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For many hearing-impaired individuals, the benefits of conventional amplification may be limited by acoustic feedback, occlusion effect, and/or ear discomfort. The MAXUM system and other implantable hearing devices have been developed as an option for patients who derive inadequate assistance from traditional HAs, but who are not yet candidates for cochlear implants. The MAXUM system is based on the SOUNDTEC Direct System technology, which has been shown to provide improved functional gain as well as reduced feedback and occlusion effect compared to hearing aids. This and other implantable hearing devices may have increasing importance as future aural rehabilitation options.

To systematically review the safety and efficacy of the 3 Food and Drug Administration–approved middle ear implant (MEI) systems currently in use for the rehabilitation of sensorineural hearing loss.

To describe a contemporary, pragmatic approach to managing cholesteatoma in the only hearing ear.

To better understand the characteristics and outcomes of facial nerve schwannomas (FNSs) over a 30-year period.

Objectives/HypothesisMastoidectomy has long been identified as an effective method of treatment for chronic ear infection. The effect of mastoidectomy on patients without evidence of active infectious disease remains highly debated and unproven. The objective in the study was to examine the impact of mastoidectomy on the repair of uncomplicated tympanic membrane perforations.

To determine the incidence of bilateral endolymphatic sac tumors in von Hippel-Lindau syndrome and to describe the technique and results of hearing preservation surgery for small endolymphatic sac tumors in a series of patients with von Hippel-Lindau syndrome.

The age of modern microsurgery has made resection of glomus tumors with extensive skull base involvement possible. Resection of extensive lesions is not without risk of major complication or new cranial nerve deficit. Because glomus tumors are rare and slow growing, data reflecting recurrence risk after resection using modern skull base techniques are lacking. These factors complicate the accurate definition of efficacy of surgical resection and its functional cost. The object of this review is to determine the current incidence of major complications, the surgical cranial nerve deficit, the long-term control rate, and the recurrence risk in patients undergoing surgical resection of glomus jugulare tumors.

The Audiant Bone Conductor has been heralded as an aid for use in conductive hearing loss; however, its possible use in unilateral sensorineural hearing loss (SNHL) has also been proposed. Between July 1987 and July 1989, profound unilateral sensorineural hearing loss has been rehabilitated In 43 patients using the Xomed Audiant Bone Conductor. Patients who were willing to participate in this clinical trial and who were felt to be good contralateral routing of signals (CROS) aid candidates were selected preoperatively. Audiometric followup, selection criteria, patient satisfaction, and complications are discussed.

Vestibular neurectomy is gaining widespread acceptance as a primary means of controlling medically refractory vertigo. However, debate continues over the adequacy of vestibular neurectomy within the cerebellopontine angle, long-term control, and the most appropriate surgical approach. To address these issues, we retrospectively reviewed 118 patients who underwent vestibular neurectomy between October 1984 and January 1988. Forty-two patients who underwent a retrolabyrinthine approach and 44 patients who underwent a retrosigmoid approach completed a written questionnaire and provided a recent audiogram. According to American Academy of Otolaryngology–Head and Neck Surgery guidelines, complete or substantial vertigo control was achieved and maintained in 95% of patients in both surgical groups. Hearing, tinnitus, and fullness results over the long term are variable. The advantages and disadvantages of the various vestibular neurectomy approaches will be detailed. On review of our results and surgical experience, we now prefer the retrosigmoid approach.

This study, merging data from two different clinics, was undertaken to evaluate the efficacy of endolymphatic mastoid shunt surgery using the Denver Inner Ear Shunt operation for Meniere's disease. This surgery was performed by the senior authors (C.G.J., J.R.E.D., and M.E.G.) between May 1984 and October 1986, after we attended the Colorado Otologic Research Center (CORC) instructional course. Sixty-six and thirty-four cases, respectively, were performed. Follow-up questionnaires were sent to this total of 100 patients. Fifty-six patients returned completed questionnaires, which formed the basis of this report. A retrospective chart study was also carried out. The results of surgery and questionnaires are reported as statistical data, and, where applicable, the 1985 AAO-HNS criteria for reporting vertigo and hearing results in the treatment of Meniere's disease are used. Preliminary analysis of these data is reported because of its profound effect upon our treatment protocol for patients with Meniere's disease. Results on use of the Denver valve do not appear to be significantly different than the results of other shunt surgery not using the valve.

We reviewed 271 intracanalicular and cerebellopontine angle lesions removed over the past ten years, 237 by the translabyrinthine or combined approach which created a mastoid defect. The patients were divided into three groups with the following results: (1) obliteration of the mastoid defect combined with older wound closure techniques in the first 188 patients produced CSF leakage in 25% and meningitis in 16% of cases; (2) not obliterating the defect intentionally in 16 patients produced CSF leakage in 50% and meningitis in 25% of cases; and (3) obliteration of the defect combined with newer packing and closure techniques in the last 33 patients produced CSF leakage and meningitis in only 6% of cases. Four problem areas were identified: The eustachian tube, middle ear, mastoid defect, and postauricular wound. Of these, obliteration of the mastoid defect was most important in minimizing postoperative CSF wound leakage, CSF rhinorrhea, and meningitis.

A previous study by the authors compared pathologic changes in the mouse sciatic nerve following prolonged continuousstimulation with either pulsed or direct current. The pulsed current stimulator was found as equally effective yet far safer than the direct current (DC) stimulator. In an attempt to reproduce more accurately the clinical setting, the Ear, Nose, and Throat Device Classification Panel of the Food and Drug Administration requested a prospective, controlled study of repeated DC stimulation at one or more sites along a peripheral motor nerve. Again, repetitive DC stimulation both at one site and at multiple sites of the same nerve produced significant myelin and axon degeneration. The authors conclude that even routine use of the DC stimulator carries with it the potential for permanent nerve damage and muscle paralysis.

Surgical intervention is indicated in Meniere's disease when symptoms persist in spite of an adequate trial of medical therapy. At The Otology Group, three surgical procedures are employed for the individual not responding to medical management. These are (1) the endolymphatic mastoid shunt, (2) the middle fossa vestibular nerve section, and (3) the translabyrinthine labyrinthectomy. The selection of the surgical procedures depends on a number of factors, including age, severity of symptoms, status of the contralateral ear, and hearing in the affected ear. As a general rule, if the patient has serviceable hearing, the first operation suggested is the endolymphatic shunt because of its low morbidity. Should this procedure fail, the middle fossa approach is recommended if the patient is not over 60 years of age. Translabyrinthine labyrinthectomy is recommended for those patients over 60 years of age as well as for younger patients with unilateral profound hearing loss.

The most significant objection to the proposal of lateral transtemporal cranial base surgery for the treatment of jugular foramen tumors is the perceived lasting morbidity attendant to aggregate cranial nerve loss. As techniques become more standardized and earlier diagnosis generates smaller tumors for treatment, outcome has become more predictable. Surgery has become the recognized management preference for these cranial base lesions. The purpose of this article is to assess the role of surgery in the treatment of jugular foramen lesions, as well as to review some of the technical highlights of conservation surgery, its clinical prerequisites, and reasonable expectations. We review 100 lateral skull base surgical cases of lesions involving the jugular foramen. The majority of these lesions—77 of 100—were paragangliomas. For these glomus tumors, cranial nerve preservation correlated well to tumor size and location. The diversity of the remaining 23 cases prevented any substantive conclusions.

• We report a case of astrocytoma of the acoustic nerve. Most gliomas arise from the brainstem, and seldom originate in the acoustic or other "true" cranial and spinal nerves. Clinical features of this rare acoustic tumor differ from those of brainstem gliomas, but are indistinguishable from typical acoustic neurilemoma. We discuss the diagnosis and histogenesis of glioma arising in the eighth cranial nerve. Demonstration of glial fibrillary acid protein, an antigen specific for astrocytes, is a new method of verifying the diagnosis. Review of the literature indicates that a few cases of epithelial-like tumors of peripheral nerves may have been of neuroepithelial origin. The evidence, however, generally is not sufficient to exclude the possibility of metastatic neoplasms or other tumors such as malignant schwannoma and melanoma. Most of these putative gliomas contained gland-like tissue, and did not have the morphologic appearance of astrocytoma, as in approximately five reported examples, and in our case.(Arch Otolaryngol 106:456-459, 1980)

Abstract: 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.

• Materials commonly employed in the preparation of otologic homografts such as ethanol and formaldehyde are effective in vitro in inactivating human immunodeficiency virus (HIV). However, to our knowledge, the complete permeation of homograft materials with preservative has not been demonstrated. Ethanol and formaldehyde have not been shown to be effective in inactivating the CreutzfeldtJakob agent. The literature on sterilization procedures for these agents is reviewed. Standard procedures for preparation of otologic homografts are examined. It is recommended that donor HIV serologic status be determined when otologic homografts must be used. Further research is required to determine the efficacy of otologic homograft sterilization techniques against HIV and CreutzfeldtJakob disease.(Arch Otolaryngol Head Neck Surg 1988;114:1252-1255)

• Auditory brain-stem response (ABR) waveforms from 61 patients with eighth nerve or cerebellopontine angle tumors were analyzed for wave presence or absence. Eighteen patients yielded ABRs lacking any readable waves; 27 patients revealed ABRs in which waves were partially present (ie, one or two waves present); and 16 patients demonstrated ABRs with all waves (I, III, and V) present. In the partially present ABR group, waves I and V were most prevalent. The group lacking waves (absent ABR group) had significantly poorer hearing sensitivity and larger tumors than the partial ABR group. Various additional group comparisons for hearing sensitivity and tumor size did not yield any significant differences among the three groups.(Arch Otolaryngol Head Neck Surg 1986;112:186-189)

• Glomus tumors of the temporal region and skull base can range in size from small microscopic lesions confined to the promontory to large destructive and neurologically aggressive agents of substantive incapacitation. Contemporary diagnostic and surgical technology has made definitive management of these historically dreaded lesions confidently practical. Prompted by these advances, a new classification of glomus tumors is disclosed. The specific problem of the large chemodectoma at the skull base is addressed and a series of such cases reviewed. Surgical therapy is viewed as definitive, and the technique employed by the members of The Otology Group, PC, is described.(Arch Otolaryngol 1982;108:401-406)

• We present two cases of lipoma of the cerebellopontine angle (CPA), which brings, to our knowledge, the total reported in the literature to six. These cases had a clinical, surgical, and postoperative course that was similar to a small acoustic neuroma. The roentgenographic aspects were in keeping with small, CPA tumors. A review of the literature of CPA tumors and lipomas of the CNS is presented. We discuss the controversy surrounding the origin of lipomas of the CNS.(Arch Otolaryngol 104:431-436, 1978)

Although the contribution of special auditory testing in the determination of site of lesion in patients with sensorineural hearing loss has been well established, there is need for ongoing investigation. A comparative evaluation of eight audiometric procedures with 100 cochlear lesion patients and 27 patients with confirmed eighth nerve tumors was made. Although the individual tests continue to be less than 100% accurate in prediction, the pattern of results obtained from the test battery continues to show a high correlation with site of lesion.

The VII-XII anastomosis has been employed for more than a decade by The Otology Group, P.C., In the facial reanimation of patients undergoing extirpation of tumors involving the cerebellopontine angle and the skull base. A retrospective review based upon a detailed questionnaire and submitted photographic documentation from 61 patients forms the basis for this review. Details and analysis of the functional results include (1) onset of function, (2) synkinetic activity, (3) corneal irritation and associated ophthalmologic problems, (4) facial tone and symmetry, and (5) volitional mimetic function. From a psychosocial perspective, evaluation was made regarding (1) workplace and home acceptance, (2) self consciousness, (3) adaption, and (4) overall satisfaction. Because of the nature of its technical performance and reliability, the VII-XII anastomosis is an important technique for the otolaryngologist to be familiar with. Cognizance of the functional and psychologic results with this procedure will ensure optimal (yet realistic) rehabilitation for this patient population.

Advances in microsurgical techniques, with the use of the laser and the ultrasonic suction aspirator, have paved the way for even more aggressive surgical approaches to large skull-base lesions, such as glomus tumors, adenocarcinomas of the temporal bone, and cranial nerve neuromas. Postoperative morbidity of multiple cranial nerve dysfunction has been aggravated in the past by cerebrospinal fluid leakage and/or gastro-intestinal dysfunction. At the Otology Group, P.C., In the course of dealing with more than 86 skull-base tumors, we have developed a standardized, multispecialty approach to such extensive skull-base lesions, including tracheotomy, gastrostomy, “ventriculoatrial” shunt placement, and rotated temporalis muscle flap closure. This article presents cases that Illustrate the evolution of the current, “standard skull-base” operative procedure and the rationale for incorporation of the shunt and the muscle flap closure as operative refinements. Their beneficial effects upon postoperative morbidity are detailed.

Chondrosarcoma of the skull base is an uncommon neoplasm that comprises 0.15% of all intracranial tumors and 6% of skull base lesions. Compression of vital neurologic structures by tumor expansion as well as the histologic characteristics contribute to the malignant potential of these neoplasms. Tumor recurrence has been associated with incomplete resection in this traditionally surgically inaccessible region. This review of five cases of chondrosarcoma of the skull base examines the surgical approaches to resection in a chronologic fashion to emphasize that total gross resection of these lesions has become possible through the evolution of lateral skull base techniques. The difficulty in pathologic diagnosis and the efficacy of postoperative radiotherapy in enhancing survival are also addressed.

Middle fossa section of the vestibular nerve is a neurotologic skull-base procedure that has found a successful, albeit limited, role in the surgical management of Meniere's disease. Incapacitating vertigo can be controlled in 94% of the cases, while serviceable hearing is maintained in 70%. Tinnitus and aural pressure are reduced in 60% and 80% of cases, respectively. The morbidity of this approach is similar to that of other destructive procedures of the labyrinth when careful patient selection and precise surgical technique are employed. In the incapacitated vertiginous patient with serviceable hearing, middle fossa vestibular nerve section should be considered as an alternative to other destructive procedures involving the otic capsule.

The surgical management of patients with slow‐growing benign temporal bone neoplasms has been criticized because of its significant morbidity and mortality compared with results after radiation therapy, but long‐term control by irradiation remains unproved. Long‐term surgical results have not been studied previously. One hundred twenty‐nine skull base operations were performed in 126 patients at the Otology Group, Nashville, Tenn., from January 1970 through May 1987. Fifty‐eight patients responded to questionnaires focusing on recovery from loss of cranial nerves. All patients regained some degree of facial function (class V or better), no alimentary tubes or tracheotomies were in use, and no patients had debilitatinQ aspiration. Long‐term compensation from the cranial nerve deficits of lateral skull base surgery can be expected in most patients and should not be used as an argument for irradiation in patients with a long life expectancy at time of diagnosis.

Hearing stabilization following middle fossa vestibular nerve section (MFVNS) has been observed to occur in a large percentage of patients (71% to 86%) after short-term followup. This study looks at the long-term audiological followup (5 to 15 years) of 46 patients who underwent a MFVNS for intractable Meniere's disease. Although the percentage of patients with stabilized hearing was relatively high within the first 2 years postoperatively (61%), it dropped to 41% after a longer followup of 5 to 15 years. When this patient population is divided into two groups based on their preoperative PTA, those patients in whom hearing had bottomed out to ≥50 dB PTA preoperatively lost only 5 dB PTA over the course of the follow-up period. The patients with better preoperative hearing (<50 dB PTA) lost 30 dB PTA over the same follow-up period. As this pattern is similar to what has been observed in nonsurgically treated Meniere's patients, we conclude that the MFVNS has no beneficial effect on the hearing loss associated with Meniere's disease.

Glomus tumors can be classified as to their origin in the temporal bone. Those limited to the middle ear and mastoid are referred to as tympanicum tumors while those originating in the jugular bulb are known as jugulare tumors. Diagnostic studies such as polytomes of the temporal bone, retrograde venograms and arteriograms now make it possible for the surgeon to determine not only the origin of the tumor but also the extent of its involvement in the temporal bone and base of the skull.

• Cases of aberrant internal carotid artery in the middle ear have been well documented. However, bilateral aberrant intratympanic carotid artery presentation, as in this case, is quite rare. In addition, in this case, there was unilateral duplication of the cervical and petrous portions of one internal carotid artery such that the duplicated portion rejoined the normal internal carotid artery in its petrous portion. The purpose of this article is to review the literature regarding aberrant internal carotid artery presentation in the middle ear and to discuss the related embryological and anatomical features as well as diagnosis of this lesion, using a unique case as an example.(Arch Otolaryngol Head Neck Surg. 1993;119:335-339)

Surgical intervention is indicated in Meniere's disease when symptoms persist in spite of an adequate trial of medical therapy. At The Otology Group, three surgical procedures are employed for the individual not responding to medical management. These are (1) the endolymphatic mastoid shunt, (2) the middle fossa vestibular nerve section, and (3) the translabyrinthine labyrinthectomy. The selection of the surgical procedures depends on a number of factors, including age, severity of symptoms, status of the contralateral ear, and hearing in the affected ear. As a general rule, if the patient has serviceable hearing, the first operation suggested is the endolymphatic shunt because of its low morbidity. Should this procedure fail, the middle fossa approach is recommended if the patient is not over 60 years of age. Translabyrinthine labyrinthectomy is recommended for those patients over 60 years of age as well as for younger patients with unilateral profound hearing loss.

The most significant objection to the proposal of lateral transtemporal cranial base surgery for the treatment of jugular foramen tumors is the perceived lasting morbidity attendant to aggregate cranial nerve loss. As techniques become more standardized and earlier diagnosis generates smaller tumors for treatment, outcome has become more predictable. Surgery has become the recognized management preference for these cranial base lesions. The purpose of this article is to assess the role of surgery in the treatment of jugular foramen lesions, as well as to review some of the technical highlights of conservation surgery, its clinical prerequisites, and reasonable expectations. We review 100 lateral skull base surgical cases of lesions involving the jugular foramen. The majority of these lesions—77 of 100—were paragangliomas. For these glomus tumors, cranial nerve preservation correlated well to tumor size and location. The diversity of the remaining 23 cases prevented any substantive conclusions.

Within the past quarter of a century, contributions to the surgical literature have steadily reflected an increasing interest in the surgical extirpation of lesions involving the skull base. While optimistic reports demonstrate a variety of operative techniques1–9and clinical experiences,10–12there has been a paucity of discussion regarding the intraoperative and perioperative management of these complex cases. Cognizant of the fact that the care of these patients is of paramount importance, we herein review our rationale and practiced protocol for management of this unique patient population. The removal of lesions that also require the sacrifice of multiple cranial nerves results in significant postoperative morbidity. With the significant cardiopulmonary challenges attendant upon long procedures, these patients require constant multisystem management. Infection surveillance, wound care, cardiopulmonary support, fluid and nutritional monitoring, vocal and pharyngeal rehabilitation, along with psychological support, manifest in a complicated array of managerial decisions, require an organized approach to optimize patient care.

This study, merging data from two different clinics, was undertaken to evaluate the efficacy of endolymphatic mastoid shunt surgery using the Denver Inner Ear Shunt operation for Meniere's disease. This surgery was performed by the senior authors (C.G.J., J.R.E.D., and M.E.G.) between May 1984 and October 1986, after we attended the Colorado Otologic Research Center (CORC) instructional course. Sixty-six and thirty-four cases, respectively, were performed. Follow-up questionnaires were sent to this total of 100 patients. Fifty-six patients returned completed questionnaires, which formed the basis of this report. A retrospective chart study was also carried out. The results of surgery and questionnaires are reported as statistical data, and, where applicable, the 1985 AAO-HNS criteria for reporting vertigo and hearing results in the treatment of Meniere's disease are used. Preliminary analysis of these data is reported because of its profound effect upon our treatment protocol for patients with Meniere's disease. Results on use of the Denver valve do not appear to be significantly different than the results of other shunt surgery not using the valve.

One hundred thirty‐nine tumor removals are reviewed with special interest in ten attempts to save hearing. Three patients with bilateral tumors had some hearing preserved. One of these individuals had a 1 cm tumor removed and his postoperative SRT and discrimination scores were the same as his preoperative ones. Overall, the authors were able to maintain some hearing in six out of ten attempts. While there is no question that patients with bilateral tumors benefit from residual hearing, this is not always true in unilateral cases. The suboccipital and middle fossa procedures are discussed as well as the relative merits of each approach in the preservation of hearing. The total series (139 tumors) is discussed in detail regarding results and complications.

The patient with a cholesteatoma in an only hearing ear presents a management dilemma: how best to treat the patient to minimize the chances of developing a severe hearing loss in that ear. Twelve patients managed surgically for cholesteatoma in their only hearing ear are reviewed. The location and extent of the cholesteatoma, the type of operation performed, and postoperative hearing results are presented. Eleven of the 12 patients maintained their bone-conducted speech reception threshold to within 5 dB of the preoperative level during follow-up periods of 2 months to 4 years. Recommendations are made regarding preoperative evaluation, perioperative use of antibiotics and steroids, operative planning, ossicular reconstruction, and postoperative care. Special attention is given to the evaluation and management of the ear that has a fistula eroding into the cochlea and semicircular canals, both when suspected from preoperative imaging studies and when discovered intraoperatively.

Ten skull-base neuromas treated at the Otology Group P.C. are presented. Each case is reported in detail. The method by which preoperative diagnosis was made is reviewed. Specific attention is paid to distinguishing these tumors from chemodectomas. Presenting signs and symptoms are enumerated. Operative—especially postoperative—management is discussed in detail.

Eighty-two revision stapedectomies were analyzed to determine causes of primary and revision stapedectomy failure. The specific cause of failure can often be anticipated preoperatively by analysis of the patient's audiogram, symptoms, type of prosthesis initially used, and the duration of time between initial surgery and the onset of symptoms. Guidelines for performance of revision stapedectomy are discussed.