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Otological disease in Turner's syndrome.

1988 was the 50th anniversary of Henry Turner's original publication of the constellation of physical features which were subsequently recognized to be associated with a deprivation of X chromosomal material. The abnormalities of phenotype and karyotype are now eponymously associated with his name. Very many phenotype stigmata have now been recognized to be associated with Turner's syndrome. However, a survey of females with the syndrome revealed that they consider that a major disability associated with the abnormal karyotype results from otological disease and that this is poorly recognized as a significant feature of the syndrome. The incidence of otological disease in patients with this syndrome has been difficult to evaluate because of ascertainment bias and the apparent diversity of otological disorders previously reported. This report reviews the literature and details a study of otological disease and hearing impairment in a sample of Turner's syndrome females enrolled from a defined area.

Adolescent↗

Implications of sound levels generated by otologic devices.

OBJECTIVE: Exposure to loud noise can result in sensorineural hearing loss. In otology, a wide variety of devices are used that have significant noise output, both to the operator and the patient. This study quantifies and compares the sound pressure levels (SPL) of a variety of otologic instruments. METHODS: Using a calibrated sound pressure level meter, the peak and impulse sound pressure levels of several otologic instruments were determined at 1 cm from their application to human temporal bones. Devices measured were an air-powered drill with a cutting burr, an electric micro-drill, and KTP, CO(2), and erbium lasers. RESULTS: Impulse sound pressure levels for the KTP laser, CO(2) laser, microdrill, air-powered drill and erbium laser were 67, 71, 90, 105, and 105 dBA, respectively. Peak sound pressure levels were 81, 89, 102, 118, and 132 dBA, respectively. CONCLUSIONS: Use of the KTP laser, CO(2) laser, and microdrill result in impulse levels of less than 100 dBA. Although the impulse levels of the erbium laser and the air-powered drill were equivalent, the peak sound pressure levels of the erbium laser were significantly higher. These high sound pressure levels may increase the chance for hearing loss. Like any tool, lasers and drills have benefits and risks. The results of this study demonstrate significant differences in SPLs of common otologic lasers and drills. Although noise-induced hearing loss is a function of both SPL and duration of exposure, surgeons should consider these differences when selecting and applying these tools, especially when used on or adjacent to the ossicular chain and stapes footplate.

Otolaryngology↗

Prevalence of vestibulopathy in benign paroxysmal positional vertigo patients with and without prior otologic history.

The purpose of this study was to determine the prevalence of reduced or absent labyrinthine reactivity (vestibulopathy) in two groups of participants with posterior canal BPPV. One group had prior diagnosis of otologic disease (positive history group). No one in the second group had ever been diagnosed with otologic disease (negative history group). Caloric responses were retrospectively analyzed for the two groups. Patients with a positive history exhibited a greater prevalence of vestibulopathy than patients with a negative history. The positive history group, on average, also exhibited a larger unilateral weakness than those patients in the negative history group. We conclude that patients with BPPV and a history of otologic disease are more likely to present with vestibulopathy, than patients with BPPV and no history of otologic disease. This finding supports the benefit of complete vestibular evaluation in patients with BPPV to ensure comprehensive and successful treatment outcome.

Adult↗

Perioperative management of intracranial aneurysms and otologic disease.

OBJECTIVE: Diagnosis and treatment of asymptomatic, unruptured intracranial aneurysms (UIA) are areas of longstanding controversy in the neurologic and neurosurgical literature. Treatment decisions require careful consideration of both the potential for aneurysm rupture as well as the risks associated with repair. Studies examining the natural history of UIA, the morbidity and mortality associated with subarachnoid hemorrhage (SAH), and the various treatment options have led to general guidelines for UIA management. In the literature, the simultaneous presence of otologic disease and UIA has not been introduced or addressed. It is unknown whether surgical treatment of middle ear disease has an effect on the natural history of UIA. STUDY DESIGN: Two illustrative patients with UIA discovered incidentally on radiographic evaluation of middle ear disease are presented. METHODS: Patients were managed according to diagnostic and treatment strategies developed and validated in the neurosurgical literature. RESULTS: Both patients underwent uneventful endovascular embolization of their intracranial aneurysms before middle ear surgery. The intra- and postoperative courses were uncomplicated without symptoms related to either their history of intracranial aneurysms or the prior embolization. CONCLUSIONS: Application of neurosurgical diagnostic and treatment algorithms to otology patients in the perioperative setting is appropriate. Established risk factors such as the size and location of the intracranial aneurysm, prior SAH, and patient age should guide treatment planning in all cases of UIA. Additional research is needed to evaluate the influence of otologic procedures on the natural history of UIA. In addition, studies are needed to evaluate the role of preoperative screening for UIA in otologic surgery candidates.

Adult↗

Neuro-otological and psychiatric abnormalities in a community sample of people with dizziness: a blind, controlled investigation.

OBJECTIVES: To determine neurootological and psychiatric abnormalities associated with complaints of dizziness in an epidemiological community sample of people of working age, and the extent of comorbidity between neuro-otological and psychiatric dysfunction. METHOD: A survey of 3884 people randomly selected from six general practice lists identified 262 people with significant dizziness, from which a subsample of 15 men and 22 women were recruited for testing. Dizzy subjects were evaluated by blind neuro-otological testing, computerised dynamic posturography, a computerised psychiatric assessment, neuro-otological and general medical examination, and diagnosis. An age matched control group of 18 men and 22 women underwent the same evaluation. RESULTS: Tests of auditory, vestibular, and oculo-motor function did not discriminate between dizzy subjects and controls, but dizzy subjects had significantly worse balance on posturographic testing, more diagnoses of medical disorder, and a higher prevalence of psychiatric morbidity. CONCLUSIONS: The findings suggest that dizziness in the community is typically characterised by mild physical disorder accompanied by some psychiatric disturbance. As the combination of minor physical and psychiatric disorder is known to be unusually persistent and handicapping, treatment programmes must be provided for this prevalent syndrome, perhaps by a partnership between primary care and neuro-otological and psychiatric hospital outpatient clinics with experience and expertise in the diagnosis and management of dizziness and psychiatric disturbance.

Adolescent↗

Preschool otologic and audiologic screening.

A study of otologic and audiologic screening of preschool children including tympanometry, acoustic reflexes, and pure-tone screening was carried out at school sites. Subsequent pure-tone threshold tests and otolaryngologic examinations were carried out in a hospital setting. Complete data are available on 1,335 subjects, ages 3 through 5 years. Results are reported in comparison with those of an earlier Pittsburgh screening study. The results of the earlier study showed that loss of hearing sensitivity could not be predicted by the otologic findings, and conversely, the otologic findings could not be predicted by the air conduction thresholds. The results of the present study showed a contribution by tympanometry and acoustic reflexes. The results are evaluated in terms of screening effectiveness in these age groups. One of the recommendations combines an interpretation of certain large negative tympanometric peak pressures (TPP) along with the absence of acoustic reflexes to certain stimuli. The method is more efficient than other aspects of middle ear measurement or of pure-tone screeniing. The efficiency of each procedure is documented. Summary matrices are used to show the effectiveness of each test compared to all other tests in the identification of subjects with hearing or middle ear dysfunction. A protocol for screening evaluation and otologic referral is suggested to effect a balance between over-referral and correct identification.

Acoustic Impedance Tests↗

Otologic history, audiometry and tympanometry as a case finding procedure for school screening.

An algorithm for identification of hearing loss and middle ear effusion based on otologic history, audiometry and tympanometry was designed and tested on 771 first grade school children. Each ear was profiled as being normal, at risk, or in need of otologic referral based on test-retest screening. In addition, each child was categorized on the basis of the worse ear. Results showed 57 percent of the children as being normal; otologic referral was indicated in 14 percent. The remaining children (29 percent) were at risk or at high risk. The combination of medical history, audiometry and tympanometry constitutes an excellent case finding procedure for screening the otologic status of young school children. Through the utilization of specific criteria, optimal referrals can be made, thereby providing better health care.

Audiometry↗

[Neuro-otologic findings in multiple sclerosis].

Neuro-otologic examination is very important in evaluation of balance system. Multiple sclerosis (MS) is the most common chronic, debilitating disease characterized by focal demyelinization that develop throughout of the central nerves system at varying time. Patients with MS presented various complaints and signs, so the aim of the study was the neuro-otologic presentation of multiple findings in patients with MS and comparison with reviewing literature. The study was carried out in 32 patients with MS (21 with relapsing-remitting, 9 with secondary progressive, 1 with primary progressive and 1 with progressive/relapsing types) the mean age was 38 +/- 10 years. Clinical neuro-otologic examinations were performed in all patients paying special attention to eye movement ability. Vertigo as the first symptom was found in 18.8% of patients but up to 68% of patients would complain this symptom at some point of disease. The hearing impairment and tinnitus complains 6.2% patients, optic neuritis 28% patients and double vision 46.9% of them. A few types of eye movement disorder like abnormalities of fixation, gaze-evoked nystagmus, acquired pendular nystagmus and vertical nystagmus in 46.8% patients were observed. The clinical signs of internuclear ophthalmoplegia were noticed in 2 patients. Disturbance in voluntary gaze either in smooth pursuit test (31.3%) and in saccadic test (43.8%) were seen. The multifocal nature of MS explain why this disease are so commonly subject to defect different part of nerve system. Careful neuro-otologic assessment of every patient with MS is necessary to confirm and monitoring the course of disease.

Adolescent↗

Otologic disease in the acquired immunodeficiency syndrome.

It appears that true otologic manifestations of AIDS are rare and that incidental otologic disease associated with AIDS is more common. A review of the literature revealed that otitis externa, acute otitis media, recurrent acute otitis media, otitis media with effusion, chronic suppurative otitis media with cholesteatoma, and herpes zoster oticus may all represent incidental otologic disease occurring in patients with AIDS. Chronic otitis media without cholesteatoma (P carinii-infected aural polyps), sensorineural hearing loss, acceleration of otosyphilis from the latent stage, and development of Kaposi's sarcoma of the external auricle or nasopharynx may represent true otologic manifestations of AIDS.

Acquired Immunodeficiency Syndrome↗

The changing nature of otological practice in a teaching centre.

The impression that otologic surgical practice is a declining segment of the specialty of otolaryngology is widespread. In an attempt to assess objectively the validity of that assumption, the authors reviewed their own experience over the 12 years 1971-82 inclusive. Five representative years out of the 12 were examined--1971, 1974, 1977, 1979, and 1982. In each of these years, the relative and absolute frequency with which six common otological procedures were performed was determined. The procedures selected for study included myringoplasty, tympanoplasty Type I, II, III and IV, and stapedectomy. In addition the relative frequency of primary vs. revision operations was assessed. The data indicate a change in the pattern of otologic surgical practice over the period, most noticeably in the ratio of primary to revision procedures. These changes have significant implications for the teaching and practice of otology in the future.

Ear Diseases↗

Wegener's granulomatosis with otological and nervous system involvement.

Wegener's granulomatosis usually presents to the otolaryngologist with nasal manifestations. Complications including otologic and central nervous system symptoms occur in 20-40 per cent of cases. Two such cases presented to our clinic with nasal manifestations initially, one subsequently developing otologic sequelae and one progressive sensory motor neuropathy. These patients were followed to determine the reversibility of the otologic and sensory motor neuropathy while on therapy. The otologic changes remain and this patient was required to use a hearing aid. The sensory motor neuropathy improved rapidly so that the other patient's ability to walk soon became normal but sensory deprivation continues below the ankle bilaterally. The literature concurs with findings that involvement of the central nervous system and special sense organs such as the cochlea have limited reversibility once they occur, and early detection, therapy, and prevention of such complications are most important.

Adult↗

The history of neuro-otology. A personal perspective.

I realize I practiced otology and neuro-otology during a golden era, but I have to admit that I didn't appreciate how important it was until I sat down to outline this article. How fortunate I was to have lived and practiced during these developing years of neuro-otology. How fortunate to have worked with the likes of Howard and William House, James Sheehy, James Crabtree, David Austin, and John Shea. How fortunate to have had the opportunity to teach residents and fellows in association with my private practice. So I envy the young otologist-neuro-otologist and the future you have. You stand on the brink of great discoveries. And like my generation, you stand on the shoulders of giants. Good luck.

Audiometry↗

Endoscopy in neuro-otologic surgery.

Endoscopy offers several distinct advantages over the operating microscope during neuro-otologic surgery that make it an excellent adjunctive tool to the microscope or independent modality during cranial base surgery. The high magnification gives excellent definition of perforating blood vessels, cranial nerves, and neural structures, which in many cases is superior to that achieved with the microscope. Furthermore, the use of angled or flexible endoscopes allows one to look around corners and behind anatomic structures blocking the view seen via a 0 degree microscope. Endoscopy also has the theoretical advantage that a less invasive operative procedure is required, which should reduce the operative morbidity. Several notable disadvantages of endoscopy include the problems associated with blood soiling the endoscope, making visualization difficult or impossible, the lack of readily available instrumentation designed specifically for endoscopic neuro-otology, and the poor overview of the operative field. This last point is an important one because the endoscope is placed adjacent to the lesion and does not allow one to look backward to prevent [figure: see text] injury to structures next to the shaft of the telescope. Furthermore, the surgeon must be cognizant of potential thermal injury to structures caused by the heat generated by the light source. The present endoscopic technology limits the image that the surgeon sees to two dimensions, which results in certain unique problems when operating in a three-dimensional milieu. Because of this, there is a steep learning curve to acquire endoscopic dexterity and three-dimensional orientation. Finally, bimanual operation requires the use of an articulated endoscope holder or the commitment of the co-surgeon to hold the endoscope. One of the limitations of the operative microscope is that the angle of view is determined by the distance of the lens to the skull, retractor, or obstructing tissue, which is a function of the lens focal length; the longer the focal length, the narrower the viewing angle. During most microsurgical procedures, the focal distance varies between 200 and 400 mm. Using a previous analogy, if one looks through a door's keyhole at close range, nearly the entire room on the opposite side of the door can be seen, although nothing can be seen when the hole is viewed from a long distance. This is similar to what happens when using the endoscope with focal lengths ranging from 5 to 20 mm: a wider angle of view can be achieved. Based on their, experience the authors believe that endoscopes can be used safely during neuro-otologic surgery. As an adjunct to or substitution for the operative microscope, this modality does improve visualization of bony, neural, and vascular structures while minimizing cerebellar retraction.

Decompression, Surgical↗

Remifentanil-based anesthesia versus a propofol technique for otologic surgical procedures.

Otologic procedures require a still surgical field and are associated with a 50% incidence of emetic symptoms. Propofol reduces nausea and vomiting but not intraoperative movement. This study compares a remifentanil/propofol anesthetic to a propofol/fentanyl combination to determine which provides the best perioperative conditions for otologic microsurgery. Eighty healthy patients were randomly assigned to receive one of the anesthetic combinations. Demographic data, hemodynamic variables, movement, and bispectral index monitoring values in addition to anesthetic emergence, nausea, vomiting, pain, and other recovery variables were compared between groups with appropriate statistical methods. Both groups were similar. Times to eye opening (7.7 +/- 0.7 vs 12.4 +/- 1.2 minutes) and extubation (9.8 +/- 0.9 vs 12.4 +/- 1.0 minutes) were shorter with remifentanil. This group also had lower hemodynamic variables and movement (23% vs 65%) under anesthesia. Postoperative pain was mild in both groups, but remifentanil patients had more than the propofol group. All other postoperative parameters were similar. Remifentanil-based anesthesia produces better hemodynamic stability, less movement, and faster emergence after otologic surgery, with propofol's antiemetic effect, for the same cost.

Adolescent↗

Comparison of 3 different anesthetic techniques on 24-hour recovery after otologic surgical procedures.

Intravenous propofol anesthesia is better than inhalational anesthesia for otologic surgery, but cost and intraoperative movement make this technique prohibitive. This study compares a propofol sandwich anesthetic with a total propofol or inhalational anesthetic for otologic surgery to determine which produces the best perioperative conditions and least expense. One hundred twenty patients undergoing ear surgery were randomly chosen to receive an anesthetic with either isoflurane (INHAL), total propofol (TPROP), or propofol used in conjunction with isoflurane (PSAND). Postoperative wakeup and the incidence and severity of nausea, vomiting, and pain were compared among groups. Antiemetic administration and discharge times from recovery and the hospital were also compared. The groups were similar, but anesthesia times were longer in the INHAL group. Emergence from anesthesia after PSAND or TPROP was more rapid than after INHAL. Recovery during the next 24 hours was associated with less nausea and vomiting with PSAND than with INHAL. The cost of the PSAND anesthetic was similar to that of INHAL, and both were less than TPROP. PSAND anesthesia may be similar to TPROP and better than INHAL for otologic procedures. PSAND was less expensive than TPROP and produced a similar recovery profile and antiemetic effect in the 24-hour period after surgery.

Adult↗

The pedicled superficial temporalis fascial flap: a new method for reconstruction in otologic surgery.

OBJECTIVES: Our goal was to describe a novel fascial flap of the temporal region and its use for reconstruction in otologic and neurotologic surgery. METHODS: The superficial temporalis fascia has an axial blood supply derived from the middle temporal artery and can be raised independently from the overlying temporoparietal fascia or the underlying deep temporalis fascia. This flap was used on 15 consecutive patients to solve a wide variety of reconstructive problems after otologic procedures. RESULTS: No additional morbidity was observed from the use of this flap. There were no complications related to the reconstruction. Adequate exposure for raising this flap was obtained using standard incisions for the otologic procedures. Follow-up ranges from 2 to 25 months. CONCLUSIONS: This fascial flap provides a wide surface area of tissue on a narrow-based pedicle capable of a wide arc of rotation. It provides thin, pliable tissue that can be adapted to the needs of various reconstructive otologic/neurotologic problems.

Adolescent↗

[Otologic surgical procedures in an Ear, Nose and Throat Department in Subotica from 1987 to 2001].

INTRODUCTION: This paper deals with otologic surgical procedures performed in a an Ear, Nose and Throat Department during a 15-year period. The authors compared the number of otologic surgical procedures with the number of laryngomicroscopies and procedures performed in Waldeyer's ring. RESULTS: From January 1, 1987 to December 31, 2001, 1184 patients underwent the following surgical procedures in total endotracheal anesthesia: 285 paracenteses, 473 ventilation tubes insertions, 175 antrotomies or mastoidectomies, 194 tympanoplasties, 34 radical mastoidectomies, 4 facial nerve decompressions, 5 exostoses or osteoma of the external auditory canal operations and 14 stapedectomies. DISCUSSION: Otologic surgical procedures are presented in table 1. In Yugoslav literature Topolac reports approximately 700 tympanoplasties in a 10-year period (1968-1978). Radonjić and associates report 2272 ear operations in the period 1975-1985. Distribution of operations is presented in table 2, whereas in table 3 we can see that the number of operated ears is much greater than the number of operated patients. CONCLUSION: We think that the number of operations is not bigger, because we operate only in critical cases. Our human and technical resources should provide advanced ear microsurgery, but only with financial and organizational support.

Hospital Departments↗

Informed consent in otologic surgery: prospective study of risk recall by patients and impact of written summaries of risk.

OBJECTIVES: To evaluate how much patients remember of the risks discussed with them about their otologic surgery and to evaluate whether a simple intervention, the addition of an information handout, improves recall. STUDY DESIGN: A prospective, randomized trial was undertaken in the setting of an academic tertiary care centre. METHODS: Fifty patients undergoing a variety of otologic procedures, including mastoidectomy, tympanoplasty, ossiculoplasty, and stapedectomy, were verbally consented by the operating surgeon with a standard checklist of potential surgical complications and side effects. Two surgeons participated in the study (40 patients and 10 patients). Patients were stratified into two groups: a higher education group and a lower education group. Within each group, patients were randomized to either a control group, consisting of a verbal explanation only, or an intervention group, which added a written handout to the verbal explanation. A follow-up telephone interview was conducted at an average of 20.6 days (range 14-53) to survey for recall of the complications discussed. MAIN OUTCOME MEASURES: The main outcome measure is risk recall. This is analyzed by education level and written sheet intervention. Other parameters examined are patient demographics, time elapsed from when the consent was obtained, and surgeon obtaining the consent. RESULTS: All 50 patients were interviewed in follow-up. Twenty-two patients received the handout (intervention arm), and 28 patients served as the control group. Overall recall, expressed as a percentage of risks explained, was 54% for the entire study group. For those who received the handout, the recall rate was 51%, whereas the rate was actually higher, at 56%, for those who did not receive the handout. This difference was not statistically significant. Of the specific risks discussed, patient recall was consistently high for the complications of facial nerve paralysis, 88%, and for complete hearing loss, at 88%. Other risks, such as dizziness, 31%, and change in taste, 36%, were not as commonly recalled. Receiving the handout made only a significant difference with one complication, facial nerve paralysis. CONCLUSION: The addition of a handout did not significantly alter recall of potential complications of otologic surgery with the exception of facial nerve paralysis.

Adolescent↗