Waldenstrom's macroglobulinemia and sensorineural hearing loss.

Waldenstrom's macroglobulinemia, (WM) first described in 1944, is an uncommon disease caused by the abnormal production of immunoglobulin M monoclonal macroglobulin. Presenting signs and symptoms most frequently include fatigue or generalized weakness; tendency to bleed from mucosal surfaces; characteristic retinal lesions, including dilated and tortuous retinal veins, retinal hemorrhages, and exudates; lymphadenopathy; hepatosplenomegaly; sensory motor peripheral neuropathy; worsening normochromic anemia; increased sedimentation rate; and extremely high serum viscosity. Hearing loss is an unusual presenting symptom of WM; only 6 cases are reported in literature. The etiology of hearing loss is unclear; however, hyperviscosity and thrombus formations are the most likely causes. We present a patient diagnosed with WM whose initial presenting symptoms were hearing loss and progressive sensory peripheral neuropathy with subsequent development of lymphadenopathy and hyperviscosity syndrome. Treatment with Fludarabine lead to improvement of her hearing and neurological deficits, as well as resolution of her other hyperviscosity-related symptoms.

Vibratory sample magnetometry of middle ear prostheses and manufacturing materials.

OBJECTIVE: To assess the magnetic properties of stapes prostheses and manufacturing materials using a vibratory sample magnetometer (VSM). DATA SOURCES: VSM was performed on 16 samples, including ferromagnetic 420F stainless steel, with an LDJ Model 9600 VSM in accordance with American Society for Testing and Materials standard A894. RESULTS: A VSM measures the magnetic dipole moment of a sample in a magnetic field. The magnetic field is swept over a range of magnetic fields, and the magnetic dipole moment is plotted as a function of field. The prostheses made of 316L stainless steel previously found to be ferromagnetic had the highest specific magnetic moments. The specific magnetic moments ranged from 0.023 electromagnetic units (emu)/g to 156 emu/g. The samples made with 316L stainless steel, which is used in otologic implants, were significantly less magnetic than was the 420F stainless steel. CONCLUSION: VSM demonstrates that prostheses made with 316L stainless steel are relatively nonferromagnetic compared with 420F stainless steel. However, none of the implants are nonmagnetic. The torque and linear force on the prosthesis is a concern. The safety of performing MRI on patients with these implants needs to be further assessed.

Incidence of carcinoma in incidental tonsil asymmetry.

OBJECTIVE: To determine the incidence of malignancy in patients with incidentally discovered unilateral tonsillar enlargement with an otherwise normal physical examination. STUDY DESIGN: A retrospective review of our institution's experience with unilateral tonsillar enlargement. RESULTS: During a five-year period, 570 patients underwent tonsillectomy at our institution. Of patients undergoing tonsillectomy, 49 patients (8.6%) underwent tonsillectomy with normal mucosa, no lymphadenopathy, and asymmetry of the tonsils. Two (4.8%) of the 49 patients with unilateral enlargement had the presence of malignancy. The two adult patients had B-cell lymphoma. Of all of the patients undergoing tonsillectomy, 0.35% had occult malignancy. In additional, the ability of the clinically assessed size (1+, 2+, 3+, and 4+) to predict volume was evaluated. The clinically assessed size and pathologically measured volume were significantly correlated (R = 0.333, P < .013). In 60.5% of the patients, the clinically assessed size and the pathologically measured volume agreed with the asymmetry reported on physical examination. CONCLUSION: A relatively high number of tonsillectomies will be performed to yield an approximately 5% positive rate of malignancy within removed tonsils that are asymmetrical with normal mucosa and no cervical adenopathy.

MR imaging of patients with carotidynia.

BACKGROUND AND PURPOSE: Carotidynia is an idiopathic neck pain syndrome associated with tenderness to palpation over the carotid bifurcation. Although well known in the otolaryngology and neurology literature, the validity of the entity has recently been questioned, in part because of the almost uniform absence of radiologic or pathologic findings. We report the MR findings in five patients with carotidynia. METHODS: During a period of 44 months, five patients with clinical signs and symptoms consistent with carotidynia were referred for imaging from the otolaryngology service. Each patient underwent MR imaging of the neck on a 1.5-T system. The studies included, as a minimum, pre- and postcontrast axial and postcontrast coronal T1-weighted images. Two patients also had axial T2-weighted imaging and another two patients underwent duplex sonography of the carotids. RESULTS: All five patients had abnormal enhancing tissue surrounding the symptomatic carotid artery centered at the level of the distal common carotid and carotid bifurcation. This tissue had intermediate signal intensity on T1-weighted images and showed marked enhancement. In all patients, the remaining visualized portions of the carotid artery were normal. Normal flow voids were present throughout the vessel, and the caliber of the vessels was always within normal limits. There was no evidence of intramural hematoma, cervical lymphadenopathy, or atherosclerotic disease of the vessel. In one patient, repeat imaging after resolution of symptoms showed an absence of the previous abnormality. CONCLUSION: The MR findings in these patients, along with the lack of any findings to suggest alternative diagnoses, support the existence of carotidynia as a distinct clinical entity.

Acanthamoeba sinusitis with subsequent dissemination in an AIDS patient.

Otolaryngologists can play an important role in the care of patients with acquired immunodeficiency syndrome (AIDS) and/or human immunodeficiency virus infection. We present the case of an AIDS patient who was hospitalized for dehydration and who was soon found to have sinusitis and subsequent disseminated infection caused by Acanthamoeba. To treat the Acanthamoeba infection, the patient was started on oral itraconazole and intravenous metronidazole; i.v. pentamidine was added 2 days later. Despite aggressive therapy, on the eleventh day of hospitalization, the patient was obtundent and provided minimal response to noxious stimuli. He died on the sixteenth day of hospitalization. This case is one of only six reported cases of Acanthamoeba associated with sinusitis. Current therapeutic regimens have not been successful for most of these patients, and the prognosis is poor.

Management of lateral sinus thrombosis.

OBJECTIVES: Review the clinical signs and symptoms, management, bacteriology and outcomes of patients treated for lateral sinus thrombosis. STUDY DESIGN: A retrospective review of six patients, treated from 1993 through 1998, with an intraoperatively confirmed diagnosis of lateral sinus thrombosis. METHODS: All charts from 1993 through 1998 coded for sinus thrombosis, meningitis, brain abscess, otitic hydrocephalus, subdural abscess, and mastoidectomy were reviewed. Operative reports, radiological examinations, laboratory data, culture data and other pertinent data were reviewed. RESULTS: The presenting symptoms ranged from headache to mental status changes. All patients had a history of chronic ear disease and all had at least one additional intracranial complication. The range of additional intracranial complications included otitic hydrocephalus, epidural abscess, and brain abscess. All of the infections were polymicrobial, with a predominance of anaerobes. There were no mortalities; morbidities included anacusis, acute respiratory distress syndrome, reoperation, seizures, septic cardiomyopathy, transfusion, ventriculoperitoneal shunt and nutritional supplementation. CONCLUSION: In patients with otologic disease, complaints of headache, earache or photophobia should warrant an evaluation. The presence of lateral sinus thrombosis mandates further investigation for additional intracranial complications. Conservative surgical intervention, consisting of removal of all perisinus infection and needle aspiration of the sinus, has been found to be effective. Lateral sinus thrombosis is an uncommon complication of otitis media, with potentially significant morbidities, necessitating a high index of suspicion.

Overview of telemedicine applications for otolaryngology.

OBJECTIVE/HYPOTHESIS: As we evolve into the managed care era, telemedicine technologies can help to provide efficiencies for the entire health care system. Our practice is centered around a residency training program in a federal facility. Our goal was to lay the groundwork for an entire service being on-line. STUDY DESIGN: Prospective. METHODS: The stepwise process is as follows: needs assessment to examine what part of the practice would lend itself to telemedicine deployment; usability studies to select the best equipment; study in-house to normalize the technology to the current standard of care comparing control subjects with experimental subjects; and deploying remote units to study remote referral. RESULTS: Video-otoscopy captures the image of an eardrum that can be sent from a remote site in a store-forward mode. After normalizing the standard of care, the potential for diagnosing and treating certain ear conditions such as hearing loss is readily apparent. An audiogram, pertinent laboratory data, and history and physical performed by the referring primary care provider would allow the patient to be seen and triaged virtually. Time savings for the patient and the otolaryngologist would be gained. Initial data indicate a greater than 90% correlation with handheld otoscopy and binocular microscopy. In speech sciences, a need existed to evaluate and treat patients from remote sites where speech therapists are unavailable. The use of live video teleconferencing to evaluate and treat patients in-house has preliminary results that show no difference in outcomes. Studying the proctoring of remote surgeons is also a need, as one of our military missions. Initial data show no difference in outcomes when residents are proctored by attending physicians from a physically separated control room in the hospital. We also participate with the Internet Tumor Board, which links four remote sites with our medical center. Different sites scroll through the slides that are listed on a Web site while linking each site with audio teleconferencing. We are initiating Internet Grand Rounds using the same technology. CONCLUSION: These efforts performed in a stepwise approach will lead to an otolaryngology--head and neck surgery service that is on-line for the next millennium, with telemedicine advances normalized to the standard of care.

Cerebrospinal fluid fistulae in a canine model.

Various diagnostic techniques currently are used to detect the presence of a cerebrospinal fluid fistula. High resolution computerized tomography scanning with the instillation of an intrathecal nonionic contrast medium yields the most accurate diagnostic results. Occasionally, even with optimal conditions, little information is gained other than the confirmation of the presence of a fistula. Intrathecal fluorescein can provide accurate information on the exact location of the fistula. The current study was designed to refine the clinical examination for cerebrospinal fluid fistulae with the use of intrathecal fluorescein. The canine model that we used also served as a vehicle to investigate the histopathologic effects of fluorescein on the central nervous system. We modified a commonly used xenon light source to enable examination with 490 nm light. In the canine model, this allowed accurate visualization of surgically created fistulae using very low doses of intrathecal fluorescein. An examination of the histopathologic features of the central nervous system of the canine model after acute instillation of a higher dose of fluorescein revealed microscopic changes consistent with the introduction of an irritant material. The changes induced by the chemical trauma may explain the serious neurologic sequelae sometimes seen in patients after the instillation of intrathecal fluorescein. Recommendations on the correct dosage of intrathecal fluorescein for diagnosis of cerebrospinal fluid fistulae are proposed.

Evaluation of lower cranial nerve deficits.

Patients with cranial base tumors often present with cranial neuropathies or develop cranial deficits as a consequence of skull base surgery. Deficits involving cranial nerves IX, X, XI, and XII can lead to significant morbidity for patients. A standard evaluation of a patient with a suspected skull base lesion or a postsurgical patient would consist of a complete history and physical examination, including a bedside examination and videofluoroscopy. Other modalities, including fiberoptic endoscopic examination of swallowing safety, ultrasound, and manofluorography, are used less frequently.