Tinnitus complaint behaviour in long-standing Menière's disorder: its association with the other cardinal symptoms.

OBJECTIVE: To explore factors that determines tinnitus complaint behaviour in patients with chronic long-standing Menière's disorder. DESIGN AND SETTING: A questionnaire-based cross-sectional investigation. This included the Oto-neurological questionnaire, the Hearing Disability and Handicap Scale (HDHS), Hearing Measurement Scale (HMS) on sound localisation and the Dizziness Handicap Questionnaire (DHQ). PARTICIPANTS: Randomly selected 183 members of the Finnish Menière's Federation. INTERVENTION: Postal questionnaire. MAIN OUTCOME MEASURE: International Tinnitus Inventory and impact of tinnitus. RESULTS: The 183 patients,[36 men and 147 women; mean age, 63 years] had their Meniere's disorder-like symptoms, with a mean of 18 years [range, 1-43], 19% of patients ranked tinnitus as their most severe symptom, and 10% experienced tinnitus as causing a severe or very severe impact. Regression analysis indicated that 41% of International Tinnitus Inventory variance and 28% of tinnitus impact variance were explained by the cardinal symptoms of Menière's disorder. Furthermore, 40% of International Tinnitus Inventory and 25% of tinnitus impact variance were explained by symptom-related disabilities (HDHS, HMS and DHQ). Aural pressure, hearing loss and gait problems were the most important predictors of tinnitus complaint. Understanding what people say and limitation of activities because of vertigo were the most important related disabilities. CONCLUSION: Tinnitus shares a significant variance with the other cardinal symptoms in patients with long-standing Menière's disorder. As the impact is significantly related to activity limitations based on hearing disability and vertigo, the results suggest that therapeutic efforts to reduce tinnitus in Menière's disorder should include the alleviation of balance and hearing problems.

Diagnostic and surgical challenge: middle ear dermoid cyst in 12 month old with branchio-oto-renal syndrome and multiple middle-ear congenital anomalies.

Described is the first case report, to our knowledge, of a middle-ear dermoid in a child with branchio-oto-renal (BOR) syndrome. Radiographic, pathologic, and intraoperative figures are shown. This was a diagnostic and surgical challenge as the presentation was similar to a congenital cholesteatoma and the child had numerous significant temporal bone abnormalities. After the intraoperative findings suggested a non-destructive process, the treatment strategy was altered. This case reiterates the need for a cautious, flexible operative approach in a syndromic child. Included is a relevant review of the literature and a detailed clinical analysis.

The presentation and management of multiple paraganglioma in head and neck.

OBJECTIVE: We report the presentation and management of an 18-year-old male, who presented with bilateral carotid body paragangliomas and a unilateral jugular body paraganglioma. METHOD: A comprehensive review of the medical literature concerning paragangliomas in the pediatric and adult population is discussed. RESULTS: Presentations of multiple paragangliomas in an 18-year-old have never been described. CONCLUSION: This is the first case of multiple paragangliomas in a 18-year-old male, who was treated with embolisation and surgical resection and remains disease free 2 years from surgery.

Analysis of the dysfunctional eustachian tube by video endoscopy.

OBJECTIVE: Human eustachian tubes with known pathologic conditions of the ear were inspected endoscopically, and video recordings were made for slow-motion analysis of the pathophysiologic changes. SETTING: Ambulatory office in a tertiary referral center. SUBJECTS: Forty-four adults with 64 ears having pathologic conditions. INTERVENTIONS: Transnasal endoscopic examination of the nasopharyngeal opening of the eustachian tube during rest, swallowing, and yawning to study the dilatory movements of the eustachian tube. MAIN OUTCOME MEASURES: Slow-motion video analysis of the dilatory movements of the eustachian tube. RESULTS: Sixty-four ears and eustachian tubes with pathologic changes were studied. Tubal function was graded on (1) the extent of lateral excursion and progression of dilatory wave as estimates of tensor veli palatini and dilator tube muscle function, reduced function being observed in 43 tubes; (2) the degree of mucosal disease, which was significant in 48 tubes; (3) obstructive mucosal changes, which were present in 15 tubes; (4) ease and frequency of tubal dilation with maneuvers-26 tubes opened moderately, 21 opened minimally, and 11 were unable to open; and (5) patulous tubes-all 6 clinically patulous tubes showed concavities in the superior third of the tube, which is convex in normal subjects. All tubes with active pathologic conditions of the ear (otitis media with effusion, tympanic membrane retraction, draining ear, cholesteatoma) had significant abnormalities. A correlation could not be made between the severity of middle ear disease and the severity of observed eustachian tube dysfunction. CONCLUSIONS: Slow-motion endoscopic video analysis is a potentially useful technique in classifying types of pathologic changes in the eustachian tube. Additional studies of dysfunctional tubes are needed to predict outcomes in operative ear cases and to design intratubal therapy for chronically dysfunctional tubes.

Etanercept therapy for immune-mediated cochleovestibular disorders: preliminary results in a pilot study.

OBJECTIVE: Immune-mediated cochleovestibular disorders (IMCVDs) continue to present a management challenge to the otolaryngologist. Antirheumatic agents, commonly used for IMCVDs, are associated with variable efficacy and sometimes with serious side effects. The authors describe the preliminary result of their experience in patients with IMCVDs who have been treated with etanercept, a tumor necrosis factor alpha receptor blocker, recently approved by the United States Food and Drug Administration for the treatment of rheumatoid arthritis. STUDY DESIGN: Retrospective case series. SETTING: Tertiary care hospital. PATIENTS: Twelve patients suspected of having IMCVD who did not respond to conventional therapies or experienced side effects of the conventional therapies. INTERVENTION: Etanercept 25 mg by subcutaneous injection twice per week. MAIN OUTCOME MEASURES: The main outcome measurement was assessment of hearing change by air conduction pure tone audiograms and/or word discrimination. When present, vertigo, tinnitus, and aural fullness were assessed as well. RESULTS: Follow-up of more than 5 months was available for all patients (range, 5-12 months). Eleven (92%) of 12 patients had improvement or stabilization of hearing and tinnitus, seven (88%) of eight patients who had vertigo and eight (89%) of nine patients who had aural fullness had resolution or significant improvement of their symptoms. The benefit persisted until the last visit (5-12 months after etanercept was begun). The condition of one patient improved dramatically at first but deteriorated after 5 months. The patient's hearing was rescued and stabilized with the addition of leflunomide to etanercept. Similarly, three other patients required a second antirheumatic agent to stabilize their hearing. There were no significant side effects from the etanercept therapy. CONCLUSIONS: Our limited data suggest that etanercept therapy is safe and may be efficacious in carefully selected patients with IMCVDs, at least on a short-term basis. These preliminary efficacy and safety results appear encouraging enough to warrant further follow-up and studies for better determination of the potential clinical utility of etanercept for IMCVDs.

Experimental CO2 laser myringotomy.

OBJECTIVE: The purpose of this study was to compare closure rates of CO2 laser myringotomies with those produced conventionally with a knife. STUDY DESIGN AND SETTING: We investigated closure rates in 3 experimental groups of chinchillas: (1) semicircular myringotomy in both ears either produced with a knife or CO2 laser; (2) CO2 laser myringotomy, round in one ear and kidney shaped in the other; (3) CO2 laser myringotomy, incisional in one ear and kidney shaped in the other. RESULTS: Patency of knife myringotomies was shorter (P < 0.01) than of similar laser myringotomies, means 10.0 and 21.8 days, respectively. The patency of kidney-shaped laser myringotomies was longer (P < 0.05) than of round ones. Incisional laser myringotomies remained open longer (P < 0.01) than round ones. CONCLUSION: Use of CO2 laser as well as certain geometries delay the closure of myringotomies. By allowing perforations of different shapes and sizes, CO2 laser myringotomy may become an alternative in the treatment of otitis media.

Autoimmune vestibulo-cochlear disorders.

Autoimmune vestibulo-cochlear disorders (AVCD) represent a group of syndromes with overlapping clinical features, manifesting as sensorineural hearing loss, often associated with vertigo, tinnitus, and aural fullness, and believed to be caused by an autoimmune mechanism. Although definitive evidence of a classic "autoimmune process" is still lacking, substantial indirect evidence has accumulated to strongly indicate such a pathogenesis. Rapidly progressing AVCD is analogous to rapidly progressive glomerulonephritis in that inner ear inflammation progresses to severe, irreversible damage within 3 months of onset (and often much more quickly). Thus patients with rapidly progressive AVCD are treated with a sense of urgency. Prompt treatment with corticosteroids and other antirheumatic/immunosuppressive agents can preserve hearing and vestibular functions. We are not aware of any randomized controlled clinical trials evaluating the efficacy of antirheumatic/immunosuppressive agents in AVCD. In this article we review reports of various therapies that have been tried in this condition and our experience of etanercept therapy in AVCD.

Tegmental defects and cerebrospinal fluid otorrhea.

Congenital tegmental defects that present as unsuspected cerebrospinal fluid (CSF) otorrhea are diagnostic and therapeutic challenges. We reviewed 5 such patients to determine an optimal strategy for evaluation. Five patients presented with watery otorrhea, 4 of them after ventilation tube placement, and only 1 with rhinorrhea. The preoperative analysis of middle ear effusion for beta(2)-transferrin was positive in 2/4, equivocal in 1/4 and false negative in 1/4. Computerized tomography (CT) revealed nonspecific tegmental defects in all 5 patients. Magnetic resonance imaging (MRI) demonstrated meningoencephalocele in 3/5 and dural irregularity in 1/5. Tegmental defects were confirmed at surgery in all cases, demonstrating meningocele or arachnoid granulations in 2/5 and encephalocele in 2/5 patients. We recommend a combination of beta(2)-transferrin analysis to verify CSF, high resolution CT (axial and coronal planes) to diagnose tegmental defects, and MRI (multiplanar) to evaluate the type of herniation. A combination mastoid and middle fossa approach for definitive repair is suggested.

Analysis of eustachian tube function by video endoscopy.

OBJECTIVE: Human eustachian tubes (ET) were inspected in vivo endoscopically, and video recordings were made for careful slow-motion analysis of normal physiologic function. SETTING: Ambulatory office in a tertiary referral center. SUBJECTS: Thirty-four adults, 17 with no history of ET dysfunction (2 of whom had tympanic membrane perforations), 17 with known ET dysfunction. INTERVENTIONS: Transnasal endoscopic examination of the nasopharyngeal opening of the eustachian tube during rest, swallowing, and yawning. MAIN OUTCOME MEASURES: Video analysis of ET opening movements. RESULTS: Normal ETs had four consistent sequential movements: (1) palatal elevation causing passive, then active, rotation of the medial cartilaginous lamina; (2) lateral excursion of the lateral pharyngeal wall; (3) dilation of the lumen, caused primarily by tensor veli palatini muscle movement beginning distally and inferiorly, then opening proximally and superiorly; and (4) opening of the tubal valve at the isthmus caused by dilator tubae muscle contraction. Dysfunctional ETs had intraluminal edema, polyps, or minimal muscle movement. CONCLUSIONS: Slow-motion endoscopic video analysis may be a useful new technique for the study of eustachian tube physiology. Consistent muscle movement patterns were demonstrated in normal subjects but were absent in abnormal subjects. More studies of normal and abnormal patterns are needed to establish useful clinical correlates.

Persistent stapedial artery: CT findings.

CT of a patient with conductive hearing loss showed a small vascular structure leaving the carotid canal and passing through the stapes to join the facial nerve canal. The small bony channel paralleled the anterior tympanic segment of the facial nerve, continuing beyond the geniculate ganglion into the middle cranial fossa. Otoendoscopic photography documented the presence of a persistent stapedial artery.

Laser-assisted myringoplasty--technical aspects.

At lower power, lasers fuse collagen fibres and weld tissues. Welded collagen fibres make a solid bond and allow tissue growth along the bonded edges. Our aim was to study applicability of lasers in myringoplasty. We used a KTP-532 laser in outpatient myringoplasty. The laser beam was delivered through a micromanipulator connected to a microscope or through a 200-400-micron silica fibre. The perichondrium was used for transplantant and harvested from the tragus. The margins of the perforation in the eardrum were evaporated, with the laser operating in a continuous mode at 2-4 W. The middle ear was filled with gelfilm to provide support for the transplant. The perichondrium was placed under the margins of the tympanic membrane and lazed at low power (0.2-1.5 W) in continuous mode. In pale tissues, venous blood, methylene blue or fluorescein was used to enhance the tissue admittance of laser energy. Surgical failures were linked to thermal tissue damage due to excessive energy during lazing. In two cases, visibility via microscope into the anterior edge was not complete and the transplantant did not adhere in the relatively limited area. One patient had epidermal growth under the tympanic membrane and developed local cholesteatoma. Laser-assisted myringoplasty provides several advantages over traditional myringoplasty: it is minimally invasive, no manipulation of the ossicles is needed and it is convenient in anterior perforations, where it can be done endoscopically. We prefer a fibre delivery system to a micromanipulator, as lazing with endoscopes is possible and thermal damage is easier to prevent.

Laser-assisted endoscopic stapedectomy: a prospective study.

OBJECTIVE: To improve the techniques required to perform a stapedotomy without prosthesis (stapedioplasty). STUDY DESIGN: New infrared lasers were evaluated for potential use in otological surgery in guinea pigs. A prospective human trial of 34 primary stapes operations using the Argon ion laser was performed, with 11 stapedioplasties and 23 conventional stapedotomies as controls. METHODS: Laser-tissue interactions were evaluated for temporal bone and live guinea pig tissues, measuring crater histology and labyrinthine temperature elevations. Patients undergoing stapedioplasty had Argon ion laser cuts with endoscopic assistance made in the anterior crus and footplate to mobilize the posterior segment of the stapes while the anterior portion remained fixed. RESULTS: Diode laser (808-nm) vaporization craters and temperature elevations in the vestibule were suitable for clinical use. Overall, stapedioplasty patients' hearing was improved with air-bone gap closure to a mean of 8.3 dB (SD +/- 9.8 dB). CONCLUSIONS: Patients with anterior footplate otosclerosis are candidates for stapedioplasty preserving the annular ring and stapes tendon and eliminating prosthesis complications. High-resolution small endoscopes, coupled with Argon ion or diode lasers promise to improve stapes visualization, enhancing the ability to perform minimally invasive surgery on the stapes footplate.

Adjunctive use of endoscopy during acoustic neuroma surgery.

OBJECTIVE/HYPOTHESIS: In specific clinical situations, endoscopes offer better visualization than the microscope during acoustic neuroma (vestibular schwannoma) surgery and can therefore decrease the incidence of the postoperative complications of cerebrospinal fluid (CSF) leakage and recurrence of tumor. This study was undertaken to determine if the use of adjunctive endoscopy provides complementary information to the operating surgeon during surgery for acoustic neuromas. METHOD: Seventy-eight patients with acoustic neuromas underwent tumor excision by two neurotologists (PAW., D.S.P.), together with their respective neurosurgical partners, via a retrosigmoid (suboccipital) approach (n = 68), translabyrinthine approach (n = 7), or middle cranial fossa approach (n = 3). Endoscopy with a rigid glass lens endoscope was used during tumor removal to examine posterior fossa neurovascular structures, and after tumor excision to inspect the internal auditory canal (IAC), inner ear, and middle ear, depending on the approach used. One of the authors (D.S.P.) has not used adjunctive endoscopy during resections via the translabyrinthine and middle cranial fossa approaches, and therefore, these cases were excluded from the data collection and analysis. RESULTS: Complete tumor excision was achieved in 73 patients. Endoscopy allowed improved identification of tumor and adjacent neurovascular relationships in all cases. In addition, residual tumor at the fundus of the IAC (n = 11) and exposed air cells (n = 24) not seen with the microscope during retrosigmoid approaches were identified endoscopically. In one of the translabyrinthine cases, the endoscope allowed identification of open air cells not visualized with the microscope. None of the 78 patients developed CSF rhinorrhea. Incorporating the endoscope did not significantly increase operative time. CONCLUSIONS: Endoscopy can be performed safely during surgery to remove acoustic neuromas. The adjunctive use of endoscopy may offer some advantages including improved visualization, more complete tumor removal, and a lowered risk of CSF leakage.

Endoscope-assisted vestibular neurectomy.

OBJECTIVE/HYPOTHESIS: In some instances endoscopes offer better visualization than the microscope and frequently allow less invasive surgery. This study was undertaken to determine whether endoscopy is safe and effective during neurectomy of the vestibular nerve. METHOD: Ten patients with intractable unilateral Meniere's disease underwent a retrosigmoid craniotomy for neurectomy of the vestibular nerve. Endoscopy with a Hopkins telescope was used during each procedure to study posterior fossa anatomic relationships and to assist the neurectomy. Preoperative and postoperative audiometric evaluation was performed in all patients undergoing vestibular neurectomy. Nine of these patients had preoperative electronystagmography, and four patients completed postoperative electronystagmography. The 1995 American Academy of Otolaryngology-Head and Neck Surgery's Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere's disease were used. RESULTS: Complete neurectomy was achieved in all 10 patients. Endoscopy allowed improved identification of the nervus intermedius and the facial, cochlear, and vestibular nerves and adjacent neurovascular relationships without the need for significant retraction of the cerebellum or brainstem. In addition, endoscopic identification of the cleavage plane between the cochlear and vestibular nerves medial to or within the internal auditory canal (n = 3) was not made with the 0-degree endoscope; however, identification was made with the 30- or 70-degree endoscope in all cases. In all patients with Meniere's disease, elimination of the recurrent episodes of vertigo (n = 10) or otolithic crisis of Tumarkin (n = 1) was achieved. CONCLUSIONS: Posterior fossa endoscopy can be performed safely. Endoscope-assisted neurectomy of the vestibular nerve may offer some advantages over standard microsurgery including increased visualization, more complete neurectomy, minimal cerebellar retraction, and a lowered risk of cerebrospinal fluid leakage.

Intratympanic gentamicin injection for the treatment of Meniere's disease.

OBJECTIVE: This study aimed to examine the effectiveness of intratympanic injection of gentamicin as a nonsurgical treatment option in the treatment of patients with unilateral Meniere's disease who are refractory to medical treatment. STUDY DESIGN: The study design was a prospective case series. SETTING: The study was conducted at a physician's office setting in a tertiary care hospital. PATIENTS: The results of 37 patients who became eligible for reporting according to the American Academy of Otolaryngology Head and Neck Surgery (AAO-HNS) guidelines for reporting treatment results of Meniere's disease were reviewed. INTERVENTION: Intratympanic injections of a prepared gentamicin concentration of approximately 30 mg/ml were given weekly until the patient reported cessation of vertigo attacks. Patients reclined for 45 minutes after each injection. MAIN OUTCOME MEASURES: The 1995 AAO-HNS guidelines were used in this report, and measures included pure-tone hearing results, word recognition scores, vertigo control scores, and ice-water calories after a minimum of 24 months of follow-up. RESULTS: Vertigo control was achieved in 32 patients (87%). Fifteen patients (41%) had complete recovery from vertigo spells, 17 patients (46%) had substantial recovery, and 5 patients (14%) had treatment failure requiring additional surgery to control vertigo. Hearing results showed that 21 patients (72%) had unchanged or better hearing, 10 patients (28%) had an average threshold shift of 10-25 dB, 4 patients (11%) had a threshold shift between 16 and 25 dB, 1 patient (3%) had a threshold shift between 26 and 40 dB, and 1 patient (3%) had a threshold shift of > 40 dB. CONCLUSIONS: The authors found intratympanic gentamicin to be a useful alternative to surgery. The flexible treatment protocol allowed for better hearing monitoring compared to the more frequent injection schedules of other studies and yielded a lower rate of severe hearing loss. It had a higher failure rate for vertigo control and a greater amount of hearing loss than the author's experience with vestibular nerve section.

Peritoneal metastases in children with cancer.

BACKGROUND: This study attempted to evaluate the childhood malignancies associated with computed tomography (CT) detected peritoneal metastases as well as the diagnostic imaging characteristics of these metastases as shown on CT. METHODS: The authors reviewed all available pathology specimens and abdominopelvic CT scans of patients identified as having peritoneal metastases at three childhood cancer centers. Patient demographics, primary diagnosis, and CT characteristics of such metastases were evaluated. RESULTS: Peritoneal metastases were identified by CT in 32 children with cancer either at diagnosis (n = 20) or up to 6.2 years from diagnosis (n = 12). On CT, peritoneal disease appeared as a mass in 26 cases, as studding in 11 cases, as peritoneal enhancement in 15 cases, and as diffuse caking in 4 cases (15 patients had > 1 category of peritoneal metastasis). Thirteen patients had concurrent metastases in other sites. Fourteen patients died of progressive disease at a median of 10 months from the time peritoneal metastases were identified on CT. At last follow-up, the remaining 18 patients were alive, with follow-up ranging from 1 month to 9.7 years. As expected, peritoneal metastases were identified in patients with germ cell tumors and colon carcinoma. However, they also were observed in patients with epithelioid carcinoma, leiomyosarcoma, pineoblastoma, neuroblastoma, melanoma, and peripheral neuroectodermal tumor. CONCLUSIONS: Peritoneal metastases have variable appearance on CT, but most commonly appear mass-like. They are associated with a wider range of primary diagnoses than reported previously. The outcome varies with the type of the primary tumor and its responsiveness to existing therapies.

Endoscope-assisted second-stage tympanomastoidectomy.

Intact canal wall mastoidectomy techniques for cholesteatoma are often followed by a planned second look for residual disease and possible ossicular reconstruction. Endoscopic techniques may reduce morbidity but introduce new concerns. Twenty-five consecutive second-look procedures were performed from July 1994 to July 1996 utilizing endoscopes in 19 cases and avoiding or terminating their use in the others because of known difficult anatomy, inadequate exposure, or excessive bleeding. Thirteen cases were prospectively explored first through a planned exclusively endoscopic approach and then opened for a conventional second look in comparison. In one of the 13 cases, endoscopy was abandoned. There were no cases in which endoscopy yielded a false-negative result. Endoscopes underestimated the size of recurrence in one case. Our experience, indications, and precautions for endoscope-assisted second-stage tympanomastoidectomy are presented.