Frequency-Specific Analysis of Hearing Outcomes Associated with Ossiculoplasty Versus Stapedotomy.

OBJECTIVE: Surgery on the ossicular chain may impact its underlying mechanical properties. This study aims to investigate comparative differences in frequency-specific hearing outcomes for ossiculoplasty versus stapedotomy. METHODS: A retrospective chart review was conducted on subjects who underwent ossiculoplasty with partial ossicular replacement prosthesis (PORP) or laser stapedotomy with self-crimping nitinol/fluoroplastic piston, and achieved closure of postoperative pure tone average air-bone gap (PTA-ABG) ≤ 15 dB. 45 PORP and 38 stapedotomy cases were included, with mean length of follow-up of 7.6 months. RESULTS: The mean change in PTA-ABG was similar for the 2 procedures (-17.9 dB vs -18.1 dB, P = .98). Postoperative ABG closure for stapedotomy was superior at 1000 Hz (8.9 dB vs 13.9 dB, P = .0003) and 4000 Hz (11.8 dB vs 18.0 dB, P = .0073). Both procedures also had improved postoperative bone conduction (BC) thresholds at nearly all frequencies, but there was no statistical difference in the change in BC at any particular frequency between the 2 procedures. CONCLUSION: Both procedures achieved a similar mean change in PTA-ABG. Stapedotomy was superior to PORP at ABG closure at 1000 Hz and at 4000 Hz, with 1000 Hz the most discrepant. The exact mechanism responsible for these changes is unclear, but the specific frequencies affected suggest that differences in each procedure's respective impact on the native resonant frequency and mass load of the system could be implicated.

Reconstruction of Mastoid Cortex Defects with Hydroxyapatite Cement for Negative Sequelae of Mastoidectomy.

OBJECTIVES: Mastoid cortex defects resulting from mastoidectomy surgery can rarely lead to negative sequelae, including unsightly post-auricular depression and discomfort. This study sought to evaluate the use of hydroxyapatite cement (HAC) to reconstruct mastoidectomy cortex defects. METHODS: Retrospective chart review was undertaken for all patients that underwent reconstruction of the mastoid cortex using HAC at a single tertiary medical center between 2013 and 2019. Collected data included demographics, indications for mastoidectomy, complications, and associated symptom status. RESULTS: Twenty-nine patients that underwent mastoid cortex repair using HAC were included, and ten of these underwent mastoid revision in a secondary procedure to reconstruct the mastoid cortex with a specific goal to eliminate negative signs and symptoms. There were no associated postoperative complications and no instances of post-auricular depression following repair. All cases of secondary reconstruction resolved the primary signs and symptoms that prompted mastoid revision. CONCLUSIONS: HAC mastoid cortex reconstruction may be a safe and effective method to resolve negative sequelae resulting from previous mastoidectomy defects. Also, upfront HAC mastoid cortex reconstruction appears viable in select instances as an option to prevent potential future complications.

Ipsilateral Cochlear Implantation in the Presence of Observed and Irradiated Vestibular Schwannomas.

OBJECTIVES: Historically, eventual loss of cochlear nerve function has limited patients with neurofibromatosis type 2 (NF2) to auditory brainstem implants (ABI), which in general are less effective than modern cochlear implants (CI). Our objective is to evaluate hearing outcomes following ipsilateral cochlear implantation in patients with NF2 and irradiated vestibular schwannomas (VS), and sporadic VS that have been irradiated or observed. METHODS: Multi-center retrospective analysis of ipsilateral cochlear implantation in the presence of observed and irradiated VS. MESH search in NCBI PubMed database between 1992 and 2019 for reported cases of cochlear implantation with unresected vestibular schwannoma. RESULTS: Seven patients underwent ipsilateral cochlear implantation in the presence of observed or irradiated vestibular schwannomas. Four patients had sporadic tumors with severe-profound contralateral hearing loss caused by presbycusis/hereditary sensorineural hearing loss, and three patients with NF2 lost contralateral hearing after prior surgical resection. Prior to implantation, one VS was observed without growth for a period of 7 years and the others were treated with radiotherapy. Mean post-operative sentence score was 63.9% (range 48-91) at an average of 28 (range 2-84) months follow up. All patients in this cohort obtained open set speech perception. While analysis of the literature is limited by heterogenous data reporting, 85% of implants with observed schwannomas achieved some open set perception, and 67% of patients previously radiated schwannomas. Furthermore, blending literature outcomes for post implantation sentence testing in quiet without lip-reading show 59.0 ± 35% for patients with CI and observed tumors and 55.7 ± 35% for patients with radiated tumors, with both groups ranging 0 to 100%. CONCLUSION: This retrospective series and literature review highlight that hearing outcomes with CI for VS patients are superior to those achieved with ABI. However, important considerations including imaging, delayed hearing loss, and observation time cannot be ignored in this population.

Histology of the Cochlear Outer Sulcus Cells in Normal Human Ears, Presbycusis, and Menière's Disease.

HYPOTHESIS: Outer sulcus cell features and distribution are hypothesized to differ throughout regions of the human cochlea and between diseased and normal specimens. BACKGROUND: Outer sulcus cells play a role in inner ear fluid homeostasis. However, their anatomy and distribution in the human are not well described. METHODS: Temporal bone specimens with normal hearing (n = 10), Menière's disease (n = 10), presbycusis with flat audiograms (n = 4), and presbycusis with sloping audiograms (n = 5) were examined by light microscopy. Outer sulcus cells were assessed quantitatively and qualitatively in each cochlear turn. One specimen was stained for tubulin immunofluorescence and imaged using confocal microscopy. RESULTS: Outer sulcus cells interface with endolymph throughout the cochlea, with greatest contact in the apical turn. Mean outer sulcus cell counts in the upper apical turn (8.82) were generally smaller (all p < 0.05) than those of the upper basal (17.71), lower middle (18.99) upper middle (18.23), and lower apical (16.42) turns. Mean outer sulcus cell counts were higher (p < 0.05) in normal controls (20.1) than in diseased specimens (15.29). There was a significant correlation between mean cell counts and tonotopically expected hearing thresholds in the upper basal (r = -0.662, p = 0.0001), lower middle (r = -0.565, p = 0.0017), and upper middle (r = -0.507, p = 0.0136) regions. Other differences in cell morphology, distribution, or relationship with Claudius cells were not appreciated between normal and diseased specimens. Menière's specimens had no apparent unique features in the cochlear apex. Immunofluorescence staining demonstrated outer sulcus cells extending into the spiral ligament in bundles forming tapering processes which differed between the cochlear turns in morphology. CONCLUSION: Outer sulcus cells vary throughout the cochlear turns and correlate with hearing status, but not in a manner specific to the underlying diagnoses of Menière's disease or presbycusis.

The Utility of Numeric Grading Scales of Middle Ear Risk in Predicting Ossiculoplasty Hearing Outcomes.

OBJECTIVE: To assess the usefulness of numeric grading scales of middle ear risk in predicting ossiculoplasty hearing outcomes. STUDY DESIGN: Retrospective review. SETTING: Tertiary care, academic medical center. PATIENTS: Adults and children undergoing ossiculoplasty between May 2013 and May 2019 including: synthetic ossicular replacement prosthesis, autograft interposition, bone cement repair, and mobilization of lateral chain fixation. INTERVENTION: Cases were scored via middle ear risk index (MERI), surgical prosthetic infection tissue eustachian tube (SPITE) method, and ossiculoplasty outcome scoring parameter (OOPS) scale. Preoperative and postoperative hearing outcomes were recorded. MAIN OUTCOME MEASURE: Statistical correlation between risk score and postoperative pure-tone average air-bone gap (PTA-ABG). RESULTS: The 179 included cases had average pre and postoperative PTA-ABGs of 30.3dB (standard deviation [SD] 12.7) and 20.3dB (SD 11.1), respectively. Mean MERI, SPITE, and OOPS scores were 4.5 (SD 2.3), 2.8 (SD 1.7), and 3.1 (SD 1.8), respectively. Statistically significant correlations with hearing outcome were noted for all three methods (MERI r = 0.22, p = 0.003; OOPS r = 0.19, p = 0.012; SPITE r = 0.27, p < 0.001). No scale predicted poor (PTA-ABG > 30dB) outcomes; only low SPITE scores predicted excellent (PTA-ABG < 10dB) outcomes (odds ratio [OR] 0.74 [Confidence Interval: 0.57 - 0.97], p = 0.032). CONCLUSIONS: Significant weak correlations between each middle ear risk score and hearing outcomes were encountered. Although only the SPITE method predicted postoperative PTA-ABG, it was not overwhelmingly superior. Current grading scale selection may be justified by familiarity or ease of use.

On the Relationship Between Menière's Disease and Endolymphatic Hydrops.

: The relationship between Menière's disease and endolymphatic hydrops is ambiguous. On the one hand, the existence of cases of endolymphatic hydrops lacking the classic symptoms of Menière's disease has prompted the assertion that endolymphatic hydrops alone is insufficient to cause symptoms and drives the hypothesis that endolymphatic hydrops is a mere epiphenomenon. Yet, on the other hand, there is considerable evidence suggesting a relationship between the mechanical pressure effects of endolymphatic hydrops and resultant disordered auditory physiology and symptomatology. A critical appraisal of this topic is undertaken, including a review of key histopathologic data chiefly responsible for the epiphenomenon hypothesis. Overall, a case is made that A) the preponderance of available evidence suggests endolymphatic hydrops is likely responsible for some of the auditory symptoms of Menière's disease, particularly those that can be modulated by mechanical manipulation of the basilar membrane and cochlear microphonic; B) Menière's disease can be reasonably considered part of a larger spectrum of hydropic inner ear disease that also includes some cases that lack vertigo. C) The relationship with endolymphatic hydrops sufficiently robust to consider its presence a hallmark defining feature of Menière's disease and a sensible target for diagnostic detection.

Pediatric Autoimmune Inner Ear Disease: A Rare, But Treatable Condition.

Autoimmune inner ear disease (AIED) is a rare, but treatable cause of sudden sensorineural hearing loss in children. Most cases present acutely and involve both ears. The precise mechanism of hearing loss in AIED is not known. Many suspected etiologies have been proposed including infections, vascular abnormalities, and trauma. However, 70% of cases are defined as idiopathic. There are no standardized diagnostic criteria for AIED, and the diagnostic process may be challenging. Positive auto antibodies and response to immunosuppressive therapy support the diagnosis. Treatment may include corticosteroids and steroid-sparing immunosuppressive medications. A high index of suspicion is recommended as the hearing loss may be stabilized or even reversed with early treatment. Long-term medical treatment failures generally have good outcomes with cochlear implantation. [Pediatr Ann. 2019;48(10):e391-e394.].

Delineation of the intratemporal facial nerve in a cadaveric specimen on diffusion tensor imaging using a 9.4 T magnetic resonance imaging scanner: a technical note.

The purpose of this study was to determine whether the intratemporal facial nerve could be delineated on 9.4 T magnetic resonance imaging (MRI) using T2-weighted and diffusion tensor imaging (DTI). DTI using a b value of 3000 and an isotropic resolution of 0.4 mm3 on a 9.4 T MRI scanner was performed on a whole-block celloidin-embedded cadaveric temporal bone specimen of a 1-year-old infant with normal temporal bones. The labyrinthine, tympanic, and mastoid segments of the facial nerve and the chorda tympani nerve were readily depicted on DTI. Therefore, DTI performed using a high b value on a high-field strength MRI scanner could help evaluate the intratemporal facial nerve in whole temporal bone ex vivo specimens.

Impact of cochlear tonotopy on electrically evoked compound action potentials (ECAPs).

BACKGROUND: A wide range of cochlear implant electrode designs exists. Lateral wall electrodes may be favored for their potential to preserve residual hearing by virtue of being thin and delicate; whereas perimodiolar electrodes may have advantages in case of profound hearing loss, due to electrode positioning in close proximity to the auditory nerve fibers. AIM: The aim of this study was to investigate the impact of these two array designs on the interaction between electrodes and the auditory nerve in different tonotopic regions of the cochlea. PATIENTS AND METHODS: A retrospective study of both adult and pediatric cochlear implant recipients (CI24RE/CI512 or CI422, Cochlear®) was undertaken. The differences of threshold Neural Response Telemetry (tNRT) acquired 12 months after surgery were analyzed with respect to the tonotopic location. RESULTS: The results of 168 implants showed that perimodiolar arrays had lowest thresholds in the basal region whereas straight arrays had lowest thresholds in the apex. Highest thresholds for both array types were encountered in the medial parts. CONCLUSIONS AND SIGNIFICANCE: tNRTs differ depending on electrode type and location inside the cochlea. This should be considered pre implantation when choosing the electrode array type and post-implantation when mapping the CI program.

Histopathology of the Mucosa of Eustachian Tube Orifice at the Middle Ear in Chronic Otitis Media With Effusion: Possible Insight Into Tuboplasty Failure.

INTRODUCTION: Balloon dilation of the cartilaginous segment of the Eustachian tube has emerged as a means to directly augment tubal dilatory function, and this has been applied as a potential treatment for otitis media with effusion (OME). Although results of clinical studies involving this modality appear promising, there are still a moderate number of ears affected by OME that do not respond. The purpose of this study was to investigate the status of mucosa of the Eustachian tube at the middle ear orifice in OME as it may relate to some cases of tuboplasty failure. METHODS: Twenty-three temporal bone specimens with OME were identified within an institutional archived collection. Each specimen was inspected for the presence of a fixed obstruction at the level of the Eustachian tube orifice at the protympanum. In addition, the mucosa at the tubal orifice was graded on a 4-point scale. RESULTS: Overall, 3 cases (13%) were normal (Grade 1), 6 cases (26%) were mildly thickened (Grade 2), 11 (48%) were severely thickened (Grade 3), and 3 (13%) were severely thickened with polypoid degeneration (Grade 4). A single case was noted to have a complete fixed obstruction in the form of a mucosal web. CONCLUSION: In ears affected by OME, the mucosa of the Eustachian tubal orifice at the middle ear is most often severely thickened. Normal mucosa, mucosa with severe polypoid changes, or a complete fixed obstruction are possible but uncommon. The majority of specimens studied had sufficiently diseased mucosa to raise questions regarding whether thickened mucosa in the tubal orifice may act as a barrier to middle ear ventilation that would not be directly addressed by cartilaginous Eustachian tube balloon dilation.

Combined petrosal approach.

PURPOSE OF REVIEW: The combined petrosal approach to the lateral skull base merges a retrolabyrinthine-presigmoid posterior fossa craniotomy with an adjacent middle fossa craniotomy, which are rendered continuous by division of the tentorium. This is a hearing-preserving approach that affords wide access to the lateral aspect of the clivus, the prepontine space, and the cerebellopontine angle. RECENT FINDINGS: This article details the historical development of the combined petrosal approach alongside a description. In particular, the critically relevant anatomy is reviewed, including the course of the vein of Labbé, with a view toward avoiding the known complications associated with this approach. Outcomes for application of this approach as applied for various lesions are also reviewed as portrayed by the current literature. SUMMARY: The combined petrosal approach affords wide access to the lateral skull base in the middle and posterior fossa. The approach and the lesions addressed by this approach involve delicate and sensitive anatomy. We review the evolution of this approach and highlight advancements that have allowed the combined petrosal approach to be a safe addition to the surgeon's armamentarium.

Human Superior Olivary Nucleus Neuron Populations in Subjects With Normal Hearing and Presbycusis.

INTRODUCTION: Normative data on superior olivary nucleus neuron counts derived from human specimens are sparse, and little is known about their coherence with structure and function of the cochlea. The purpose of this study was to quantify the neuron populations of the divisions of the superior olivary nucleus in human subjects with normal hearing and presbycusis and investigate potential relationships between these findings and histopathology in the cochlea and hearing phenotype Methods: Histopathologic examination of temporal bone and brainstem specimens from 13 subjects having normal hearing or presbycusis was undertaken. The following was determined for each: number and density of superior olivary nucleus and cochlear nucleus neurons, inner and outer hair cell counts, spiral ganglion cell counts, and pure tone audiometry. RESULTS: The results demonstrate a significant relationship between cells within structures of the cochlear nucleus and the number of neurons of the medial superior olivary nucleus. No relationship between superior olivary nucleus neuron counts/density and cochlear histopathology or hearing phenotype was encountered. CONCLUSION: Normative data for superior olivary nucleus neuron populations are further established in the data presented in this study that includes subjects with normal hearing and also presbycusis.

In Vitro Susceptibility of Ciprofloxacin-Resistant Methicillin-Resistant Staphylococcus aureus to Ototopical Therapy.

Objective Despite the rising incidence of methicillin-resistant Staphylococcus aureus (MRSA) otologic infections, choice of treatment remains controversial. Only fluoroquinolone-containing ototopical preparations are approved by the US Food and Drug Administration for middle ear application. Furthermore, American Academy of Otolaryngology-Head and Neck Surgery Foundation guidelines advocate ototopical monotherapy for both tympanostomy tube otorrhea and acute otitis externa. Unfortunately, MRSA may be ciprofloxacin resistant. This causes confusion regarding antibiotic selection, because susceptibility profiles reflect a minimum inhibitory concentration (MIC), referenced against systemic, not ototopical, drug delivery dosing. The goal of this study is to determine the ciprofloxacin MIC for ciprofloxacin-resistant MRSA isolates from otologic infections and compare that value to the expected drug concentration achieved by fluoroquinolone ear drops and determine MRSA genotype for each isolate. Study Design In vitro assay with retrospective medical record review. Setting Tertiary care university hospital. Subjects and Methods Thirty otologically sourced ciprofloxacin-resistant MRSA isolates collected from adult and pediatric patients. MICs were calculated by broth dilution method. Isolates underwent multilocus sequence typing and polymerase chain reaction for arcA and Panton-Valentine leukocidin to establish the genotype. Results MICs ranged from 16 to 1025 µg/mL. There was a relationship between MIC and genotype; of the 7 isolates with an MIC value greater than 512 µg/mL, 6 were sequence type (ST)8. Conclusion These findings support the practice of ototopical monotherapy for patients with uncomplicated ciprofloxacin-resistant MRSA otitis externa. However, they raise concern that ototopical therapy may not be adequate to treat highly resistant strains of MRSA infecting the middle ear space.

Newly Diagnosed Meniere's Disease: Clinical Course With Initiation of Noninvasive Treatment Including an Accounting of Vestibular Migraine.

OBJECTIVE: To describe the course of Meniere's disease with noninvasive treatment during the first few years after initial diagnosis. METHODS: A retrospective review of consecutive patients with newly diagnosed definite Meniere's disease between 2013 and 2016 and a minimum follow-up of 1 year. Patients received a written plan for low sodium, water therapy, and treatment with a diuretic and/or betahistine. Subjects were screened and treated for vestibular migraine as needed. Vertigo control and hearing status at most recent follow-up were assessed. RESULTS: Forty-four subjects had an average follow up of 24.3 months. Thirty-four percent had Meniere's disease and vestibular migraine, and 84% had unilateral Meniere's disease. Seventy-five percent had vertigo well controlled at most recent follow-up, with only noninvasive treatments. Age, gender, body mass index, presence of vestibular migraine, bilateral disease, and duration of follow-up did not predict noninvasive treatment failure. Worse hearing threshold at 250 Hz and lower pure tone average (PTA) at the time of diagnosis did predict failure. Fifty-two percent of ears had improved PTA at most recent visit, 20% had no change, and 28% were worse Conclusions: Encountering excellent vertigo control and stable hearing after a new diagnosis of Meniere's disease is possible with noninvasive treatments. Worse hearing status at diagnosis predicted treatment failure.

Advantages and Complications of Fisch Partial Mastoidotympanectomy for Tumors of the Retromandibular Fossa and Poststyloid Space.

Objective This study aims to characterize a single surgeon's experience with resection of tumors of the retromandibular fossa and poststyloid space utilizing the Fisch partial mastoidotympanectomy (FPMT) technique of partial temporal bone resection. Design Retrospective review. Setting Academic medical center. Participants Seven patients who underwent tumor resection with FPMT. Main Outcome Measures Complications. Results Six males and one female underwent FPMT, with ages ranging from 44 to 79 years. The facial nerve was sacrificed in six cases, no cases were converted to more extensive temporal bone resection procedures, and no postoperative hearing loss occurred. The only intraoperative complication experienced was a violation of the external auditory canal in four cases, which resulted in postoperative wound healing issues in two cases. Conclusions FPMT allows for the release of tumors of the poststyloid space and retromandibular fossa without compromise of hearing status. Preservation of external auditory canal soft tissues is challenging, and the violation is experienced relatively frequently due to shearing forces during soft tissue retraction. When encountered, robust repair of defects is encouraged to prevent further wound healing complications.

Age-Related Change in Vestibular Ganglion Cell Populations in Individuals With Presbycusis and Normal Hearing.

HYPOTHESIS: We sought to establish that the decline of vestibular ganglion cell counts uniquely correlates with spiral ganglion cell counts, cochlear hair cell counts, and hearing phenotype in individuals with presbycusis. BACKGROUND: The relationship between aging in the vestibular system and aging in the cochlea is a topic of ongoing investigation. Histopathologic age-related changes the vestibular system may mirror what is seen in the cochlea, but correlations with hearing phenotype and the impact of presbycusis are not well understood. METHODS: Vestibular ganglion cells, spiral ganglion cells, and cochlear hair cells were counted in specimens from individuals with presbycusis and normal hearing. These were taken from within a large collection of processed human temporal bones. Correlations between histopathology and hearing phenotype were investigated. RESULTS: Vestibular ganglion cell counts were positively correlated with spiral ganglion cell counts and cochlear hair cell counts and were negatively correlated with hearing phenotype. There was no statistical evidence on linear regression to suggest that the relationship between age and cell populations differed significantly according to whether presbycusis was present or not. Superior vestibular ganglion cells were more negatively correlated with age than inferior ganglion cells. No difference in vestibular ganglion cells was noted based on sex. CONCLUSION: Vestibular ganglion cell counts progressively deteriorate with age, and this loss correlates closely with changes in the cochlea, as well as hearing phenotype. However, these correlations do not appear to be unique in individuals with presbycusis as compared with those with normal hearing.

Surgery for Cholesteatomatous Labyrinthine Fistula.

OBJECTIVE: There is uncertainty regarding the ideal surgical management of cholesteatomatous labyrinthine fistulae. The objective was to review the published evidence to determine whether a difference exists in hearing outcome for cholesteatoma matrix removal or matrix exteriorization. DATA SOURCES: Systematic MEDLINE and Web of Science searches identified publications describing hearing results after cholesteatoma matrix removal or matrix exteriorization. REVIEW METHODS: Three reviewers appraised the studies for quality, level of evidence, and extracted data. Fistula characteristics such as single-site, multisite, size, grade, and follow-up time were extracted for subanalyses, and when appropriate, data were pooled for statistical analysis. RESULTS: Twenty-eight articles met inclusion criteria, and the level of evidence was judged no better than level 3b. There was no difference in hearing preservation detected between matrix removal and exteriorization (87% for matrix removal, 95% CI, 0.82-0.90; 95% for exteriorization, 95% CI, 0.85-0.98). An analysis of the individual cohort studies that compared these groups directly did not show a difference in calculated odds ratio (OR), 0.96 (95% CI, 0.66-1.40). CONCLUSION: The level of evidence on which to base surgical decision making related to cholesteatomatous labyrinthine fistula is poor, and the data do not demonstrate significant differences in hearing outcomes based on surgical technique.