation are much lower than they are in most environments.1,2
Surround-Sound Goes Clinical
Cleveland Clinic’s Section of Audiology recently installed new technology that helps us overcome these shortcomings by allowing us to assess hearing in a more realistic setting of background noise. The R-SPACE™ sound system reproduces environmental noise by using an eight-speaker set-up that is arrayed around the patient. The result is that patients have a more realistic sense that they are immersed in everyday noise, such as in a busy restaurant.
The use of this technology benefits both the patient and the clinician; the patient has the sense that the clinician understands the types of challenging environments in which they find themselves, and the clinician is able to obtain valuable information about how these types of environments affect the patient’s ability to communicate effectively. Cleveland Clinic’s cochlear implant program is one of just a handful of programs in the U.S. that offers this technology.
Compton-Conley et al demonstrated that the R-SPACE™ system yields speech-in-noise intelligibility scores that are very similar to those in real-world conditions for normal hearing listeners.3
Revit et al2 demonstrated the same in hearing-impaired listeners, and Gifford and Revit4 did likewise in patients with cochlear implants.
The R-SPACE™ system provides tremendous flexibility, allowing the audiologist to create a variety of speech-in-noise and reverberant speech reproductions while presenting a variety of target word or sentence information from one or more directions.
The introduction of this new technology to our Audiology Clinic has created a variety of clinical and research opportunities. Clinically, the incorporation of the R-SPACE™ system will allow clinicians to objectively assess improvements in patients with various hearing device options, which will be helpful when generating recommendations and developing post-fitting expectations. Patients will benefit from having the opportunity to experience the potential for hearing improvement in a real-world listening environment — in some cases before committing to a device selection.
The exciting addition of this new technology will enhance the experience of the patients we serve by better addressing their day-to-day hearing needs and by providing them with the peace of mind of knowing that their selected device will offer positive benefits in their real life.
- Revit LJ, Schulein RB, Julstrom SD. Toward accurate assessment of real-world hearing aid benefit. The Hearing Review. Aug 2002.
- Revit LJ, Killion MC, Compton-Conley CL. Developing and testing a laboratory sound system that yields accurate real-world results. The Hearing Review. Oct 2007.
- Compton-Conley CL, Neuman AC, Killion MC, Levitt H. Performance of directional microphones for hearing aids: real-world versus simulation. J Am Acad Audiol. 2004;15(6):440-455.
- Gifford RH, Revit LJ. Speech perception for adult cochlear implant recipients in a realistic background noise: effectiveness of preprocessing strategies and external options for improving speech recognition in noise. J Am Acad Audiol. 2010;21(7):441-451.
Dr. Sydlowski is Audiology Director of the Hearing Implant Program. She can be reached at 216.444-0354 or firstname.lastname@example.org.