Pathogenic Potential of Turicella otitidis and Staphylococcus auricularis: A Case Report.

Objective: Turicella otitidis and Staphylococcus auricularis have been considered normal aural flora. Their significance in active infection is controversial. We examined a series of patients presenting with acute and chronic otitis media whose ear canal culture isolated T. otitidis and S. auricularis and explored possible pathogenicity, associated factors, and outcomes. Methods: This is a retrospective chart review of patients who presented to a tertiary center outpatient clinic between 2017 and 2022 with otologic microscopic examination of active infection and ear canal culture isolating T. otitidis or S. auricularis only. Clinical course was collected including history, microscopic otoscopy findings, interventions given, outcomes, and sensitivity results. Results: A total of 13 patients (10 with T. otitidis and 3 with S. auricularis) were included. Majority of the patients had a history of otologic surgery (92%) and tympanic membrane perforation (62%). All were treated with combinations of antibiotic otic drops (ie, fluoroquinolone, sulfa, or aminoglycoside based) ± oral antibiotics (ie, penicillin or trimethoprim/sulfamethoxazole). Otorrhea resolved among majority of patients. Otorrhea and mucosalization returned or continued among 4 patients. Sensitivity results demonstrated that 2 of 3 strains of T. otitidis were resistant to clindamycin. There was no resistance against S. auricularis for tested antibiotics. Conclusions: Our findings suggest the potential pathogenicity of T. otitidis and S. auricularis, especially among patients with prior ear surgery and tympanic membrane perforation. Violation of the epithelial barrier from surgery or trauma may contribute to their pathogenicity. Future study is warranted to elucidate pathogenicity of normal aural flora and its mechanisms.

Association of Sleep Characteristics with Tinnitus and Hearing Loss.

Objective: The impact of poor sleep on tinnitus has been mainly attributed to central processes. There is an association between sleep disorders and hearing loss, but whether hearing levels mediate the association between sleep disorders and tinnitus is unknown. This study investigates the association between sleep characteristics, tinnitus, and hearing loss. Study Design: Cross-sectional. Setting: National Health and Nutrition Examination Survey (NHANES). Methods: Study cohort includes 9693 adults (≥20 years) from the NHANES 2005 to 2018 who completed audiometric testing and questionnaires on tinnitus and sleep characteristics. Multivariable regression analyses were performed to quantify associations between sleep characteristics, tinnitus, and hearing loss. Results: In this cohort, 29% (95% confidence interval [CI]: 28%-31%) reported trouble sleeping and 9% (95% CI: 8%-10%) reported being diagnosed with sleep disorders. Negative sleep characteristics (less hours of sleep, diagnosis of a sleep disorder, trouble sleeping, or OSA symptoms) were not associated with audiometry-measured hearing loss in multivariable models adjusted for demographics and comorbidities but were significantly associated with bothersome tinnitus. This association remained significant without substantial attenuation in multivariable models additionally adjusting for hearing levels: sleeping <8 h/day (vs ≥8) (odds ratio [OR]: 1.28 [95% CI: 1.08-1.52]), trouble sleeping (OR: 1.78 [95% CI: 1.45-2.19]), diagnosis of sleep disorders (OR: 1.57 [95% CI: 1.14-2.15]), and report of OSA symptoms (OR: 1.42 [95% CI: 1.08-1.88]). Conclusion: Negative sleep characteristics were associated with tinnitus while there was no clinically meaningful association between sleep and hearing loss. Our findings suggest that the relationship between poor sleep and tinnitus is likely contributed by central processes without a major role of mediation via the peripheral auditory system.

Congenital Cytomegalovirus-Associated Sensorineural Hearing Loss in Children: Identification Following Universal Newborn Hearing Screening, Effect of Antiviral Treatment, and Long-Term Hearing Outcomes.

OBJECTIVES: Congenital cytomegalovirus (cCMV) is the most common cause of nongenetic sensorineural hearing loss (SNHL) in children. We examined the longitudinal hearing outcomes of children with cCMV in relation to their newborn hearing screening findings, and their use of antiviral therapy. DESIGN: The study was based on a retrospective chart review using a database of pediatric patients (N = 445) seen at the University of Minnesota Lions clinic. Chart review identified infants with cCMV, and records were reviewed for information about universal newborn hearing screen (UNHS) results, the clinical course of SNHL, and the use of antiviral therapy. RESULTS: A total of 44 children were identified with cCMV. In this group, 33 (75%) had SNHL of varying degree and age at onset. Notably, 17 (39%) children passed UNHS bilaterally. Of those children, 6 (35%) ultimately acquired bilateral or unilateral SNHL, detected at a mean age of 20 months (median age, 12 months). Five out of 10 children (50%) that did not pass UNHS in one ear acquired late-onset hearing loss in the contralateral ear, identified at a mean age of 24 months (median age, 4 months). Eleven (25%) children passed UNHS bilaterally and continued to demonstrate normal hearing in both ears at their most recent follow-up visit at a mean age of 19 months (SD, 18 months). Of the 33 children with cCMV and SNHL, 18 (55%) received antiviral medication (ganciclovir and/or valganciclovir). While, on average, both treated and untreated ears experienced a progression of hearing loss over time, the group that received antiviral treatment experienced less overall hearing change compared with the untreated group (baseline-adjusted expected mean difference, -10.5 dB; 95% confidence interval, -28.1 to 7.2 dB). CONCLUSIONS: Among children with cCMV included in this study who passed UNHS in both ears, 35% demonstrated delayed-onset SNHL. Notably, of those children who referred unilaterally, 50% later demonstrated SNHL in the contralateral ear. These findings have implications for audiological monitoring, and potentially antiviral therapy, of children with cCMV. As implementation of universal cCMV screening moves forward, a key aspect of follow-up will be appropriate long-term audiologic monitoring.

Magnetic Resonancy Imaging Safety in Active Osseointegrated (Osia®) and Cochlear Implants: New Technology Creating Confusion.

OBJECTIVE: There is increased confusion regarding MRI-compatible CIs and BAHAs. This report describes two cases when patients underwent MRIs with non-MRI compatible devices. RESULTS: One patient with bilateral Cochlear Osias experienced dislocation of both internal magnets after 1.5 Tesla MRI. Both magnets were outside the silastic sheath, with the left magnet flipped. A second patient with a legacy CI experienced similar internal magnet dislocation and inversion after 3 Tesla MRI. CONCLUSIONS: This study describes internal magnet dislocation/inversion with the Cochlear Osia and a legacy CI after MRI. Our findings suggest the need for improved patient education and simplified radiology guidelines. Laryngoscope, 134:393-396, 2024.

Neurotology Workforce in the United States: Gender Diversity and Geographic Distribution.

OBJECTIVE: The workforce of neurotology has changed with increasing numbers of accredited programs and diverse representation among trainees over the past several decades. This study aims to describe the characteristics, density, and geographic variation of the current neurotology workforce in the United States. STUDY DESIGN: Cross-sectional study. SETTING: American Board of Otolaryngology-Head and Neck Surgery portal and online search. METHODS: The study cohort included physicians certified in Neurotology by the American Board of Otolaryngology as of 2021 (n = 372). Physician characteristics including years of practice, gender, practice setting, and location were collected. Geographic variation analysis was performed by the state, county, and hospital referral region. Associations between the number of neurotologists per population and socioeconomic characteristics were assessed using multivariable regression analysis. RESULTS: Among 372 neurotologists, 65% practiced in academic settings and 13% were female. The percentage of female neurotologists increased from 0% among neurotologists with ≥30 years of practice to 23% among <10 years of practice. There were no differences in a practice setting by gender. The geographical analysis demonstrated that the average number of neurotologists was 1.1 per 1 million Americans. In a multivariable model, the density of neurotologists was significantly higher within counties with the highest quartiles of college education (ß = .6 [95% confidence interval, CI: 0.3-0.8]) and income (ß = .3 [95% CI: 0.1-0.6]). CONCLUSION: The number of board-certified neurotologists has gradually increased and there have been trends toward greater gender diversity. The geographical distribution of neurotology practice was concentrated in counties with higher socioeconomic status as expected given the referral-based nature of the subspecialty. There should be efforts to reach out to low socioeconomic communities to ensure equivalent access to neurotological care.

Tinnitus Neuroimaging.

This article reviews the use of human neuroimaging for chronic subjective tinnitus. Evidence-based guidance on the clinical use of imaging to identify relevant auditory lesions when evaluating tinnitus patients is given. After introducing the anatomy and imaging modalities most pertinent to the neuroscience of tinnitus, the article reviews tinnitus-associated alterations in key auditory and nonauditory networks in the central nervous system. Emphasis is placed on how these findings support proposed models of tinnitus and how this line of investigation is relevant to practicing clinicians.

Bevacizumab for Hearing Preservation in Neurofibromatosis Type 2: Emphasis on Patient-Reported Outcomes and Toxicities.

OBJECTIVE: Bevacizumab for hearing preservation in patients with neurofibromatosis type 2 (NF2) is an emerging practice. We set out to characterize the effectiveness and toxicity of bevacizumab in our patient group. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Seventeen consecutive patients with NF2 received bevacizumab treatment for vestibular schwannomas, including 2 patients treated to maintain cochlear implant performance. Volumetric analysis of serial magnetic resonance imaging scans was used to evaluate radiographic response, and hearing response was evaluated with serial audiograms. Patient-reported outcomes were also assessed, including subjective hearing improvement, changes in tinnitus, vertigo, headaches, ear pain, and improvement in ability to communicate via telephone. RESULTS: A positive radiographic response occurred in 8 of 17 (47%) patients and the median tumor volume change was a tumor decrease of 19%. A positive hearing response was recorded in 5 of 9 (56%) patients. Two patients had a word recognition score improvement over 40%. There was an approximately 40% improvement in patient-reported outcomes. Primary toxicities included hypertension, proteinuria, dysgeusia, and amenorrhea. CONCLUSION: Bevacizumab treatment was followed by hearing improvement in 56% of patients, while decreased tumor volume was noted in 47%. These outcomes agree favorably with prior reported series. There were significant improvements in patient-reported outcomes that have not been described previously.

Analysis of archived newborn dried blood spots (DBS) identifies congenital cytomegalovirus as a major cause of unexplained pediatric sensorineural hearing loss.

PURPOSE: Congenital cytomegalovirus (cCMV) infection is the most common non-genetic cause of sensorineural hearing loss (SNHL). However, accurate diagnosis of cCMV as the etiology of SNHL is problematic beyond the neonatal period. This study therefore examined whether cCMV infection could be identified retrospectively in children presenting with unexplained SNHL to a multidisciplinary diagnostic outpatient otolaryngology clinic at an academic medical center in Minnesota. METHODS: Over a 4-year period, 57 patients with an age range of 3months to 10years with unexplained SNHL were recruited to participate in this study. Informed consent was obtained to test the archived dried blood spots (DBS) of these patients for cCMV infection by real-time PCR, targeting a highly conserved region of the CMV UL83 gene. Results were normalized to recovery of an NRAS gene control. Chart review was conducted to identify subjects who underwent genetic testing and/or neurodiagnostic imaging to investigate possible genetic, syndromic, or anatomical causes of SNHL. RESULTS: In total, 15 of the 57 children with unexplained SNHL tested positive for CMV DNA in their DBS (26%). A mean viral load of 8.3×104 (±4.1×104) [range, 1×103-6×105] copies/µg DNA was observed in subjects retrospectively diagnosed with cCMV. No statistically significant correlation was found between viral load and SNHL severity. CONCLUSIONS: A retrospective DBS analysis demonstrated that 26% of patients presenting with unexplained SNHL in childhood had cCMV. DBS testing is useful in the retrospective diagnosis of cCMV, and may provide definitive diagnostic information about the etiology of SNHL.

Paraganglioma presenting as cholesterol granuloma of the petrous apex.

We report the unique finding of a petrous apex cholesterol granuloma associated with a paraganglioma, also known as a glomus jugulare tumor, in a 52-year-old woman who presented to our department with pulsatile tinnitus, hearing loss, aural fullness, and disequilibrium. She had been treated for a petrous apex cholesterol granuloma 20 years earlier, at which time she had undergone drainage of the granuloma via subtotal petrous apicectomy. When she came to our facility approximately 20 years later, she had signs and symptoms consistent with a jugular paraganglioma, which was likely to have been present at the time of her initial presentation for the cholesterol granuloma. In fact, microscopic bleeding from the paraganglioma might have led to the formation of the cholesterol granuloma. The metachronous presentation of these two entities, which to our knowledge has not been reported previously in the literature, indicates the potential association of paragangliomas with the formation of cholesterol granulomas of the petrous apex.

Symptom timeline preceding cochlear implant failure: an institutional experience.

OBJECTIVE: Review cochlear explants and determine the incidence of device and medical failures and develop a pattern of symptoms indicating probable implant failure. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Subjects were selected if they underwent cochlear explantation. Data were reviewed to determine initial symptom and symptom timeline preceding implant failure. RESULTS: A total of 847 implants were performed from 1988 to 2008, with 128 devices explanted. Of total implants, 72% were Advanced Bionic devices, and 28% were Cochlear Nucleus devices. Overall failure rate was 128 of 847 (15%), with 51 (6%) medical failures and 77 (9%) device failures. Patients with Advanced Bionic devices underwent 102 explants (16.7% failure rate), with 35% medical failures and 65% device failures. Patients with Nucleus devices underwent 26 explants (11% failure rate), with 58% medical failures and 42% device failures. Medical failures included infected devices and wound dehiscence, with the pattern averaging 4.5 months to explantation. Hard device failures included sudden malfunction and slow decline in function, with pattern of failure over 4.2 months. Soft failures included tinnitus and discomfort, with failure over 8 months. CONCLUSIONS: There were common patterns when evaluating both medical and device failures. By recognizing patterns of symptoms that may indicate probable implant failure, otolaryngologists can better counsel patients on what to expect with their implants when they present with certain symptoms.

Asymmetric sudden sensorineural hearing loss: is all this testing necessary?

OBJECTIVE: A number of factors have been identified that contribute to the presentation of asymmetric sudden sensorineural hearing loss (ASSNHL). Routinely, patients presenting with ASSNHL undergo a battery of serologic testing and imaging in an attempt to determine a cause. The objective of this study was to assess the utility of this diagnostic evaluation in elucidating a cause of an ASSNHL and to assess the average cost associated with this battery of testing. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care facility. SUBJECTS AND METHODS: Charts from patients presenting to the otolaryngology clinic of a tertiary care facility between December 1, 2002, and November 30, 2007, with ASSNHL confirmed by audiometric evaluation were reviewed. Diagnostic tests included in the workup of ASSNHL and test results were recorded. The percentage of positive tests was determined and compared to national data. Cost analysis of the diagnostic battery was performed. RESULTS: The battery of testing performed for sudden sensorineural hearing loss included antineutrophil antibody, cholesterol, creatinine, anti-DNA antibody, erythrocyte sedimentation rate, blood glucose, Lyme titer, rheumatoid factor, rapid plasmin reagent, triiodothyronine, thyroid-stimulating hormone, and magnetic resonance imaging with gadolinium. The average cost associated with the full diagnostic evaluation is greater than $2000. The diagnostic impact of these tests is extremely low. CONCLUSIONS: The utility of the comprehensive ASSNHL evaluation should be reconsidered. The choice of diagnostic evaluation should be directed by patient risk factors and exposures.

A combination antioxidant therapy prevents age-related hearing loss in C57BL/6 mice.

OBJECTIVE: Age-related hearing loss (ARHL) is characterized by gradual, progressive sensorineural hearing loss, which impairs communication, lending to clinical depression and social withdrawal. There are currently no effective treatments for ARHL. The purpose of this study is to evaluate the potential of a combination antioxidant therapy in preventing ARHL. STUDY DESIGN: Randomized controlled trial. SETTING: Animal study. SUBJECTS AND METHODS: C57BL/6 mice, a recognized animal model of ARHL, were assigned to one of three groups: early treatment (n = 12), late treatment (n = 9), or control group (n = 9). Treatment groups of mice were fed with a combination agent comprising six antioxidant agents that target four sites within the oxidative pathway: L-cysteine-glutathione mixed disulfide, ribose-cysteine, NW-nitro-L-arginine methyl ester, vitamin B12, folate, and ascorbic acid. Auditory brainstem response (ABR) thresholds were recorded at baseline and every three months following initiation of treatment. RESULTS: Threshold shifts from baseline were decreased in the treatment groups when compared to the control group at all tested frequencies (P < 0.001). The ABR threshold shift at 12 months of age for the control group was 34.7 dB with a 95% confidence interval (CI) of +/-1.6. The mean threshold shifts for the early and late treatment groups were 7.5 dB (+/-0.87, 95% CI) and 9.2 dB (+/-1.6, 95% CI). CONCLUSION: Combination antioxidant therapy effectively decreased threshold shifts on ABR within an animal model of ARHL. Combination antioxidant therapy, with further research and investigation, may provide a safe and cost-effective method of preventing presbycusis in the growing elderly population.

Cost analysis of asymmetric sensorineural hearing loss investigations.

OBJECTIVES/HYPOTHESIS: The purpose of this study is to critically evaluate the typical cost of asymmetrical sensorineural hearing loss (ASNHL) work-up, and to compare the positive predictive value from this common presenting symptom. STUDY DESIGN: Retrospective chart review from two major otolaryngology centers. METHODS: We reviewed charts from patients presenting to New York Eye and Ear Infirmary between January 1, 2006 and December 31, 2006, and the University of Minnesota between December 1, 2002 and November 30, 2007 with ASNHL. Diagnostic information included magnetic resonance imaging (MRI) and serum laboratory values (antinuclear antibodies, erythrocyte sedimentation rate, Lyme, rapid plasma reagin, and thyroid-stimulating hormone). We calculated positive rate according to each item of diagnosis. To estimate cost-benefit, we further calculated the average cost for identifying a patient with a positive result. RESULTS: The total cost was $263,535, whereas the average cost for identifying a positive patient was $146,40.81. The total lab cost was $16,935 and the total imaging cost was $246,600. The average cost for identifying a positive patient based on MRI was $61,650 and $2,109 based on lab values. Of the 247 patients, only six patients (2.4%)-one patient with acoustic neuroma, two patients with syphilis, and three patients with Lyme--were identified with treatable pathology. CONCLUSIONS: A comprehensive ASNHL work-up may not be applicable to all patients. Laboratory serologic tests are highly cost effective in diagnosing treatable causes of ASNHL, such as syphilis and Lyme. Although radiographic imaging with MRI is not as cost effective, its value in detecting for acoustic neuroma is undeniable.

Sudden hearing loss as the presenting symptom of systemic sclerosis.

OBJECTIVES: Sudden sensorineural hearing loss (SSHNL) is an emergency in otolaryngology. In most cases, the exact cause cannot be identified, but different immunologic disorders and microvascular events have been suggested to play a role in its pathogenesis. Sudden sensorineural hearing loss can be caused by collagen vascular disorders, but it has rarely been reported as the presenting symptom of these diseases. This case presented with bilateral hearing loss and was finally diagnosed as a systemic sclerosis (SSc) patient. This is the first case of SSc that presented with SSNHL as an initial symptom. PATIENT: A 65-year-old man presented with bilateral hearing loss. He also complained of generalized fatigue and intermittent paresthesia. Brain imaging and blood tests were negative except for antinuclear antibodies. Administration of high-dose prednisone did not make any improvement. After extensive workup, follow-up, and referral to rheumatologist, the diagnosis of SSc was made. The patient's hearing improved after receiving intravenous immunoglobulin along with other symptoms of the disease. CONCLUSION: This case illustrates the importance of follow-up and appropriate of SSNHL patients with other systemic symptoms.

Evaluation of the short hybrid electrode in human temporal bones.

HYPOTHESIS: The current hybrid electrode can be inserted without trauma to the temporal bone and, after insertion, assumes a position within the scala tympani near the outer cochlear wall just beneath the basilar membrane. BACKGROUND: Conservation of residual hearing after cochlear implant electrode insertion requires a special insertion technique and an atraumatic short electrode. This allows electroacoustic stimulation in ears with significant residual hearing. METHODS: Human cadaveric temporal bones were implanted with soft surgical technique under fluoroscopic observation. Dehydrated and resin-impregnated bones are dissected. Real-time electrode insertion behavior and electrode position were evaluated. The bones are examined for evidence of insertion-related trauma. RESULTS: No gross trauma was observed in the implanted bones, and the electrode dynamics evaluation revealed smooth scala tympani insertions. CONCLUSION: Atraumatic insertion of the 10-mm hybrid electrode can be accomplished using an appropriate cochleostomy and insertion technique.

Revision cochlear implantation.

Reoperation on a patient with an indwelling cochlear implant is uncommon. When necessary, surgery is performed for explantation of an existing device with immediate or delayed reimplantation, or for scalp flap revision and receiver-stimulator repositioning in the case of infection or device migration. Rarely, revision surgery is performed to reintroduce intracochlear electrodes that may have partly or entirely extruded from the cochlea or were placed inappropriately. Successful revision cochlear implant surgery requires attention to certain surgical principles. Good outcomes, asa measured by speech perception tests, are common, but are not guaranteed. This article outlines the indications for revision cochlear implant surgery, the recommended surgical principles, and published outcomes from reimplantation.

Modiolar coiling, electrical thresholds, and speech perception after cochlear implantation using the nucleus contour advance electrode with the advance off stylet technique.

OBJECTIVE: Perimodiolar electrode arrays were developed in an attempt to improve stimulation of specific neural populations and to decrease electrical thresholds, thereby decreasing power consumption. Postoperative radiographs show that coiling of the arrays is variable. Our previous study explored the relationship between the angle of coiling, threshold levels, and functional outcomes using the Nucleus Contour electrode array. This study compares coiling angle, electrical threshold levels, and speech perception measures with the Nucleus Contour Advance electrode array implanted using the new advance off stylet technique versus the Nucleus Contour electrode array implanted using the standard technique. STUDY DESIGN: Retrospective review. SETTING: University medical center. PATIENTS: Forty-two adults and children with normal cochlear anatomy implanted with the Nucleus CI24RCA electrode using the advance off stylet technique with at least 1-year follow-up. INTERVENTION: Therapeutic. MAIN OUTCOME MEASURES: Computer-aided radiographic analysis of perimodiolar electrode placement, electrical threshold measurements, and speech perception outcome measures at 1 year postimplantation. RESULTS: The degree of modiolar coiling was tighter using the new electrode and technique in comparison with standard insertion technique using the Nucleus Contour electrode array. The tighter coiling tended to result in higher electrical thresholds. Lower speech perception outcome measures tended to correlate with a higher degree of coiling. CONCLUSION: The Nucleus Contour Advance electrode array combined with the advance off stylet technique resulted in a more consistent perimodiolar position. However, the tighter coiling resulted in statistically significant increased electrical thresholds and decreased speech perception outcomes. This finding may be secondary to multiple factors, not just coiling angle.

Total tympanic membrane reconstruction: AlloDerm versus temporalis fascia.

BACKGROUND AND OBJECTIVE: Patients who require surgery for chronic otitis media with perforation and cholesteatoma frequently provide no residual tympanic membrane that is usable in grafting procedures. A novel technique of total tympanic membrane reconstruction (TTMR) is described that maximizes perforation closure rate in these situations while minimizing mucosalization, incomplete healing, and anterior blunting. The specific aim of this report is to assess the safety and efficacy of TTMR and to compare the results obtained with AlloDerm compared with temporalis fascia as a grafting material. METHODS: The records of 50 patients operated within the years 1999 and the 2004 were reviewed. TTMR with intact canal wall was performed in all cases. Both clinical and audiometric data were analyzed. RESULTS: Overall perforation closure rate was 92%. There was no statistical significance in closure rate when grafting with AlloDerm versus temporalis fascia. A statistically significant shortened healing time was observed with AlloDerm grafting. CONCLUSIONS: TTMR is a highly effective and safe technique.