Evaluation of Type III Tympanoplasty Using Cartilage Ossiculoplasty in Cholesteatoma Ear Surgery.

This prospective observational study evaluates the role of tympanoplasty type III in cholesteatoma ear disease during same sitting with mastoid surgery using cartilage ossiculoplasty. Forty patients of chronic suppurative otitis media-cholesteatoma disease were recruited in the study. All the patients had extensive cholesteatoma and underwent canal wall down mastoid surgery. Tympanoplasty type III, that is, stapes columella, minor columella, or major columella, was done in each case along with mastoid surgery depending upon the remnant ossicular status. Conchal cartilage graft was used for ossiculoplasty along with temporalis fascia graft. Hearing and graft uptake results were evaluated at the end of 6 months postoperatively. Of the 40 cases, 3 cases failed tympanoplasty. In the remaining 37 cases, a statistically significant hearing improvement (air-bone gap of 33 dB) was observed postoperatively. Seven cases underwent stapes columella, 13 cases underwent minor columella, and 17 cases underwent major columella tympanoplasty type III. Although a hearing improvement was recorded in all these subgroups, a statistically significant hearing gain was present only in tympanoplasty type III minor columella cases thereby underlying the importance of intact stapes. However, it is difficult to discern the type of tympanoplasty type III that the patient would undergo prior to the ear surgery.

Ossicular chain defects in adults with chronic otitis media.

BACKGROUND AND OBJECTIVE: Chronic otitis media (COM) is a common condition characterized by the perforation of the tympanic membrane and inflammation of the mucosal lining the hollow space in the middle ear and airy spaces of the temporal bone for at least 2-6 week. This study was carried out to find out the status of the middle ear ossicles in patients with COM and to correlate their status with clinical parameters. METHODS: This retrospective clinical study was conducted on 107 COM patients (52 males and 55 females; age range: 18 to 75 years) submitted to surgery in the Otology Clinic at Imam Khomeini Hospital, Ahvaz Jundishapur University of Medical Sciences, Iran. Initially, a comprehensive case-history was obtained from patients and their hearing thresholds were recorded. Then, ossicles status and their junction condition were evaluated intraoperatively. RESULTS: The malleus was found intact in 70 (65.42%), absent in 10 (9.34), and eroded in 27 (25.24%) patients. Our results revealed that the incus was intact in 33 (30.84%), eroded in 55 (51.41%) and absent in 19 (17.75%) subjects. Stapes was found intact in 54 (50.46%) cases and eroded in 53 (49.54%) cases. The mean Pure Tone Average (PTA) and Air-Bone Gap (ABG) comparisons in "intact" and "discontinuous" ossicular chain groups was not significant (Independent sample t-test, p>0.05). CONCLUSION: Our results demonstrated that incus was the most susceptible middle ear ossicle to erosion in COM, whereas the malleus was the most resistant ossicle. Furthermore, ABG and PTA values cannot be considered as a potential preoperative predictor for ossicular chain status.

Bone conduction hearing in the Guinea pig and the effect of artificially induced middle ear lesions.

Although human bone conduction (BC) hearing is well investigated, there is a lack of information about BC hearing in most other species. In humans, the amount of conductive loss is estimated as the difference between the air conduction (AC) and BC thresholds. Similar estimations for animals are difficult since in most species, the normal BC hearing thresholds have not been established. In the current study, the normal BC thresholds in the frequency range between 2 kHz and 20 kHz are investigated for the Guinea pig. Also, the effect of a middle ear lesion, here modelled by severing the ossicles (ossicular discontinuity) and gluing the ossicles to the bone (otosclerosis), is investigated for both AC and BC. The hearing thresholds in the Guinea pigs were estimated by a regression of the amplitude of the compound action potential (CAP) with stimulation level and was found robust and gave a high resolution of the threshold level. The reference for the BC thresholds was the cochlear promontory bone velocity. This reference enables comparison of BC hearing in animals, both intra and inter species, which is independent on the vibrator and stimulation position. The vibration was measured in three orthogonal directions where the dominating vibration directions was in line with the stimulation direction, here the ventral direction. The BC thresholds lay between -10 and 3 dB re 1 µm/s. The slopes of CAP growth function were similar for AC and BC at low and high frequencies, but slightly lower for BC than AC at frequencies between 8 and 16 kHz. This was attributed to differences in the stimulus levels used for the slope estimation and not a real difference in CAP slopes between the stimulation modalities. Two kinds of middle ear lesions, ossicular discontinuity and stapes glued to the surrounding bone, gave threshold shifts of between 23 and 53 dB for AC while it was below 16 dB when the stimulation was by BC. Statistically different threshold shifts between the two types of lesions were found where the AC threshold shifts for a glued stapes at 2 and 4 kHz were 9-18 dB greater than for a severed ossicular chain, and the BC threshold shifts for a glued stapes at 4 and 12 kHz were 8-9 dB greater than for a severed ossicular chain.

Round window and promontory movements during bone conduction with different middle ear conditions in Thiel embalmed specimens.

BACKGROUND: Thiel conservation is mainly based on a watery solution of salts. We have shown that bone conduction (BC) evokes motion in normal middle ears of Thiel embalmed specimens that is comparable to the motion for other cadaveric models. AIMS/OBJECTIVES: We evaluated whether promontory and round window (RW) motion identifies differences in BC transmission for different middle ear conditions. METHODS: We investigated the conditions of mobile ossicle chain, cement-fixed stapes and stapedectomy in seven ears. A retroauricular bone anchored hearing system provided BC stimulation. The motions of the promontory and the RW were measured using single point laser Doppler vibrometer (LDV, HLV1000, Polytec). RESULTS: The averaged differences between the conditions were small for RW motion and for promontory motion. However, for RW motion we found differences of more than one standard deviation at some frequencies. These differences in RW motion were more apparent when we limited the analysis to three selected specimens. CONCLUSIONS AND SIGNIFICANCE: Extracochlear measurement of the RW motion with LDV allowed differentiation between BC for different middle ear conditions. These changes could be detected best in a small frequency range in selected specimens. Promontory motion could not be used to differentiate between different conditions of the middle ear. ABBREVIATIONS: LDV: laser Doppler vibrometry; Prom: cochlear promontory; RW: round window; ST: stapes; TM: tympanic membrane; VProm: velocity of the promontory; VRW: velocity of the round window.

Influence of angular positioning of the prosthesis in stapes surgeries with a NiTiBond prosthesis: Investigation in cadaveric temporal bones.

In incus stapedotomy surgeries, the longitudinal direction of the piston prosthesis should ideally be perpendicular to the stapes footplate. However, in reality, some amounts of angular deviation of the prosthesis from the ideal angular position is unavoidable due to anatomical constraints and surgical conditions. This study aims to evaluate the influence of angular positioning of the prosthesis on surgical outcomes in incus stapedotomy and to provide surgical guidelines related to practical tolerance of the angular positioning. In this study, this influence was assessed with a Kurz NiTiBond prosthesis (0.4-mm diameter) and fenestra sizes of 0.5- and 0.6-mm diameter in cadaveric temporal bones (n = 7 including 2 preliminary tests). Angular position of the prosthesis relative to the footplate was modulated by rotating the stapes about the long and short axes of the footplate. At each angular position, the tympanic membrane was acoustically stimulated in the frequency range of 0.2-10 kHz, and motion of the prosthesis was measured using a Laser Doppler vibrometer (LDV). Furthermore, micro-computed tomography (micro-CT) data of the middle-ear ossicles were used for anatomical analysis of angular positioning of the prosthesis. The results showed that changes of angular position of the prosthesis relative to the stapes footplate do not cause significant changes of prosthesis motion until a certain angular position threshold, and sharply attenuate prosthesis motion when the angular position reaches the threshold. The threshold of the angular position, as the tilting angle of the prosthesis from the direction normal to the stapes footplate, was 26.9 ±â€¯2.5° with the fenestration hole of 0.5-mm diameter and 30.6 ±â€¯3.0° with the fenestration hole of 0.6-mm diameter (n = 5, p < 0.01 for difference between the two fenestra sizes). Analysis of the middle-ear anatomy in this study revealed that the tolerances of the angular positions of the prosthesis does not always cover possible positions of prosthesis crimping. This study suggests that if an anterior offset of the stapes head and/or the thickened footplate is suspected, efforts to locate prosthesis crimping closer to the tip of the incus and/or to make a sufficiently large fenestration hole are favorable.

Stapes hypermobility as a possible cause of hyperacusis.

OBJECTIVE: Hyperacusis is a reduction of normal tolerances for everyday sounds. Although several publications have been produced demonstrating that minimally invasive surgical procedures may improve patient symptoms, the precise etiology of hyperacusis often remains elusive. This study describes 21 patients, 7 of whom stapes hypermobility is believed to be a mechanical genesis of their hyperacusis symptoms. STUDY DESIGN: A prospective, repeated-measure single-arm design was used for this study. SETTING: All patients were evaluated and treated at a tertiary level otologic referral center. SUBJECTS AND METHODS: 21 patients (Cohort A) with severe hyperacusis underwent oval and round window reinforcement. Seven patients (Cohort B) intraoperatively appeared to have subjective hypermobility of the stapes. Additional reinforcement of the stapes superstructure was performed in these patients. RESULTS: In Cohort A, loudness discomfort level (LDL) values improved on average from 72.7 dB to 81.9 dB. Hyperacusis questionnaire (HQ) scores improved from 30.1 to 14.7. Numeric Rating Scale scores (0-10) decreased from 8.5 to 4.0. In Cohort B, values similarly improved from an average of 72.4 dB to 88.2 dB. HQ scores improved from 35.8 to 18.9. Numeric Rating Scale scores fell from 10.0 to 3.7. Postoperatively there were no complaints of hearing loss. Sixteen out of 21(76%) reported improved quality of life and diminished symptoms of hyperacusis. CONCLUSION: It is possible that patients suffering from hyperacusis may have a mechanical cause for their symptoms. Further research is necessary to clarify stapes mobility in patients with these symptoms. Excess temporalis tissue reinforcement of the stapes along with round window reinforcement shows promise as a minimally invasive surgical option for patients suffering from hyperacusis.

Drill-induced Cochlear Injury During Otologic Surgery: Intracochlear Pressure Evidence of Acoustic Trauma.

HYPOTHESIS: Drilling on the incus produces intracochlear pressure changes comparable to pressures created by high-intensity acoustic stimuli. BACKGROUND: New-onset sensorineural hearing loss (SNHL) following mastoid surgery can occur secondary to inadvertent drilling on the ossicular chain. To investigate this, we test the hypothesis that high sound pressure levels are generated when a high-speed drill contacts the incus. METHODS: Human cadaveric heads underwent mastoidectomy, and fiber-optic sensors were placed in scala tympani and vestibuli to measure intracochlear pressures (PIC). Stapes velocities (Vstap) were measured using single-axis laser Doppler vibrometry. PIC and Vstap were measured while drilling on the incus. Four-millimeter diamond and cutting burrs were used at drill speeds of 20k, 50k, and 80k Hz. RESULTS: No differences in peak equivalent ear canal noise exposures (134-165 dB SPL) were seen between drill speeds or burr types. Root-mean-square PIC amplitude calculated in third-octave bandwidths around 0.5, 1, 2, 4, and 8 kHz revealed equivalent ear canal (EAC) pressures up to 110 to 112 dB SPL. A statistically significant trend toward increasing noise exposure with decreasing drill speed was seen. No significant differences were noted between burr types. Calculations of equivalent EAC pressure from Vstap were significantly higher at 101 to 116 dB SPL. CONCLUSION: Our results suggest that incidental drilling on the ossicular chain can generate PIC comparable to high-intensity acoustic stimulation. Drill speed, but not burr type, significantly affected the magnitude of PIC. Inadvertent drilling on the ossicular chain produces intense cochlear stimulation that could cause SNHL.

The human otitis media with effusion: a numerical-based study.

Otitis media is a group of inflammatory diseases of the middle ear. Acute otitis media and otitis media with effusion (OME) are its two main types of manifestation. Otitis media is common in children and can result in structural alterations in the middle ear which will lead to hearing losses. This work studies the effects of an OME on the sound transmission from the external auditory meatus to the inner ear. The finite element method was applied on the present biomechanical study. The numerical model used in this work was built based on the geometrical information obtained from The visible ear project. The present work explains the mechanisms by which the presence of fluid in the middle ear affects hearing by calculating the magnitude, phase and reduction of the normalized umbo velocity and also the magnitude and phase of the normalized stapes velocity. A sound pressure level of 90 dB SPL was applied at the tympanic membrane. The harmonic analysis was performed with the auditory frequency varying from 100 Hz to 10 kHz. A decrease in the response of the normalized umbo and stapes velocity as the tympanic cavity was filled with fluid was obtained. The decrease was more accentuated at the umbo.

Finite element analysis of auditory characteristics in patients with middle ear diseases.

CONCLUSION: This study validates that a finite element model of the human ossicular chain and tympanic membrane can be used as an effective surgical assessment tool in clinics. OBJECTIVE: The present study was performed to investigate the application of a finite element model of ossicular chain and tympanic membrane for fabrication of individualized artificial ossicles. METHODS: Twenty patients (20 ears) who underwent surgery for middle ear disease (n = 20) and 10 healthy controls (10 ears) were enrolled in the hospital. Computed tomography (CT) and pure tone audiometry were performed before and after surgery. A finite element model was developed using CT scans, and correlation analysis was conducted between stapes displacement and surgical methods. An audiometric test was also performed for 14 patients before and after surgery. RESULTS: Stapes displacement in the healthy group (average = 3.31 × 10-5 mm) was significantly greater than that in the impaired group (average = 1.41 × 10-6 mm) prior to surgery. After surgery, the average displacement in the impaired group was 2.55 × 10-6 mm, which represented a significant improvement. For the patients who underwent the audiometric test, 10 improved hearing after surgery, and stapes displacement increased in nine of these 10 patients.

A point mutation in Fgf9 impedes joint interzone formation leading to multiple synostoses syndrome.

Human multiple synostoses syndrome (SYNS) is an autosomal dominant disorder characterized by multiple joint fusions. We previously identified a point mutation (S99N) in FGF9 that causes human SYNS3. However, the physiological function of FGF9 during joint development and comprehensive molecular portraits of SYNS3 remain elusive. Here, we report that mice harboring the S99N mutation in Fgf9 develop the curly tail phenotype and partially or fully fused caudal vertebrae and limb joints, which mimic the major phenotypes of SYNS3 patients. Further study reveals that the S99N mutation in Fgf9 disrupts joint interzone formation by affecting the chondrogenic differentiation of mesenchymal cells at the early stage of joint development. Consistently, the limb bud micromass culture (LBMMC) assay shows that Fgf9 inhibits mesenchymal cell differentiation into chondrocytes by downregulating the expression of Sox6 and Sox9. However, the mutant protein does not exhibit the same inhibitory effect. We also show that Fgf9 is required for normal expression of Gdf5 in the prospective elbow and knee joints through its activation of Gdf5 promoter activity. Signal transduction assays indicate that the S99N mutation diminishes FGF signaling in developmental limb joints. Finally, we demonstrate that the conformational change in FGF9 resulting from the S99N mutation disrupts FGF9/FGFR/heparin interaction, which impedes FGF signaling in developmental joints. Taken together, we conclude that the S99N mutation in Fgf9 causes SYNS3 via the disturbance of joint interzone formation. These results further implicate the crucial role of Fgf9 during embryonic joint development.

Effectiveness of stapedotomy in improving hearing sensitivity for 53 otosclerotic patients: retrospective review.

BACKGROUND: Otosclerosis is a common cause of progressive hearing impairment that causes fixation of the stapes. Surgical intervention is the preferred treatment approach to ameliorate the conductive hearing loss associated with stapedial otosclerosis. However, given that it is a difficult and delicate procedure, the surgery may fail for a number of reasons. Therefore, it is very important to evaluate the success rate of the surgical approach used in each regional center. OBJECTIVE: To examine the effectiveness of stapedotomy in improving hearing sensitivity for otosclerotic patients at King Abdul Aziz University Hospital in Riyadh. DESIGN: Retrospective chart review with an analysis of pre- and postoperative surgical treatment. SETTING: Tertiary referral otolaryngology clinic. PATIENTS AND METHODS: All cases who underwent stapedotomy between 1997 and 2009 were retrospectively reviewed. Preoperative and postoperative audiometric assessments were conducted using conventional pure tone audiometry. Differences were analyzed by two-way repeated measures ANOVA. MAIN OUTCOME MEASURE(S): Pre- and postoperative pure tone thresholds for air and bone conduction. RESULT: Fifty-three patients underwent stapedotomy. Stapedotomy yielded significant improvements in mean (SD) postoperative air-conduction thresholds of about 18.7 (11.7) dB (P < .0001) and mean (SD) post.operative bone-conduction thresholds of about 2 (7.2) dB (P < .05). Additionally, a significant correlation was found between improvement in air-conduction thresholds and the size of preoperatve air-bone gap (P < .01) About 70%of patients achieved an air-bone gap of 20 dB or better. None of the cases examined in this study exhibited sensorineural hearing loss or adverse complications following stapedotomy. CONCLUSIONS: Stapedotomy is a safe and effective treatment option for patients with otosclerosis. Given that the majority of participants in this study exhibited mixed hearing loss preoperatively, the results further suggest that stapedotomy can also be effective in improving thresholds for these patients. LIMITATIONS: The sample size was relatively small.

Acoustic effects of the reconstructed lateral epitympanic wall in a temporal bone and clinical study.

OBJECTIVE: Acoustic evaluation of reconstruction of the lateral epitympanic wall with bone or cartilage in a temporal bone study, and evaluation of audiometric data of patients who underwent cholesteatoma surgery with reconstruction of the lateral epitympanic wall with horseshoe-shaped cartilage. STUDY DESIGN: Temporal bone study and retrospective chart review. METHODS: Preparation of temporal bones included reconstruction of the epitympanic wall with fixated and loose cartilage and bone. The volume velocities of the stapes footplate were measured from the inner-ear side of the footplate by laser scanning doppler vibrometry following sound stimulation in the outer ear canal. Additionally, the audiometric data of 13 consecutive patients who underwent epitympanic cholesteatoma surgery, with an intact ossicular chain and reconstruction of the scutum with a horseshoe-shaped cartilage in contact with the malleus' neck, were evaluated retrospectively. RESULTS: The experimental results showed similar volume velocities at the stapes footplate for the fixated and unfixated cartilage as well as for the unfixated bone. However, the fixated bone yielded significantly reduced volume velocities. Clinical data confirmed that the cartilaginous horseshoe- technique allowed for a stable reconstruction of the scutum with satisfying audiometric outcome. CONCLUSION: In case of cholesteatoma surgery and the need for the reconstruction of the scutum, no adverse effects on hearing outcome are to be expected by using the malleus' neck as an anchoring point for cartilaginous scutum reconstruction. LEVEL OF EVIDENCE: NA. Laryngoscope, 127:1427-1434, 2017.

Benefits of active middle ear implants in mixed hearing loss: Stapes versus round window.

OBJECTIVES/HYPOTHESIS: We compared the audiologic benefits of active middle ear implants with those of passive middle ear implants with hearing aids in mixed hearing loss, and also compared the outcomes of stapes vibroplasty with those of round window vibroplasty. STUDY DESIGN: Retrospective chart review. METHODS: Thirty-four patients with mixed hearing loss due to chronic otitis media were treated with a middle ear implant. Of these, 15 were treated with a passive middle ear implant (conventional ossiculoplasty with a partial ossicular replacement prosthesis), nine with an active middle ear implant coupling to the stapes, and 10 with an active middle ear implant coupling to the round window. Patients underwent pure-tone/free-field audiograms and speech discrimination tests before surgery and 6 months after surgery, and the results of these tests were compared. RESULTS: The active middle ear implant resulted in better outcomes than the passive middle ear implant with hearing aids at mid to high frequencies (P < .05). Patients who received either a stapes vibroplasty or a round window vibroplasty showed comparable hearing gain except at 8,000 Hz (48.9 dB vs. 31.0 dB, P < .05). Patients who received a stapes vibroplasty showed an improvement even in bone conduction at 1,000 Hz and 2,000 Hz (both P < .05). CONCLUSIONS: Active middle ear implantation could be a better option than treatment with passive middle ear implants with hearing aids for achieving rehabilitation in patients with mixed hearing loss. Vibroplasty via either oval window or round window stimulation shares similar good results. LEVEL OF EVIDENCE: 4 Laryngoscope, 127:1435-1441, 2017.

Inside implant criteria or not? - Detection of non-organic hearing loss during cochlear implant assessment.

OBJECTIVES: The diagnosis of non-organic hearing loss (NOHL) is a difficult but important issue during the assessment process for cochlear implantation (CI). We aim to identify the key factors in identifying patients with NOHL during CI assessment and present our local screening protocol for NOHL. METHODS: A retrospective review of patients referred to the Yorkshire Auditory Implant Service (YAIS) between 2003 and 2015 who were subsequently diagnosed with NOHL during the assessment. Patient demographic data, audiological and functional assessments were assessed. RESULTS: Thirty-two patients were included in the study. Mean age was 43 years (range 14-82 years). Male to female ratio was 1:1.7. Indicators of possible NOHL included a sudden deterioration in hearing (n = 21; 66%), mismatches in observed behaviour and either pure-tone audiogram (PTA) (n = 27; 84%) or functional testing (n = 20; 80%) and stapedial reflexes below reported audiological thresholds (n = 12; 46%). A mismatch in functional hearing and PTA was seen in 72% of patients. Patients with suspected NOHL were referred for further objective testing. All 23 patients who underwent objective testing had better hearing levels compared to reported hearing thresholds thus placing them outside of implant criteria. Five candidates were found to have normal hearing thresholds. DISCUSSION: NOHL can present a significant challenge to the implant team, particularly in the subgroup with a pre-existing organic hearing loss with non-organic overlay. We discuss the common features in this cohort of patients. CONCLUSIONS: To facilitate the identification of patients with NOHL, the YAIS has developed a screening protocol.

Effects of Continuous Positive Airway Pressure on Middle Ear Pressure and Acoustic Stapedial Reflex.

OBJECTIVE: This study investigated the effects of continuous positive airway pressure (CPAP) on middle ear pressure and acoustic stapedial reflex and the correlation between CPAP and middle ear pressure. STUDY DESIGN: Prospective cohort study. SETTING: Tertiary hospitals. SUBJECTS AND METHODS: Fifty patients with obstructive sleep apnea-hypopnea syndrome were assigned to the study group, and 50 healthy volunteers were assigned to the control group. The subjects underwent standard tympanometry while wearing a CPAP device (ie, simulated CPAP treatment), which was set to 0, 5, 10, and 15 cm H2O, respectively. Tympanometry was performed before and after swallowing at each pressure of CPAP treatment. RESULTS: The mean middle ear pressures were 21.2, 22.6, 22.7, and 23.4 daPa (before swallowing) and 21.6, 42.6, 81.4, and 118.6 daPa (after swallowing) in the study group and 17.6, 18.7, 19.5, and 20.8 daPa (before swallowing) and 17.7, 44.2, 85.6, and 120.5 daPa (after swallowing) in the control group at the CPAPs of 0, 5, 10, and 15 cm H2O, respectively. While the CPAPs were at 0 and 15 cm H2O, the stapedial muscle reflex at 1.0 kHz did not have a significant difference between the 2 groups (χ(2) = 0.521, P = .470). The Pearson correlation coefficient of the CPAP pressure and the middle ear pressure after swallowing was 0.812 (P < .001). CONCLUSION: CPAP affected middle ear pressure and was directly proportional to the pressure of the CPAP. However, CPAP treatment had no significant effect on stapedial muscle reflex.

Mutations and altered expression of SERPINF1 in patients with familial otosclerosis.

Otosclerosis is a relatively common heterogenous condition, characterized by abnormal bone remodelling in the otic capsule leading to fixation of the stapedial footplate and an associated conductive hearing loss. Although familial linkage and candidate gene association studies have been performed in recent years, little progress has been made in identifying disease-causing genes. Here, we used whole-exome sequencing in four families exhibiting dominantly inherited otosclerosis to identify 23 candidate variants (reduced to 9 after segregation analysis) for further investigation in a secondary cohort of 84 familial cases. Multiple mutations were found in the SERPINF1 (Serpin Peptidase Inhibitor, Clade F) gene which encodes PEDF (pigment epithelium-derived factor), a potent inhibitor of angiogenesis and known regulator of bone density. Six rare heterozygous SERPINF1 variants were found in seven patients in our familial otosclerosis cohort; three are missense mutations predicted to be deleterious to protein function. The other three variants are all located in the 5'-untranslated region (UTR) of an alternative spliced transcript SERPINF1-012 RNA-seq analysis demonstrated that this is the major SERPINF1 transcript in human stapes bone. Analysis of stapes from two patients with the 5'-UTR mutations showed that they had reduced expression of SERPINF1-012 All three 5'-UTR mutations are predicted to occur within transcription factor binding sites and reporter gene assays confirmed that they affect gene expression levels. Furthermore, RT-qPCR analysis of stapes bone cDNA showed that SERPINF1-012 expression is reduced in otosclerosis patients with and without SERPINF1 mutations, suggesting that it may be a common pathogenic pathway in the disease.

First results of a novel adjustable-length ossicular reconstruction prosthesis in temporal bones.

OBJECTIVES/HYPOTHESIS: The performance of an ossicular replacement prosthesis (ORP) is influenced by its alignment and appropriate tension between the tympanic membrane and the stapes footplate. A novel ORP with a flexible element that potentially allows for length adjustment in situ is presented and tested for acoustic performance. STUDY DESIGN: Laser Doppler vibrometry in fresh human cadaveric temporal bones was used to test the acoustic performance of the adjustable ORP relative to standard prostheses used for ossiculoplasty. METHODS: The three-dimensional (3D) velocity of the stapes posterior crus was measured in the 0.2- to 20-kHz range using a Polytec CLV-3D laser Doppler vibrometer. The middle ear cavity was accessed through a facial recess approach. After measuring the normal response, the incus was removed and stapes velocity was measured in the disarticulated case, then after insertion of the new prosthesis, a conventional prosthesis (Kurz BELL Dusseldorf type), and a sculpted autologous incus prosthesis in each temporal bone. The 3D stapes velocity transfer function (SVTF) was calculated for each case and compared. RESULTS: The novel ORP design restored stapes velocity to within 6 dB (on average) of the intact response. No significant differences in 3D-SVTF were found between the new, conventional, or autologous ORPs. CONCLUSIONS: The inclusion of an in situ adjustable element into the ORP design did not adversely affect its acoustic performance. The adjustable element may increase the ease of achieving optimal ORP placement, especially through a facial recess approach. LEVEL OF EVIDENCE: NA Laryngoscope, 126:2559-2564, 2016.

The pathophysiology of otosclerosis: Review of current research.

Otosclerosis is a complex disease of the human otic capsule with highest incidence in adult Caucasians. So far, many possible etiological factors like genetics, HLA, autoimmunity, viruses, inflammation, and hormones have been investigated but still the development of the disease remains unclear. Currently, the surgical replacement of stapes (stapedotomy) remains the best possible treatment option. In this review, we analyze different etiological factors studied so far in otosclerosis pathophysiology and discuss most recent findings and possible new research pathways.

Efficacy of pre-operative computed tomography evaluation of the tympanic cavity for hearing improvement after stapes surgery for tympanosclerosis with stapes fixation.

OBJECTIVE: Our aim was to determine if stapes surgery is useful for treating inflammatory ear diseases. MATERIALS AND METHODS: Thirteen patients underwent single-stage or staged surgery for stapes fixation due to tympanosclerosis alone or with cholesteatoma. Operative criteria were: no tympanic membrane retraction, perforation or adhesion; middle-ear cavity with aeration >1 year; a fixed stapes. Computed tomography was used to analyse the relation between operative success and pre-operative pneumatisation. RESULTS: Success rate at six months was 75 per cent. Hearing results were stable with little deterioration and no complications. Patients with poor pneumatisation had good results (with improved air-bone gap) only after staged surgery. Well-aerated ears heard better even with single-stage surgery. CONCLUSIONS: Pre-operative computed tomography and intra-operative findings are necessary to determine the pneumatisation status of tympanic mastoid cavities. If criteria approved, poorly pneumatised patients underwent staged surgery. Stapedectomy achieved good hearing results for inflammatory middle-ear disease with stapes fixation.