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We report a family with the Buschke-Ollendorff syndrome and several unusual features, including one individual with congenital spinal stenosis and another with hearing loss, probably due to otosclerosis. Other reported abnormalities associated with this syndrome, including otosclerosis, are reviewed.
Thirty-five consecutive patients with the clinical diagnosis of fenestral otosclerosis were evaluated with high-resolution computed tomography (CT). Twenty-six were diagnosed as having this disorder by CT evidence of abnormal bony excrescences at or adjacent to the oval window. The diagnosis was made upon examination of 1.5-mm-thick targeted sections obtained at 0.5-mm intervals in the axial projection. Coronal sections were also included. Sections were also evaluated for evidence of plaque formation elsewhere in the lateral wall of the labyrinth and for surgical obstacles such as an abnormally wide cochlear aqueduct, a high jugular vein, and a dehiscent facial nerve. It is concluded that fenestral otosclerosis may be accurately diagnosed with proper CT techniques.
Otosclerosis (otospongiosis) is a primary focal disease of the labyrinthine capsule. The stapes footplate is fixed when the spongiotic focus expands and invades the oval window. Persons with stapedial otosclerosis experience a progressive conductive hearing loss. In many cases, cochlear degeneration is observed, in which a mixed hearing loss occurs. Using computed tomography (CT), we studied the ears of 45 selected patients with conductive or mixed hearing loss. CT proved valuable in determining otosclerotic changes of the oval window and otic capsule. Spongiotic changes of the otic capsule are better appreciated by CT than complex motion tomography. The usefulness of CT in diagnosing other causes of conductive or mixed hearing loss is also described.
Chondrosarcoma constitutes 6% of all primary bone tumors and 11% of malignant primary bone tumors. Nevertheless, in a review of the tumor registry of the University of Michigan covering a period of 50 years, there were only 3 cases involving the temporal bone. A case of a woman with a chondrosarcoma of the temporal bone that was partially resected by means of an infratemporal approach at the skull base is presented. This patient had previously undergone surgical treatment for otosclerosis of the other ear. Several considerations regarding survival factors in this type of tumor are dealt with in terms of the histological features, therapeutic options and anatomic location. The possibility that this lesion may originate from the persistence of the cartilaginous inclusions that some authors consider to be involved in the origin of otosclerosis is discussed.
The central corneal thickness was measured in 53 patients with osteogenesis imperfecta. The average thickness was found to be 0.443 +/- 0.003 (SEM) mm. This value was significantly lower than the central corneal thickness in 35 patients with otosclerosis (0.515 +/- 0.004 mm) and in 35 sex- and age-matched normal control subjects (0.522 +/- 0.004 mm). No difference in corneal thickness between otosclerosis and controls was observed. Measurements of the central corneal thickness may be of great value in the diagnosis of osteogenesis imperfecta.
This paper studies the changes in speech discrimination following stapes surgery in patients with advanced otosclerosis. Two thirds of these patients showed a postoperative improvement in the discrimination score with obvious correlation with closure of bone-air gap. The results were slightly worse in comparison to those obtained on otosclerotic patients with good bone conduction. In advanced otosclerosis even a minimal impairment in bone conduction and/or an incomplete closure of the gap interfere with discrimination.
We report the case of a 35-year-old man suffering from otosclerosis and unilateral auditory hallucinations as well as other psychotic symptoms that disappeared completely after surgery for otosclerosis. The patient experienced a change of his acoustic sensations: the tinnitus was transformed into music, and 4 months later the music changed into commenting and imperative voices. However, on both occasions the transformation from one form to another occurred during an alcohol withdrawal syndrome characterized mainly by vivid visual hallucinations. Some theoretical considerations on hallucinatory predisposition, development of hallucinations, and psychological factors determining psychotic symptoms will be discussed.
The differences in hearing gain 1 year after stapedotomy, partial platinectomy, or total stapedectomy for otosclerosis were studied separately at 0.5, 1, 2, 4, and 8 kHz with the data available from 311 consecutive primary operations performed between 1980 and 1982 in the University of Nijmegen Department of Otorhinolaryngology. Three groups of patients were matched for age, sex, clinical type of otosclerosis, and type of 0.6-mm all-Teflon piston. The hearing gain for air conduction was significantly better by an average of 7.4 dB for all frequencies combined after either stapedotomy or partial platinectomy compared to total stapedectomy.
Profound deafness has received increasing attention in recent years, largely because of the availability of cochlear implants. Consequently, it is especially important for otolaryngologists to remember that a "blank" audiogram does not necessarily mean total or even profound deafness. Patients with far-advanced otosclerosis may have no measurable hearing with routine audiometric testing even in the presence of serviceable sensorineural hearing. Review of nine patients (10 ears) who underwent stapedectomy from 1980 to 1987 reveals that seven of the nine (78%), who had been unable to use a hearing aid preoperatively, obtained serviceable hearing with hearing aids following surgery. Otolaryngologists should depend on a good history and tuning fork examination to avoid being misled by the audiogram, and should not hesitate to offer stapes surgery to patients with far-advanced otosclerosis.
The goal of this temporal bone study was to quantify the relationship between specific histologic changes at the stapes footplate and the magnitude of the air-bone gap in otosclerosis. The study material comprised 26 specimens with otosclerosis and 37 age-matched controls. Detailed anatomic measurements were made on each histologic section through the stapes footplate in each bone, resulting in 30 different measurement parameters for each bone. For frequencies 250 to 2,000 Hz, the conductive hearing loss correlated highly with (p < .01) and appeared to be caused primarily by narrowing and loss of the annular ligament, especially at the posterior stapediovestibular joint space. The size of the air-bone gap appeared to be determined by the extent and degree of this pathologic change. Schuknecht's hypothesis that bony ankylosis of the footplate would be associated with an air-bone gap of >30 dB was supported by our data. However, the degree and extent of bony footplate ankylosis could not be reliably predicted by the size of the air-bone gap.
Preoperative bone conduction threshold measurements were made in the otosclerotic and opposite ears of 25 patients with unilateral otosclerosis to tabulate the sensorineural hearing impairment in this disease. Middle ear otosclerosis was confirmed at surgery in all cases. The bone conduction thresholds at all frequencies measured were similar in the otosclerotic and opposite ears. Adequate masking was applied to the nontest ear to measure an accurate bone conduction threshold in the test ear without producing overmasking.
A prospective study of 47 patients with otosclerosis was undertaken to investigate the possible etiologic role of vitamin D undernutrition. The population comprised 27 women and 20 men, with a mean age of 46.4 years (range 21 to 79). The disease was bilateral in 43 patients, and cochlear involvement was present in 84.4%. The mean duration of symptoms was 17.1 years. Vitamin D status was evaluated by measuring the plasma 25-hydroxy vitamin D3 (25-OHD), which is the main storage metabolite. Abnormally low 25-OHD levels were found in 10 patients (21.7%) and borderline low levels in another two. Raised serum alkaline phosphatase levels were present in 32.6%, calcium in 6.5%, and inorganic phosphate in 4.3%. Calcium and vitamin D replacement therapy resulted in significant hearing improvement in 3 of 16 patients; these data support a causal correlation. Vitamin D deficiency is probably a factor in the etiology of some cases of otosclerosis and is important, since the deafness resulting from cochlear involvement may be reversible.
Twelve patients with surgically proven otosclerosis and sensorineural hearing loss were studied with high-resolution computed tomography (CT). The purpose was to identify abnormal bony changes that could be diagnostic of cochlear otosclerosis. Of the 24 temporal bones thus studied, 10 showed positive findings. The findings in our study--as well as those of others--show a pericochlear zone of radiolucency. The precise origin of this is undetermined.
The incisor absent (ia) rat is introduced as an animal model for the study of otosclerosis. Previous animal models have failed to accurately reflect the dynamic nature of this disease. Auditory brainstem response testing suggested a conductive hearing loss in the incisor absent rat as compared to age-matched normal controls. The hearing loss, which was manifested during puberty, was progressive in nature up to 18 weeks of age. Microscopic dissection of the middle ear revealed bony abnormalities of the ossicles and oval window in the incisor absent rat. Scanning electron microscopy of the ossicles demonstrated bony lesions at the incudostapedial joint and stapes footplate. Histologic examination demonstrated thickened spongiotic bone involving the otic capsule and ossicles. The incisor absent rat model possesses an inheritable defect of the otic capsule and ossicles that results in a progressive conductive hearing loss. The genetically transmitted lesion appears histologically similar to otospongiosis. The bony pathology in the incisor absent rat is caused by defective osteoclasts and transplantation of bone marrow cells from normal rats to the incisor absent rats corrects the cellular abnormality. The incisor absent rat may represent the best animal model to date for the study of otosclerosis, its cause, and clinical treatment.
In this study, we determined the effects of indomethacin and calcitonin on bone resorption in otosclerosis-like lesions in rats. Morphometric analysis showed that both indomethacin and calcitonin inhibited active otosclerosis-like lesions (bone resorption) and rats immunologically induced with type II collagen, and indomethacin had a much higher inhibitory effect than calcitonin. In in vitro studies we found that conditioned medium from splenic lymphocytes of rats immunized with type II collagen stimulated collagenase production by macrophages and fibroblasts. Collagenase is the major enzyme for degradation of the organic components of bone. Treatment of the immunized rats with indomethacin and calcitonin significantly reduced the stimulatory effect of the lymphocyte-conditioned medium on collagenase production. Indomethacin caused a greater reduction of the stimulatory effect of the lymphocytes on collagenase production than calcitonin. These findings are in agreement with results of the morphometric study. Results of the present study also suggest that cell-to-cell interaction plays an important role in collagenase production for degradation of organic components of bone resorption in otosclerotic lesions.
Hearing levels of 174 patients (213 ears operated on) with otosclerosis undergoing stapedectomy were analyzed. All patients had followup of 10 years or more, the mean follow-up period being 13.4 years. Eighty-seven ears (41%) underwent posterior crus stapedectomy, and in the remaining 126 ears (59%) a prosthesis was inserted. Large fenestra technique was used in all cases. In the long run, both air conduction and bone conduction thresholds of ears operated on showed remarkable deterioration from the best values obtained 6 to 12 months postoperatively. Ten years after surgery both air conduction and bone conduction thresholds of ears operated on were significantly worse than those of normal controls. At 10 years, 70 percent of the ears operated on had hearing levels (at 0.5 to 2.0 kHz) of 30 dB or better and 88 percent had 40 dB or better. At the last follow-up examination, in 90% of the patients the better hearing ear had a hearing level of 40 dB or better. In 90% of patients with bilateral otosclerosis who had operations in only one ear, the ear operated on had better hearing function than the opposite ear that had not been operated on.
Our study aimed to show whether it is useful to preserve the stapedial muscle during the surgical treatment of otosclerosis. The study was carried out on 67 patients with tympanoscopic evidence of otosclerosis. In group A (34 patients) the stapedial muscle was intra-operatively preserved as follows: the stapedial suprastructure with preserved muscle tendon was transpositioned onto the longer arm of the incus and secured with a wire loop. The mean uncomfortable level for sounds was measured after surgery and the results of group A patients were compared with those obtained in group B (33 patients, who underwent standard surgery). One month after surgery, in group A patients, the cochleostapedial reflex was evoked in 21 patients (61.8 per cent) and two months later in 32 patients (94.1 per cent). One month after operation, in group A patients the mean uncomfortable level was 108.8 dB, while three months later it increased to 114.1 dB. In group B, the relevant values were 97.8 dB one month after surgery, and 98.0 dB three months later. Our presumption that stapedial muscle preservation is necessary has been proved by the fact that the patients with the preserved muscle had a higher noise discomfort threshold.
Although it is generally accepted that 20% or more of patients with clinically manifest otosclerosis have a concomitant sensorineural hearing loss, it is still debated whether otosclerosis is the cause of the neural loss. Three cases are presented of other causes of sensorineural loss in otosclerotic patients who underwent stapes surgery. One of these cases, the Mondini malformation, is discussed in detail.