Twenty-year report of the first auditory brain stem nucleus implant.

We present the 20-year case report of an auditory implant placed on the dorsal cochlear nucleus with long-term electrical stimulation in a patient with neurofibromatosis 2. The patient has continued to use her implant daily for 20 years, and it has greatly enhanced her quality of life. There have been no adverse sequelae.

Surgical rehabilitation of deafness.

Cochlear implants are now an accepted treatment in the rehabilitation of sensorineural hearing impairment. An overview of our understanding of how implants work, the clinical indications, and the current expected results of implantation are briefly reviewed. This is not a comprehensive review but rather a brief summary on the current knowledge of cochlear implants.

The middle fossa transpetrous approach for petroclival meningiomas.

Seventeen patients with petroclival meningioma were operated on through a middle fossa transpetrous approach. This approach exposes the anterior cerebellopontine angle through a middle fossa craniotomy with removal of the petrous apex medial to the cochlea and petrous carotid artery. This approach may be enlarged by transection of the superior petrosal sinus and tentorium. The surgical technique and application of the middle fossa transpetrous approach for petroclival meningiomas is presented.

Differential diagnosis of virus-like particles in the human inner ear.

The entire endolymphatic duct and sac as well as the vestibular epithelia were obtained from four patients with Meniere's disease during translabyrinthine (TL) eighth nerve section and from 12 patients undergoing TL resection of acoustic schwannomas. After these specimens were processed for routine transmission electron microscopy (TEM), they were studied for morphologic evidence of viral infection. Although no virus particles were identified, numerous regularly occurring cell components and artifacts were found to morphologically mimic viruses. An atlas of these structures is presented.

Extended middle fossa and retrolabyrinthine approaches in acoustic neuroma surgery: case reports.

Hearing conservation surgery for small acoustic neuromas is well accepted. At present, two approaches are primarily used: the suboccipital and the middle fossa. The middle fossa approach to the internal auditory canal has the advantage of using bony landmarks to identify and protect the facial nerve. Because of anatomic constraints presented by the superior semicircular canal however, its uses are limited to intracanalicular tumors or tumors protruding only slightly into the cerebellopontine angle. By extending the approach through the superior semicircular canal, a wide access to the cerebellopontine angle can be safely obtained. In this study three procedures, two through the superior semicircular canal and one through the posterior semicircular canal, were utilized for hearing conservation surgery. By immediately sealing off the canal ends, hearing preservation was accomplished in two out of three of these cases. The dictum that labyrinthine opening invariably leads to anacusis should be reconsidered. By utilizing approaches through the semicircular canal, it is possible that morbidity from this surgery may be reduced.

Middle fossa approach for acoustic tumor removal.

The middle fossa approach is well suited for the removal of small acoustic tumors with possible hearing preservation. The most appropriate candidates have tumors with less than 5 mm extension into the cerebellopontine angle and good preoperative hearing (speech reception threshold less than or equal to 30 dB, speech discrimination score greater than or equal to 70%). Measurable postoperative hearing can be preserved in 31% to 59% of patients, and normal or near normal facial function occurs in 86% to 89%. Serious postoperative complications are rare with this approach. With the advent of gadolinium-enhanced MRI, it is now possible to diagnose acoustic tumors reliably when small and before hearing has been significantly affected. The middle fossa approach provides excellent access for the removal of these small tumors.

A technique to obtain and process surgical specimens of the human vestibular aqueduct for histopathological studies of the endolymphatic duct and sac.

The endolymphatic sac (ES) may play a crucial role in the pathophysiology of Ménière's disease. This paper presents a technique to obtain and process fresh human specimens of the endolymphatic duct (ED) and the presumably more active intraosseous portion of the ES obtained at surgery. The specimens are preserved with an intact bony shell around the ED and the intraosseous ES. This allows ultrastructural histopathological evaluation of the intraluminal contents, the epithelium and the subepithelial tissue as well as the mutual relationships of these structures. Various factors influencing ES ultrastructure are discussed. The results obtained from this method may increase our understanding of the possible role of the ES in the etiology of different inner ear disorders.

Cochlear implants: histopathologic findings related to performance in 16 human temporal bones.

This paper presents results of a histologic study of 16 temporal bones with cochlear implants from 13 subjects. Damage caused by electrode insertion in the basal turn of the cochlea was evaluated. Dendrite and spiral ganglion cell populations were compared to clinical performance scores to determine structures necessary for stimulation and the minimum number needed for electrical stimulation. Results show that damage from insertion of long electrodes was located mainly at the most anterior part of the basal turn; that despite total degeneration of dendrites in the area near the electrode, some spiral ganglion cells remained; and that spiral ganglion cells or possibly axons are the stimulated structures and that fewer of them than previously thought are necessary to achieve a hearing sensation from electrical stimulation.

Electrode insertion trauma in cochlear implantation.

Residual hearing conservation may be important in cochlear implantation of children and of adults with disabling tinnitus responsive to extracochlear stimulation. Damage to the neural elements of the cochlea during electrode insertion may have a negative impact on residual hearing conservation. Histologic analysis of eight temporal bones with cochlear implants reveals trauma at essentially two locations: the round window insertion site and along the basal turn of the cochlea. In four of the bones, insertion at the round window resulted in damage to the osseous spiral lamina and the electrode was inserted through the scala media into the scala vestibuli. Evidence of secondary reactive osseous formation was also noted in these bones. This paper relates the surgical anatomy of the round window to histologic findings and microanatomical dissections. Recommendations for implantation surgery include creation of a cochleostomy by removal of the floor of the round window niche and a superior-to-inferior angle of electrode insertion.

Cochlear implant histopathology.

Twenty-two temporal bones and one brain stem from 13 cochlear implant patients were examined histologically. Sixteen temporal bones had undergone one or more implant procedure. Results of analysis suggested that the ganglion cells were the responding elements to the implant and that useful auditory sensation could result from as few as 10 percent of the normal number of ganglion cells. All implanted bones exhibited varying amounts of fibrosis (some ossified) in the basal turn of the cochlea and beyond in some cases. Usually there was damage to the surviving elements of the organ of Corti and the dendrites throughout the extent of the electrode insertion. However, the ganglion cell population was not affected. Prolonged electrical stimulation (up to 14 years) did not affect ganglion cell survival in three cases, and had no effect on the cochlear nerves in two cases or on cochlear nuclei in one case.

Ultrastructural evidence of a merocrine secretion in the human endolymphatic sac.

The results of a light and transmission electron microscopic analysis of an endolymphatic sac (ES) from a patient suffering from episodic vertigo, tinnitus, and hearing loss are presented. A biopsy of the intraosseous portion of the ES was obtained during a translabyrinthine approach to section the vestibular nerve in the internal acoustic meatus. The material consisted mainly of tubular epithelial structures filled with heavily stained material. Pathologically dilated and degranulated rough endoplasmic reticuli and disaggregation of polyribosomes with accumulation of solitary ribosomes in the cytosol and endoplasmic reticulum suggested a disturbed epithelial cell protein synthesis. Ultrastructural evidence of an increased merocrine secretion of glycoprotein conjugates into the ES was noted. This made it possible to analyze the presumed intracellular secretory pathways. An increased number of intraepithelial lymphocytes and monocytes was observed. Since the inner ear had been subjected to surgical intervention before the vestibular nerve section, no conclusions can be drawn as to whether the patient's symptoms were related to the disturbed protein metabolism and hypersecretion of glycoprotein conjugates into the ES. The findings support earlier experimental results that indicate that the ES has not only a resorptive function but also a secretory one.