Melanoma of the petrous apex of the temporal bone.
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Biomedical subjects
Publications and source records attributed to D B Welling.
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A review of the recent literature concerning hearing loss and Meniere's disease is presented. A compilation of the change in hearing resulting from endolymphatic sac surgery, vestibular nerve section, and intratympanic gentamicin injection and a summary of the results of pharmacologic interventions is tabulated for 1986 to 1996. Although vertigo control is variable with the above treatment strategies, hearing loss does not appear altered by any of them when compared with the natural history of hearing loss associated with Meniere's disease. Further investigation is necessary to understand the underlying cause of hearing loss in Meniere's disease and to eventually control its progression.
OBJECTIVE: The main goal of this study was to examine the vestibular ganglia from 11 patients with intractable classic Menière's disease (MD) for the presence or absence of DNA from three neurotropic viruses (herpes simplex virus, cytomegalovirus, and varicella zoster virus) using exquisitely sensitive molecular biologic techniques. STUDY DESIGN: This was a prospective controlled study with vestibular ganglia from patients with MD and from patients with small vestibular schwannomas undergoing resection. Polymerase chain reaction was used for viral DNA detection from the ganglia along with known positive and negative polymerase chain reaction control subjects. SETTING: The study was performed in an academic tertiary referral center. PATIENTS: Patients for inclusion had medically uncontrolled MD, including documented fluctuating sensorineural hearing loss, episodic vertigo, and tinnitus who elected to undergo vestibular nerve section. Control patients were undergoing vestibular schwannoma removal. INTERVENTIONS: The intervention was vestibular nerve section with removal of vestibular ganglion. MAIN OUTCOME MEASURES: The presence or absence of viral DNA (herpes simplex virus, cytomegalovirus, and varicella zoster virus) in vestibular ganglion tissues detected by polymerase chain reaction. RESULTS: No viral DNA was detected in the vestibular ganglia of patients with MD (p = 0.028) nor in the control group. The likelihood of a type II or beta type error was < 10%. CONCLUSIONS: In patients with MD requiring surgical intervention, infection with herpes simplex virus, cytomegalovirus, or varicella zoster virus of the vestibular ganglia does not appear to play a major role in the pathoetiology of the disease.
Using a heteroduplex approach and direct sequencing, we have completed the screening of approximately 88% of the neurofibromatosis type 2 (NF2)-coding sequence of DNA extracted from 33 schwannomas from NF2 patients and from 29 patients with sporadic schwannomas. The extensive screening has resulted in the identification of 33 unique mutations. Similarly to other human genes, we have shown that the CpG sites are more highly mutable in the NF2 gene. The frequency, distribution, and types of mutations were shown to differ between the sporadic and familial tumors. The majority of the mutations resulted in protein truncation and were consistent with more severe phenotype, however three missense mutations were identified during this study and were all associated with milder manifestations of the disease.
The pathoetiology of Meniere's disease remains elusive. Histopathologic and imaging studies have implicated congenital or developmental abnormality of the endolymphatic duct as a likely predisposing factor to the development of endolymphatic hydrops and clinical Meniere's disease. Recently, improved high-resolution magnetic resonance imaging (MRI) protocols have allowed better demonstration of the soft tissues of the endolymphatic duct and sac. This study is a prospective evaluation of the ability of submillimeter MRI to detect the endolymphatic duct in Meniere's disease patients and control subjects. In addition, the development of the temporal bone in the region of the endolymphatic sac and duct is evaluated by measurements of the distance from the posterior semicircular canal to the subarachnoid space and the distance from the vestibule to the subarachnoid space. Visualization of the endolymphatic duct was found to be significantly less frequent in the Meniere's disease patients (29%) than in the control subjects (91%). Temporal bone measurements in the region of the endolymphatic duct showed patients with Meniere's disease to have smaller dimensions than control subjects.
The role of the endolymphatic sac in the pathoetiology of Menière's disease has been extensively studied but remains controversial. Likewise, the mechanism behind the efficacy of the endolymphatic-mastoid shunt procedure in Menière's disease remains obscure. The central hypothesis of this study is that the endolymphatic-mastoid shunt procedure is a destructive rather than a drainage procedure. Therefore removal of the extraosseous endolymphatic sac with scarring of the intraosseous portion may be more destructive and thereby more effective in the treatment of Menière's disease than conventional shunt placement alone in the extraosseous endolymphatic sac. This study reports a 2-year follow-up on the effect of complete removal of the extraosseous endolymphatic sac for 10 patients with intractable Menière's disease and 10 matched control Menière's disease patients undergoing conventional endolymphatic-mastoid shunt surgery. This study showed no statistical difference in the change in hearing, vertigo, or disability in the two groups.
Lipoma of the internal auditory canal is a rare tumor that may be confused clinically with the much more common vestibular schwannoma. We present two cases of lipoma of the internal auditory canal. The clinical presentation is indistinguishable from that of vestibular schwannomas. The high signal intensity on T1-weighted magnetic resonance imaging, both with and without contrast, is consistent with other reports of lipoma. Review of the literature shows that lipomas of the internal auditory canal are histopathologically similar to lipomas of the cerebellopontine angle. The symptoms, erosive effect on the auditory canal, and gross appearance of this uncommon tumor are sometimes difficult to differentiate from those of a vestibular schwannoma. The diagnosis can be established by intraoperative examination of frozen sections.
PURPOSE: To evaluate the utility of submillimeter resolution MR imaging for direct depiction of functional soft-tissue components of the intraosseous endolymphatic duct and sac in healthy subjects and in patients with Menière disease. METHODS: Axial MR images were acquired of 14 patients with Menière disease and 14 healthy volunteers at 1.5 T with a short-echo-time steady-state 3-D gradient-echo sequence. Seven volunteers and eight patients were also studied with a T1-weighted 3-D spoiled gradient-echo sequence. T1/T2 relaxation times were estimated from studies with multiple flip angles. RESULTS: Independent of the acquisition method, intraosseous endolymphatic ducts and sacs were seen unambiguously in the ears of 20 of 21 healthy subjects but in only four of 12 asymptomatic and two of 10 symptomatic ears of patients with Menière disease. Other labyrinthine structures were well depicted in all subjects. Furthermore, shorter relaxation times were measured for the contents of the vestibular aqueduct than for other labyrinthine structures. CONCLUSION: In our high-resolution study, the intraosseous portions of the endolymphatic ducts and sacs were depicted in most of the healthy subjects. They were frequently not seen in either ear of patients with unilateral Menière disease, presumably because of their small size.
Immune-mediated inner ear disease, first described by McCabe in 1979, typically presents with an idiopathic, rapidly progressive bilateral sensorineural hearing loss. The course of the hearing loss occurs over weeks to months. It may occur in both sexes and at a variety of ages, but is most common in middle-aged females. It may be accompanied by tinnitus, Meniere's-like vertigo, or more commonly, ataxia or unsteadiness. Approximately 30% of patients will have associated systemic immune-mediated disease. Although refinements in laboratory tests for specific inner ear antigens are being made, and non-specific laboratory indicators of inflammatory or systemic immune disease may be useful in confirming the diagnosis, the most important diagnostic finding is the improvement in hearing seen with a trial of immunosuppressants. This report includes a typical evaluation of the patient with suspected immune-mediated inner ear disease and an illustrative case. Sensorineural hearing loss due to immune-mediated inner ear disease, although unusual as a cause of hearing loss, is important to recognize because early diagnosis and treatment can have a marked effect on the clinical outcome.
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A prospective randomized study analyzing the safety and efficacy of a single dose of ototopical antibiotics following human middle ear tympanostomy tube insertion was performed. Fifty children undergoing bilateral tympanostomy tube insertion were studied by the placement of 0.5 mL of Cortisporin Otic Suspension (COS; Burroughs Wellcome Co., Research Triangle Park, N.C.) to one middle ear space by random assignment. Preoperative and postoperative audiograms were obtained, and the presence of otorrhea was noted. In one patient sensorineural hearing loss of 6 dB developed bilaterally, which was symmetric in both the treated and the untreated ear. This preliminary study showed no statistical difference in hearing loss or postoperative otorrhea associated with a single application of Cortisporin to the middle ear space.
This study investigated the relative effects of vestibular rehabilitation (VR) and social reinforcement (SR) on recovery following ablative vestibular surgery. Twenty-four subjects were randomly assigned to three treatment groups of either VR with SR, VR without SR, or general range of motion (ROM) exercises with SR. Outcome measures included equilibrium scores in dynamic posturography, asymmetry index in rotation testing, motion sensitivity quotient (MSQ), and dizziness handicap inventory (DHI). A multiple comparison of the overall outcome measures showed no significant differences in group performance over an 8-week period. When individual outcome measures were compared, MSQ and DHI results at the end of the 8-week treatment period revealed less motion sensitivity and dizziness handicap in groups who received VR, with or without SR, as compared with the group who received ROM exercises. These results suggest that after a vestibular injury most patients can effectively utilize central compensation mechanisms to recover from such an injury, regardless of the type of therapeutic intervention used. On the other hand, the reduction in motion sensitivity and dizziness handicap for patients who received VR could indicate a more rapid and complete recovery for these patients. This investigation is continuing as a long-term follow-up study to determine whether there are any long-term benefits in participating in a VR program.
Chronic tympanic membrane (TM) perforation is a common problem worldwide. Recent reports have shown epidermal growth factor (EGF) to stimulate healing in approximately 80% of chronic TM perforations in chinchillas when applied in three doses over 1 week. The objective of this controlled study is to evaluate the efficacy of long-term EGF in the closure of TM perforations. Chronic chinchilla TM perforations were treated with EGF for up to 6 weeks. One hundred percent (17 of 17) of treatment group perforations completely healed. However, two new findings with this long dosing scheme were reperforation on long-term follow-up and three TMs with cholesteatomas. It is likely that reperforation was due to a progressive thinning seen with prolonged EGF application. Long-term EGF use is not recommended for the treatment of TM perforations because of possible wound healing impairment and possible cholesteatoma induction.
Stimulation of the semicircular canals provides an alternative method of vibratory stimulation of the auditory system. The frequency response characteristics of stimulation of the tympanic membrane, malleus, stapes, round window, and fenestrated semicircular canal are presented in this article.
The recent demonstration of free-floating particles in the endolymph of the posterior semicircular canal in patients with benign paroxysmal positional vertigo (BPPV) has renewed interest in the physiology and treatment of this entity. The particle repositioning maneuver (PRM) relocates the free-floating particles from the posterior semicircular canal back into the utricle, relieving the patient of bothersome, often long-standing vertigo. This report represents a prospective study of 27 consecutive patients seen with a diagnosis of BPPV. Eighty-four percent of the patients treated with the particle repositioning maneuver who had no other associated pathology were cured or significantly improved with this new technique. Two patients who failed conservative management went on to surgical intervention with the posterior semicircular canal occlusion. The authors find the particle repositioning maneuver effective for many patients with benign positional vertigo and recommend it as the first-line treatment modality for BPPV.
Neurotropic viruses have been postulated to play a role in the development of Menière's disease (MD). The purpose of this study was to evaluate the endolymphatic sacs of patients undergoing surgery for MD in a single-blind study for evidence of herpes simplex virus (HSV), varicella zoster (VZ), or cytomegalovirus (CMV) DNA. Polymerase chain reaction (PCR) was used as the method of detection because of its sensitivity, specificity, and applicability to fresh, as well as fixed tissues. Twenty-two patients with MD and 11 control patients with vestibular schwannomas had a portion of the endolymphatic sac removed at the time of surgery. The specimens were then evaluated for herpes simplex type and 2, varicella zoster, and cytomegalovirus DNA. Herpes simplex virus DNA was detected in 2 of the 22 extracts from the endolymphatic sacs obtained from patients with MD. There was no evidence of a positive signal obtained with any of the other viral DNA probes when PCR was performed on the control tissue extracts or the other MD tissue extracts. These results do not demonstrate a significant difference and do not statistically support the postulate that ongoing viral infection in the endolymphatic sac is a frequent factor in the development of Menière's disease.
Iatrogenic injury to the carotid artery as it traverses the middle ear in its normal anatomic position is a rare but potentially life-threatening event. Predisposing factors include a thin or dehiscent plate of bone, which normally protects the artery at the level of the eustachian tube, erosive middle ear disease, or previous trauma. Prevention and management of carotid artery injuries in the middle ear are discussed with an applicable case report.
Insertional trauma to the cochlea from three different multichannel cochlear implant electrodes was evaluated in a single-blind controlled study in fresh human temporal bones. Sixteen fresh human temporal bones were implanted with one of three types of multichannel electrodes (Symbion/InnerAid, Cochlear/Nucleus, or Storz/UCSF). Seven temporal bones were used as controls where a cochleostomy only was created. The temporal bones were evaluated histologically and cochlear histograms of the trauma were created. Although the three electrode designs caused damage which was unlikely to hinder implant performance, a distinct pattern of trauma was seen with each of the three electrode types. The least traumatic of the three electrode designs in this study was the Nucleus type. The degree of insertional trauma may be relevant to changing indications for insertion of cochlear implants as well as for patients with device failure who require reimplantation.