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Otitis media (OM), a very common disease in young children, can result in hearing loss. In order to potentially replicate previously reported associations between OM and PLG, exome and Sanger sequencing, RNA-sequencing of saliva and middle ear samples, 16S rRNA sequencing, molecular modeling, and statistical analyses including transmission disequilibrium tests (TDT) were performed in a multi-ethnic cohort of 718 families and simplex cases with OM. We identified four rare PLG variants c.112A > G (p.Lys38Glu), c.782G > A (p.Arg261His), c.1481C > T (p.Ala494Val) and c.2045 T > A (p.Ile682Asn), and one common variant c.1414G > A (p.Asp472Asn). However TDT analyses for these PLG variants did not demonstrate association with OM in 314 families. Additionally PLG expression is very low or absent in normal or diseased middle ear in mouse and human, and salivary expression and microbial α-diversity were non-significant in c.1414G > A (p.Asp472Asn) carriers. Based on molecular modeling, the novel rare variants particularly c.782G > A (p.Arg261His) and c.2045 T > A (p.Ile682Asn) were predicted to affect protein structure. Exploration of other potential disease mechanisms will help elucidate how PLG contributes to OM susceptibility in humans. Our results underline the importance of following up findings from genome-wide association through replication studies, preferably using multi-omic datasets.

A genetic basis for otitis media is established, however, the role of rare variants in disease etiology is largely unknown. Previously a duplication variant within A2ML1 was identified as a significant risk factor for otitis media in an indigenous Filipino population and in US children. In this report exome and Sanger sequencing was performed using DNA samples from the indigenous Filipino population, Filipino cochlear implantees, US probands, Finnish, and Pakistani families with otitis media. Sixteen novel, damaging A2ML1 variants identified in otitis media patients were rare or low-frequency in population-matched controls. In the indigenous population, both gingivitis and A2ML1 variants including the known duplication variant and the novel splice variant c.4061 + 1 G>C were independently associated with otitis media. Sequencing of salivary RNA samples from indigenous Filipinos demonstrated lower A2ML1 expression according to the carriage of A2ML1 variants. Sequencing of additional salivary RNA samples from US patients with otitis media revealed differentially expressed genes that are highly correlated with A2ML1 expression levels. In particular, RND3 is upregulated in both A2ML1 variant carriers and high-A2ML1 expressors. These findings support a role for A2ML1 in keratinocyte differentiation within the middle ear as part of otitis media pathology and the potential application of ROCK inhibition in otitis media.

Hypothesis: A cochlear implant electrode within the cochlea contributes to the air-bone gap (ABG) component of postoperative changes in residual hearing after electrode insertion. Background: Preservation of residual hearing after cochlear implantation has gained importance as simultaneous electric-acoustic stimulation allows for improved speech outcomes. Postoperative loss of residual hearing has previously been attributed to sensorineural changes; however, presence of increased postoperative ABG remains unexplained and could result in part from altered cochlear mechanics. Here, we sought to investigate changes to these mechanics via intracochlear pressure measurements before and after electrode implantation to quantify the contribution to postoperative ABG. Methods: Human cadaveric heads were implanted with titanium fixtures for bone conduction transducers. Velocities of stapes capitulum and cochlear promontory between the two windows were measured using single-axis laser Doppler vibrometry and fiber-optic sensors measured intracochlear pressures in scala vestibuli and tympani for air- and bone-conducted stimuli before and after cochlear implant electrode insertion through the round window. Results: Intracochlear pressures revealed only slightly reduced responses to air-conducted stimuli consistent with previous literature. No significant changes were noted to bone-conducted stimuli after implantation. Velocities of the stapes capitulum and the cochlear promontory to both stimuli were stable after electrode placement. Conclusion: Presence of a cochlear implant electrode causes alterations in intracochlear sound pressure levels to air, but not bone, conducted stimuli and helps to explain changes in residual hearing noted clinically. These results suggest the possibility of a cochlear conductive component to postoperative changes in hearing sensitivity.

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.

Non-secretor status due tohomozygosity for the commonFUT2 variant c. 461G> A(p. Trp154*) is associated witheither risk for autoimmune diseases or protection against viral diarrhea and HIV. We determined the role of FUT2 in otitis media susceptibility by obtaining DNA samples from 609 multi-ethnic families and simplex case subjectswith otitis media. Exome and Sanger sequencing, linkage analysis, and Fisher exact and transmission disequilibrium tests (TDT) were performed. The common FUT2 c. 604C> T (p. Arg202*) variant co-segregates with otitismedia in a Filipino pedigree (LOD = 4.0). Additionally, a rare variant, c. 412C> T (p. Arg138Cys), is associated with recurrent/ chronic otitismedia in European-American children (p = 1.2310(-5)) and US trios (TDT p = 0.01). The c. 461G> A (p. Trp154*) variant was also overtransmitted in US trios (TDT p = 0.01) and was associated with shifts inmiddle ear microbiota composition (PERMANOVA p 20 were combined, FUT2 variantswere over-transmitted in trios (TDTp = 0.001). Fut2 is transiently upregulated inmouse middle ear after inoculation withnon-typeable Haemophilus influenzae. Four FUT2 variants-namely p. Ala104Val, p. Arg138Cys, p. Trp154*, and p. Arg202*-reduced A antigen in mutant-transfected COS-7 cells, while the nonsense variants also reduced FUT2 protein levels. Common and rare FUT2 variants confer susceptibility to otitis media, likely by modifying the middle ear microbiome through regulation of A antigen levels in epithelial cells. Our familiesdemonstratemarked intra-familial genetic heterogeneity, suggesting thatmultiple combinations of common and rare variants plus environmental factors influence the individual otitis media phenotype as a complex trait.

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.

HYPOTHESIS Bone-anchored hearing systems (BAHSs) provide sound location-dependent input to the normal ear for reducing the head shadow effect in the case of single-sided deafness (SSD). BACKGROUND Patients with SSD can be fit with a BAHS positioned on the impaired side. Despite successful outcomes and some reports of spatial hearing capabilities, little data are available regarding the physiologic performance of BAHSs in response to free-field sounds. METHODS Cochlear microphonics (CMs) were recorded from five chinchillas before and after destruction of one cochlea. A BAHS (Cochlear Baha) was fitted on the deafened side. CM measurements were made in response to tones, with and without the BAHS, to free-field sounds presented ipsilateral to the SSD, on the side of the normal ear, and along the midline. Stimuli were also presented directly through the BAHS and an earphone to generate sounds with interaural time and level differences approximating free-field sounds. RESULTS With the BAHS, CM thresholds were decreased (re: no BAHS) by approximately 10 dB for sources ipsilateral to the SSD, approximately 14 dB for midline sources, and approximately 5 dB for sources contralateral to the SSD. Changes in CM amplitudes and thresholds were sound location dependent. CM amplitudes were modulated by interaural time and level differences generated by the linear interaction of BAHS and acoustic signals. CONCLUSION This study suggests that BAHS can provide input to the normal ear that is modulated by sound location, which serves to reduce the head shadow effect and may also offer cues to sound location.

Long-term retained foreign bodies in the human body have been reported across many specialties, but relatively few exist in the ENT literature.We present a case report of a patient with a broken dental needle fragment in the posterior oral cavity with subsequent migration to the cochlea over the course of 4 years, eventually leading to hearing loss. CT scan and middle ear exploration demonstrated a 4-cm metallic fragment abutting the base of the cochlea, immediately adjacent to the internal carotid artery.The needle segment was removed through an endaural approach without complication.Postoperatively, the patient had improvements in PTA and speech discrimination, as well as the resolution of chronic otalgia and jaw pain. Imaging, audiologic results, and surgical details and pictures are presented herein.To our knowledge, based on a thorough PubMed and Google Scholar search, there are no reports of such a foreign body migration from the oral cavity to the skull base.

Purpose: This paper presents longitudinal case studies of children who received (cochlear implants) CIs and a controlled sample of children with normal hearing (NH). Phoneme discrimination (i.e., /sa-ma/, /a-i/, /a-u/, /u-i/, /ta-da/, /pa-ka/) was assessed prior to receiving CIs and monthly for 3 mo following CI activation. Research Design: Case studies. Study Sample: Three cochlear implant recipients and seven NH control participants were recruited through the University of Colorado Hospital and the University of Colorado, Boulder. Data Collection and Analysis: The study utilized the visual reinforcement audiometry and interactive play assessment of speech pattern contrasts (VRASPAC) algorithm. A comparison of scoring was conducted using Cohen's kappa to determine interrater reliability. Results: Findings from this study revealed that CI recipients could discriminate at least three out of five phoneme contrasts at mastery level (≥90%) by 2 mo of device use. None of the CI recipients reached mastery prior to implantation. Following 3 mo of CI use there was no difference in contrast discrimination performance between the CI users and their NH age-matched peers (with the exception of /pa-ka/ for one CI user). Conclusions: The CI users in this case study, who were implanted between 12 and 16 mo of age, were able to master the phoneme contrasts regardless of bilateral or unilateral CI, socioeconomic status, or language spoken at home.

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.

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, …

Long-term changes in the parameters of the vestibulo-ocular reflex (VOR) were evaluated using recently developed multilevel statistical modelling techniques in a group of patients who had undergone ablative inner ear procedures. Data from 84 patients were available for study. The most dramatic recovery in the VOR occurred during the first 60 postoperative days, however, slow changes continued, levelling off at about 120 days. While frequency was a highly significant predictor of outcomes in all parameters, age showed significant predictive value only to phase measures (p = 0.006) and gender had no effect. Sum gain at the lowest frequency tested remained below the 5th percentile and at the higher frequencies remained below the 50th percentile for normal subjects. Phase decreased exponentially, but remained above the 95th percentile for normal subjects, except at the highest frequency where it remained above the 50th percentile. DC bias decreased exponentially, dropping to the 50th percentile by day 146. Asymmetry stayed between the 50th and 95th percentile, except at the highest frequency, where it was at the 50th percentile by day 125.

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)

To study the effects of various active middle ear implant loading parameters on round window stimulation in an animal model.Physiological measurements of the cochlear microphonic and stapes velocity were made from active middle ear implant-generated sinusoidal stimuli with controlled changes in loading parameters.Prospective study at an academic research institution.Cochlear microphonic and stapes velocities (H(EV)) were measured in 6 study subjects (Chinchilla lanigera) in response to active middle ear implant (Otologics MET, Boulder, Colorado) round window stimulation with assessment of effects of varying parameters of loading pressure, interposed connective tissue, and angle of stimulation with respect to the round window membrane.The measured performance variabilities in repeated applications of the active middle ear implant to the round window were 2.5 dB and 5.0 dB for H(EV) and cochlear microphonic thresholds, respectively. Loading pressure applied to the round window (51-574 dynes) and angle of approach (±30° with respect to coronal plane) did not have a significant effect on cochlear microphonic thresholds or H(EV). Significant improvements in cochlear microphonic thresholds and H(EV) were observed for interposed connective tissue regardless of tissue type.Variability in performance due to repeated couplings of the active middle ear implant to the round window is small and reproducible. Interposition of connective tissue significantly improves vibration energy transfer to the cochlea. Neither changes in loading pressure nor angle of stimulation of the round window affected active middle ear implant performance.

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.

• 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 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.

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.

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.

The sensory hair cells of the inner ear are responsible for converting balance and hearing stimuli into electrical signals. Until recently, all previous studies of hair cell physiology had been performed on tissue obtained from non-mammals and rodents. In primates, hair cells are difficult to access, because they rest within the densest structure of the body, the otic capsule of the temporal bone. In this report, we describe a technique that we have used in physiological studies to harvest living human hair cells. We collected vestibular and cochlear tissue specimens from adult humans undergoing translabyrinthine and transotic surgical approaches for resection of lateral skull base tumors. Viable hair cells were identified and visualized with light microscopy. The ability to study normal hair cells from humans may further the study of normal and pathological human sensation, hair cell regeneration, and genetic causes of balance and hearing disorders.

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.

Summary The goals of this study were to collect normative data on asymptomatic, ambulatory, community-dwelling adults on a standard diagnostic test of vestibular function in balance and to determine if their responses differ significantly from younger adults. Subjects were divided into four age groups, 18-44 years (young), 45-69 years (middle-aged), 70-79 (old), and 80-89 (elderly). Subjects were seen in the neurotologic diagnostic laboratory at a tertiary care facility. Their dynamic balance was tested under a variety of sensory conditions using the EquiTest (NeuroCom), a standard diagnostic test. The data from one subtest, the Sensory Organization Test (SOT) were evaluated. These data showed significant age-associated declines in overall score and changes in movement strategy. These results suggest that those parts of the vestibular system involved with balance have age-related declines through the end of the life span, even in asymptomatic people, and that these changes do not level off but continue into the ninth decade. Therefore, when elderly people are evaluated for balance disorders age-appropriate norms should be used. These results also suggest that declines in motor performance on laboratory tests are not directly related to reduced independence in essential activities of daily living in elderly people.

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)

Background: Pure tones stimulated oscillopsia in a 30-year-old man with bilateral hearing loss and Tullio's phenomenon. Design: The subject's eye movements were recorded with electro-oculography while he was seated with his head stabilized facing a visual array. Pure tones were given in each ear, individually, from 500 to 2000 Hz, 90 to 110 dB. The subject reported the intensity of oscillopsia, if any, during each trial. He was also tested while standing on a force platform and receiving pure tones. Results: No increased postural sway was found, but oscillopsia and vertical eye movements followed stimulation to the more impaired ear. Conclusions: These results support the hypothesis that Tullio's phenomenon is caused by inner ear pathology and suggest that oscillopsia is caused by the occurrence of unplanned vertical eye movements, the perceptual consequences of which were unexpected. (Arch Otolaryngol Head Neck Surg. 1995;121:459-462)

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.

(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.

With advances in imaging, intracanalicular acoustic neuromas are identified readily in patients including, oftentimes, those with serviceable hearing. Fourteen patients were operated on in an attempt to preserve hearing. Hearing was retained in 10 (71%) of the 14. Serviceable hearing was present in 7 (50%), borderline serviceable hearing in 2 (14%), and nonserviceable hearing in 1 (7%). The problems, advantages, and disadvantages of the differing approaches are discussed.

• 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)

• 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)

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.

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.

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)

Suprathreshold morphologic characteristics of the acoustic stapedius reflex are reported for five subjects with acoustic tumor. Results support the hypothesis that changes in suprathreshold morphology may be more sensitive to acoustic tumor than conventional reflex threshold and decay measures. Both the afferent relative amplitude index (AI) and measures of sequential variability of individual waveforms appear to be especially promising.

• 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)

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.

Individual speech tracking data were analyzed in relation to other variables in 11 adult users of the Nucleus multichannel cochlear implant. Patients were categorized according to postoperative speech tracking results. The resultant groups could also be differentiated on the basis of postoperative MAC scores, surgeon's rating of overall success, and duration of deafness before implantation. The speech tracking paradigm appears to be a useful technique for the evaluation of performance with a cochlear prosthesis.

The clinical features of laryngeal abscess have changed markedly since the introduction of antibiotics and are rarely seen by clinicians today. Three cases of acute laryngeal abscess associated with malignancy, airway instrumentation and a preexisting laryngocele respectively are presented. Management of these cases included immediate tracheostomy, intravenous antibiotics and surgical drainage. Current etiological factors and modes of presentation are discussed. Laryngeal abscess remains a serious and potentially lethal problem requiring emergent treatment. Serious sequelae may result from this disease.

• Brachial plexus neoplasms are uncommon. When this diagnosis is suspected, the functional and anatomical integrity of the brachial plexus and cervical spinal cord must be carefully assessed. A thorough search for other signs of neurofibromatosis (von Recklinghausen's disease) must also be completed. The distinction between neurilemoma and neurofibroma is an important and useful one to know. Evaluation by a pathologist who is well versed in neural tumors is mandatory for appropriate treatment of these usually benign lesions. Surgical intervention, adequately prepared on the basis of the patient's age, the amount of neural impairment, and the extent and histology of the tumor requires a surgeon who is experienced in peripheral nerve surgery and in microdissection techniques. Long-term follow-up is necessary to monitor the growth of known tumors, the detection of malignant change, and the appearance of other stigmata of von Recklinghausen's disease. (Arch Otolaryngol103:653-657, 1977)

Concurrent with the increase in handgun crime in the United States is a rising incidence of gunshot wounds to the temporal bone. These injuries present challenging diagnostic and management problems to the otolaryngologist. A timely multicenter review of this entity revealed 22 cases evaluated and treated by the authors between 1975 and 1984. A wide variety of injuries were encountered, involving anatomic structures within or contiguous with the temporal bone. These included: eight intracranial injuries; 11 traumatized facial nerves; seven vascular wounds; 19 mastoid, middle, or inner ear injuries; and 11 cases of damage to the external auditory canals. A rational scheme for immediate and long-term management of each type of injury is outlined. Illustrative cases representing vascular, neural, temporal bone, and central nervous system injury are presented.