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.

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.

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.

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.