Jack Shohet

Malignant Glomus Tumors

Objectives/Hypothesis: To ascertain the incidence of malignancy in a large glomus and skull base series and to compare the clinical course of such patients with those who do not have malignant skull base lesions. Study Design: Retrospective review of all skull base surgery cases treated at the Otology Group between 1970 and 1995. Results: In a series of 175 jugulotympanic glomus tumors, 9 cases (5.1%) were identified. The surgery required for their extirpation is more extensive than nonmalignant glomus tumors. Attendant deficits and mortality from these procedures are accordingly greater. Five‐year survival in this limited population was 72%. Prolonged periods of survival are possible with distant metastases. Conclusions: This rate of malignancy should advocate against a watchful, waiting approach. Radiation therapy is not advocated as a primary modality for this type of tumor, as it may lead to recurrence/persistence with either subsequent malignant degeneration and metastases or local recurrence.

The management of pediatric cholesteatoma

Pediatric cholesteatoma can be congenital or acquired. The two types appear to be separate and distinct entities based on molecular studies and clinical course. Pediatric cholesteatoma behaves differently from cholesteatomas in the adult. This may have more to do with anatomic and physiologic differences than with the molecular structure of the cholesteatoma. Treatment requires an individualized approach taking into account the experience of the operative surgeon and the high risk of recidivism of this disease.

The Envoy Esteem Implantable Hearing System

This article discusses the Envoy Esteem implantable hearing system, a completely implantable hearing device. The device is indicated for patients older than 18 years with stable moderate to severe sensorineural hearing loss and good speech discrimination. The device is placed through an intact canal wall tympanomastoidectomy with a wide facial recess approach. The implant is typically activated at 6 to 8 weeks postoperatively and usually requires several adjustments for optimal performance. The sound processor/battery lasts 4.5 to 9.0 years and can be replaced through a minor outpatient procedure.

Normative Data of Incus and Stapes Displacement During Middle Ear Surgery Using Laser Doppler Vibrometry

Objectives To report normative data for incus and stapes motion using laser Doppler vibrometry (LDV) during middle ear surgery and to discuss possible limitations of the procedure. Study Design Institutional review board–approved, retrospective study of data from patients undergoing the Envoy Esteem implantable device at 3 institutions. Setting Quaternary referral health system. Patients Eligibility criteria: patients sucsessfully implanted with an Esteem device. Methods Data from 70 patients undergoing the Envoy Esteem procedure were reviewed. Sound at 100 dB and 50 frequencies ranging from 125 to 8,000 Hz were used during the procedure. LDV was performed to measure the displacment of the body of the incus and the posterior crus of the stapes to assess whether there was sufficient ossicular mobility to allow for implantation. Results The average displacement of the ossicles for all 70 patients was collected and analyzed. The trend was an average displacement around 100 nm from 125 to 500 Hz for both the incus and stapes with a linear decline starting at 1,000 Hz for the incus and 500 Hz for the stapes, with slightly greater displacement of the stapes at higher frequencies. Conclusion This is the first article to report in vivo measures of ossicular mobility. These data help to understand the micromechanics of ossicular motion as well as the use and limitations of LDV. This information may lead to a prescreening process for implanted middle ear devices that function by overdriving the stapes.

Esteem Treatment of Profound High-Frequency Hearing Loss

been diagnosed. Special focus was on the age at diagnosis, the localization, and the size of the tumor. The size of the tumor was registered as either intrameatal or with the largest extrameatal diameter. Incidence: The annual number of diagnosed VS has increased from 15 in 1976 to 120 in 2004. Since 2004 the annual number of diagnosed cases has decreased. The size of the diagnosed tumors has decreased from a median of 35 mm in 1979 to 10 mm in 2009. In the first years the large and giant tumors dominated, in contrast to the recent years where the intrameatal and small tumors dominated. The median age at the time of diagnosis has been slowly increasing from 50 years in the beginning of the period to almost 60 years in the end of the period. In patients with 100% speech discrimination, hearing seems to be stable, even after 10 years. In patients with even a slight discrimination loss at diagnosis, almost half lose good hearing during wait and scan. Discussion: If the decreasing size of the tumor and the increasing incidence of VS should be explained only by earlier diagnosis and easier access to MR scanning one should expect that the median age at the time of diagnose would decrease simultaneously. In our study, the median age at the time of diagnosis has been almost unchanged through the 33 year long period. This paradox can be explained by the fact that with the easier access to MR scanning, the examination has been offered also to the elderly patients, in whom the small and intrameatal tumors are dominating. CONCLUSION: The annual number of diagnosed vestibular schwannomas is increasing, the tumor size is decreasing, and the age at diagnosis is increasing. About 25% of tumors are growing and in patients with 100% speech discrimination, good hearing remains even after several years.

09:10: Phase II Results: Esteem Totally Implantable Hearing System

diagnosing various types of hearing loss. METHODS: Prospective randomized study investigating the validity of the hum test to diagnose hearing loss. One hundred consecutive patients presenting to a subspecialty neurotologic clinic were randomly assigned to the study over a four-month period. Patients were asked to hum and report which side they heard the hum. Tuning fork Weber test at 512Hz as well as a full audiologic profile was done and blindly correlated to the results of the hum test. RESULTS: The hum and Weber test had a 95% correlation to one another and to the pure tone audiogram in identifying conductive hearing loss. The tests had a 70% correlation to one another in diagnosing sensorineural hearing loss. CONCLUSIONS: The hum test is an accurate means of remotely diagnosing conductive hearing loss without the need for patient training or instrumentation. The test can easily be performed remotely by an otolaryngologist or even general physicians with minimal training to confirm conductive hearing loss and prevent unnecessary office visits.

Skull Base Surgery

Publisher Summary Advances in otolaryngology, plastics and reconstruction surgery, ophthalmology, oral or maxillofacial surgery, and neurosurgery have resulted in improved treatment for skull-base tumors. This chapter provides an overview of the diagnostic evaluation of a skull-base tumor patient, the anesthetic and intraoperative monitoring options available, the common pathologies encountered, the surgical approaches for tumors involving the cranial base, and the potential complications arising from skull-base surgery. The evaluation of the patient with a cranial base lesion begins with a thorough history and physical examination. Then, a complete head and neck examination, along with a neurological assessment, is performed. Particular emphasis in the examination is placed on the region of tumor involvement. Narcotic agents are favorable for skull-base surgery as they decrease the cerebral oxygen consumption rate, have fewer hypotensive side effects, avoid elevation of the intracranial pressure, and are reversible—allowing for rapid emergence from anesthesia. Diuretics are often administered to shrink the brain during the intracranial portion of the surgery to enhance surgical exposure. One of the most common complications in skull base surgery is a cerebrospinal fluid (CSF) leak. The incidence of a CSF leak varies proportionally to the size of the dural defect created. A CSF leak detected in the postoperative period occurs typically through the surgical wound, ear canal (otorrhea), or nose (rhinorrhea). Early identification and measures to correct this are important to minimize the risk of CSF contamination and the development of meningitis.