The future of otology.

BACKGROUND: The field of otology is increasingly at the forefront of innovation in science and medicine. The inner ear, one of the most challenging systems to study, has been rendered much more open to inquiry by recent developments in research methodology. Promising advances of potential clinical impact have occurred in recent years in biological fields such as auditory genetics, ototoxic chemoprevention and organ of Corti regeneration. The interface of the ear with digital technology to remediate hearing loss, or as a consumer device within an intelligent ecosystem of connected devices, is receiving enormous creative energy. Automation and artificial intelligence can enhance otological medical and surgical practice. Otology is poised to enter a new renaissance period, in which many previously untreatable ear diseases will yield to newly introduced therapies. OBJECTIVE: This paper speculates on the direction otology will take in the coming decades. CONCLUSION: Making predictions about the future of otology is a risky endeavour. If the predictions are found wanting, it will likely be because of unforeseen revolutionary methods.

Signed, sealed and delivered: "big tobacco" in Hollywood, 1927-1951.

OBJECTIVE: Smoking in movies is associated with adolescent and young adult smoking initiation. Public health efforts to eliminate smoking from films accessible to youth have been countered by defenders of the status quo, who associate tobacco imagery in "classic" movies with artistry and nostalgia. The present work explores the mutually beneficial commercial collaborations between the tobacco companies and major motion picture studios from the late 1920s through the 1940s. METHODS: Cigarette endorsement contracts with Hollywood stars and movie studios were obtained from internal tobacco industry documents at the University of California, San Francisco (UCSF) Legacy Tobacco Documents Library and the Jackler advertising collection at Stanford. RESULTS: Cigarette advertising campaigns that included Hollywood endorsements appeared from 1927 to 1951, with major activity in 1931-2 and 1937-8 for American Tobacco Company's Lucky Strike, and in the late 1940s for Liggett & Myers' Chesterfield. Endorsement contracts and communication between American Tobacco and movie stars and studios explicitly reveal the cross-promotional value of the campaigns. American Tobacco paid movie stars who endorsed Lucky Strike cigarettes US$218,750 in 1937-8 (equivalent to US$3.2 million in 2008) for their testimonials. CONCLUSIONS: Hollywood endorsements in cigarette advertising afforded motion picture studios nationwide publicity supported by the tobacco industry's multimillion US dollar advertising budgets. Cross-promotion was the incentive that led to a synergistic relationship between the US tobacco and motion picture industries, whose artefacts, including "classic" films with smoking and glamorous publicity images with cigarettes, continue to perpetuate public tolerance of onscreen smoking. Market-based disincentives within the film industry may be a solution to decouple the historical association between Hollywood films and cigarettes.

Neurofibromatosis 2 and malignant mesothelioma.

Mutations of the neurofibromatosis 2 (NF2) tumor suppressor gene cause the inherited disorder NF2 and are also common in malignant mesothelioma, which is not a characteristic feature of NF2. The authors report an asbestos-exposed person with NF2 and malignant mesothelioma. Immunohistochemical analysis of the mesothelioma confirmed loss of expression of the NF2 protein, and comparative genomic hybridization revealed losses of chromosomes 14, 15, and 22, and gain of 7. The authors propose that a person with a constitutional mutation of an NF2 allele is more susceptible to mesothelioma.

Contrast-enhanced MR imaging of the facial nerve in 11 patients with Bell's palsy.

Contrast-enhanced MR images (at 1.5 T) were obtained in 11 patients with facial palsy. The group included five people with acute idiopathic facial (Bell's) palsy, three with chronic idiopathic facial palsy, and one each with acute facial palsy after local radiation therapy, acute facial palsy resulting from herpes zoster virus infection, and facial palsy caused by facial neuroma. Eight of the 11 patients demonstrated marked enhancement of the affected facial nerve from the labyrinthine portion through the descending canal. Three patients also demonstrated mild enhancement of the distal canalicular portion of the facial nerve, simulating small distal acoustic neuromas. No difference in the pattern of enhancement between the acute or chronic Bell's palsy patients was seen. Radiographic resolution appeared to lag behind clinical resolution. The facial neuroma appeared distinct from the other lesions as a focally enhancing mass. The enhancement pattern in the Bell's group correlated with the histopathologic features of Bell's palsy and is consistent with the viral hypothesis of the syndrome. Thin-section contrast-enhanced MR scans are recommended for individuals with atypical presentation of facial paralysis. In the proper clinical setting, contrast-enhanced MR imaging may permit a positive radiographic diagnosis of Bell's palsy, which has previously been a diagnosis of exclusion.

MR imaging in two cases of subacute denervation change in the muscles of facial expression.

SUMMARY: Denervation changes in muscle following damage to cranial and peripheral nerves can be observed on both CT and MR imaging studies. These findings are well described for cranial nerves (CN) V, X, XI, and XII. The CT findings of denervation atrophy due to CN VII dysfunction have been reported. We describe the MR imaging findings in two patients with perineural spread of tumor along CN VII. Both patients showed T2 prolongation and postcontrast enhancement in muscles of facial expression, suggestive of subacute denervation changes.

Hearing preservation in patients undergoing vestibular schwannoma surgery: comparison of middle fossa and retrosigmoid approaches.

OBJECT: The goal of this retrospective study was to evaluate hearing preservation after surgery for vestibular schwannoma in which the middle fossa (MF) or retrosigmoid (RS) approaches were used. Hearing preservation in vestibular schwannoma surgery can be achieved by using either the MR or RS approach. Comparative outcome data between these approaches are lacking, and, as a result, selection has generally been determined by the surgeon's preference. METHODS: The authors have compared removal of small vestibular schwannomas via MF and RS approaches with regard to hearing preservation and facial nerve function. The study group was composed of consecutively treated patients with vestibular schwannoma, 48 of whom underwent operation via an MF approach and 50 of whom underwent the same number of RS operations. Tumors were divided into size-matched groups. Hearing results were recorded according to the American Academy of Otolaryngology-Head and Neck Surgery criteria, and facial nerve outcome was recorded as the House-Brackmann grade. Overall, 26 (52%) of the patients treated via the MF approach achieved a Class B or better hearing result compared with seven (14%) of the RS group. Some hearing was preserved in 32 (64%) of the patients in the MF group and in 17 (34%) of the RS group. The results obtained by using the MF approach were superior for intracanalicular tumors (p=0.009, t-test), and for tumors with a cerebellopontine angle (CPA) component measuring 0.1 to 1 cm (p=0.006, t-test). For tumors in the CPA that were 1.1 to 2 cm in size, our data were inconclusive because of the small sample size. Facial weakness was seen more frequently after MF surgery in the early postoperative period, but results were equal at 1 year. CONCLUSIONS: The results of this study have demonstrated a more favorable hearing outcome for patients with intracanalicular tumors and tumors extending up to 1 cm into the CPA that were removed via the MF when compared with the RS approach.

Bilateral temporal bone encephaloceles after cranial irradiation. Case report.

Irradiation of the central nervous system may cause significant morbidity, including endocrine dysfunction and intellectual impairment. The authors report a case of bilateral temporal bone encephaloceles in a 21-year-old man who had received prophylactic central nervous system irradiation for acute lymphocytic leukemia in early childhood. Endaural encephaloceles are uncommon, and most occur as a complication of mastoid surgery. The etiology, clinical features, radiological diagnosis, and surgical treatment of temporal bone encephaloceles are discussed.

Anterior epitympanic cholesteatoma with facial paralysis: a characteristic growth pattern.

Epitympanic cholesteatoma may involve the facial nerve at several anatomic locations resulting in functional impairment. The most common site of nerve compression is the middle-ear segment where the nerve is frequently devoid of bony covering. In five patients with facial palsy due to epitympanic cholesteatoma, a characteristic pattern of growth was recognized in which the cholesteatoma traversed the anterior epitympanum rather than taking the more common posterior route. Extension anteromedial to the head of the malleus leads to compression of the facial nerve in the region of the geniculate ganglion at the level of the middle cranial fossa floor. The most probable pathogenesis of this lesion is extension of disease along the embryologic course of either the saccus anticus or the anterior saccule of the saccus medius. Management of these lesions is surgical and may require, in addition to mastoidectomy with anterior and/or posterior atticotomy, middle fossa craniotomy and/or partial removal of the labyrinth for complete excision.

Exophytic brain tumors mimicking primary lesions of the cerebellopontine angle.

The vast majority of cerebellopontine angle (CPA) tumors are extraaxial masses arising from either the eighth nerve (acoustic neuroma) or meninges (meningioma). Rarely, a tumor that arises from the brain parenchyma may protrude laterally to present with a clinical and radiographic picture simulating that of the much more common extraaxial lesions. Three individuals with CPA lesions that ultimately proved at operation to be exophytic brain tumors (pontine medulloblastoma, cerebellar astrocytoma, and fourth ventricular ependymoma) are described. The clinical manifestations of these lesions, although not entirely typical, fell well within the possible range of presentation of benign tumors primary to the CPA. In two of these cases the tumor actually penetrated into the internal auditory canal. As the optimal management strategy for treating parenchymal tumors differs substantially from that for extraaxial lesions, it is essential that the surgeon have a preoperative awareness of the lesion's nature before embarking on a surgical endeavor. Critical to arriving at the correct diagnosis is the close examination of preoperative imaging studies. The clinician should be alerted to the possibility that a CPA tumor is of intraaxial origin when the preoperative magnetic resonance imaging scan shows 1) blurring of the margin between the tumor and brainstem or cerebellum; 2) a degree of peritumoral hyperintensity on T2-weighted scans disproportionate to the size of the extraaxial mass; and 3) dilation of the lateral recess of the fourth ventricle.

Lipoid meningitis due to aseptic necrosis of a free fat graft placed during neurotologic surgery.

We present two unusual cases of aseptic postoperative lipoid meningitis resulting from necrosis of a free fat graft placed into a skull base craniotomy defect. Two patients underwent translabyrinthine resection of acoustic neuroma and received abdominal fat grafts to ablate the surgical defect. Both patients presented postoperatively with persistent cerebrospinal fluid (CSF) wound leak and severe headache. Computed tomography demonstrated hydrocephalus and widely dispersed intracranial fat droplets throughout the CSF circulation. Both patients ultimately required CSF diversion for management of persistent communicating hydrocephalus. The clinical and radiographic correlates of lipocephalus and lipoid meningitis are presented, and a review of free fat graft physiology is discussed.

Role of the mastoid in tympanic membrane reconstruction.

Otologists have long debated the importance of the mastoid in determining the success or failure of tympanic membrane reconstruction. The pneumatic spaces within the mastoid represent an "air reservoir" which can be drawn upon during periods of eustachian tube dysfunction and buffer the middle ear against the development of detrimental negative pressures. Mastoid inflammatory disease, if untreated, may result in recurrent suppuration and graft failure. Small mastoid volume, aside from its well known association with chronic infectious middle ear disease, has been shown to effect adversely graft survival following myringoplasty. In 48 patients undergoing myringoplasty with simple mastoidectomy, neither small mastoid size nor inflammatory mastoid disease significantly decreased the rate of graft healing. This suggests that simple mastoidectomy is an effective means of repneumatizing the mastoid and eradicating mastoid sources of infection. The successful surgical creation of a pneumatized mastoid cavity in communication with the middle ear was confirmed by postoperative computerized tomographic (CT) scans. In failed cases, CT scanning predictably identified residual mastoid disease. Simple mastoidectomy is considered to be a safe and useful adjunct to myringoplasty in selected cases of chronic otitis media with perforation.

Atypical hearing loss in acoustic neuroma patients.

Audiologic data from 126 patients treated at the University of California, San Francisco for newly diagnosed acoustic neuromas (ANs) from 1986 to 1990 were reviewed. Subjectively normal hearing was present in 15% of patients and was most frequent in patients with small (< 1 cm) tumors. Only 4% had objectively normal hearing on the basis of speech reception threshold (SRT), speech discrimination score (SDS), and high-frequency pure-tone loss. This was most frequent in patients with < 1 cm tumors. Abnormal but symmetrical hearing is usually not considered to be indicative of a unilateral AN. In the present series, 7% of patients with ANs possessed symmetrical hearing. High-frequency asymmetry was a more sensitive indicator of the presence of an AN than differences in either SRT or SDS. The clinician must be aware of the relatively high incidence of atypical audiologic findings in acoustic tumor patients.

The large vestibular aqueduct syndrome.

It has long been known that abnormally large vestibular aqueducts may accompany congenital malformations of the cochlea and semicircular canals. Recently, enlargement of the vestibular aqueducts as the sole radiographically detectable inner ear anomaly has been recognized as a distinct pattern of congenital inner ear malformation. Pathogenesis of the large vestibular aqueduct syndrome probably stems from an early derangement in the embryogenesis of the endolymphatic duct. This anomaly appears to be relatively common in children with sensorineural hearing loss and is probably significantly underdiagnosed. Hearing loss is typically bilateral and progressive, with stepwise rather than fluctuant hearing decrements often triggered by relatively minor head trauma. A review of 17 patients (33 ears) revealed an average hearing level at presentation of 57 dB with a speech discrimination score of 66%. Considerable variability exists in hearing level among affected ears, ranging from normal hearing (4%) to profound deafness (39%). In 12 patients (23 ears) with an average long-term follow-up of 7.3 years, the hearing loss progressed by an average of 25 dB, with a drop of 29% in speech discrimination over the period of observation. An endolymphatic to subarachnoid shunt was performed on seven ears in an effort to stabilize hearing. Four of these ears had a substantial immediate postoperative drop in hearing. For this reason, endolymphatic sac surgery is not recommended for patients with this deformity.

Sound detection with the cochlear implant in five ears of four children with congenital malformations of the cochlea.

To determine if the cochlear implant can enable sound detection in children with a congenitally deformed cochlea, we reviewed warble-tone thresholds in five ears of four children in the implant clinical trials program. Of the five ears, there were two common cavity deformities, two cochlear hypoplasias, and one incomplete partition. Four of the five ears had an auditory response to stimulation by the implant at the same level as ears deafened by other disorders. One ear that had an auditory response to stimulation also produced facial stimulation that precluded use of the implant. The one ear that did not stimulate was the incomplete partition. This ear had a very narrow internal auditory canal. Three patients are now using the implant in three ears to detect sound. Results show that a cochlear implant may enable sound detection in a patient with a malformed cochlea but that a very narrow internal auditory canal (less than 1.5 mm) detected preoperatively on radiographs may contraindicate an implant. Such anatomy suggests only a rudimentary audiovestibular nerve or no such nerve and only a facial nerve.

Congenital malformations of the inner ear: histologic findings in five temporal bones.

The histopathologic findings in five temporal bones from three patients with congenital malformation of the inner ear are described. The external contour of the cochlea was deformed in two temporal bones, while the internal architecture was abnormal in all five temporal bones. Intracochlear abnormalities included defects in the interscalar septum, deficiencies in the modiolus, and a paucity of neural elements. Spiral ganglion cell populations varied, but were substantially diminished in all five temporal bones. Enlargement of the vestibule and semicircular canals (lateral and posterior) was seen in two temporal bones. A large vestibular aqueduct and saccular hydrops were found in one temporal bone each. An attempt was made to relate the histologic patterns of deformity to the proposed clinical classification of inner ear malformations. Radiographic abnormality of the cochlea would have been detectable in two bones (incomplete partition pattern), while two other bones would have appeared as vestibule-semicircular canal syndromes. One bone would have been radiographically normal.

Congenital malformations of the inner ear: a classification based on embryogenesis.

Approximately 20% of patients with congenital sensorineural hearing loss have radiographic abnormalities of the inner ear. A broad spectrum of anomalous patterns have been described, most of which have been lumped together under the term "Mondini's dysplasia." We feel that this grouping of many dissimilar entities under a single umbrella term is unwarranted. Based on a review of 63 patients with 98 congenitally malformed ears, we have been able to recognize a number of distinct anatomic patterns from their radiographic appearance. A remarkable similarity between these morphologies and the appearance of the inner ear at various stages of embryogenesis was found. This led us to propose a classification system based upon the theory that these deformities result from an arrest of development during varying stages of inner ear organogenesis.

Stapedectomy: long-term hearing results.

The initial improvement in hearing following stapedectomy usually deteriorates with the passage of time. We studied the long-term results of stapedectomies performed on 42 patients (49 ears) between 1959 and 1969 who had a minimum follow-up of 18 years. Both air conduction (AC) and bone conduction (BC) thresholds progressively deteriorated over the long term. The degree of BC loss paralleled that expected from presbycusis alone. A greater deterioration was noted in the AC levels, producing a recurrent conductive hearing loss in the speech frequencies. Age at the time of surgery had no effect on the long-term outcome. Comparison of the average preoperative speech discrimination scores (SDS) to the 1-year postoperative SDS and the long-term SDS revealed a 1.1% and 16.7% drop, respectively. The improvement in the average speech reception threshold (SRT) obtained 1 year postoperatively deteriorated by less than 1 dB per year over the long term. Patients with a higher SDS (more than 95%) preoperatively fared better in the maintenance of speech discrimination than those with a lower SDS (less than 95%). The preoperative SRT level was predictive of the timing for the requirement of hearing amplification. The postoperative SRT level was predictive of the timing for the requirement of hearing amplification. The caused by presbycusis, combined with a recurrent conductive loss in the speech frequencies rather than cochlear otosclerosis. Although the decline in hearing following stapedectomy exceeds the rate of hearing loss due to presbycusis, many individuals, after successful stapes surgery, are able to delay the need for hearing amplification for longer periods than had been previously reported.

Computed tomography in suppurative ear disease: a correlation of surgical and radiographic findings.

Forty-two patients with chronic otitis media underwent preoperative CT scanning followed by surgical exploration of the middle ear and mastoid. The CT finding of abnormal soft tissue density associated with bone erosion was highly correlated with the surgical finding of cholesteatoma. By contrast, the total absence of abnormal soft tissue on CT essentially excluded cholesteatoma. However, 50% of all patients had abnormal soft tissue on CT scan not accompanied by bone erosion. In this largest group of patients it was not possible to diagnose or exclude cholesteatoma on the basis of CT findings alone. Also, CT occasionally gave the erroneous impression of lateral semicircular canal fistulization, tegmen tympani erosion, and facial nerve involvement due to volume averaging of these structures with adjacent soft tissues. CT scan has a role in the evaluation of selected patients with chronic otitis media, but must be interpreted cautiously in view of its limitations and numerous pitfalls.

The changing clinical presentation of acoustic tumors in the MRI era.

The application of magnetic resonance imaging (MRI) scanning in the diagnosis of acoustic neuroma (AN) has increased the relative incidence of smaller tumors and has impacted on the typical clinical presentation of AN patients. The charts of 126 patients treated at the University of California, San Francisco for newly diagnosed AN from 1986 to 1990 were reviewed. Twenty-four percent of tumors fell into the smallest size category (< 1 cm); this was a substantial improvement over earlier series. However, 16% of tumors remained undiagnosed until they achieved large size (> 3 cm). The incidence of hearing loss, dysequilibrium, headache, facial numbness, and diplopia all increased with increasing tumor size, while the incidence of vertigo decreased. Diagnosticians should not overemphasize "typical" symptom complexes, as substantial variability in clinical manifestations exists. An improved awareness by clinicians of the variability of AN presentation will improve diagnostic efficiency and continue the trend toward earlier diagnosis of these lesions.

Surgical considerations and hearing results with the UCSF/Storz cochlear implant.

Sixteen patients have been implanted with the UCSF/Storz multichannel implant, 11 of whom have been fitted with their external speech processors and transmitters and administered postoperative audiological evaluations. Both the surgical procedures used and the hearing results for these patients are presented. The potential medical/surgical complications of implant surgery and the future direction of research and development within the UCSF/Storz implant program are discussed.