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Otosclerosis and endolymphatic hydrops.

It has been postulated that otosclerosis may produce vertigo by several mechanisms. One mechanism is by causing endolymphatic hydrops. We present six temporal bones in which otosclerosis and endolymphatic hydrops coexist. We consider that there is a spectrum-like interrelationship between these two entities. At one end of the spectrum the relationship is coincidental, while at the other end of the spectrum we consider the massive amount of active otosclerosis to be a causative factor in the development of the endolymphatic hydrops.

Aged↗

[Impedance measurements and otosclerosis (author's transl)].

Impedance measurements (model Madsen ZO 72) were performed on 158 patients with otosclerosis and compared with the operative findings. It was found that otosclerosis shows in 95% of the cases a symmetrical tympanogram, type A. Its amplitude does not differ from the standard values: that means that otosclerosis does not correlate with a flattened amplitude of the tympanogram. In addition there is no correlation between the intraoperative finding and the ampitude. The stapedius reflex could not be elicitated by acoustic stimuli.

Audiometry↗

The effect of stapedectomy on hearing of patients with otosclerosis and Meniere's disease.

To determine new guidelines for stapedectomy in patients with both Meniere's disease and otosclerosis, we studied the position of the saccular membrane and Reissner's membrane in relation to the stapes footplate in eight temporal bones from patients with Meniere's disease. We also reviewed charts of four patients with both otosclerosis and Meniere's disease who had stapedectomy. Histologic and clinical findings were compared with preoperative bone conduction levels at 500 Hz and at high frequencies. We found that the saccular and Reissner's membranes did not contact the stapes footplate ain bones of patients with preoperative bone conduction levels of 35 dB or better at 500 Hz and no high-frequency loss. We also found that stapedectomy was successful in patients with the same criteria. We therefore conclude that stapedectomy does not increase the risk of sensorineural hearing loss for patients with otosclerosis and Meniere's disease who have bone conduction levels of 35 dB o better at 500 Hz and no high-tone loss, but it is contraindicated for patients with 45 dB at 500 Hz or worse and with high-tone loss.

Hearing Loss, Sensorineural↗

Neurologic perspectives of otosclerosis.

Of 500 patients with roentgenographically verified otosclerosis, 230 had vestibular manifestations. Because of its frequency in general practice, otosclerosis has a major impact on the differential diagnosis of vertigo and related symptoms. The vestibular syndrome encompasses the full symptomatic spectrum common to diseases of the vestibular system, from episodic violent rotational vertigo to a sense of almost continuous imbalance, and cannot usually be distinguished from idiopathic endolymphatic hydrops. Otosclerosis is easy to diagnose when vestibular symptoms occur in conjunction with conductive or mixed hearing loss and a normal tympanic membrane that excludes middle ear disease. However, in approximately one-third of patients with a hearing loss that is purely sensorineural, the diagnosis can be confirmed only by compound-motion tomography of the petrous bones.

Adolescent↗

[Significance of dynamic measurements of acoustic impedance for early detection of hypoesthesia in otosclerosis].

To clarify the role of dynamic registration of acoustic impedance in early detection of hypoacusis in otosclerosis, the authors analyzed impedometry readings in subjects with unilateral otosclerosis. Special emphasis is laid on cases with minor audiogram bone-air intervals. Based on comparison of contra- and ipsilateral reflexes, registration of the acoustic reflex may provide early signs of otosclerosis.

Acoustic Impedance Tests↗

Antibodies to the minor cartilage collagen type IX in otosclerosis.

The presence of antibodies to collagens type I, II, III, VI, IX, and XI was studied in patients with otosclerosis, using enzyme-linked immunosorbent assays. Levels of antibodies to collagens type II and IX were significantly higher in these patients as compared to sex- and age-matched control subjects, whereas no differences were found between the levels of antibodies to collagens type I, III, VI, and XI. These observations for the first time document the presence of autoantibodies against a minor collagen type IX in patients with otosclerosis and support a possible role for collagen autoimmunity in the etiology of otosclerosis.

Adult↗

Physical examination and clinical evaluation of the patient with otosclerosis.

A detailed history, otoscopic examination, and testing with tuning forks are essential to the diagnosis of otosclerosis. These steps, including a differential diagnosis to rule out those middle ear conditions that can masquerade as otosclerosis, can help the examining physician make the presumptive diagnosis of otosclerosis.

Diagnosis, Differential↗

Audiologic characteristics of the patient with otosclerosis.

The audiologic manifestations of otosclerosis are as distinctive as they are varied. When pure stapedial otosclerosis is present, the most prominent audiologic signs are elicited in response to the use of low-frequency stimuli, reflecting the effect that increased stiffness has on the transfer function of the middle ear. Characteristic abnormalities appear on tympanometry, acoustic reflexes, and the pure-tone audiogram. When the disease process extends into the cochlea, a sensorineural hearing loss develops, which may be accompanied by vestibular symptoms and/or tinnitus. Complete audiologic evaluation may prove to be uniquely helpful both in establishing a diagnosis by recognizing the characteristic patterns of stapes fixation and in planning for the surgical, medical, or hearing aid management of the patient with otosclerosis.

Audiometry↗

Inner ear inflammation and round window otosclerosis.

Recently, it has been suggested that otosclerosis represents the host's ongoing immunologic response to measles or other viral antigens. Documentation of past inflammation within the inner ear would serve as further evidence that this mechanism may be at play in the pathogenesis of the disease. Among the characteristic signs of prior inflammation in the inner ear is the presence of lamellar bone at the site of inflammation. This has been described in the temporal bone of a patient with immune-mediated deafness and with the temporal bones of experimental models of immune-mediated inner ear disease. Review of temporal bones with round window otosclerosis from the Eastern Temporal Bone Bank at the Massachusetts Eye and Ear Infirmary show that in four of ten cases there are characteristic signs of a prior severe inflammatory event centered in the scala tympani adjacent to the otosclerotic lesion. Otosclerosis, therefore, may have an inflammatory stage that is the consequence of a host response to an inciting event.

Autoimmunity↗

Simultaneous presentation of facial nerve neuroma and otosclerosis.

Otosclerosis often occurs as a unilateral mixed or conductive hearing loss. In the absence of retrocochlear findings, otologists usually do not pursue further diagnostic testing. A patient who presented to the Warren Otologic Group with a unilateral mixed hearing loss is discussed. He was followed for 1 year with the intent of scheduling a stapedectomy. Two weeks prior to the surgical date, the patient developed a sudden hearing loss and was admitted to the hospital for treatment. Magnetic resonance imaging demonstrated a tiny, enhancing mass in the lateral internal auditory canal, measuring 7 mm in diameter. At surgery, the tumor was found to originate at the union of the nervus intermedius and the facial nerve. The simultaneous occurrence of facial nerve neuroma and otosclerosis is discussed, with emphasis on a thorough evaluation of all unilateral mixed hearing losses, including those attributable to otosclerosis.

Adult↗

[Human leukocyte antigen and otosclerosis].

Fifty patients (32 women, 18 men) who underwent stapedectomy [FISCH, V. (1), 1982] for otosclerosis were studied. Histo-compatibility antigens were typed in all patients using the microlymphocytic test to determine antigens of the HLA system loci A and B. Statistical comparison of results with those obtained in a control group of 339 persons without otosclerosis showed that otosclerosis has a genetic HLA-related component.

Chromosomes, Human, Pair 6↗

[Content of fluoride and calcium in stapedial bone in otosclerosis].

The study dealing with the content of fluoride and calcium in stapedial bone and canal wall bone in otosclerosis was performed in 69 subjects (48 females and 21 males) out of the patients, in whom bone rebuilding activity had earlier been studied isotopically. The control group comprised 20 normal stapedies taken during autopsies. Fluoride content was determined by means of fluoride ions meter, the content of calcium was assessed by resorting to atomic absorptiometer. The content of studied elements was compared with the bone rebuilding activity and some clinical features such as the patient's age and duration of the disease. High fluoride content in otosclerotic stapes was revealed, being several times greater than in the bone of normal stapes. Concurrently the stapedial bone in otosclerosis contained less calcium as compared with the bone of normal stapes. In principle, that referred to otosclerotic focus and next to stapedial crura. The bones of stapedial footplates and otosclerotic foci with rebuilding activity lover han the means value had statistically significant, higher content of fluoride and calcium than the bones with greater rebuilding activity. Fluoride content in stapedial bone during otosclerosis dramatically increased with the patient's age and the length of the disease duration period, however, the calcium content had the tendency to decrease.

Adult↗

[Surgical treatment of otosclerosis in the aged. Results of retrospective analysis].

Otosclerosis is, per se, a disease which rarely occurs after the age of 50. This is why stapes surgery is seldom performed in advanced age and there are few reports on the topic in the literature. The authors have performed a retrospective analysis of patients over the age of 65 who had undergone surgery for otosclerosis in the last 27 years. Out of a total of 3585 surgical procedures, 106 patients were analyzed. Most of the cases were in the advanced stages. Assessment of the outcome included pre- and post-operative audiometry, one year after surgery. Inner ear performance was established by evaluating air and bone conduction at 0.5, 1, 2, 3 and 4 kHz. The results showed that performing otosclerosis surgery is worthwhile even in the elderly with mixed auditory impairment, as long as the air-bone gap is limited to 20-30 dB and the surgical procedure is through. In this light, stapedotomy appears to be the most adequate technique. When evaluating the results the possibility of adopting a less powerful hearing aid should also be considered a success.

Age Factors↗

In vivo performance of the Nitinol shape-memory stapes prosthesis during hearing restoration surgery in otosclerosis: a first report.

The limitations of manual prosthesis crimping in hearing restoration surgery for otosclerosis are thought to have a key role in the occurrence of incomplete postoperative elimination of conductive hearing loss and postoperative recurrences of conductive hearing loss. To eliminate manual crimping, the self-crimping, shape-memory alloy Nitinol stapes piston was introduced in nine otosclerosis patients. The results were compared with those in a database of surgeries performed with conventional titanium pistons. The effects of the self-crimping Nitinol prosthesis on the postoperative elimination of conductive hearing loss and its postoperative variations were investigated. The variations of postoperative residual conductive hearing loss were significantly smaller and the extent of conductive hearing-loss elimination greater in the Nitinol group. The mean postoperative residual conductive hearing loss was smaller in the Nitinol group. The postoperative stability of conductive hearing loss elimination was similar in both patient groups. Preliminary results suggest that the self-crimping shape-memory alloy Nitinol stapes piston overcomes the drawbacks of manual crimping in hearing restoration surgery for otosclerosis.

Adult↗

Temporal bone showing otosclerosis, paget's disease and adenocarcinoma.

In a pair of temporal bones serial sectioning revealed widespread Paget's disease with otosclerosis on both sides and, at the right side, adenocarcinoma added. Stapedial fixation was produced on the right by otosclerosis, and on the left by Paget's disease. In the contest to reach the inner cochlear space, Paget was the winner on the left, where, at the tympanic scala, otosclerosis and Paget's disease almost met, while the immediately adjacent organ o Corti was uninvolved.

Adenocarcinoma↗

Revision cochlear implantation for facial nerve stimulation in otosclerosis.

OBJECTIVE: To find if patients experiencing postsurgical facial nerve stimulation caused by underlying disease process (ie, otosclerosis) can improve their hearing performance with their cochlear implant by reimplantation and by an optimal programming strategy. DESIGN: Retrospective analysis. SETTING: Academic tertiary referral center. PATIENTS: Two cochlear otosclerosis patients with resistant facial nerve stimulation (FNS). Both patients were initially implanted with Nucleus 22 devices (Cochlear Corporation, Englewood, Colo) and they developed FNS after a period of use. Owing to the decreasing number of active electrodes, concurrent decreases in speech understanding occurred. INTERVENTIONS: Various programming approaches were used to address the FNS. Both subjects ultimately received Nucleus 24 devices. One was reimplanted in the same ear, and the other was implanted in the opposite ear. Both have been followed up for 8 months following the reimplantation. MAIN OUTCOME MEASURES: Cochlear implant programming levels, cochlear implant performance, and facial nerve stimulation. RESULTS: The FNS was managed for more than 3 years through optimized programming. However, the FNS progressed until performance dropped below acceptable levels. Reimplantation was believed to be the only option for improvement. After reimplantation and programming, both subjects showed immediate improvement in speech discrimination. One user increased his consonant-nucleus-consonant word score from 12% preoperatively to 42%, and the other's performance increased from 0% to 86%. CONCLUSIONS: Our results suggest that having more programming options with newer devices is critical in otosclerotic or ossified users who experience FNS. Also, reimplantation may be a useful tool to improve performance.

Cochlear Implantation↗

Preoperative bone-conducted electrocochleography in otosclerosis.

Prior to surgery, pure-tone audiometry and bone-conducted (BC) electrocochleography (ECOG) were performed on 17 patients with preoperatively diagnosed otosclerosis. Short tone bursts, approximately 5 ms in duration and presented by means of a bone conductor on the exposed surface of the mastoid, were used as stimuli in BC ECOG. The thresholds thus obtained were compared by regression analysis with conventional psychoacoustic thresholds. Thresholds recorded at BC ECOG showed a close correlation to preoperative pure-tone BC thresholds, and there was no difference in predictability of postoperative hearing thresholds between preoperative BC ECOG and conventional preoperative pure-tone BC thresholds. The results lend support to the conclusion that BC ECOG, offering a monaural, objective estimation of cochlear function, is a valuable complement to conventional audiometry in the preoperative evaluation of hearing in otosclerosis.

Audiometry↗

Otosclerosis and sensorineural hearing loss. A clinical study.

A causal relationship between otosclerosis and sensorineural hearing loss is thought to exist. Forty-nine patients with unilateral otosclerosis were studied by comparing the bone conduction threshold in the otosclerotic ear to the bone conduction threshold in the uninvolved ear. This difference was analyzed using the paired Student's t test, and significant differences were found at 1,000, 2,000, and 4,000 Hz but not at 500 Hz. This effect on the higher frequencies may be due to a change in the motion mechanics of the basilar membrane and is greatest at the basal end of the cochlea.

Audiometry↗