Annals of Otology,Rhinology & Laryngology

Malignant external otitis is an infection which begins in the external auditory canal. It is uniformly caused by the Gram negative Pseudomonas aeruginosa organism and mainly affects elderly diabetics. It spreads to the soft tissues beneath the temporal bone and, if not properly treated leads to facial nerve palsy, mastoiditis, sepsis, osteomyelitis of the base of the skull, sigmoid sinus thrombosis, multiple cranial nerve palsies and death. Experience with 72 patients in varying stages of the disease is summarized. Stressed are the diagnostic criteria of nonresponsiveness to the usual methods of treatment, continued suppuration, and the continuing reformation of granulation tissue in the floor of the external auditory canal. Medical treatment is recommended with hospitalization and intravenous carbenicillin and gentamicin. Minor surgical debridement is helpful. All patients should be treated medically for as long as improvement continues, reserving surgical intervention only in the event a plateau is reached or symptoms and signs become worse under treatment. With or without a major surgical procedure, it is imperative to continue treatment for at least seven days after apparent cure in order to avoid recurrent disease possibly at a site distant from the canal.

The transsphenoid approach to cystic lesions of the petrous tip has been made possible by the advances in techniques for diagnosis. These include polytomography, improved angiography, and computer tomographic (CT) brain scanning. The results of these studies can determine the size, shape, and thickness of the cystic lesion, as well as its exact relationship to the sphenoid sinus. A number of surgical approaches have been used to reach the cystic lesion of the petrous apex. Most notably, these include the middle fossa extradural craniotomy and posterior fossa craniotomy. These surgical techniques allow the cyst to be biopsied but do not establish permanent drainage. The translabyrinthine approach accomplishes wide access to the petrous apex, but cochlear and vestibular functions are sacrificed. The transsphenoid approach to cystic lesions of the petrous apex should be the ideal operation provided that the anterior aspect of the cyst abuts against the posterior wall of the sphenoid sinus. The cyst can be completely evacuated and permanent fistulization established into the sphenoid sinus using a septal mucosal flap and a silicone drainage device. The operation is accomplished without destruction to the inner ear.

Seventy percent of 80 surgically proven acoustic neuromas were visualized on preoperative computed tomography (CT) scans. Many more tumors were seen on contrast enhanced scans than on unenhanced scans (55 vs 25). No intracanalicular tumors and only one of 15 tumors less than 2 cm in size were visualized on CT scan. Most medium size tumors (2.0–3.0 cm) and all large tumors (>3.0 cm) were visualized on CT scan when optimal technique was used. Positive CT scans often replace more invasive diagnostic studies in the investigation of acoustic neuromas. Negative scans do not rule out tumors as intracanalicular, and small tumors are usually not visualized with current techniques. If there is clinical suspicion of an acoustic neuroma, positive contrast posterior fossa cisternography should be done when the CT scan is negative.

Modifications of external ear anatomy following tympanomastoid surgery can have significant effects on the hearing threshold. Experiments are described that demonstrate the acoustical effect of changes in the dimensions of the concha and ear canal on external ear sound pressure gain. An attempt is made to correlate these findings with results in 30 post-tympanomastoidectomy ears and develop some basic principles of external ear surgery that might be clinically useful.

This is an analysis of 1,024 primary cases of mastoid surgery for cholesteatoma operated upon during a ten-year period at the Otologic Medical Group, inc. Our philosophy of management of the mastoid in these cases has been as follows: 1) avoid an open mastoid cavity when possible; 2) perform the operation in two stages if necessary; 3) reexplore the mastoid and middle ear for residual cholesteatoma when indicated. One-third of 380 revised cases had residual cholesteatoma, disease left by the surgeon. In 260 cases in which the surgeon felt it unlikely that there was residual disease he found it in 23%. In 4% this residual cholesteatoma was found in the mastoid. The incidence of residual cholesteatoma was higher in children and in planned, as opposed to unplanned, revisions. Residual cholesteatoma was detected in the middle ear more frequently than in the epitympanum, and in the epitympanum more frequently than the mastoid. Indications for, and timing of, the reexploration are discussed. Recurrent cholesteatoma refers to a retraction pocket and must be differentiated from residual cholesteatoma; the causes, prevention and treatment are different. Recurrent cholesteatoma was detected in 5%. The most common complication of the disease was a labyrinthine fistula (10%). Operative facial nerve damage occurred in one case. The most common postoperative complication was graft failure (3%). Intact canal wall tympanoplasty with mastoidectomy should be performed as a two-stage procedure in most cases when used in the treatment of aural cholesteatoma.

This is a prospective in-depth study of patients with sudden idiopathic sensorineural hearing loss. We found that 65% recover completely to functional hearing levels spontaneously and independent of any type of medical treatment. The majority do so within 14 days and many within the first few days. Prognosis can be predicted according to the slope of the initial audiogram (low-frequency losses do better than high-frequency losses), hearing at 8 kHz, erythrocyte sedimentation rates, in some select instances spatial disorientation symptoms, and speech discrimination scores. There was a very poor correlation between hearing and vestibular test abnormalities, except hypoactive calorics. There were no correlations with age (excepting the very elderly), with antecedent respiratory infections. hypertension, diabetes, or other chronic diseases. We conclude that there is a fundamental difference in the behavior of apical and basal cochlea losses, that hearing recovery is always better at low than at high frequencies, that because of the high spontaneous recovery rates, tympanotomies seeking perilymph fistulas should be delayed ten days unless there is a progressive hearing loss, and that none of the current recommended treatments, especially histamine, have any effect on the outcome.

Pathogenesis of otitis media was studied in humans and various animal models primarily from a pathological and chemical point of view. Findings were correlated and interpreted for various forms of otitis media in longitudinal and parallel studies, including acute purulent otitis media (POM), serous otitis media (SOM), mucoid or secretory otitis media (MOM), and chronic suppurative otitis media (COM), especially as regards the continuum or interrelated changes of various groups. Purulent otitis media was produced in chinchillas by direct inoculation of less than 100 pneumococci into the middle ear space. Serous otitis media was produced in chinchillas and cats following Eustachian tube obstruction with silicone. Mucoid otitis media followed the development of SOM in cats after two to four weeks of tubal occlusion. Samples of middle ear effusion (MEE) and serum, obtained from children with SOM and MOM after myringotomy for ventilation tube placement, were evaluated. The three components studied were MEE, epithelium and the subepithelial space (SES). Inflammatory changes in the SES were significant for all forms of otitis media, but especially for POM and SOM. Epithelial metaplasia to secretory cells was most prominent in MOM. Chemical factors involved in pathogenesis and defense were studied. Lactic dehydrogenase and lysozyme, chemical indicators of inflammatory activity, were greater in POM and MOM than in SOM. Immunoglobulins (A, G, & M) were greater in MOM than in SOM. The similarity of findings between the groups suggests a strong relationship between them. The ability of certain types of otitis media to evolve into another substantiates the concept of the continuum for some patients. Pathogenesis is dependent upon various extrinsic factors of etiopathogenesis, while the form that otitis media takes seems to rely mostly on relative activity of the SES and the epithelium.

Inner ear fluids were studied biochemically in guinea pigs from 1 to 16 weeks after producing endolymphatic hydrops by obstructing the endolymphatic duct. Fluid collected from beneath the footplate showed changes of 50% of the animals indicating distension of the saccule. There was an increase in potassium concentration and decrease in sodium concentration of the collected fluid, indicating a traumatic mixing of endolymph and perilymph produced by rupture of the saccule during collection of the fluid. The smallest changes occurred at one week, and the greatest changes were found four months after endolymphatic sac obstruction. Similar findings were observed in studying inner ear fluids obtained from patients with Ménière's disease. Cochlear endolymph showed biochemical alterations after endolymphatic duct blockage. There was a two- to three-fold increase in sodium concentration and a decrease in potassium concentration with the total ionic concentration remaining approximately the same. Some guinea pigs showed similar changes in vestibular endolymph. This study indicates that in the guinea pig, endolymph obtained in the distended endolymph compartment has a slightly different sodium-potassium ratio as compared to the normal ear. In patients with Ménière's disease there may also be an elevation of endolymph sodium concentration. The significance of this change and hearing loss observed in experimental endolymphatic hydrops and Ménière's disease is open to speculation at the present time.

After bilateral ablation of the auditory areas of the cerebral cortex, experimental animals have a severe deficit in ability to discriminate between temporal patterns of tonal stimuli and to localize sound in space. These two kinds of discrimination are basic for communication and for attack or avoidance of prey and predator. Recognition of which ear is stimulated may also depend upon excitation of auditory cortex contralateral to the given ear. Binaural discriminations are dependent upon interaction of nerve impulses from the two ears at a low level in the auditory nervous system. Similar hearing losses have been reported for human patients.

The ultrastructural pathology in an ear with long-standing profound deafness is presented. Endolymphatic hydrops was present, although there had been no vestibular symptoms. Although hair cells were absent, the intercellular junctional complex at the endolymphatic surface of the organ of Corti was maintained, suggesting “healing” by migration or proliferation of supporting cells to fill the empty spaces. Severe atrophy of the stria vascularis was present, and crystalline deposits were found within the strial remnant. An encapsulated tectorial membrane was interpreted in light of ultrastructural findings as either herniation of the fibrillar connective tissue of the spiral limbus into the scala media or alternatively demonstrating abnormal continuity between the connective tissue of the spiral limbus and the remnant of tectorial membrane. Neural degeneration was severe in all cochlear turns. However, more nerve fibers were seen in the proximal portion of Rosenthal's canal than at the habenula perforata or in the organ of Corti, and central processes from the ganglion area were more numerous than peripheral processes. The ganglion cell population was reduced to approximately 10% of normal, and only unmyelinated cell bodies were found. Few recognizable neural fibers were seen within the organ of Corti, although bizarre collections of apparent cellular debris and cell processes containing many mitochondria or vesicles were seen in regions normally containing neural fibers.

Several significant histopathological findings were noted in a case of sudden hearing loss in a patient with chronic lymphocytic leukemia. The major pathological findings were leukemic hemorrhage into both perilymphatic and endolymphatic spaces in the cochlear and vestibular systems, endolymphatic hydrops in the cochlea and sacculus, and a relatively narrowed and straightened vestibular aqueduct and endolymphatic sac. Additional interesting findings include: loss of hair cells in the organ of Corti and vestibular end-organs; destruction of the stria vascularis (possibly the origin of the blood); fibrosis in the perilymphatic spaces in the cochlea and the vestibule, and in the endolymphatic space in the vestibule; and new bone formation in the perilymphatic spaces in the vestibule. The leukemic infiltrate observed in both the cochlea and the vestibule was not considered to be significant. Hemorrhage into the cochlea is thought to be the most reasonable cause of the sudden hearing loss in this case. Also discussed are fibrosis and osteogenesis as a late consequence of hemorrhage, and the coexistence of endolymphatic hydrops with an anomaly of the vestibular aqueduct and endolymphatic sac.

A total of 32 otosclerotic stapes is thin-sectioned without decalcification and examined using transmission and scanning electron microscopes, with a nondispersive x-ray analyzer attached to the latter. These otosclerotic stapes are classified as spongiotic, sclerotic, or preotosclerotic, according to their pathologic characteristics and state of mineralization. Either diffuse or patchy demineralization in the ground substance appears to be the initial stage of otosclerosis, and this area coincides with preotosclerotic lesions (also known as blue mantle) in light microscopy. Therefore, it is interpreted that demineralization precedes the destruction of ground substance in the preotosclerotic lesion. Bone mineral deposits in new otosclerotic bone appear to be related to the collagen fibrils that are embedded in the ground substance. No mineral deposit could be seen without the ground substance deposition; therefore, it is suggested that this ground substance is the single most important factor in the poor mineralization of the otosclerosis. The sclerotic lesions are well mineralized and show a typical pattern of hydroxyapatite by x-ray diffraction study. We could not confirm the notion that the sclerotic lesion is hypermineralized as compared to the normal stapes. The spongiotic lesions are poorly mineralized, with low calcium salt. Using the Ca/P ratio and x-ray diffraction pattern as criteria, it was determined that spongiotic lesions belong to unstable, immature bone.

We report our postmortem findings in the temporal bones of a patient who, at the age of 13, after having had a cold, experienced a sudden and profound hearing loss in her right ear. She died of nasopharyngeal carcinoma at 41. The patient's right temporal bone showed a complete loss of cochlear neurons, but the organ of Corti was well-preserved. These findings indicate a primary degeneration of the cochlear nerve. The authors speculated that the possible cause of this sudden deafness was severe neuronitis of the cochlear nerve.

The pathogenesis of Bell's palsy is presented as retrograde epineurial compression edema with ischemia of the facial nerve. Although the etiology is unknown, an attractive theory is vasospasm, from any cause, along any facial nerve branch, with the chorda tympani, perhaps, the usual primary involvement. Retrograde vascular distension and edema, within the epineurium of the bony facial canal, compresses the nerve from outside its perineurial sheath. The compression force may be mild or severe, resulting in varying degrees of reversible or irreversible ischemic degeneration of myelin sheaths and axons, with varying degrees of cellular reaction to myelin breakdown. The edema may be resorbed, leaving reversible or irreversible nerve damage, or may stimulate collagen formation within the epineurium, with persisting fibrous compression (entrapment) neuropathy of the facial nerve. This concept is consistent with the varying results of Bell's palsy, and depends on the severity and duration of edema, and whether fibrosis occurs within the epineurium of the facial canal. Epineurial fibrosis also results in disturbance of metabolic exchange through the epineurial-permeurial-endoneurial tissues, and may ultimately result in obliteration of vascular drainage. Two temporal bone cases of Bell's palsy, one occurring ten years before death, with residual paralysis. and one two years before death, with clinical recovery, are added to the previously described four cases in the literature, three of early Bell's palsy, and one of remote palsy with almost complete recovery.

Eighty patients with marginal velopharyngeal competency received Teflon® injections to improve closure of the nasopharyngeal port. Sixty percent of these experienced no further voice problem and an additional 18% exhibited improved voice quality.