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PubMed1982

Sensorineural hearing loss and somatic insufficiency.

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 ectomesodermal 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 semiaxial tomography. These patients were often X-ray positive and among them there were many cornea-positive individuals. Cases with noise-induced hearing impairment may also be cornea-positive, i.e. they may exhibit, somatic insufficiency indicating that their hearing organs are characterized by a decreased resistance to noise. Corneometry has shown its value in the diagnosis of insufficient somatic constitution typical of individuals at risk.

Cornea↗

PubMed2001

Short- and long-term results of stapedectomy in children.

Otosclerosis in children and teenagers is very uncommon. Typical otosclerosis presents as a slowly progressive conductive hearing loss and tinnitus in the third to fourth decade of life. Stapedectomy is a well-established procedure for correction of conductive hearing loss in adult patients with otosclerosis. The aim of this work is to assess short- (6 months) and long-term (7 years) results after stapedectomy performed in 10 patients under 20 years of age. In 1 patient cochlear otosclerosis was diagnosed and in 1 patient incus anomaly was found during the operation. Pure tone audiograms obtained 6 months after stapedectomy showed air-bone gap closure to 10 dB in 6 patients, to 15 dB in 8 patients and in all patients to 20 dB. Average air conduction threshold improved by 26.5 dB and average bone conduction threshold by 5.9 dB. 7 years after stapedectomy air-bone gap was similar to the one measured 6 months postoperatively. Stapedectomy in children provides good and stable hearing results.

Adolescent↗

PubMed2004

Use of Remifentanil for sedo-analgesia in stapedotomy: personal experience.

Stapedotomy for otosclerosis presents particular anaesthesiology demands as the surgeon has to assess functional results during the operation, work with some bleeding, be ensured the collaboration of the patient, and limit the occurrence of intra- and post-operative symptoms (dizziness, nausea, vomiting and pain). Remifentanyl, a micro-opioid selective agonist characterised by short latency and duration, has been used for about 2 years at the Otolaryngological Unit of the "Federico II" University of Naples for patients with otosclerosis undergoing stapedotomy. Aim of the study was, therefore, to assess: efficacy and tolerability of Remifentanyl in combination with a local anaesthetic in surgical procedures for otosclerosis; intra- and post-operative reduction in patient symptoms of dizziness, nausea, vomiting and pain; reduction of intra-operative bleeding; degree of patient collaboration and optimisation of anaesthesiological and vital parameters monitored during surgery. The study was carried out on 92 patients with otosclerosis, (17 M, 75 F), median age 41 years (range 25-56), undergoing stapedotomy. Patients were randomly assigned to one of two groups, which were homogeneous as far as concerns age, sex and pre-operative hearing: i. Group A (50 patients), received Remifentanyl infusion in combination with canal injection for local anaesthesia with Mepivacaine 2% and Adrenalin 1/100,000; ii. Group B (42 patients), received only local anaesthetic by infiltration of the external canal ear. Remifentanyl led to an improvement over the local anaesthetic technique previously used, with a clear decrease in intra- and post-operative neurovegetative symptoms such as dizziness, nausea, vomiting and pain, as well as reduced bleeding.

Adult↗

PubMed1978

Incidence of Ménière's disease.

The incidence of Ménière's disease in Sweden for the year 1973 has been calculated as one case in a population of 2,163. This gives an overall incidence of 46/100,000. Extrapolating the Swedish incidence data to the population of the United States, there should have been 97,000 cases of Ménière's disease treated as inpatients or outpatients during the year 1973. The incidence in the United States of Ménière's disease, cochlear type, is considered to be even higher. In addition, the Swedish statistic for some other disorders, including otosclerosis, are reported. For otosclerosis, the yearly incidence in Sweden is 12/100,000 or one case in a population of 8,414. This could be extrapolated to 25,000 patients with clinical otosclerosis in the United States for the year 1973. By comparison, Ménière's disease, by stringent diagnostic criteria, is approximately four times as common as clinical otosclerosis. It is also more common than all laryngeal carcinomas, all salivary gland tumors, both benign and malignant, as well as acute epiglottitis and acute nephritis.

Adolescent↗

PubMed1999

Stapedectomy in children.

Stapes surgery for correction of conductive hearing loss in adults with otosclerosis is a well-established procedure. Its effectiveness in children, however, has received less scrutiny in the literature. Previous studies from our and other institutions demonstrated similar results in children and adults. Between 1980 and 1994 stapedectomies were done on 95 ears of 81 patients younger than 18 years (83 primary and 12 revisions). Data regarding age of onset, family history, associated anomalies, surgical findings, technique, hearing results, and complications were reviewed. Two groups were identified: congenital stapedial fixation and juvenile otosclerosis. Patients with congenital stapedial fixation had an earlier onset of hearing loss (3 vs 10 years, P < 0.001), a greater incidence of abnormalities of the malleus and incus (25% vs 3%, P < 0.001), and a slightly greater preoperative air-bone gap (35.2 +/- 12.9 vs 27.8 +/- 8.9, P = 0.002). Patients with otosclerosis had a greater frequency of a positive family history of deafness (53% vs 10%, P < 0.001). Overall, 79% of primary cases and 89% of revision cases had an improvement in hearing, with mean postoperative air-bone gaps of 15 dB and 22 dB, respectively. The gap did not widen significantly during the entire length of follow-up (mean 72 months). In primary cases, 59.1% obtained a postoperative air-bone gap of 10 dB or less. Eighty-two percent of children operated on for otosclerosis obtained excellent results (postoperative air-bone gap < or = 10 dB), compared with only 44% of children with congenital stapedial ankylosis (P = 0.02). In revision surgery, 29% of children obtained excellent results. Poorer results in both cases of congenital stapedial fixation and revision stapedectomy appear to be related to the greater incidence of associated anomalies of the malleus and incus. Indications, technique, complications, and considerations pertinent to childhood stapedectomy are discussed.

Audiometry↗

PubMed1997

[Differences in temporal bone development in various ear diseases].

To investigate the differences in temporal bone development in various ear diseases--bilateral Meniere's disease (n = 13), unilateral Meniere's disease (n = 41), unilateral chronic otitis media (COM) (n = 25), temporal bone fracture (n = 9) and otosclerosis (n = 12)--the following 4 distances on a temporal bone CT slice encompassing the lateral semicircular canal (LSC) were measured by using the NIH Image program. These distances included the minimal distance between the posterior semicircular canal (PSC) and the posterior petrous surface (PPS) (P-P distance), the minimal distance between the PSC and the LSC (P-L distance), the minimal distance between the vestibule and the PPS (V-P distance) and the minimal distance between the PSC and the anterior margin of the sigmoid sinus (P-S distance). Both the P-P and V-P distances showed significant differences among the ear diseases, but the P-L and P-S distance did not. Meniere's disease showed significantly shorter P-P and V-P distance especially in bilateral Meniere's disease than those in COM and otosclerosis (p < 0.05). Affected ears in unilateral Meniere's disease showed a shorter P-P distance than non-affected ears (p < 0.01). In contrast, both ears with otosclerosis showed significantly longer P-P and V-P distances than those in Meniere's disease, COM (p < 0.01) and temporal bone fracture (p < 0.05). COM and temporal bone fracture showed intermediate P-P and V-P distances, without a difference between the affected and non-affected ears. In conclusion, the development of the posterior part of the temporal bone is reduced in Meniere's disease and greatly increased in otosclerosis. These findings by CT may be useful for diagnosing ear diseases.

Ear Diseases↗

PubMed2003

Bone and air conduction after stapedectomy.

UNLABELLED: Otosclerosis causes loss of air conduction (AC) but also bone conduction (BC) may be deteriorated. It is reported that also BC may improve after surgical treatment of otosclerosis. The investigation was carried out in a group of 50 patients treated due to otosclerosis in our institution. The group included 34 women and 16 men, at the age 24 to 55 years. Pure-tone audiometry for bone and air conduction threshold was performed in all the patients. Hearing tests were carried out before and 2-4 months after the operation by the same technician Partial. Only patients with air-bone gap closure were included in the study. Stapedectomy was performed under local anaesthesia. RESULTS: Significant improvement of AC was obtained after stapedectomy. There was also significant improvement of BC at all the frequencies examined of 6-12 dB and it depended on BC threshold before surgery. In the group of 37 patients with BC threshold before surgery > or = 30 dB a significant improvement of BC after stapedectomy developed. In the group of 14 patients with BC threshold before surgery < 30 dB the change of BC after stapedectomy was not significant. Successful stapedectomy improves bone conduction. It means that in patients with otosclerosis there is a reversible BC pseudo hearing loss and it is related to BC threshold before the operation.

Adult↗

PubMed1977

Surgery and histopathology of the stapes in osteogenesis imperfecta tarda.

Fourteen stapedectomies were performed on ten patients with osteogenesis imperfecta tarda. Clinically, the footplates were softer and more tenuously fixed than in otosclerosis and floating footplates seemed more common. Stapes and stapes fragments from all 14 operations were examined microscopically. The histopathology of osteogenesis imperfecta was contrasted to that of otosclerosis. We believe that the greater degree of structural disorganization and greater area occupied by resorption spaces clearly distinguish the new bone formed in osteogenesis imperfecta from otosclerosis.

Adolescent↗

PubMed2006

Stapedectomy in congenital stapes fixation: are hearing outcomes poorer?

OBJECTIVE: To evaluate outcomes of stapedectomy surgery for congenital stapes fixation. STUDY DESIGN: Retrospective chart review. METHODS: The charts of 463 stapedectomies performed by 1 surgeon from 1996 to 2003 were reviewed. Patients with a history of childhood hearing loss and operative findings consistent with congenital fixation of the stapes were included. Patients with a history of trauma or chronic otitis media and those with otosclerosis were excluded. Thirty-six patients underwent stapedectomy for congenital fixation. Revision cases and those with inadequate postoperative bone or air conduction data were excluded. Inclusion criteria were met for 25 patients. Operative findings and hearing outcomes were evaluated. RESULTS: The study group was comprised of 25 stapedectomies. Closure of the air-bone gap to less than 10 dB was achieved in 48% of cases, and 80% had closure within 20 dB. A gap of more than 30 dB remained in 3 cases. There were 2 cases of sensorineural hearing loss with worsening of the bone conduction thresholds by 15 dB and 30 dB. There were no instances of perilymph gusher. In addition to an excluded case that was not reconstructed because of facial nerve position, 3 of the included patients were found to have a dehiscent facial nerve at surgery. There were no facial nerve injuries, and fixation of the malleus or incus was not found in any of the included patients. When hearing outcomes were compared with our results for otosclerosis over the same time period, rates of closure to within 10 dB and 20 dB were significantly worse in the congenital group. CONCLUSION: Closure of the air-bone gap in this population differs from our results in stapedectomy done for otosclerosis. This difference likely reflects subtle anatomic variations in the congenital group that affect the effectiveness of the prosthesis. Despite the difference in results, stapedectomy for congenital fixation remains an effective method to achieve significant hearing improvement in the majority of patients. EBM RATING: B-3b.

Adolescent↗

PubMed1975

Otosclerotic obliteration of oval window niche: an analysis of the results of surgery.

1. The massive otosclerotic focus, obliterating the oval window niche, has a relatively high case incidence of 11-2 per cent in South Australia. The three classes of obliterated footplate are defined. 2. Basic data of 109 consecutive surgical cases of obliterative otosclerosis are given. A brief description of operative techniques--vein graft and polyethylene tube in nine, and piston technique in 100--is given and the difficulties and complications arising at or after surgery are discussed. 3. Post-operative follow-up of patients with yearly audiometric assessment has been achieved in 105/109 (= 96-33%) at four years, and in 94/109 (= 86-26%) at five years. 4. The results of surgery are presented by many and varied methods including bar diagrams, post-operative hearing gains, post-operative bone-air gaps, speech discrimination studies and standard statistical analysis techniques. 5. The vein graft technique (nine cases) is very much inferior to the piston technique. Vein graft cases had a 33% incidence of profound sensori-neural loss due to bony reclosure of the window. 6. The piston technique (100 cases) gave hearing gains of 20 decibels or more in 91%, 30 decibels or more in 71%, and 40 decibels or more in 41% of cases at five years post-operatively. The bone-air gap at five years post-operatively was diminished to 20 decibels or less in 88%, to 15 decibels or less in 85%, to 10 decibels or less in 77%, and complete closure or over-closure occurred in 52% of the patients. 7. Data analysis has established there is no change with time of the post-operative mean bone-air gap 500--2,000 Hz. over a period of at least five years, and thus that the piston operation gives a persisting and stable hearing result. 8. In predicting the effect of the piston operation only a rough guide can be obtained from other variables. In particular bone hardness, mucosal characteristics, sex and piston diameter appear to be irrelevant. Patients with a large bone conduction reading or a large air-bone gap appear to do slightly worse than patients with smaller values for these variables. There are no grounds for excluding a patient from operation on account of age, or the finding of active otosclerosis (soft bone and thickened vascular muco-periosteum). 9. From the data of piston operations, the estimated mean bone-air gap for the five-year examination period was 4-14 db. with a standard deviation of 12-5 db. 10. The piston operation is highly recommended as a safe and suitable method of alleviating, in the long-term, the conductive hearing loss of patients with obliterative otosclerosis of the oval window.

Adolescent↗

PubMed1988

Thirty years of stapes surgery.

The modern stapedectomy with prosthesis insertion and living oval window seal, like the modern cataract extraction with lens replacement, is now performed, very much the same, throughout the world. I have reviewed the evolution of stapes surgery during these last thirty years and tried to gain some agreement for several important facts about otosclerosis and several basic principals of stapes surgery. While a well-performed stapedectomy can eliminate the conductive component, the sensorineural hearing loss continues and, in about one-third, will progress till the patient, after age 65, must return to a hearing aid. A piston prosthesis gives the best hearing results: 0.6 mm diameter, when half the footplate is removed and a living oval window seal interposed, and 0.6 mm diameter when a small opening is made in the footplate obliterated by otosclerosis. I prefer a teflon prosthesis to stainless steel because it can be altered by the surgeon at operation, and vein as an oval window seal. I have presented a rare group of patients who develop facial palsy 5-1/2 days after uncomplicated stapedectomy, of whom all recover quickly and completely. I am confident that progress will continue to be made in the understanding of otosclerosis, and the performance of stapes surgery, but in these last thirty years we have made a good beginning.

Aged↗

PubMed2000

Predilection of otosclerotic foci related to the bone turnover in the otic capsule.

Using multiple fluorochrome tagging of eight mongrel dogs and a newly established methodology of measuring bone turnover (BTO) in the otic capsule, it has previously been demonstrated that BTO in the canine otic capsule is highly reduced close to the perilymphatic spaces (PLS) compared to the normal level of BTO in the periphery. Reanalysis of these data shows that this inhibition of BTO is far more pronounced around the cochlea and vestibule than around the semicircular canals. Similar tendencies are seen for the numerical density and mean label area of the bone remodelling units. With increasing distance to the PLS, these patterns are weakened, but still recognizable. In otosclerosis, foci of abnormal bone deposition are particularly frequent around the oval and round windows and in the cochlear capsule, i.e. where inhibition of bone remodelling is most prominent. A surpassing (or failure) of this pronounced inhibition must precede the tumultuous but delimited osteogenesis of otosclerosis. Otosclerosis may be a deviation of the normal BTO process in an osteometabolically abnormal site, perhaps initiated as an osteogenetic response to abnormal stress exposure.

Animals↗

PubMed2004

Effect of 17 beta-estradiol on diastrophic dysplasia sulfate transporter activity in otosclerotic bone cell cultures and SaOS-2 cells.

OBJECTIVE: Diastrophic dysplasia sulfate transporter (DTDST) is involved in the regulation of bone turnover, and its activity in otosclerosis has been shown to be abnormally high. Taking into account the role of estrogens in the progression of otosclerosis, the possible effect of estrogens on DTDST was investigated in otosclerotic bone cell cultures and in SaOS-2, a human osteoblastic cell line. MATERIAL AND METHODS: Primary bone cell cultures of stapes and external auditory canal (EAC) bone were obtained from 33 patients with otosclerosis and 18 control patients undergoing cerebellopontine angle tumor surgery. These cultures were assessed in parallel with SaOS-2 cells. Estrogen receptors (ERs) were detected using reverse transcriptase polymerase chain reaction. DTDST activity was assessed by sulfate uptake at baseline and after 24 h of incubation with 17 beta-estradiol at concentrations ranging from 10(-12) to 10(-6) M. RESULTS: Stapes and EAC cultures predominantly expressed mRNA of ER alpha, while ER beta expression was predominant in SaOS-2 cells. In stapes and EAC cultures no modification of DTDST activity was observed with 10(-8) M 17 beta-estradiol. In SaOS-2 cells, DTDST activity was inhibited by 17 beta-estradiol (93.5+/-9.21 vs 83.6+/-8.83 pmol/mg protein/5 min, n=29; mean of differences=10.0+/-3.22, paired t-test, p<0.01). CONCLUSION: DTDST activity is regulated by estrogens in SaOS-2 cells, but not in primary cell cultures from stapes and EAC. This difference in the regulation mechanisms may be related to the type of estrogen receptor expressed.

Adult↗

PubMed2000

Multifrequency multicomponent tympanometry in normal and otosclerotic ears.

A multifrequency multicomponent admittance meter was used to evaluate 70 ears of patients affected by fenestral otosclerosis (Os ears), monolateral (16 cases) or bilateral (27 cases). The 16 contralateral ears of the patients with monolateral otosclerosis who presented a pure-tone air-bone gap less than 10 dB were evaluated separately (Cos ears). A group of 48 ears belonging to 24 otologically normal subjects (N ears), with hearing thresholds better than or equal to 10dB HL in the frequencies between 250 and 8000 Hz served as a control group. The purpose of the study was to evaluate the acoustic admittance characteristics of the three groups of ears, with particular regard to the parameters represented by the resonance frequency (RF), the acoustic conductance value (G) at RF and the individual interaural differences in these two parameters in the N and Cos groups. The degree to which fenestral otosclerosis can influence variations of RF and the correlation between the value of RF and conductive hearing loss in patients with clinically confirmed pictures and in the controlateral ears in the cases where the disease was clinically unilateral were also investigated. The study reveals statistically significant differences between the RF means in the N group (1085 +/- 244Hz) vs the Os group (1264 +/- 320 Hz) (p < 0.001) and between the G means in the N group (5.33 +/- 1.72 mmhos) vs the Os group (4.46 +/- 2.54 mmhos) (p = 0.04) and N group vs Cos group (3.42 +/- 2.27 mmhos) (p < 0.001). No correlation was found between the value of RF and conductive hearing loss. This study also shows how prognostic value may also be attributed to conductance at middle-ear pressure balancement: extremely low values for this parameter at RF are indicative of initial otosclerotic involvement of the oval window.

Acoustic Impedance Tests↗

PubMed1995

Value of computed tomography in patients with persistent vertigo after stapes surgery.

RATIONALE AND OBJECTIVES: The value of computed tomography was evaluated in 14 patients with persistent vertigo after otosclerosis operation. METHODS: High-resolution computed tomography was performed with 1-mm slice thickness and table feed in the axial and coronal planes. RESULTS: Computed tomography scans showed in 13 case findings that related to symptoms. An air bubble at the end of the prosthesis as a new, indirect sign of a perilymphatic fistula was found in 6 cases. An incorrect position of the stapes prostheses was diagnosed in 7 patients. One patient had recurrence of otosclerosis, and two others developed scarring around the prosthesis. In 1 case, an incus necrosis was found. Some patients showed two signs simultaneously. The computed tomography results were confirmed by retympanotomy in 12 cases. CONCLUSIONS: Computed tomography proved to be a valuable method in the diagnosis of persistent vertigo after otosclerosis operation. The indication for a repeat operation was facilitated by the use of computed tomography.

Follow-Up Studies↗

PubMed1998

Short- and long-term results of stapedectomy in children.

Studies have indicated that stapedectomy can be an effective procedure in children for correcting conductive hearing losses due to juvenile otosclerosis. However, because childhood otosclerosis is rare and children commonly choose to use hearing aids in lieu of undergoing surgery, little outcome data are available. The purpose of this retrospective study was to provide additional outcome data in both the short and the long term. Stapedectomies were performed on 47 children. Preoperative hearing results were compared with 6-month postoperative hearing results. Hearing results for the children who had long-term follow-up (5 years or more) were compared with the 6-month postoperative results. Stapedectomy was successful (postoperative air conduction pure-tone average [PTA] within 10 dB of the preoperative bone conduction PTA) in 91.7% of the cases. The mean overclosure of the preoperative bone conduction PTA by the postoperative air conduction PTA was 0.2 dB. The mean PTA hearing improvement was 32.8 dB. Results from the 21 children (28 ears) who had long-term follow-up indicated an average 0.7 dB/year PTA worsening from the 6-month postoperative PTA. Results from this study provide additional evidence that stapedectomy can be an effective procedure for correcting conductive hearing losses due to juvenile otosclerosis.

Adolescent↗

PubMed2003

Predictive factors in pediatric stapedectomy.

OBJECTIVE/HYPOTHESIS: The objective of the study was to investigate predictive factors in the postoperative hearing outcomes in pediatric stapedectomy. STUDY DESIGN: Retrospective case series. The study was performed in a tertiary academic otological practice. METHODS: The outcome of 66 stapedectomies in children 17 years of age and younger were analyzed according to the 1995 American Academy of Otolaryngology-Head and Neck Surgery Committee on Hearing and Equilibrium guidelines. Factors evaluated included patient age, underlying diagnosis (tympanosclerosis, otosclerosis, congenital fixation), type of footplate graft and type of prosthesis used, associated ossicular abnormalities, and revision surgery. RESULTS: The mean postoperative air-bone gap following stapedectomy in children with tympanosclerotic footplate fixation (24.9 dB [+/-11 dB]) was significantly worse than in patients with an underlying diagnosis of congenital stapes fixation (15.7 dB [+/-9 dB]) or otosclerosis (13.1 dB [+/-3 dB]) (P =.024). Revision stapedectomy was also associated with a poorer outcome, but patient age and prosthesis and graft type did not contribute to the outcome in a statistically significant manner. CONCLUSIONS: Pediatric patients with stapes fixation resulting from tympanosclerosis showed poorer outcomes from stapedectomy than patients with congenital or otosclerotic fixation. Outcomes for congenital or otosclerosis fixation more nearly matched the outcomes in the literature for adult series.

Adolescent↗

PubMed1978

Nonotosclerotic ankylosis of the stapedial footplate.

A woman with a long history of chronic otitis media had a stapedectomy after a surgical diagnosis of otosclerosis. In the histologic study of the temporal bones, done after her death four years later, the main portion of the stapes footplate was present but fractured and no otosclerosis was present. New celluluar bone which simulated otosclerosis filled the anterior oval window niche and was continuous with the middle ear surface of the footplate fragments. The new bone extended anteriorly to overlie a tympanosclerotic focus and inferiorly was continuous with the periosteum of the middle ear. At least part of the new bone formation occurred after stapedectomy. This may explain the poor long-term results frequently encountered when a stapedectomy is done on patients with tympanosclerotic fixation of the stapes footplate.

Diagnosis, Differential↗