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Beginning in the late 20th century, implantable hearing aids were developed and used as an alternative for individuals who were unable to tolerate conventional hearing aids. Since that time, several devices have been developed, with four currently remaining on the international market (Med‐el Vibrant Soundbridge, Envoy Esteem, Ototronix MAXUM, and Cochlear Carina). This review will briefly examine the history of middle ear implant development, describe current available devices, evaluate the benefits and limits of the technology, and consider the future directions of research in the field of implantable hearing aids.

Previous genetic studies on susceptibility to otitis media and airway infections have focused on immune pathways acting within the local mucosal epithelium, and outside of allergic rhinitis and asthma, limited studies exist on the overlaps at the gene, pathway or network level between the upper and lower airways. In this report, we compared [1] pathways identified from network analysis using genes derived from published genome-wide family-based and association studies for otitis media, sinusitis, and lung phenotypes, to [2] pathways identified using differentially expressed genes from RNA-sequence data from lower airway, sinus, and middle ear tissues, in particular cholesteatoma tissue compared to middle ear mucosa. For otitis media, a large number of genes (n = 1,806) were identified as differentially expressed between cholesteatoma and middle ear mucosa, which in turn led to the identification of 68 pathways that are enriched in cholesteatoma. Two differentially expressed genes CR1 and SAA1 overlap in middle ear, sinus, and lower airway samples and are potentially novel genes for otitis media susceptibility. In addition, 56 genes were differentially expressed in both tissues from the middle ear and either sinus or lower airways. Pathways that are common in upper and lower airway diseases, whether from published DNA studies or from our RNA-sequencing analyses, include chromatin organization/remodeling, endocytosis, immune system process, protein folding, and viral process. Taken together, our findings from genetic susceptibility and differential tissue expression studies support the hypothesis that the unified airway theory wherein the upper and lower respiratory tracts act as an integrated unit also applies to infectious and nonallergic airway epithelial disease. Our results may be used as reference for identification of genes or pathways that are relevant to upper and lower airways, whether common across sites, or unique to each disease.

This study examined the safety and efficacy of a fully implantable active middle ear (AMEI) system. Outcome measures assessed AMEI performance compared with an optimally fitted conventional hearing aid (CHA). Fifty adults with stable, symmetric moderate-to-severe sensorineural hearing loss were implanted at 9 ambulatory settings. Consonant-Nucleus-Consonant (CNC) words, Bamford-Kowel-Bench Speech in Noise test (BKB-SIN), Abbreviated Profile of Hearing Aid Benefit (APHAB), and unaided hearing thresholds in the implanted ear were compared to baseline measures obtained using a personal CHA. Changes in thresholds were observed from pre- to 12-month postoperative assessments. CNC word scores decreased (within 10%), and the BKB-SIN showed no change from pre- to 12-month postoperative time points. The APHAB revealed improvement. Findings suggest no difference in performance between an appropriately fit CHA and the AMEI at 12 months. This study indicates AMEIs have the potential to help individuals who choose not to use CHAs.

HYPOTHESIS: Simulated otitis media with effusion reduces intracochlear pressures comparable to umbo velocity. BACKGROUND: Otitis media with effusion is a common cause of temporary hearing loss, particularly in children, that can produce conductive deficits of 30–40 dB. Previous studies have measured the effects of simulated effusion on ossicular mechanics; however, no studies have measured cochlear stimulation directly during effusion. Here, we compare pressures in the scala vestibuli and tympani to umbo velocity, before and after induction of simulated effusion in cadaveric human specimens. METHODS: Eight cadaveric, hemi-cephalic human heads were prepared with complete mastoidectomies. Intracochlear pressures were measured with fiber optic pressure probes, and umbo velocity measured via laser Doppler vibrometry (LDV). Stimuli were pure tones (0.1 – 14 kHz) presented in the ear canal via a custom speculum modified to accept speaker and microphone tubing, and sealed with a glass cover slip. Effusion was simulated by filling the mastoid cavity and middle ear space with water. RESULTS: Acoustic stimulation with middle ear effusion resulted in decreased umbo velocity up to ~26 dB, whereas differential pressure at the base of the cochlea, the driving force to the basilar membrane, decreased by only ~16 db. CONCLUSION: Simulating effusion leads to a frequency-dependent reduction in intracochlear sound pressure levels consistent with audiological presentation and prior reports. Results reveal that intracochlear pressure measurements (P(SV) & P(ST)) decrease less than expected, and less than the decrease observed in the differential pressure (P(Diff)). The observed decrease in umbo velocity is greater than in the differential intracochlear pressures, suggesting that umbo velocity overestimates the induced conductive hearing loss. These results suggest that an alternate sound conduction pathway transmits sound to the inner ear during effusion.

Mechanical stimulation through a cochlear third window into the scala tympani in a chinchilla model with normal and fixed stapes can generate cochlear responses equivalent to acoustic stimuli.Cochlear stimulation via the round window (RW) using active middle ear implants (AMEIs) can produce physiologic responses similar to acoustic stimulation including in a model of stapes fixation. However, pathologic conditions, such as advanced otosclerosis, can preclude delivery of sound energy to the cochlea through the oval window and/or the RW.Cochlear microphonic (CM) and laser Doppler vibrometer measurements of stapes and RW velocities were performed in 6 ears of 4 chinchillas. Baseline measurements to acoustic sinusoidal stimuli (0.25-8 kHz) were made. Measurements were repeated with an AMEI driving the RW or a third window to the scala tympani before and after stapes fixation.AMEI stimulation of the third window produced CM waveforms with morphologies similar to acoustic stimuli. CM thresholds with RW and third-window stimulation were frequency dependent but ranged from 0.25 to 10 and 0.5 to 40 mV, respectively. Stapes fixation, confirmed by laser Doppler vibrometer measurements, resulted in a significant frequency dependent impairment in CM thresholds up to 13 dB (at3 kHz) for RW stimulation and a nonsignificant frequency-dependent improvement of up to 10 dB (at3 kHz) via third-window stimulation.AMEI mechanical stimulation through a third window into the scala tympani produces physiologic responses nearly identical to acoustic stimulation including in a model of stapes fixation with decreased efficiency.

HYPOTHESIS: Mechanical stimulation of the round window (RW) with an active middle ear implant (AMEI) with and without experimentally induced stapes fixation (SF) results in equivalent electrophysiologic measures of cochlear microphonic (CM), compound action potential (CAP), and auditory brainstem response (ABR). BACKGROUND: Where normal oval window functionality is mitigated, the RW provides a pathway to mechanically stimulate the inner ear. METHODS: Measurements of the CM, CAP, and ABR were made in 5 ears of 4 chinchillas with acoustic stimulation and with application of the AMEI to the RW with and without experimentally induced SF using pure-tone stimuli (0.25-20 kHz) presented at differing intensities (-20 to 80 dB SPL vs. 0.01 mV to 3.16 V). RESULTS: Morphologies of the CM, CAP, and ABR were similar between acoustic and RW stimulation with and without SF. Stapes fixation increased CM thresholds relative to RW stimulation without fixation by a frequency-dependent 4- to 13-dB mV (mean, 7.9 +/- 3.2 dB mV). Although the thresholds changed with SF, CM sensitivities and amplitude dynamic range were identical to normal. The CAP in all conditions demonstrated equivalent decreasing amplitudes and increasing latency with decreasing intensity (decibel sound pressure level versus decibel millivolt). Stapes fixation increased the CAP thresholds at all frequencies, ranging from 9 to 24 dB mV (mean, 17.7 +/- 4.9 dB mV). Auditory brainstem response waveforms were preserved across experimental conditions. CONCLUSION: Mechanical stimulation of the RW in an animal model of SF generates functionally similar inputs to the cochlea as normal acoustic and RW mechanical inputs but with increased thresholds. With further study, AMEIs may provide a surgical option for correction of otosclerosis and ossicular chain disruption.

Objectives The safety of the Otologics Fully-Implantable MET Ossicular Stimulator was assessed in adult patients with bilateral moderate to severe sensorineural hearing loss. Methods Surgical implantation of the ossicular stimulator was performed. A repeated-measure, within-subjects design assessed safety and aided sound field thresholds and speech performances with the subject’s own, appropriately fitted, walk-in hearing aid(s) and the Otologics Fully-Implantable MET Ossicular Stimulator. Results Twenty patients were implanted and activated as part of the Phase I clinical trial. Results demonstrated 10-20 dB of functional gain across audiometric frequencies. Pure tone averages and monaural word recognition scores were slightly better for the walk-in−aided condition, while patient benefit scales favored the postoperative implant-aided conditions. Conclusion/Significance Although monaural word scores and aided thresholds favored the walk-in−aided condition, preliminary results indicate that the Otologics MET Fully-Implantable Ossicular Stimulator is an alternative to currently available hearing aids in patients with sensorineural hearing loss.

Cavernous hemangiomas are rare lesions that may be difficult to distinguish from acoustic neuromas, schwannomas, or other lesions of the cerebellopontine angle on imaging studies. The clinical history, workup including radiologic evaluation, histologic assessment, and a brief review of the literature are presented.

This study was conducted to demonstrate the safety and efficacy of the Otologics Middle Ear Transducer (MET) Ossicular Stimulator and, in particular, to compare the audiologic benefits of this novel form of electromechanical stimulation with those of conventional acoustical amplification.A total of 282 patients were implanted with the device in Europe and the USA. Pure-tone audiometry, speech recognition and subjective assessment of benefit were tested before the surgery and 2, 3, 6 and 12 months afterwards. The US patients were fitted with a digital hearing aid for a minimum of 4 weeks prior to surgery, and the same benefit measures were performed with the digital hearing aid and their "walk-in" hearing aid.Group mean postoperative bone and air conduction thresholds did not change significantly from preoperative levels. Postoperative air conduction thresholds decreased slightly in some individual patients, due to the mass loading effect exerted by the transducer on the ossicles. Sufficient gain was available to reach target prescription levels for moderate to severely impaired hearing individuals. Speech and subjective assessment of patient preference indicated that patients did as well or better with the MET Ossicular Stimulator than with their "walk-in" aid or the standardized digital aid.The capability of the MET Ossicular Stimulator to provide appropriate gain as a function of degree of hearing loss indicates that the device is a viable treatment for moderate to severe sensorineural hearing loss in adults.

Objectives/Hypothesis: Delayed-onset facial paralysis (beginning more than 3 d after the procedure) has been described as a complication of many different types of otological procedures. The incidence of this problem in vestibular neurectomy and the relationship to surgical approach are detailed in the study. Study Design: Retrospective case review. Methods: In the setting of a tertiary referral center, vestibular neurectomy was performed in 70 individuals with disabling vertigo unresponsive to medical therapy who elected vestibular neurectomy. The main outcome measure was incidence of delayed onset facial paralysis. Results: Delayed-onset facial paralysis was significantly more common after the middle fossa (18%) and translabyrinthine (11%) approaches compared with the retrosigmoid approach (0%) Conclusions: Surgical approach influences the incidence of delayed-onset facial paralysis. Measures to prevent this complication such as prophylactic antiviral medication or labyrinthine segment decompression may be considered in middle fossa and translabyrinthine operations.

Coeliac disease is caused by T cell sensitisation of the intestine to cereal prolamins, which results in a range of mucosal abnormalities that may lead to malabsorption.1 The population prevalence in western countries is ∼1 in 200.2–4 Evidence for an inherited predisposition to coeliac disease comes from studies of first degree relatives of patients and studies of twins.5,6 A strong association is seen between coeliac disease and the HLA-DQ (α1*05, β1*02) heterodimer (DQ2) which is present in approximately 95% of patients,7–9 compared with 20-30% of healthy subjects.10,11 The difference in concordance rates between monozygotic twins and HLA identical sibs (80-100% v 25%) implicates non-HLA genes in the genetic predisposition to coeliac disease.11 The overall relative risk in sibs is at least 20 and is therefore four-fold higher than that attributable to HLA alone under model of inheritance.7 Genome linkage searches carried out on Irish,12 Italian,13, and UK14 coeliac disease families have identified a number of potential sites for the location of non-HLA linked genes. The putative candidate loci detected in the three studies are, however, largely inconsistent and the findings have not been replicated in other populations.15–19 Here we report the results of a genome screen of 24 multiplex families with coeliac disease and discuss the findings of this study in relation to previously published analyses. Twenty-four families with two or more members affected with coeliac disease were recruited for this study (fig 1). Nine of these families (Nos 1, 3, 4, 6, 30, 32, 39, 43, and 44) have been used in a previous study of candidate regions.16 All the families were of northern European ancestry. Twelve of the families were recruited from the UK, nine from Sweden, two from Switzerland, …

In order to determine which variables influence recovery from acoustic neuroma resection, acutely postoperative patients were divided into two groups and treated twice daily at bedside as inpatients at a tertiary care center. Treatment times were increased gradually from 5 to 30 min. Exercise subjects underwent passive and active head movements and walking with augmented head movements. Control subjects received attention without exercise. Pre- and post-tests included vertigo intensity and frequency, low-frequency vestibulo-ocular reflex (VOR), posturography and path integration. Post-tests were given at discharge and at approximately 4-week intervals for 3 months. Multilevel statistical analyses showed that neither group assignment nor age affected the outcome. All measures were abnormal at discharge but improved towards normal within 3 weeks. VOR on the unoperated side returned to normal values but on the lesioned side did not. Therefore, compensation is influenced by tumor size but not by age or early postoperative vestibular rehabilitation. Most compensation occurs within 3 weeks, probably due to central mechanisms, although some measures may not recover completely.

The sinus tympani (ST) is a critical anatomic region of the temporal bone. It lies medial to the facial nerve, between the ponticulus and the subiculum, and therefore is not easily visualized by routine surgical approaches to the middle ear and mastoid. This limited access makes the ST a site that is notorious for residual cholesteatoma. An extensive evaluation of the anatomic dimensions of the ST was made from human temporal bones. Three hundred twenty-seven bones were examined at four standardized levels to describe the dimensions and anatomic relationships of the ST with other structures of the temporal bone. The region of the stapedial tendon was found to be the most limited anatomic substructure in the vicinity of the ST. This study demonstrates the feasibility of a retrofacial approach to the ST as an aid in eradication of otherwise hidden disease.

Objective: The purpose of this study was to determine the level of disability among patients with Meniere's disease, information needed by clinicians when evaluating patients for coverage under the Americans With Disabilities Act. We hypothesized that the unpredictability of vertiginous episodes or "Meniere's attacks" would be the most disabling problem, combined with the lack of a safe place to sit down during Meniere's attacks. Design: All patients seen in the otolaryngology faculty practice at Baylor College of Medicine, Houston, Tex, and diagnosed as having Meniere's disease over a 3-year period were sent a self-administered questionnaire. Setting: This practice is located at a tertiary care center. Subjects: One hundred forty-nine potential subjects constituted the pool, from which data from 50 subjects provided complete data sets. Results: The data from 50 adults suggested that the most problematic symptom was vertigo, followed by hearing loss. The unpredictability of Meniere's attacks and the lack of a safe place to rest during attacks was a significant problem for few subjects. (Arch Otolaryngol Head Neck Surg. 1995;121:29-33)

Because of the diminishing number of patients diagnosed with otosclerosis, the adequacy of residency training in stapedectomy techniques remains controversial. A prospective study, conducted from 1986 to 1991, assessed whether or not surgical outcome obtained by residents, under close supervision by otologic faculty, could be improved using a single stapedectomy technique. Comparison of pre- and postoperative hearing results (pure-tone averages of 500, 1000, and 2000 Hz) from 49 cases revealed closure of the air-bone gap to within 10 dB in 68 percent of procedures. Complications included failure to improve the conductive hearing loss, tympanic membrane perforations, transient facial nerve weakness, subluxation of the incus, and adhesions. The failure to improve the success rate in resident-performed procedures relates to the individual learning curve and the limited number of training cases.

HYPOTHESIS Driving the oval window directly with an active middle ear implant (AMEI) can produce high levels of input to the inner ear. BACKGROUND Treatment of otosclerosis bypasses the stapes with a piston that penetrates the vestibule. Although this treats the conductive component of hearing loss, it does not treat the sensorineural part, which can be improved using an additional conventional hearing aid. Active middle ear implants have been proposed to be an alternative in treating otosclerosis in cases of mixed hearing losses. METHODS Seven temporal bones were prepared to expose the stapes and round window (RW). Stapes and RW velocities were measured while driving with an AMEI the stapes head with a bell-shaped tip. The stapes footplate was then fixed with acrylic cement; fixation was confirmed through attenuated RW velocities. A cylinder tip (0.5 mm) was then used to drive the inner ear through a stapedotomy with and without interposition of fascia. RESULTS Driving the stapes with an AMEI produced mean maximum equivalent ear canal sound pressure levels (SPL) of 138 dB (0.25-8 kHz at 1 V [RMS]). Stapes fixation caused a approximately 25-dB attenuation. Driving with a cylinder tip through the stapedotomy produced 114 dB SPL (24 dB less than normal) and 110 dB SPL (28 dB less than normal) performance with and without fascia, respectively. Performance with fascia was greater than without. CONCLUSION Driving the oval window with an AMEI in a scenario of stapes fixation was demonstrated to be feasible, with performance comparable to traditional AMEI coupling to the incus or stapes. These possibilities offer new perspectives to treat mixed hearing loss in case of fixed footplate.

• The temporal bone histopathologic findings of a patient having received a Nucleus 22-channel cochlear implant (Cochlear Corp, Englewood, Colo) following bilateral transverse otic capsule fractures is presented. The case was complicated by perilymph fistulas, labyrinthitis, meningitis, and death due to drowning. The left cochlea revealed complete labyrinthitis ossificans; the implanted right cochlea demonstrated new bone formation in the basal turn of the scala tympani, degeneration of the organ of Corti, but normal ganglion cell counts. Results of psychophysical and speech perception 4 months after implantation are presented. ( Arch Otolaryngol Head Neck Surg. 1992;118:1257-1265)

Objective To analyze test-retest reliability of vestibular evoked myogenic potential (VEMP) responses with and without the use of electromyography (EMG) monitoring in people with normal audiovestibular function. Patients Twenty adult volunteers with no history of ear disease, normal otoscopic examination, normal pure-tone audiometry thresholds, and normal tympanograms. Interventions Prospective evaluation of VEMP responses with and without the use of EMG monitoring in 2 separate sessions 1 to 4 weeks apart. Main outcome measures Threshold repeatability, p13 and n23 latency, p13-n23 interlatency, and interamplitude and interaural amplitude difference from the first and the second sessions were assessed via the intraclass correlation coefficient. Results Test-retest reliability of p13-n23 interamplitude was found to be excellent, and the reliability of threshold and latency was found to be fair to good (with the exception of poor reliability for p13 latency in the EMG monitoring condition). Conclusion Overall, VEMP response parameters were found to have fair to good test-retest reliability. The intraclass correlation coefficient value for amplitude was found to be more reliable than latency, with the latency of n23 more reliable than the latency of p13. Clinicians should consider these findings when interpreting VEMP responses. Maintenance of symmetric head rotation with and without EMG monitoring produced reliably reproducible results, the VEMP amplitude being the best criteria.

OBJECTIVE To assess the safety of the Otologics fully implantable hearing system after 1 year of use in a Phase I clinical trial. STUDY DESIGN Repeated-measures within-subjects design. SETTING Procedures were performed in a variety of facilities, including a university, military, and private hospital's ambulatory surgical center and outpatient clinical audiologic test facilities. PATIENTS Adult patients with bilateral moderate to severe sensorineural hearing loss. INTERVENTION(S) Surgical insertion of this prosthesis included an atticotomy to expose the incus, securing the transducer to the mastoid bone, attaching the transducer tip to the incus via insertion into a laser-drilled hole, and postauricular implantation of the microphone/battery/electronics capsule. MAIN OUTCOME MEASURE(S) Subjective patient benefit, aided sound field thresholds, and speech discrimination with the subject's own, appropriately fit, walk-in hearing aid(s) and the prosthesis were assessed. RESULTS There were no pre-post-implant differences noted for bone conduction: slight differences were noted in the pre-post-implant air conduction results (p < 0.05). These differences were attributed to the healing process and reversed to almost preimplant assessment levels by the third-month evaluation. Pure-tone averages and monaural word recognition scores were slightly better for the walk-in-aided condition (p < 0.05), whereas the patient benefit scales favored the postoperative implant-aided conditions.Adverse effects of the implant were encountered on 14 occasions after the implantation of the 20 subjects. With the exception of partial device extrusions (that occurred later), all were rectified by the time of initial activation. At the 12-month data collection point, problems that had been encountered by subjects included 1) partial device extrusion (3 subjects), necessitating explantation in 2; 2) loss of external communication (2 subjects), resulting in 1 explantation; and 3) increased charging times beyond 1.5 hours (7), resulting in 3 explantations and 2 patients not using their device while awaiting explantation. CONCLUSION Phase I trial results provide evidence that this fully implantable device can provide sound amplification to sensorineural hearing loss patients, with performance results similar to the patients' walk-in hearing aids.

Objective: The goal was to measure quality of life (QOL) and hearing-specific functional status before and after treatment of conductive hearing loss (CHL). Study Design and Setting: A prospective, longitudinal, outcomes-based study was performed at an academic medical center. Results: Mean baseline QOL scores in CHL patients were comparable with those of the general population. Audiometric studies indicated significant improvements in hearing threshold in all treated patients. There was no significant improvement in mean global QOL scores after treatment of CHL, although there was little room for improvement over baseline. The hearing-specific instrument did demonstrate significant improvements in emotional and social/situational hearing status after treatment. Patients treated with hearing aids had poorer baseline QOL and hearing status than patients treated with surgery and tended to show declines in QOL and only partial improvement in hearing-specific functional status after treatment. Conclusions: Treatment of CHL resulted in improvements in hearing-specific functional status, although changes were difficult to detect with a global QOL instrument. (Otolaryngol Head Neck Surg 2000;123:527-3.)

Balance disorders in elderly patients are associated with an increased risk of falls but are often difficult to diagnose because of comorbid chronic medical problems. We performed a cross-sectional study to determine the prevalence of unrecognized benign paroxysmal positional vertigo (BPPV) and associated lifestyle sequelae in a public, inner-city geriatric population. Dizziness was found in 61% of patients, whereas balance disorders were found in 77% of patients. Nine percent were found to have unrecognized BPPV. Multivariate analysis demonstrated that the presence of a spinning sensation and the absence of a lightheadedness sensation predicted the presence of unrecognized BPPV. Patients with unrecognized BPPV were more likely to have reduced activities of daily living scores, to have sustained a fall in the previous 3 months, and to have depression. These data indicate that unrecognized BPPV is common within the elderly population and has associated morbidity. Further prospective studies are warranted.

Iron deficiency anemia is a frequently occurring clinical disorder. Despite the suggested association with hearing loss in the literature, cochlear sequelae of iron deficiency have yielded conflicting results in experimental studies. Auditory function was tested in iron-deficient and normal male Wistar albino rats using distortion product otoacoustic emissions and auditory brainstem response audiometry for the clarification of the opposing results in the literature. Hemoglobin, hematocrit, serum iron and albumin levels were monitored to verify iron deficiency. Although dramatic differences in weight gain and blood test parameters were noted, no significant change in auditory function due to iron deficiency was detected.

Transverse fractures often violate the otic capsule and commonly, although not invariably, result in immediate and profound sensorineural hearing loss. The pathogenic mechanisms producing sensorineural hearing loss include direct trauma to the cochlear nerve, disruption of the membranous labyrinth, vascular compromise or hemorrhage into the inner ear, perilymphatic fistula, and endolymphatic hydrops. The last two mechanisms account for the occasional patient who develops progressive sensorineural hearing loss after the initial insult. On early posttraumatic evaluation three patients were discovered to have transverse fractures and residual auditory function. Deterioration of hearing led to middle-ear exploration with closure of perilymphatic fistulas. Hearing was preserved in two patients. Diagnosis of posttraumatic perilymphatic fistulas requires early audiometric assessment, high-resolution computed tomographic scanning of the temporal bones to detect fracture of the otic capsule and pneumolabyrinth, and a high index of clinical suspicion. (Arch Otolaryngol Head Neck Surg. 1995;121:795-799)

Temporal bone fractures often cause loss of audiovestibular function. Those patients with bilateral profound sensorineural hearing losses secondary to temporal bone fractures become candidates for cochlear implantation. The authors present the histopathology of five temporal bone fractures in three patients, evaluating specifically the traumatic effects on the neural elements of the inner ear. Transverse fractures of the temporal bone result in severe loss of hair cells, ganglion cells, and other supporting cells in the inner ear. Occasionally labyrinthitis ossificans may occur as a consequence of trauma or infection. While longitudinal fractures do not violate the otic capsule, these same neural elements may be damaged by concussion.

The frequency of gunshot injuries is escalating in American urban centers; consequently, the otolaryngologist is confronting increasing numbers of penetrating injuries to the head and neck. The incidence of temporal bone involvement in penetrating head injuries approximates 20% [1], facial nerve paralysis develops in over 50% of these cases [4]. The damage to the intratemporal facial nerve creates major challenges to the restoration of neural continuity and function. The following retrospective study reviews recent experience with this unique form of facial nerve trauma.

(Editorial comment: Invasive mycotic infection of the inner ear is rare and is almost invariably associated with an immune disorder. Radical mastoidectomy and high-dose amphotericin B apparently irradicated the disease in this patient with acquired immunodeficiency syndrome.] Invasive fungal infections have become increasingly common and represent a situation in which ubiquitous fungal species become aggressive opportunistic pathogens in immunocompromised hosts. Invasive aspergillosis is most frequently encountered in patients with lymphoproliferative disorders. Additional risk factors include prolonged courses of antibiotics, cytotoxic chemotherapy, steroids, neoplasia, organ transplantation, and disorders of cell-mediated and humoral immunity.lPzV3 Manifestation of invasive aspergillosis in the head and neck are infrequent and generally limited to the paranasal sinuses.* Involvement of the inner ear is rare, and to the authors’ knowledge, this represents the first report of invasive aspergillosis confined to the temporal bone presenting with acute facial paralysis and sensorineural hearing loss.

• Currently, the diagnosis of otosyphilis applies to any patient with otologic symptoms of unknown cause and a positive fluorescent treponemal antibody—absorption (FTA-ABS) test result. However, the FTA-ABS assay can have false-positive results and does not distinguish between active and treated disease. The VDRL test, which has been used to distinguish inactive disease, frequently yields negative results in patients who are nonetheless given the diagnosis of otosyphilis. A new Western blot assay can eliminate the possibility of a false-positive result and can confirm whether the infection is active. This assay was used to diagnose unsuspected active syphilis in a patient believed to have autoimmune ear disease. In three other cases of suspected otosyphilis, the diagnosis was refuted when the Western blot demonstrated that the FTA-ABS result was false-positive or that the infection was inactive. ( Arch Otolaryngol Head Neck Surg . 1990;116:617-621)

Lateral surgical approaches to the base of the skull through the temporal bone often result in a large cavity with exposed dura and vascular structures and no possibility of reconstruction of the middle ear conductive hearing mechanism. Subtotal petrosectomy with tympanomastoid obliteration provides a relatively safe and secure closure of the surgical defect in the temporal bone and eliminates the problems associated with an open mastoid cavity. Eradication of all accessible air cell tracts and mucosa in the petrous pyramid, obliteration of the eustachian tubal orifice, closure of the external auditory canal, and fat obliteration of the middle ear and mastoid clefts are essential in the procedure. Over the last 10 years this technique has been utilized in 372 base of skull procedures with a complication rate of less than 5%. Infection occurred only in those cases with draining cavities or contaminated wounds.

• Since a previous study conducted at the Ear, Nose, and Throat Department, University Hospital, in Zurich, Switzerland, 16 additional cases of glomus jugulare of the temporal region have been evaluated and treated surgically in this department. This article describes our experience with these additional cases and gives an in-depth description of the surgical techniques that are used in the treatment of more complicated tumors. We report one case in detail. ( Arch Otolaryngol 1981;107:209-214)

The carbon dioxide (CO2) laser has been advocated for removal of diseased tissue from the middle ear cleft. The effect of CO2 electromagnetic radiation on mucosa and the otic capsule, however, is thermal, and the dissipation of heat into tissue surrounding the area of impact is potentially deleterious to normal structures. A CO2 laser with a 150-microns spot size at a focal length of 300 mm and a power output ranging from 0.25 to 1.5 watts was used to vaporize the mucosa overlying the horizontal portion of the fallopian canal with pulses of 0.2- to 0.5-second duration. Damage to the facial nerve was directly related to laser pulse energy and was most pronounced adjacent to the area of impact but spread distally and proximally at higher energy levels because of the heat conductivity of the bony fallopian canal. Facial movement was an unreliable indicator of injury during the vaporization process. Because the application of electromagnetic energy over the fallopian canal can result in facial paralysis, a knowledge of facial nerve anatomy remains a prerequisite to the safe extirpation of diseased tissue in the middle ear cleft.

Primary tumors of the facial nerve are relatively rare and have a variety of presenting symptoms. This article reviews 248 cases of facial neuroma in the world's literature and adds seven cases that were managed at the Baylor College of Medicine. Facial weakness was most common symptom, with facial spasm or tics, hearing loss, and masses in the external auditory canal also being frequently seen. At surgery, the tympanic, vertical, and labyrinthine segments were the most commonly involved areas. On the basis of this review, general principles have been drawn up to help the clinician in diagnosis and management of this (at times) difficult problem.

Pathologic changes in the temporal bone are described in a case of polyarteritis nodosa in a 48 year old man in whom the onset of sudden unilateral deafness and vertigo occurred seven months prior to death as one of the early manifestations of the disease. The patient had received only a seven week course of prednisolone and, earlier, a two week course of anti-inflammatory agents. Autopsy revealed involvement of the arteries supplying the kidneys, testes, and pancreas. Changes within the temporal bone were seen bilaterally and there was thickening of the mucosa of the middle ear. Inner ear involvement was mainly limited to the cochlea, the deaf ear showing more pronounced changes. These changes included loss of the organ of Corti in the hook portion of the basal coil, absence of the tectorial membrane, and atrophy of the stria vascularis. The scala tympani was obliterated by fibrosis and new bone formation. The scala media showed hydrops, and a marked decrease in the spiral ganglion cells and nerve fibers supplying this portion of the cochlea was evident. Focal changes were seen throughout the remainder of the cochlea. The vestibular structures showed no detectable pathologic changes. Small vessel arteritis was found in the dural and subarcuate vessels in both temporal bones.

The diminishing number of cases of otosclerosis limits the training experience in stapes surgery during residency. Results are suboptimal. Closure of the preoperative air conduction to within 10 dB of bone conduction was obtained in only 64 % of resident cases Efforts to maximize experience and improve results include closer faculty supervision, use of one stapedectomy technique, and a prospective study of individual residents' performance.

Eosinophilic granuloma is a benign lytic lesion of bone, probably arising from an abnormal proliferation of histiocytes. It may occur within the temporal bone and be confused with more common disorders such as aural polyps or chronic suppurative otitis media. If untreated, the disease may destroy the bony labyrinth and spread to the middle and posterior cranial fossas. Temporal bone lesions may represent only one manifestation of a more extensive multifocal disorder (i.e., Hand-Schüller-Christian or Letterer-Siwe disease). From 1967 to 1982, 25 patients with eosinophilic granuloma of the skull were treated in the radiation therapy department of The Methodist Hospital in Houston. Six of these patients had disease involving the temporal bone. After analyzing these cases and reviewing the literature, we conclude that the management of the patient with eosinophilic granuloma in the temporal bone requires a diagnosis based on microscopic examination of the affected tissue, a definition of the extent of disease, therapy consisting of biopsy and low-dosage radiotherapy in the unifocal disease, and careful and continued follow-up.

• Small-fenestra stapedotomy has recently been popularized in efforts to reduce the incidence of sensorineural hearing loss following stapes surgery for otospongiosis. Lasers have been advocated as a tool to fenestrate the stapes footplate. Conversion of radiant energy from the laser into heat in the vestibule represents the greatest potential risk to the inner ear. Using a carbon dioxide laser with a focal point of 150 μm at 300 mm, fenestrations of the stapes footplate were performed in a series of 14 anesthetized cats. The laser power output ranged from 0.47 to 3.05 W, with pulse durations of 0.2 and 0.5 s. Resultant temperature elevations in the vestibule, measured by a thin-wire thermocouple, ranged from 0 to 4.4 °C (0 to 8 °F) and directly correlated to wattage and duration of the laser-beam pulse. Temperature changes could be reduced by use of lower wattage, shorter pulse duration, timed intervals between pulses, and convection cooling of the promontory. (Arch Otolaryngol1985;111:601-605)

Summary Osteoblastomas of the head region are rare lesions. This paper describes a case of a 4-year-old child with an osteoblastoma of the temporal bone presenting as a facial paralysis. Only 6 previous cases have been reported and none with nerve involvement. A review of the tumor, its differential diagnosis, clinical course and management is presented.

Epidermoids are blastomatous malformations of abnormal epithelial proliferation. Intradiploic epidermoids are very rare lesions that may occur in all flat bones of the calvarium and in the paranasal sinuses, maxilla, temporal bone, and sphenoid bone. This article describes the unique case of an intradiploic epidermoid tumor located in the greater sphenoid wing. Theories of pathogenesis, clinical behavior, diagnosis, and treatment of diploic epidermoids are reviewed.

• A volumetric study on the tympanic isthmus and its relationship to temporal bone pneumatization was conducted in 110 serially sectioned temporal bones with and without middle ear pathology. With a microcomputer and digitizing tablet, the area of the tympanic isthmus and the degree of pneumatization in sample sections were calculated. Nonpathologic temporal bones showed a significant correlation between the volume of the tympanic isthmus and the degree of pneumatization. The narrowest area of the tympanic isthmus and the degree of pneumatization were also highly correlated. The result indicated a definite inhibition of pneumatization due to chronic middle ear infection when compared with nonpathologic temporal bones. ( Arch Otolaryngol Head Neck Surg 1987;113:401-404)

• While auditory involvement in multiple sclerosis is not infrequent, sensorineural hearing loss as the initial manifestation of this disease is rare. Auditory brain-stem evoked responses, speech audiometry, acoustic reflexes, and electronystagmography yield significant objective information concerning brain-stem involvement. Magnetic resonance imaging objectively documents white matter demyelinization. The acute onset of the retrocochlear type of auditory dysfunction without other evidence of central nervous system involvement can be an early manifestation of this disease. Two patients with multiple sclerosis had sudden sensorineural hearing loss, with one patient experiencing this as the sole initial complaint. ( Arch Otolaryngol Head Neck Surg 1989;115:41-45)

Tuberculous osteomyelitis of the temporal bone is a rare and dangerous entity that should be included in the differential diagnoses of infectious processes of the base of skull. A 21-year-old man presented with petrous apicitis, extradural and retromandibular abscesses, and paresis of the facial nerve. Immediate middle fossa craniotomy and drainage of the extradural abscess, in combination with a mastoidectomy, incision and drainage of the facial abscess, and antimicrobial therapy for gram-positive cocci, failed to check the destructive nature of the infection. The patient subsequently developed labyrinthitis, sensorineural hearing loss, and meningitis. Intraoperative biopsy specimens confirmed the presence of tuberculoid granulomas, and the infectious process responded to triple-drug therapy. Tuberculosis of the temporal bone should not always be considered an indolent infection. The management of tuberculous infection of the temporal bone is outlined.

• A 10-year-old boy developed a porencephalic cyst of the mastoid in conjunction with otitic meningitis and cerebrospinal otorrhea. A defect of the tegmen tympani allowed extrusion of the cyst into the antrum and the mesotympanum. The otorrhea was controlled by closure of the defect. Our review of 251 cases in the literature showed no prior incidence of mastoid involvement by a porencephalic cyst. ( Arch Otolaryngol 102:563-565, 1976)

• Forty-eight patients with Meniere's disease underwent psychological assessment with the Minnesota Multiphasic Personality Inventory and Diagnostic Inventory of Personality and Symptoms. Using the presence or absence of recurrent vertigo or chronic dysequilibrium as the differentiating feature, the results of two groups were compared. A clinical diagnosis of depression (axis I) was evident in 80% of the patients with active vestibular symptoms examined by the Minnesota Multiphasic Personality Inventory and 70% by the Diagnostic Inventory of Personality and Symptoms compared with 32% (Minnesota Multiphasic Personality Inventory) and 39% (Diagnostic Inventory of Personality and Symptoms) in the inactive group. The data support the addition of depression to the clinical picture of active Meniere's disease. ( Arch Otolaryngol Head Neck Surg . 1989;115:1355-1357)

The argon laser has been used clinically to perform stapedotomies in patients with otosclerosis. In order to determine if this device induces histologic changes in the inner ear, stapedotomies were performed in cats. Saccular membrane fistulas, in line with the path of the laser beam, were found in 3 of 8 ears. The unpredictable nature of the argon laser in the ear is discussed.

The vestibular and optokinetic ocular control systems were studied in 10 patients with cerebellar atrophy and in 10 normal subjects using (1) constant velocity optokinetic stimulation, (2) sinusoidal rotation in the dark, and (3) sinusoidal rotation in the light with a surrounding fixed optokinetic drum. The gain (maximum slow component velocity/maximum head or drum velocity) of induced nystagmus was calculated from electro-oculographic recordings. Optokinetic nystagmus was abnormal in seven patients and the average optokinetic gain in the patients was significantly (p less than 0.01) less than that of the normal group. Three patients with "clinically pure" cerebellar atrophy had increased vestibular responses, and one patient with clinical signs of peripheral neuropathy had decreased responses, probably due to associated vestibular nerve disease. The average vestibulo-ocular reflex gain in patients did not differ significantly from controls (p greater than 0.05). Three patients had normal vestibular and optokinetic responses when tested independently, but had abnormal visual-vestibular interaction. These patients probably had selective disorders of the midline cerebellar pathways that mediate visual-vestibular interaction. By studying each system, both independently and during interaction, all patients were identified as abnormal, and a more precise anatomic localization of the atrophy was obtained.

• Autoimmune sensorineural hearing loss was first described in 1979. This may be secondary to a systemic autoimmune disease, or in some cases, to a direct autoimmune insult to the labyrinth. A case of active ulcerative colitis and progressive sensorineural hearing loss responded to corticosteroid therapy. ( Arch Otolaryngol 1984;110:810-812)

The potential usefulness of impedance studies is reviewed and a case of osteogenesis imperfecta, having bilateral ossicular discontinuity and intact acoustic reflexes, is presented. This seeming paradox was explainable on the basis of surgically confirmed, bilateral disruption of the stapes crura, central to the stapedial tendon insertion.

A series of 26 patients undergoing translabyrinthine acoustic neuroma surgery was evaluated pre- and postoperatively using rotatory vestibular testing. Patients were tested immediately prior to surgery and followed long-term at frequent intervals for periods of one week to two years, depending on availability and length of time postoperative. Rotatory testing was performed at multiple frequencies of 0.0125, 0.05, and 0.2 Hz, and the vestibulo-ocular reflex was measured using electro-oculographic techniques. Dependent variables of the slow component of the vestibulo-ocular reflex were derived by computer analysis, including gain, phase, DC bias, ampullopetal and ampullofugal responses of the remaining labyrinth, and asymmetry. These variables changed in a predictable fashion as related to the reciprocal of time. Results of this data are fitted with a linear statistical model, validating the changes.