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Introduction Otosclerosis presents as conductive or mixed hearing loss depending upon the stage of the disease. Isolated sensorineural hearing loss though known to occur has been rarely reported. Pure cochlear otosclerosis presenting as sensorineural hearing loss should be considered as a differential diagnosis in young patients presenting with progressive hearing loss. The aim of this article is to note the prevalence of cochlear otosclerosis in young patients presenting with progressive sensorineural hearing loss. Materials and Methods Retrospective chart analysis of 19 patients who presented with progressive SNHL with unknown etiology was done. Results Otosclerosis involving the pericochlear region was diagnosed in 27 ears which was identified by high resolution CT scan of temporal bone. In four ears, in addition, there was demineralization just anterior to oval window without clinical evidence of stapes fixation. Conclusion In young patients with progressive SNHL, high resolution CT scan of temporal bone should be done to identify progressive cochlear otosclerosis. Medical management should be initiated in these patients to halt the progression of disease.

OBJECTIVE To investigate the effects of cavitating lesions involving the internal auditory canal (IAC) in subjects with cochlear otosclerosis with regard to poststapedotomy hearing outcome. STUDY DESIGN Retrospective study. SETTING Tertiary referral center. PATIENTS A retrospective chart review of 134 subjects with otosclerosis treated from January 2011 to June 2017 at Seoul National University Bundang Hospital was conducted. Sixteen subjects (23 ears) with temporal bone computed tomography (TBCT)-confirmed cochlear otosclerosis who underwent stapedotomy were included in the study. MAIN OUTCOME MEASURES Pure tone audiometry (PTA) (i.e., air and bone conduction; AC and BC, respectively) thresholds and air-bone gap (ABG), measured at 6 months postoperatively were compared between cochlear otosclerosis with and without IAC involvement (IAC group and non-IAC group, respectively). RESULTS A total of 14 of 23 ears showed involvement of the IAC. There were no significant differences in age, side of otosclerosis, or preoperative hearing threshold between the two groups. The mean postoperative AC and BC thresholds and ABG of the IAC group were significantly poorer (45.7 dB, 33.8 dB, and 11.8 dB, respectively) than those of the nonIAC group (24.1 dB, 20.0 dB, and 4.1 dB, respectively). CONCLUSIONS Cochlear otosclerosis with cavitating lesions involving the IAC showed significantly poorer postoperative audiological outcomes than those without any cavitating lesion. Cavitation extending to the IAC may act as a third window providing a route for sound energy shunting, and thus precluding successful hearing outcome in some subjects with cavitating otosclerosis after stapedotomy.

No cochlear otosclerosis in infants with congenital bilateral SNHL has been reported.We report an infant male with bilateral cochlear otosclerosis, suggesting that cochlear otosclerosis may be a congenital disease and to further analyze the etiology of and genetic expression in congenital bilateral cochlear otosclerosis. We also describe the clinical characteristics and experience of patients with bilateral cochlear otosclerosis treated with cochlear implants (CIs).Seven patients, including an infant, who were diagnosed with cochlear otosclerosis underwent CI surgery. Their medical records, audiological and radiological results, surgical procedures, and CI outcomes were collected and reviewed.The median age at hearing loss was 38 years, ranging from 0 to 47 years. The child had bilateral hearing loss at birth and received a CI at 1 year of age. He also had growth retardation and was diagnosed with 3q+/3p- syndrome. All patients (8 ears) had better postoperative auditory performance than that preoperatively.Although cochlear otosclerosis often starts at middle age and progresses slowly, it may be a congenital disease that is related to chromosome abnormality. This disease presents with SNHL or MHL, and treatment with a CI is beneficial.

Objectives: This is a qualitative study to explore the utility of gray-scale inversion or the “invert” function of high-resolution computed tomography (HRCT) scans in the diagnosis of temporal bone anatomy and pathology. Methods: This is a case series describing an innovative application of an existing image processing tool to visualize temporal bone anatomy and pathology. Illustrative patients at a tertiary referral center with otologic symptoms and findings leading to HRCT scans of the temporal bone were included. Diagnostic HRCT scans were evaluated utilizing the gray-scale inversion function (invert function). Results: Nine illustrative cases which demonstrate conditions such as persistent stapedial artery, membranous stapes footplate, total ossicular prosthesis migration into the vestibule, third window syndrome such as superior semicircular canal dehiscence (SSCD) and cochlea-facial nerve dehiscence, otosclerosis, and ossicular chain discontinuity are included. The enhanced visualization was confirmed surgically in 3 cases, and 1 had physiological confirmation using cervical vestibular evoked myogenic potentials (cVEMP). Conclusions: Gray-scale inversion can be used to improve visualization of temporal bone anatomy and pathologic changes when diagnoses are in doubt. The invert function is a useful adjunct in the armamentarium of both radiologists and otologists when evaluating HRCT of the temporal bone.

Hypothesis: Connexin-26 (Cx26) expression is diminished in the spiral ligament of subjects with hearing loss and cochlear otosclerosis (CO). Background: Human temporal bone (HTB) studies have demonstrated that CO is associated with hyalinization of the spiral ligament. We hypothesize that hyalinization is associated with a loss of fibrocytes with a consequent decline in Cx26 expression. Cx26 and Connexin-30 (Cx30) encode gap junction proteins expressed in supporting cells of the organ of Corti, the spiral limbus, stria vascularis, and in fibrocytes of the spiral ligament. These gap junctions are critical for potassium recycling and maintenance of the endocochlear potential. Diminished expression of these proteins would likely be associated with hearing dysfunction. Methods: Histopathology and clinical characteristics of 45 HTB specimens with CO and spiral ligament hyalinization were reviewed. Those with sensorineural or mixed hearing loss but normal or near-normal hair cell counts were analyzed with light microscopy, and Cx26-immunoreactive (IR) signal was qualitatively assessed. Results: H&E staining demonstrated hyalinization in the spiral ligament and loss of type II and type III fibrocytes. Cx26-IR was diminished throughout the cochlea affected with CO compared with normal controls. Conclusions: Cx26-IR reduction in the spiral ligament of subjects with CO likely plays a role in hearing loss.

Diagnostic audiologic procedures for otosclerosis are effective tools in identifying this condition. Audiometric data usually demonstrate a conductive hearing loss at the early stages of otosclerosis. Modern middle ear analysis procedures are becoming more popular in the better diagnosis of otosclerosis. In clinical practice, cochlear otosclerosis can also be observed. Audiologic rehabilitation of otosclerosis includes fitting of hearing aids and implantable hearing devices. Current hearing technology enables patients who do not pursue surgical correction to function well and significantly improve their communication and quality of life. Otosclerosis may also be associated with annoying tinnitus, and tinnitus management is important in the rehabilitation process.

Objective To assess long-term hearing outcomes in patients treated with third-generation bisphosphonates for otosclerosis-related progressive sensorineural hearing loss (SNHL). Study Design Retrospective case series review Methods We performed a retrospective case series review of patients with otosclerosis and progressive SNHL. Patients were treated with either risedronate or zoledronate after a diagnosis of otosclerosis with a significant SNHL component. Bone conduction pure tone threshold averages (BC-PTAs) and word recognition scores (WRS) before and after bisphosphonate administration in long-term follow-up was analyzed. Significant change in BC-PTA was defined as greater than 10dB or between 4% and 18% in WRS based on binomial variance. Results Seven patients were identified and 14 ears met inclusion criteria. Three patients were female and the mean age was 48.3 ± 10.3 years. The mean duration between treatment with bisphosphonate administration and long-term post-treatment follow-up audiometry was 87.6 ± 18.3 months, with a range of 61.6 to 109.1 months and median of 89.2 months. Analysis using BC-PTA and WRS demonstrated that 11 ears remained stable while 2 improved and 1 worsened. No patient experienced any major complication as the result of bisphosphonate therapy. Conclusion Treatment with third-generation bisphosphonates is associated with stability in otosclerosis-related sensorineural hearing over 5- to 9-year period. These results suggest that such medications may prevent the progression of SNHL in patients with otosclerosis. Level of Evidence 4 (Case series)

Background:Patients with advanced otosclerosis can present with hearing thresholds eligible for cochlear implantation. This study sought to address whether stapes surgery in this patient group provides a clinically significant audiological benefit.Objectives:To assess pre- and post-operative hearing outcomes of patients with advanced otosclerosis, and to determine what proportion of these patients required further surgery including cochlear implantation.Methods:Between 2002 and 2015, 252 patients underwent primary stapes surgery at our institution. Twenty-eight ears in 25 patients were deemed to have advanced otosclerosis, as defined by pure audiometry thresholds over 80 dB. The patients’ records were analysed to determine audiological improvement following stapes surgery, and assess whether any further surgery was required.Results:The audiological outcome for most patients who underwent primary stapes surgery was good. A minority of patients (7 per cent) required revision surgery. Patients who underwent cochlear implantation after stapes surgery (10 per cent) also demonstrated a good audiological outcome.Conclusion:Stapes surgery is a suitable treatment option for patients with advanced otosclerosis, and should be considered mandatory, before offering cochlear implantation, for those with a demonstrable conductive component to their hearing loss. A small group of patients get little benefit from surgery and subsequently a cochlear implant should be considered.

Bilateral vestibulopathy is a rare condition. It is one of the most difficult balance problems to treat because nearly all patients experience long-term unsteadiness. In this report, a 39-year-old woman gradually developing chronic dizziness and instability due to bilateral vestibulopathy as a result of progressive severe cochlear otosclerosis was presented with review of the literature.

Patients with chronic renal failure often suffer from hearing loss and the most common cause is sensorineural hearing loss. Sensorineural hearing loss can be caused by cochlear otosclerosis with early symptoms such as decreased petrous part of the temporal bone density due to narrowing of the internal auditory canal. Finding a correlation between the petrous part of the temporal bone density and the anteroposterior diameter of the internal auditory canal in sensorineural hearing loss in patients with chronic renal failure. An observational analytic, cross-sectional study, using a consecutive sampling technique. The petrous part of the temporal bone density decreased in patients with chronic renal failure. The anteroposterior diameter of the internal auditory canal remained normal, there was no association with sensorineural loss. There is a significant correlation between the petrous part of the temporal bone density and sensorineural hearing loss in patients with chronic renal failure. High-resolution CT scans of the mastoid can assist clinicians in determining cochlear otosclerosis and the subsequent detection of the early presence of sensorineural hearing loss.

Controversies have been associated with the etiology, diagnosis, evaluation, and management of otosclerosis since Valsalva first described stapes fixation as a cause of hearing loss. Although the exact mechanism of the bone remodeling associated with otosclerosis remains uncertain, stapedotomy has been accepted as the surgical treatment of most patients with stapedial otosclerosis. There remains a disparity of opinion, however, regarding the role of preoperative imaging, surgical technique, implant selection, and medical therapy for cochlear otosclerosis. In addition, opinions vary regarding the optimal postoperative care of patients undergoing stapedotomy and a patient's ability to participate in activities that may result in barotrauma.

Objective To assess long‐term hearing outcomes in patients treated with third‐generation bisphosphonates for otosclerosis‐related progressive sensorineural hearing loss (SNHL). Study Design Retrospective case series review Methods We performed a retrospective case series review of patients with otosclerosis and progressive SNHL. Patients were treated with either risedronate or zoledronate after a diagnosis of otosclerosis with a significant SNHL component. Bone conduction pure tone threshold averages (BC‐PTAs) and word recognition scores (WRS) before and after bisphosphonate administration in long‐term follow‐up was analyzed. Significant change in BC‐PTA was defined as greater than 10dB or between 4% and 18% in WRS based on binomial variance. Results Seven patients were identified and 14 ears met inclusion criteria. Three patients were female and the mean age was 48.3 ± 10.3 years. The mean duration between treatment with bisphosphonate administration and long‐term post‐treatment follow‐up audiometry was 87.6 ± 18.3 months, with a range of 61.6 to 109.1 months and median of 89.2 months. Analysis using BC‐PTA and WRS demonstrated that 11 ears remained stable while 2 improved and 1 worsened. No patient experienced any major complication as the result of bisphosphonate therapy. Conclusion Treatment with third‐generation bisphosphonates is associated with stability in otosclerosis‐related sensorineural hearing over 5‐ to 9‐year period. These results suggest that such medications may prevent the progression of SNHL in patients with otosclerosis. Level of Evidence 4 (Case series).

Rehabilitation of patients with sensorineural hearing loss is an urgent task of otorhinolaryngology. One of the diseases leading to a pronounced hearing loss is the cochlear form of otosclerosis. The article describes a clinical case of rehabilitation of a patient with this pathology by means of cochlear implantation. The classification of otosclerosis based on the interpretation of computer tomography of temporal bones is presented.Реабилитация пациентов с сенсоневральной тугоухостью является актуальной задачей оториноларингологии. Одним из заболеваний, приводящим к выраженному снижению слуха, является кохлеарная форма отосклероза. В статье представлена классификация отосклероза на основании интерпретации данных компьютерной томографии височных костей, описан клинический случай реабилитации пациента с данной патологией посредством кохлеарной имплантации.

The increased likelihood of facial nerve stimulation (FNS) with cochlear implantation in advanced cochlear otosclerosis is due to a lowering of the facial nerve excitation threshold with increasing bone demineralization.Facial nerve stimulation can complicate cochlear implant fitting, often necessitating the deactivation of certain electrode contacts.High-resolution computed tomographic scans were used to estimate anatomic features of the cochlea and the facial nerve canal. These features were added to a detailed computational model of the implanted human cochlea to examine the consequences of increased conductivity of the bone of the otic capsule. The model took into account the electrode contact type (banded or otherwise) and position (perimodiolar or lateral wall) of the electrode array.Contrary to the hypothesis, facial nerve thresholds were found to be slightly elevated with increased conductivity of the surrounding bone. However, the threshold and most comfortable loudness levels of the auditory nerve increase more rapidly owing to the reduced current density in the scala tympani as current leaks more easily out of the cochlea. Lateral wall electrodes were predicted to result in an increased likelihood of FNS. A progressively reduced probability of FNS was indicated for the full-band, half-band, and plated electrode arrays, respectively.The clinical observation of increased FNS in cases of cochlear otosclerosis has been demonstrated in a computational model. Rather than decreased FN threshold, it is the increased levels for cochlear stimulation that is the main factor. Particularly, perimodiolar designs with more shielding against lateral spread of current could reduce the likelihood of FNS.

Most cases of otosclerosis show pathological changes only around the oval window. On the other hand, it is known that cavernous changes extending to the cochlea induce progressive sensorineural deafness. Three patients with severe bilateral deafness and severe cochlear otosclerosis as diagnosed by CT underwent cochlear implantation. The postoperative hearing abilities were satisfactory in two of the three patients at one year after the operation. However, one patient with advanced otosclerosis suffered from the complication of facial nerve stimulation two months later after the cochlear implantation. His available cochlear implant electrodes gradually decreased due to the facial nerve stimulation and at last the cochlear implant was no longer beneficial for reacquisition of his hearing ability. His CT densitometry revealed marked degradation of the CT value in the cochlear basal turn circumference in comparison with that in other patients showing good courses after the cochlear implantation. In conclusion, a cochlear implant operation is valuable in patients with severe sensorineural deafness with cochlear otosclerosis. However, we should cautious in performing cochlear implantation in patients with severe demineralization of the cochlear optic capsule.

Introduction: Constant histological changes in otosclerosis lead to progressive hearing loss which may end up in a profound hearing loss and then be treated by means of cochlear implants. These progressive changes could be followed by changes in cochlear implants fitting and speech discrimination results over the years. Objectives: The aim of the study is to correlate the progressive histological changes to the cochlear implant clinical outcomes (fitting and speech discrimination results). Also main complications (facial nerve stimulation and difficulties at insertion) and new complications will be discussed. Design: A 5-year prospective case-control study was performed in order to compare cochlear implant results in otosclerosis patients to those in a matched-pair control group. Materials and Methods: Fifteen otosclerosis patients were followed throughout the study. Preoperatively temporal bone high-resolution computed tomography, electrically evoked auditory brainstem responses and speech discrimination tests were performed in order to select the patients to be implanted. Results: Not only difficulties with electrode guide insertion were reported, but also difficulties with fitting over the years, due to increasing difficulties to spread the electrical stimuli, which provokes increasing thresholds, maximum comfort levels and charges needed to stimulate hearing cells in basal and medial turn electrodes (p < 0.05), which required deactivating some basal and medial turn electrodes in order to improve cochlear implant effectiveness. The results demonstrated no statistical differences in speech discrimination in otosclerosis patients compared to the control group (p > 0.05). Several complications were reported: facial nerve stimulation (7.14%) and sudden episodes of tinnitus and headaches (14.28%). Conclusions: Although progressive histological changes in otosclerosis lead to increasing thresholds, maximum comfort levels and charges needed to stimulate hearing cells, speech discrimination results support the cochlear implantation in otosclerosis.

Local delivery of bisphosphonates results in superior localization of these compounds for the treatment of cochlear otosclerosis, without ototoxicity.Otosclerosis is a common disorder of abnormal bone remodeling within the human otic capsule. It is a frequent cause of conductive hearing loss from stapes fixation. Large lesions that penetrate the cochlear endosteum and injure the spiral ligament result in sensorineural hearing loss. Nitrogen-containing bisphosphonates (e.g., zoledronate) are potent inhibitors of bone remodeling with proven efficacy in the treatment of metabolic bone diseases, including otosclerosis. Local delivery to the cochlea may allow for improved drug targeting, higher local concentrations, and the avoidance of systemic complications. In this study, we use a fluorescently labeled bisphosphonate compound (6-FAM-ZOL) to determine drug localization and concentration within the otic capsule. Various methods for delivery are compared. Ototoxicity is evaluated by auditory brainstem responses and distortion product otoacoustic emissions.6-FAM-ZOL was administered to guinea pigs via intraperitoneal injection, placement of alginate beads onto the round window membrane, or microfluidic pump infusion via a cochleostomy. Hearing was evaluated. Specimens were embedded into resin blocks, ground to a mid-modiolar section, and quantitatively imaged using fluorescence microscopy.There was a dose-dependent increase in fluorescent signal after systemic 6-FAM-ZOL treatment. Local delivery via the round window membrane or a cochleostomy increased delivery efficiency. No significant ototoxicity was observed after either systemic or local 6-FAM-ZOL delivery.These findings establish important preclinical parameters for the treatment of cochlear otosclerosis in humans.

Objective This current single-subject, repeated-measures study was to describe our experience with 30 patients who had been diagnosed with "far-advanced otosclerosis" and who were included in our program of cochlear implants. We analyzed the history of the patients and their families before implantation, the surgical findings, and the performance over a follow up of 3 years. Material and methods All patients met one or more of the after criteria: 1) previous surgical intervention as a treatment of their otosclerosis; 2) signs of pericochlear hypodensities in high resolution computed tomography (HRCT) scans; and 3) family precedents of otosclerosis. All underwent standard surgical cochlear implantation. Results In 78% of the cases, a stapedectomy had previously been realized. Cochlear otosclerosis could be appreciated in HRCT in 78% of the patients. A family history of otosclerosis was found in 40%, and 33.3% of patients had familial precedents of nonfilial hypoacusis. The mean results in the two-syllable test were 20% preimplantation, 54% 6 months after implantation, and 52%, 62%, 54% at 1, 2, and 3 years after implantation. In the CID sentence test, they were in the order of 32% preimplantation and of 64% at 6 months, 66% after 1 year, of 68% after 2 years, and reaching 72% after 3 years. No complications related to the surgery were detected. Conclusion Patients diagnosed with far-advanced otosclerosis have a good prognosis with cochlear implantation comparable to that of other patients in whom postlingual implants are performed.

Objectif Decrire l’interet des differentes sequences IRM dans le cas d’une otospongiose a extension peri-cochleaire. Methode Realisation d’un scanner en coupes millimetriques axiales et coronales et d’une IRM en sequences T1, T1 avec injection de Gadolinium et T2. Confirmation par une exploration chirurgicale de l’ankylose stapedo-vestibulaire bilaterale. Resultats Le scanner met en evidence un aspect de pseudo quatrieme tour de la cochlee. Celui-ci apparait en IRM en signal intermediaire en T1 et en hyper signal en T2. Ce foyer est rehausse apres injection de gadolinium, ce qui est en faveur d’une lesion active. Le scanner montre un autre foyer en regard de la cochlee qui atteint l’endoste mais qui n’est pas visible en IRM, meme apres injection de Gadolinium, ce qui traduit une lesion inactive. Discussion Analyse des donnees de la litterature concernant le scanner et l’IRM du labyrinthe et les resultats obtenus dans l’otospongiose. Conclusion L’IRM du labyrinthe et notamment les sequences T1 avec injection de gadolinium pourraient etre d’un interet diagnostique dans l’otospongiose pour differentier les lesions actives des lesions inactives. Cependant des etudes prospectives semblent necessaires afin de confirmer cette hypothese.

Background: The literature provides conflicting information regarding the prevalence and cause of sensorineural hearing loss (HL) in individuals with otosclerosis. Objective: The purpose of this study was to further evaluate the association between involvement of the cochlear endosteal layer with otosclerosis and sensorineural HL. Study Design: Retrospective case review. Methods: Temporal bones and audiograms from five individuals with otosclerosis were evaluated. The cochlear elements were quantified. The location and extent of the cochlear element loss was correlated with the location and extent of endosteal involvement with otosclerosis. Results: A reduction in the population of cochlear elements was observed in most individuals; however, the reduction was not proportional to the extent of endosteal involvement with otosclerosis. The cochlear elements remained normal adjacent to some areas of endosteal involvement with otosclerosis. One individual with extensive cochlear otosclerosis had normal hearing and predominantly normal cochlear elements. Conclusion: This study demonstrates a variable amount of degeneration of the cochlear elements in individuals with otosclerotic involvement of the endosteum. The reduction in the population of cochlear elements was not related to the extent and location of endosteal involvement with otosclerosis. These findings suggest that factors that limit the effect of otosclerotic endosteal involvement on the cochlear elements or processes that effect the cochlear elements directly and are independent of bone involvement may be present.

Background: Results after cochlear implant surgery may be complicated by postoperative facial nerve stimulation. Aim of the study presented was to evaluate postoperative results in implanting the straight Nucleus® electrode array and the preformed Contour array in patients with deafness due to cochlear otosclerosis. Methods: A retrospective analysis of intra- and postoperative reports of all patients with cochlear otosclerosis was carried out. Results with the Nucleus® straight electrode array and the Contour" array were compared with regard to postoperative facial nerve stimulation. Results and Conclusion: None of the Contour patients (n = 7) presented with postoperative facial nerve stimulation. This is in contrast to the majority (4 of 6) of patients being implanted with the straight electrode array. Our results indicate that the use of the Contour array is advantageous in patients being at risk for facial nerve stimulation. In addition intraoperative reports suggest a more reliable insertion of the Contour electrode array in cochlear otosclerosis with partial obliteration.

Objectives:Usually, the diagnosis of otosclerosis is based on clinical and audiometric findings, and its confirmation requires surgical exploration of the middle ear. More recently, it has been suggested that high-resolution computed tomography of the temporal bone (TTB) could diagnose and classify otosclerosis into fenestra otosclerosis (FO) and retrofenestral or cochlear otosclerosis (RFO). However, there are relatively few studies that relate these tomographic features with clinical and surgical findings. The aims of the study were to: (1) classify otosclerosis patients with preoperative TTB into fenestra or retrofenestral otosclerosis, and (2) compare these image findings with pre- and postoperative auditory thresholds and epidemiological findings.Methods:Retrospective study by reviewing medical records of otosclerosis patients (2006-2011) undergoing estapedostomy in the Clinical Hospital of the University of Chile (tertiary institution), who were evaluated with preoperative TTB and had full audiometr...

Objective: Quantify and illustrate the location of otosclerosic foci using morphologic images and 3D reconstruction. Evaluate the impact of multiple otosclerotic foci on hearing loss.Method: We investigated our histopathological collection to identify all the temporal bones with: 1) oval window otosclerosis (n = 200), 2) oval window and multiple otosclerotic foci (n = 35), 3) oval window plus cochlear otosclerosis (n = 133), and 4) oval window foci with cochlear and multiple other otosclerotic foci (n = 31). Foci were tabulated for each specimen. Cochlear function (hearing) and morphology (otosclerotic foci quantity and location) were compared.Results: Of 574 temporal bones with evidence of otosclerosis, 67 had multiple foci. Ectopic foci were found in the internal auditory canal, the peri-carotid area, semicircular canals and anterior to the cochlea. There was no influence of the number of foci (not involving the cochlea) on hearing loss and cochlear morphology.Conclusion: This is the first systematic ev...

A 51-year-old man had progressive hearing loss over more than 15 years. He had bilateral sensorineural hearing loss (SNHL). Computed tomography (CT) showed extensive bilateral demineralization of the cochlear capsule, which is characteristic of diffuse cochlear otosclerosis. Three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) of magnetic resonance imaging before enhancement revealed high signals in the cochlea and vestibule. Postcontrast 3D-FLAIR revealed enhancement of the basal turn of the left cochlea. This is the first published case of the breakdown of the blood-labyrinth barrier in a patient with cochlear otosclerosis. Our findings suggest that the breakdown of the blood-labyrinth barrier is associated with a part of SNHL in cochlear otosclerosis.

Otosclerosis is a disease of the human otic capsule1. Otosclerosis is well known to the otologist, as it is the most common cause for progressive conductive hearing loss in early adulthood. As hearing loss progresses, a sensorineural component is sometimes seen, resulting in a mixed hearing loss. In 1% of cases the disease is considered purely cochlear, presenting with a sensorineural hearing loss only, as the otosclerosis involves areas other than the footplate1.

OBJECTIVE The objective of this study was to discuss cochlear implantation in the setting of severe cochlear otosclerosis and review programming challenges in a patient whose performance is deteriorating as a result of advancing disease. STUDY DESIGN We conducted a case report and literature review. SETTING Tertiary care medical center. PATIENTS, INTERVENTION, AND RESULTS: A case is presented of a 66-year-old man with otosclerosis who initially had good benefit from a cochlear implant but gradually lost benefit even with reimplantation. Imaging studies demonstrated severely distorted otic capsule anatomy from cochlear otosclerosis. CONCLUSIONS Advancing cochlear otosclerosis can result in a severely thinned and distorted otic capsule. Although cochlear implantation is generally beneficial in cochlear otosclerosis, implantation in certain severe cases may be complicated as a result of difficulties with cerebrospinal fluid leak, programming challenges, and other potential hazards that can occur with a distorted anatomy.

1. The Historical Background of Otosclerosis 2. The Pathology of Otosclerosis 3. Physical Examination and Clinical Evaluation of the Patient with Otosclerosis 4. Audiological Evaluation of the Patient with Otosclerosis 5. Radiological Imaging of Otosclerosis 6. Cochlear Otosclerosis 7. Medical Treatment of Otosclerosis 8. Hearing Aids and Otosclerosis 9. Lasers in Otosclerosis 10. Stapedectomy 11. Obliterative Otosclerosis 12. Poststapedectomy Perilymph Fistulae 13. Sudden Sensorineural Hearing Loss Immediately Following Stapedectomy 14. Stapedectomy versus Stapedotomy 15. Bilateral Otosclerosis 16. Special Conditions and Complications in Otosclerosis Surgery 17. Otodystrophies Appendix: Quiz

A 64-year-old Japanese male patient, status post-stapedectomy, presented with progressive, bilateral SNHL of 30 years' duration. CT showed extensive demineralization of the otic capsule, suggestive of otospongiotic change in both ears. There were no hyperostotic lesions found, which would have been suggestive of otosclerotic change. MRI showed a morphologically intact membranous labyrinth in both ears, with no obstruction of the cochlear lumen. It was concluded that the patient has progressive otospongiotic change of the otic capsule, but no otosclerotic change.

Bilateral sensorineural hearing loss can be caused by a variety of temporal bone abnormalities including primary cochlear otoscierosis, local and systemic bony diseases and some metabolic conditions. These may be identified using computerized tomography (CT), with attenuation recordings taken across the cochlear capsule (CT densitometry). Eighty patients with bilateral sensorineural hearing loss were screened over a period of six and a half years using this technique, and only three cases (3.8 per cent) of treatable disease were detected. Positive yields may be increased by screening selected cases with other clinical or biochemical stigmata of temporal bone disease.

The temporal bones of 40 patients with surgically confirmed otosclerosis were studied with high-resolution CT. The hearing of these patients was re-examined 5 years later. Foci of demineralization in the cochlear capsule were detected in 36% of operated ears and in 44% of otosclerotic ears not operated on. Mean bone conduction (BC) thresholds of ears with positive radiological findings did not differ significantly from those of otosclerotic ears with normal CT, either at the time of CT examination or 5 years later. Over 5 years, elevation of BC thresholds by 10 dB or more was observed significantly more often in operated ears with abnormal CT than in operated ears with normal CT, but in otosclerotic ears not operated on the corresponding difference was non-significant. It was concluded that CT examination is of little value in predicting sensorineural hearing loss in patients with otosclerosis.

To the Editor .—It is somewhat intriguing to read a letter to the editor in one journal complaining about an article printed in another publication. Yet that is what George E. Shambaugh, Jr, has done in writing his letter in the October 1990 issue of theArchivesconcerning his continued use of sodium fluoride therapy for otosclerosis as a rebuttal to a commentary written by Drs Gail Kerr and Gary Hoffman 1 in the June 1989 issue of the Ear Nose Throat Journal . As Chief Editor of the Ear Nose Throat Journal , and the person responsible for the publication of the article by Kerr and Hoffman 1 in the Journal , I have chosen to respond to Dr Shambaugh's remarks, since a response is very much warranted. Over the past years, Dr Shambaugh has promulgated the use of fluoride therapy for treating cochlear otosclerosis or otospongiosis and has encouraged other clinicians

This paper presents an analysis of the relationship of the amount of hyalinization of the spiral ligament, secondary to cochlear otosclerosis (spongiosis), to the amount of hearing loss. This relationship was previously studied by Parahy and Linthicum. It was found that the larger the amount of hyalinization, the greater the hearing loss. This hyalinization is a measure of the amount of toxic enzymes being excreted into the inner ear fluid. These enzymes are suspected to affect the metabolic function of the hair cells.

The mixed form of otosclerosis, in various age groups, involves the processes at the region of the oval window and stapes as well as of the cochlear bone capsule. In most cases roentgenograms aid in the diagnosis.

The purpose of this paper is to describe the audiologic techniques currently used at the Otologic Medical Group, Inc. Auditory threshold in children is determined by a combination of behavioral and objective audiometric techniques. When behavioral techniques fail, auditory brainstem response audiometry combined with impedance audiometry gives a good estimate of hearing thresholds. Impedance audiometry is a valuable addition to the diagnosis of cochlear otosclerosis. This technique is also of benefit in the neurotologic evaluation. Auditory brainstem response audiometry is the most accurate method of detecting an acoustic tumor. We no longer use SISI, tone decay, or Bekesy tests. The newer audiometric studies have greatly improved our evaluation of the auditory system.

Studies of clinical, audiometric and histological defects in patients with conductive otosclerosis, demonstrated the occurrence of subclinical manifestations in the hair, nails, joints and teeth. Light-microscopic examination of the skin in these patients has shown metachromasia of the ground substance and degenerative abnormalities in the collagen and elastic fibres. Structural abnormalities, in the organs developing from the ectoderm and mesoderm, led to the conclusion that otosclerosis is a universal disease characterized by ecto-mesodermal insufficiency, a somatic insufficiency. Optical measurement of the corneal thickness by corneometry has been introduced as an objective method for the diagnosis of somatic insufficiency. This method, now used routinely in our clinic, shows a high frequency of abnormal corneal thickness among patients with otosclerosis. Such a person is termed ‘cornea-positive’. Cochlear otosclerosis with pure sensorineural hearing loss has been studied by X-ray in axial and semiaxia...

Ninety patients who had suspected or confirmed fenestral or cochlear otosclerosis underwent CT examination. Foci of demineralization in the otic capsule were discovered in 20 ears (12 patients). Audiometric studies of the 12 patients revealed sensorineural hearing loss (SNHL) with distinct correlation of CT findings with progressivity and with involvement of the frequency level subtended by the specific area of the cochlea involved. Foci of abnormal increased density, presumably representing the healed phase of this disorder, were found less frequently than expected. There was a predilection for the basilar turn. All patients had static SNHL in the higher frequencies. The healed phase of this disorder is probably not consistently diagnosable with CT.