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ΒΙΟΒΛΙΟΓΡΑΦΙΑ ΘΕΡΑΠΕΙΑΣ ΩΤΟΣΚΛΗΡΥΝΣΗΣ

 
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Δρ Δημήτριος Ν. Γκέλης,

Ιατρός, Οδοντίατρος, Ωτορινολαρυγγολόγος, Διδάκτωρ της Ιατρικής Σχολής του Πανεπιστημίου Αθηνών

Ιατρείο έρευνας και αντιμετώπισης της Ωτοσκλήρυνσης

Δαμασκηνού 46, Κόρινθος 20100

τηλ. 2741026631, 6944280764, pharmage@otenet.gr

www.gelis.gr, www.pharmagel.gr, www.orlpedia.gr; www.allergopedia.gr, www.gkelanto.gr,www.zinc.gr. www.curcumin.gr www.gkelikosagiorgitiko.gr www.pharmagel.gr

 

 

 

Int J Clin Pract. 2010 Jan;64(2):256-65.

Otosclerosis 2: the medical management of otosclerosis.

Uppal S, Bajaj Y, Coatesworth AP.

Department of ENT, York Hospital, York, UK. uppalsandeep@yahoo.com

Abstract

The rationale for medical therapy for otospongiosis is to slow down and eventually stop the phase of bone resorption. There is some increase in the incidence of stapedial otospongiosis in a low-fluoride area compared with a high-fluoride area. Sodium fluoride treatment has a role to play in preventing the onset and progression of hearing loss in patients suffering from otosclerosis. Sodium fluoride therapy has been shown to have some beneficial effect on dizziness associated with otosclerosis. In view of the possibility of systemic side effects of sodium fluoride therapy, a regular follow up of patients is warranted. Biphosphonates can be used as an alternative treatment to sodium fluoride in cases where the patient is intolerant to sodium fluoride therapy. Hearing aid is also a treatment option, but it does not halt the disease process.

 

Ann Otol Rhinol Laryngol. 2009 Dec;118(12):859-65.

Surgical and medical treatment of hearing loss in mixed otosclerosis.

Félix-Trujillo MM, Valdez-Martínez E, Ramírez JE, Lozano-Morales R.

Department of Otorhinolaryngology-Head and Neck Surgery, General Hospital of the National Medical Center La Raza, Mexican Institute of Social Security Mexico, DF, Mexico.

Abstract

OBJECTIVES: We assessed the effect of using preoperative sodium fluoride (NaF) on the difficulty of working with the footplate during stapedectomy and its effect on the postsurgical hearing gain in patients with mixed otosclerosis (i.e., otosclerosis and/or otospongiosis).

METHODS: There were 2 groups of patients with mixed otosclerosis. The experimental group was made up of patients with an active focus of the disease; they received NaF for 6 months and then underwent stapedectomy. The control group was made up of patients with an inactive focus of the disease; they underwent stapedectomy without administration of NaF.

RESULTS: The difference between the study groups in the degree of difficulty in extracting the footplate was not found to be statistically significant. Both groups had hearing gains in bone and air conduction. The level of posttreatment hearing gain in the group exposed to NaF was greater than that in the nonexposed group.

CONCLUSIONS: Sodium fluoride administered to patients with mixed otosclerosis and an active focus of disease does not increase the difficulty of working with the footplate during stapedectomy, and it increases the hearing gain after the procedure.

Ann Otol Rhinol Laryngol. 2005 Sep;114(9):709-16.

Otosclerosis: incidence of positive findings on high-resolution computed tomography and their correlation to audiological test data.

Naumann IC, Porcellini B, Fisch U.

Department of Otolaryngology-Head and Neck Surgery, Indiana University, Indianapolis, Indiana, USA.

OBJECTIVES: Computed tomographic (CT) scanning with slices of 1 mm or more has not been sufficient to demonstrate otosclerotic foci in most cases to date. METHODS: We investigated the validity of CT scans with a 0.5-mm cubical scan technique, with and without planar reconstruction, and correlated these findings with audiological data. Forty-four temporal bone CT scans from 30 patients with conductive or mixed hearing loss were evaluated. RESULTS: Otosclerotic foci were visualized in 74% of the cases. With reconstruction at the workstation, the sensitivity increased to 85%. Whereas in fenestral otosclerosis a correlation was found between the size of the focus and the air-bone gap, no correlation was seen between the size of the focus and bone conduction thresholds with cochlear involvement. Otosclerotic foci in patients treated with sodium fluoride were smaller than those in patients without treatment. This finding may indicate a beneficial effect of sodium fluoride on otosclerotic growth. CONCLUSIONS: High-resolution CT scans are a valid tool that can be used to confirm, localize, and determine the size of clinically suspected otosclerotic foci.

Otol Neurotol. 2003 Nov;24(6):854-62.

Increased activity of the diastrophic dysplasia sulfate transporter in otosclerosis and its inhibition by sodium fluoride.

Grayeli AB, Escoubet B, Bichara M, Julien N, Silve C, Friedlander G, Sterkers O, Ferrary E.

INSERM Unité 426, Faculté Xavier Bichat, Université Paris 7, Paris, France. alexis.bozorg-grayeli@bjn.ap-hop-paris.fr

HYPOTHESIS: This study investigates the function of the diastrophic dysplasia sulfate transporter (DTDST) in otosclerotic bone and the effect on it of sodium fluoride (NaF). BACKGROUND: Otosclerosis is a localized bone dystrophy with increased bone turnover. DTDST is implicated in the regulation of the bone turnover. MATERIALS AND METHODS: Primary cultures of cells were obtained from the stapes and external auditory canal (EAC) of 26 patients with otosclerosis and from nine control patients. Sulfate uptake was quantified under basal conditions and with NaF. The NaF signaling pathways were investigated using forskolin and verapamil. RESULTS: The relative initial rates of sulfate uptake and the apparent Vmax values were: otosclerotic stapes > EAC > control stapes = control EAC. The sulfate uptake by the otosclerotic stapes was correlated with the loss of sensorineural hearing. The amounts of DTDST mRNA (RNase protection assay) in the four subgroups did not differ. NaF (10(-6)M, 1 hr) inhibited sulfate uptake by the otosclerotic stapes and EAC cells but not by control samples. CONCLUSION: The authors believe that whether the increased DTDST activity is a cause or an effect of otosclerosis, it appears to be a specific target for NaF treatment.

Acta Otorhinolaryngol Ital. 1998 Aug;18(4 Suppl 59):59-65.

[Progressive sensorineural hearing loss in cochlear otosclerosis]

[Article in Italian]

Sellari-Franceschini S, Ravecca F, De Vito A, Berrettini S.

Dipartimento di Neuroscienze, Università degli Studi di Pisa.

Otosclerosis is a bone dysplasia limited to the otic capsule causing abnormal resorption and redeposition of bone. The existence of the entity "pure labyrinthine otosclerosis" or "cochlear otosclerosis" is not accepted by all authors; however, there is clinical and histologic evidence to support the existence of a progressive sensorineural hearing loss due to otospongiotic-otosclerotic lesions of the labyrinthine capsule, although diagnosis of this condition may be difficult. The involvement of the inner ear is described as degenerative changes in the spiral ligament, stria vascularis, organ of Corti, and cochlear neurons. The most frequent audiometric configuration is a "bite-type" curve, but flat or rising shapes can also be observed; speech discrimination appears unusually good for a pure sensorineural hearing loss and recruitment is frequently absent. A cochlear otosclerosis should be suspected when there is a family history of otosclerosis, the onset of the hearing loss occurs from the third to fifth decade, and worsening of the hearing loss is observed during periods of intense hormonal and endocrine activity, a positive Schwartze sign is present and bilateral sensorineural loss is associated with signs of unilateral stapedial ankylosis. A definitive diagnosis of cochlear otosclerosis can be made only with computed tomography, which allows a quantitative assessment of the involvement of the labyrinthine capsule by spongiotic or sclerotic areas. The factors to be considered are: otosclerotic foci 1 mm or more in diameter and a density different from that of the normal otic capsule, partially or completely erased contour of the capsule, double ring effect, bony neoformation in the labyrinthine spaces, and increased thickness of the cochlear capsule. The medical management of cochlear otosclerosis is based on sodium fluoride, in association with calcium and vitamin D; some authors have also proposed diphosphonates as inhibitor agents of bone resorption. Surgery may be useful only in those patients presenting a hearing loss so severe that the bone threshold cannot be evaluated and a gap between air and bone conduction cannot be excluded; in these cases stapes operations can improve hearing to a level that may be useful in hearing aid application.

Am J Otol. 1994 Jul;15(4):536-9.

Mixed hearing loss in otosclerosis: indication for long-term follow-up.

Ramsay HA, Linthicum FH Jr.

House Ear Institute, Los Angeles, CA 90057, USA.

This retrospective study of 146 ears with long-term follow-up after otosclerosis surgery evaluated the stability of hearing results, the incidence of sensorineural hearing loss, and the effect of fluoride treatment. Follow-up was at least 15 years (mean, 25.2 yr; range, 15-44 yr). There were 97 large fenestra stapedectomy operations, 23 lateral canal fenestrations, 7 mobilizations, and 19 revision stapes operations. The level of air-bone gap achieved at surgery remained stable over time; the mean deterioration rate was only 0.2 dB per year. Profound sensorineural hearing loss ( > or = 65 dB bone conduction average) at the most recent follow-up occurred in 13 ears (8.9%). Such hearing loss occurred in all operative groups. Mean bone conduction average immediately postoperatively was significantly higher in these ears than in others in the study. This finding indicates that a mixed hearing loss at surgery is a factor that increases the risk of later profound cochlear loss. Only 3 percent of ears with pure conductive hearing loss, but 28 percent of patients with mixed hearing loss at surgery eventually suffered profound cochlear loss. Sodium fluoride was used to treat 11 ears with progressive cochlear loss. The rate of bone conduction hearing deterioration decreased in all ears after treatment, and none developed profound hearing loss. Follow-up after the first postoperative year is not necessary if pure conductive hearing loss is present at surgery. Annual follow-up with audiograms is recommended if a mixed hearing loss is present. Fluoride treatment is recommended if inner ear hearing loss progresses.


Am J Otol. 1993 Sep;14(5):482-90.

Sodium fluoride therapy.

Causse JR, Causse JB, Uriel J, Berges J, Shambaugh GE Jr, Bretlau P.

Clinique d'Otologie Jean Causse Traverse de Beziers, Colombiers, France.

As a medical treatment for otospongiosis-otosclerosis, sodium fluoride influences the underlying bony changes in the labyrinth so as to arrest or prevent the onset of hearing loss. Sodium fluoride is an enzyme inhibitor and reduces osteoclastic bone resorption and, if the dosage is more than 60 mg a day, may rebuild pseudohaversian bone.

Am J Otol. 1989 Jan;10(1):20-2.
Otospongiosis and sodium fluoride. A clinical double-blind, placebo-controlled study on sodium fluoride treatment in otospongiosis.

Bretlau P, Salomon G, Johnsen NJ.

Department of Otolaryngology and Audiology, Gentofte Hospital, Copenhagen, Denmark.

The effect of sodium fluoride treatment in patients with otospongiosis has been evaluated in a prospective clinical double-blind, placebo-controlled study of 95 patients. The results showed a statistically significant greater deterioration of hearing loss in the placebo group than in the group actively treated with 40 mg of sodium fluoride daily. These results support the view that sodium fluoride can change otospongiotic, active lesions to more dense, inactive otosclerotic lesions.

 

Ann Otol Rhinol Laryngol. 1985 Mar-Apr;94(2 Pt 1):103-7.
Otospongiosis and sodium fluoride. A blind experimental and clinical evaluation of the effect of sodium fluoride treatment in patients with otospongiosis.

Bretlau P, Causse J, Causse JB, Hansen HJ, Johnsen NJ, Salomon G.

The effect of sodium fluoride treatment in patients with otospongiosis has been evaluated blindly in a morphological and microchemical element analysis of otospongiotic specimens together with a prospective clinical double-blind, placebo-controlled study. The results show that using the calcium/phosphorus ratio as an indication for bone maturity, the sodium fluoride treatment can stabilize otospongiotic lesions in retaining calcium relative to phosphorus. The clinical double-blind, placebo-controlled study of 95 patients showed a statistically significant worse deterioration of the hearing loss in the placebo group than in the active treated (40 mg sodium fluoride daily) group, supporting the view that sodium fluoride can change otospongiotic, active lesions to more dense, inactive otosclerotic lesions. We have postulated in the past that the actual mechanism of the cochlear loss is toxic enzymes produced by histiocytes at the periphery of the microfoci, and it may be that sodium fluoride has some effect on these enzymes.

Am J Otol. 1986 Mar;7(2):121-5.
Sodium fluoride: effectiveness of treatment for cochlear otosclerosis.

Forquer BD, Linthicum FH, Bennett C.

We examined the effectiveness of sodium fluoride in treatment of cochlear otospongiosis in ninety-four patients with cochlear otosclerosis and ninety-eight patients with stapedial otosclerosis and sensorineural hearing loss. The drug halted or slowed the progression of sensorineural hearing impairment in 63% of the patients with cochlear otosclerosis and 46% of the patients with stapedial otosclerosis. The single factor that best predicted which patients would respond most favorably to treatment was rate of progression before treatment. Sodium fluoride therapy was successful for 79% of the patients losing their hearing at a rate of 5 dB or more per year at one or more of the speech frequencies. The finding that patients with more rapid rates of progression responded most favorably to sodium fluoride therapy suggests that patients with the most active otospongiotic processes will be the most responsive to treatment.

 

Laryngoscope. 1980 Sep;90(9):1481-7.
Otosclerosis and vestibular dysfunction.

Freeman J.

Vestibular dysfunction associated with otosclerosis has been observed for many years. In a recent clinical study of patients exhibiting progressive sensorineural hearing loss of unknown origin, it was demonstrated that approximately 50% of the cases had cochlear otosclerosis/otospongiosis as the most probable cause for the hypacusis. Of these, approximatley 55% had vestibular symptoms. In the present study, the effect of sodium fluoride therapy in this group of patients is analyzed. It will be demonstrated that such therapy is beneficial not only in controlling the progressive sensorineural hearing loss but also the symptoms of vestibular dysfunction in patients with cochlear otosclerosis (otospongiosis).

Am J Otol. 1989 Mar;10(2):146-7.
How and when to prescribe sodium fluoride.

Shambaugh GE Jr.

Department of Otolaryngology, Northwestern University, Chicago, Illinois.

Sodium fluoride has now been used for 24 years in an effort to slow down or arrest sensorineural hearing nerve deterioration in patients with stapedial otosclerosis or after stapedectomy, as well as in patients with pure cochlear otosclerosis. Extensive clinical experience in thousands of patients with this therapy has demonstrated its value in arresting previously progressive sensorineural hearing loss. For a long time there were those who objected to this therapy on the basis that it had not been adequately proven by double-blind, placebo-controlled studies. They have been answered by Bretlau's study in Denmark and Fisch's from Switzerland; both investigators confirmed on small groups the value of sodium fluoride by double-blind, placebo-controlled studies. Extensive research by Professor Petrovic of Strasbourg while at our tissue culture laboratory at Northwestern University demonstrated the action of sodium fluoride on bone. A nicely designed study with radioactive strontium by Linthicum, House, and Althaus demonstrated its value in promoting maturation of a spongiotic focus. Today there is no reason to hesitate in prescribing this useful, effective, and safe medication to promote maturation of otospongiotic lesions, and thus to slow down or to arrest progression in sensorineural hearing loss.

Acta Otolaryngol. 2001 Jan;121(2):174-7.
Fluoride therapy for cochlear otosclerosis? an audiometric and computerized tomography evaluation.

Derks W, De Groot JA, Raymakers JA, Veldman JE.

Department of Otorhinolaryngology, University Medical Center Utrecht, The Netherlands.

The progress of sensorineural hearing loss (SNHL) in patients with cochlear otosclerosis was compared for 19 patients treated with fluoride for 1-5 years and 22 untreated controls. CT scans of eight patients before and after fluoride treatment were evaluated visually. Fluoride therapy arrested the progression of SNHL in the low (250, 500 and 1,000 Hz) (p < 0.001) and high (2 and 4 kHz) (p = 0.008) frequencies. It seemed to be more effective for the higher frequencies in cases with an initial SNHL of < 50 dB. Fluoride administration for 4 years did not seem to be superior to a shorter treatment period (1-2 years). For six patients followed up after discontinuing fluoride therapy there was minimal deterioration in SNHL. There was no clear relationship between the size and site of otospongiotic lesions on CT and the severity of SNHL. Follow-up with CT evaluation did not provide reliable information as to the efficacy of fluoride therapy.

Am J Otol. 1994 Jul;15(4):536-9.

Related Articles, Links


Mixed hearing loss in otosclerosis: indication for long-term follow-up.

Ramsay HA, Linthicum FH Jr.

House Ear Institute, Los Angeles, CA 90057, USA.

This retrospective study of 146 ears with long-term follow-up after otosclerosis surgery evaluated the stability of hearing results, the incidence of sensorineural hearing loss, and the effect of fluoride treatment. Follow-up was at least 15 years (mean, 25.2 yr; range, 15-44 yr). There were 97 large fenestra stapedectomy operations, 23 lateral canal fenestrations, 7 mobilizations, and 19 revision stapes operations. The level of air-bone gap achieved at surgery remained stable over time; the mean deterioration rate was only 0.2 dB per year. Profound sensorineural hearing loss ( > or = 65 dB bone conduction average) at the most recent follow-up occurred in 13 ears (8.9%). Such hearing loss occurred in all operative groups. Mean bone conduction average immediately postoperatively was significantly higher in these ears than in others in the study. This finding indicates that a mixed hearing loss at surgery is a factor that increases the risk of later profound cochlear loss. Only 3 percent of ears with pure conductive hearing loss, but 28 percent of patients with mixed hearing loss at surgery eventually suffered profound cochlear loss. Sodium fluoride was used to treat 11 ears with progressive cochlear loss. The rate of bone conduction hearing deterioration decreased in all ears after treatment, and none developed profound hearing loss. Follow-up after the first postoperative year is not necessary if pure conductive hearing loss is present at surgery. Annual follow-up with audiograms is recommended if a mixed hearing loss is present. Fluoride treatment is recommended if inner ear hearing loss progresses.

Ann Otol Rhinol Laryngol. 1984 Mar-Apr;93(2 Pt 1):105-11.

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Histologically proven cochlear otosclerosis with pure sensorineural hearing loss.

Balle V, Linthicum FH Jr.

This paper discusses cochlear otosclerosis without stapedial fixation as a cause of sensorineural hearing loss. Because many cases of cochlear otosclerosis may remain undiagnosed, we believe that patients with a progressive sensorineural hearing loss and a family history of cochlear otosclerosis should be suspected of having cochlear otosclerosis. A positive stapedial reflex on-off effect or polytomographic findings help make the diagnosis. We prescribe sodium fluoride, calcium, and vitamin D for these patients in an effort to prevent progression of the loss.

Ear Nose Throat J. 1995 Aug;74(8):534-8, 540, 542.

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Meniere's syndrome: an approach to therapy.

Brookler KH, Glenn MB.

Neurotologic Associates, P.C., New York, NY 10021, USA.

There are patients with symptoms similar to those of Meniere's disease who do not have Meniere's disease and therefore do not respond to conventional medical or conservative surgical management. Some have subtle disorders of carbohydrate and lipid metabolism and inner ear otosclerosis, which are responsible for the production of their symptoms. The response to dietary therapy confirms that glucose tolerance levels which are not diabetic nor hypoglycemic may be sufficiently significant in relation to inner ear function to produce the symptoms like those of Meniere's Disease. Management of inner ear otosclerosis with supplemental sodium fluoride, calcium carbonate, and Vitamin D was also effective in controlling the symptoms in some patients

Am J Otol. 1991 May;12(3):195-8.

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Effect of sodium fluoride on early stages of otosclerosis.

Colletti V, Fiorino FG.

ENT Department, University of Verona, Ospedale Borgo Roma, Verona, Italy.

The present investigation evaluates the effectiveness of NaF treatment in modifying the natural course of subclinical otosclerosis, as monitored by the stapedius reflex. The study was carried out on 128 relatives of patients suffering from surgically confirmed footplate otosclerosis. The diagnosis of subclinical otosclerosis was made on the basis of presence of the on-off effect. One group of subjects was treated with NaF in doses ranging from 6 to 16 mg according to age. The treatment lasted 2 years. A second group served as a control. Changes in stapedius reflex morphology were evaluated at 1, 2, and 5 years from the onset of treatment. The investigation demonstrated that NaF has a stabilizing effect on early otosclerosis. This drug, in fact, arrests the disease process in more than 60 percent of ears at the 2-year follow-up and in more than 50 percent at 5 years. A program of secondary prevention of otosclerosis by NaF is suggested.

Am J Otol. 1984 Jan;5(3):211-23.

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Otospongiosis as a genetic disease. Early detection, medical management, and prevention.

Causse JR, Causse JB.

Extensive research into the inmost mechanism of otospongiotic disease and an extended study of otosclerotic patients' ancestry have led the authors to a plan for prevention of this disease, which appears to be a genetic deafness with autosomal dominant inheritance and about 40 percent penetrance of genes. Progress in impedance audiometry has permitted early detection of stapedial fixation by means of systematic audiometric investigations, particularly the elicitation of stapedius reflex showing a very special pattern called the "on-off effect." The evidence supporting an enzymatic origin of the sensoringeural component of hearing loss in otosclerotic families has led us to treat otospongiotic children with very low doses of sodium fluoride, with no risk of stunting growt. We have been applying this procedure for four years to the families of stapedectomized otosclerotic patients. We believe it would be advisable to extend this type of prevention to schoolchildren by means of systematic audiometric investigations, including the elicitation of stapedius reflex to detect a possible on-off effect. We also studied sodium fluoride therapy, which derives from the enzymatic origin of the otospongiotic disease. This treatment, based on enzymogenesis inhibitors, should be given to young otospongiotic/otosclerotic patients detected by systematic audiometric investigations. Sodium fluoride could even be prescribed for otospongiotic mothers to prevent disease in their newborn children, exactly as in dental prevention. Otospongiotic/otosclerotic disease can be easily controlled by medical treatment, combined with surgery if needed. This treatment is effective thanks to early detection.

Acta Otorhinolaryngol Ital. 1998 Aug;18(4 Suppl 59):59-65.

Related Articles, Links


[Progressive sensorineural hearing loss in cochlear otosclerosis]

[Article in Italian]

Sellari-Franceschini S, Ravecca F, De Vito A, Berrettini S.

Dipartimento di Neuroscienze, Universita degli Studi di Pisa.

Otosclerosis is a bone dysplasia limited to the otic capsule causing abnormal resorption and redeposition of bone. The existence of the entity "pure labyrinthine otosclerosis" or "cochlear otosclerosis" is not accepted by all authors; however, there is clinical and histologic evidence to support the existence of a progressive sensorineural hearing loss due to otospongiotic-otosclerotic lesions of the labyrinthine capsule, although diagnosis of this condition may be difficult. The involvement of the inner ear is described as degenerative changes in the spiral ligament, stria vascularis, organ of Corti, and cochlear neurons. The most frequent audiometric configuration is a "bite-type" curve, but flat or rising shapes can also be observed; speech discrimination appears unusually good for a pure sensorineural hearing loss and recruitment is frequently absent. A cochlear otosclerosis should be suspected when there is a family history of otosclerosis, the onset of the hearing loss occurs from the third to fifth decade, and worsening of the hearing loss is observed during periods of intense hormonal and endocrine activity, a positive Schwartze sign is present and bilateral sensorineural loss is associated with signs of unilateral stapedial ankylosis. A definitive diagnosis of cochlear otosclerosis can be made only with computed tomography, which allows a quantitative assessment of the involvement of the labyrinthine capsule by spongiotic or sclerotic areas. The factors to be considered are: otosclerotic foci 1 mm or more in diameter and a density different from that of the normal otic capsule, partially or completely erased contour of the capsule, double ring effect, bony neoformation in the labyrinthine spaces, and increased thickness of the cochlear capsule. The medical management of cochlear otosclerosis is based on sodium fluoride, in association with calcium and vitamin D; some authors have also proposed diphosphonates as inhibitor agents of bone resorption. Surgery may be useful only in those patients presenting a hearing loss so severe that the bone threshold cannot be evaluated and a gap between air and bone conduction cannot be excluded; in these cases stapes operations can improve hearing to a level that may be useful in hearing aid application.

Laryngoscope. 1978 Dec;88(12):1918-21.

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Intestinal absorption of fluoride preparations.

Deka RC, Kacker SK, Shambaugh GE Jr.

Gastric intestinal absorption of five different preparations of sodium fluoride was measured by means of ten hour urinary excretion. Best absorption was by nonenteric coated sodium fluoride. All three enteric coated preparations showed poorer absorption, with marked individual variations. Florical, the currently available preparation, showed better absorption than any enteric coated tablets and only slightly less than sodium fluoride alone. For active cochlear otospongiosis two capsules three times a day should be prescribed.

Laryngoscope. 1983 Jun;93(6):717-20.

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Otosclerosis: relationship of spiral ligament hyalinization to sensorineural hearing loss.

Parahy C, Linthicum FH Jr.

The sensorineural component of a mixed hearing loss due to otosclerosis is generally accepted as due to the otosclerotic lesion. The existence of pure cochlear otosclerosis without stapes fixation has been questioned. However, we are documenting 7 such cases in a separate publication. Results of this study, which evaluates 46 temporal bones with clinical or cochlear otosclerosis, demonstrate that the degree of sensorineural loss is directly related to the amount of hyalinization of the spiral ligament. The hyalinization occurs adjacent to active otospongiotic lesions but not next to inactive otosclerotic lesions. Both types of lesions may involve the cochlear endosteum. Small channels through the endosteal bone from the lesion to the spiral ligament have been found. The hyalinization spreads laterally from these channels. The hyalinization is presumably a result of the passage of toxic substances (proteolytic enzymes) from the lesion to the ligament. Strial atrophy is most pronounced on ligaments with the greatest degree of hyalinization. Hyalinization in only one ear produces decreased hearing compared to the other ear.

Ann Otolaryngol Chir Cervicofac. 1980 Apr-May;97(4-5):325-51.

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[Otospongiosis has a genetic disease its early detection its medical management (author's transl)]

[Article in French]

Causse JR, Causse JB.

Progress in new audiometric techniques, chiefly in impedance-audiometry, and better knowledge of the innermost mechanism of the otospongiotic disease, have led the authors to plan prevention of the otospongiotic disease, which appears to be a genetic deafness with an autosomal dominant inheritance and about 40% penetrance of genes. The bases of this prevention are: first, the early detection of stapedial fixation by means of systematic audiometric investigations, particularly elicitation of the on-off effect by impedance-audiometry; second, the treatment of oto-spongiotic children with very low doses of sodium fluoride without any risk of stunting the growth. The authors have been applying these means for four years to families of stapedectomized otosclerotic patients, but they think that it would be advisable to extend this type of prevention to the systematic detection of otospongiosis in school-children by means of audiometry investigations, including the elicitation of the stapedius reflex in order to detect a possible on-off effect. Moreover, the prescription of a fluoride treatment to otospongiotic mothers for prevention in their new-borns (exactly as in dental prevention), would allow us, with the support of parents and public authorities, to eradicate this severe disease in the near future. Twenty years ago, otospongiosis still seemed incurable, but at present this disease can be very easily controlled by a medical treatment based on enzymogenesis regulators, associated with surgery if needed.

Laryngoscope. 1984 Apr;94(4):508-12.

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Otosclerosis and otospongiosis: clinical and histological comparisons.

Parahy C, Linthicum FH Jr.

The simultaneous occurrence of otosclerosis and otospongiosis in the same lesion is well documented. The logical sequence of events would seem to be softening and destruction of the bone by the otospongiotic lesion, which is subsequently converted to otosclerosis. Examination of 46 temporal bones has led us to further classify the lesion as: 1. an active lesion, both otospongiotic and otosclerotic, 2. an inactive lesion, also both otosclerotic and otospongiotic, or 3. a fibrous lesion. Hyalinization of the spiral ligament only occurs adjacent to active otospongiotic or very large otosclerotic lesions. If the lesion adjacent to the spiral ligament is inactive, there is no hyalinization. It seems illogical that a hyalinized spiral ligament will return to normal when the lesion changes from active otospongiosis to inactive otosclerosis. Otospongiotic and otosclerotic lesions are found side by side at the periphery of lesions, and both are adjacent to normal bone. These findings have implications concerning the mode of action of sodium fluoride and other medications that are under evaluation for the control of the sensorineural hearing loss due to otosclerosis.

Adv Otorhinolaryngol. 1977;22:43-56.

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Cochlear otospongiosis etiology, diagnosis and therapeutic implications.

Causse J, Shambaugh GE, Chevance LG, Bretlau P.

First, the authors discuss the most valuable way to correlate specific morphological changes encountered in cochlear otospongiosis with sensorineural hearing loss. They think that biochemical factors may be responsible for this association of cochlear otospongiosis and histopathologic changes, and they explain their enzymatic concept resulting from experimental findings and cyto-clinical relationship. Second, the authors analyze clinical, audiometric and X-Rays investigations enabling the diagnosis of cochlear otospongiosis, in its pure pereceptive form as well as in the perceptive component added to the conductive loss in far-advanced mixed audiometric types in surgical otospongiosis. They present two typical cases of cochlear otospongiosis: one combines clinical history, audiometric test and post mortem investigations;-the other shows the passage from a pure cochlear otospongiosis to a secondary stapedial fixation, ten years later, thus confirming by audiometric data and by stapedectomy the otospongiotic etiology of this previous pure sensorineural loss. Finally, they insist upon the great interest of establishing an early diagnosis in cochlear otospongiosis on account of its therapeutic implication, particularly from the enzymatic point of view.

Otolaryngol Head Neck Surg. 1981 Jul-Aug;89(4):646-50.

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Otospongiosis: morphologic and microchemical investigation after NaF-treatment.

Bretlau P, Hansen HJ, Causse J, Causse JB.

Element analysis of microareas of otospongiotic specimens is described. A total of 36 otospongiotic stapes are ultrasectioned without decalcification and examined using a transmission and a scanning electron microscopy (STEM-mode). The latter was equipped with an energy dispersive x-ray analyzer. Twenty of the stapes came from patients who have had sodium fluoride (NaF) treatment (15 to 45 mg/day) for a minimum of 12 months. The otospongiotic stapes are classified as spongiotic and sclerotic according to their pathologic characteristics and state of mineralization. Using the Ca/P ratio as criterion--measured by the characteristic x-ray fluorescence--it was shown in a blind study that the NaF-treated otospongiotic stapes had a statistically higher Ca/P ratio, indicating that the fluoride may stabilize otospongiotic lesions, particularly the spongiotic type with unstable mineralization

Otol Neurotol. 2003 Nov;24(6):854-62.

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Increased activity of the diastrophic dysplasia sulfate transporter in otosclerosis and its inhibition by sodium fluoride.

Grayeli AB, Escoubet B, Bichara M, Julien N, Silve C, Friedlander G, Sterkers O, Ferrary E.

INSERM Unite 426, Faculte Xavier Bichat, Universite Paris 7, Paris, France. alexis.bozorg-grayeli@bjn.ap-hop-paris.fr

HYPOTHESIS: This study investigates the function of the diastrophic dysplasia sulfate transporter (DTDST) in otosclerotic bone and the effect on it of sodium fluoride (NaF). BACKGROUND: Otosclerosis is a localized bone dystrophy with increased bone turnover. DTDST is implicated in the regulation of the bone turnover. MATERIALS AND METHODS: Primary cultures of cells were obtained from the stapes and external auditory canal (EAC) of 26 patients with otosclerosis and from nine control patients. Sulfate uptake was quantified under basal conditions and with NaF. The NaF signaling pathways were investigated using forskolin and verapamil. RESULTS: The relative initial rates of sulfate uptake and the apparent Vmax values were: otosclerotic stapes > EAC > control stapes = control EAC. The sulfate uptake by the otosclerotic stapes was correlated with the loss of sensorineural hearing. The amounts of DTDST mRNA (RNase protection assay) in the four subgroups did not differ. NaF (10(-6)M, 1 hr) inhibited sulfate uptake by the otosclerotic stapes and EAC cells but not by control samples. CONCLUSION: The authors believe that whether the increased DTDST activity is a cause or an effect of otosclerosis, it appears to be a specific target for NaF treatment.

Acta Otolaryngol. 1987 Nov-Dec;104(5-6):447-53.

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Stapedius reflex in the monitoring of NaF treatment of subclinical otosclerosis.

Colletti V, Fiorino FG.

ENT Department, University of Verona, Italy.

Stapedius reflex testing was utilized to monitor the progression of subclinical otosclerosis and evaluate the efficacy of NaF treatment in stabilizing the disease process. The investigation was performed on 93 relatives of patients suffering with surgically confirmed otosclerosis. Subjects were divided into two groups. The first group was treated with NaF in doses ranging from 6 to 16 mg in relation to age. The treatment lasted two years. The second group served as control subjects. Before entering the research, all subjects presented reflex abnormalities represented by a partial or complete on-off effect in one or both ears. Changes in stapedius reflex morphology (from partial to complete on-off and from complete on-off to absence of the reflex) were evaluated at 1 and 2 years. The groups had not developed significant differences at 1 year. At 2 years, the incidence of stable stapedius reflex findings was 91.5% in the treated ears and 77% in the control group. The difference is significant (p less than 0.05) and indicates a stabilizing effect of NaF on the disease process.

Clin Otolaryngol Allied Sci. 1980 Oct;5(5):329-37.

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Eighteen-year report on stapedectomy. II. Postoperative therapy.

Causse J, Causse JB.

This second section deals with the basis of postoperative therapy following stapedectomy. They develop first the immediate postoperative care, then maintenance therapy, mainly NaF therapy to slow down or arrest cochlear enymatic deterioration, if needed.

Arch Otolaryngol. 1980 May;106(5):260-1.

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Effect of fluoride on alpha-chymotrypsin.

Kacker SK, Shambaugh GE Jr.

Sodium fluoride has been demonstrated by spectrophotometric experiment to have an inhibitory effect on the hydrolyzing action of alpha-chymotrypsin. Sodium fluoride in moderate therapeutic dosage possibly can retard or completely inhibit the activity of bone-resorbing enzymes in the otospongiotic focus, thus retarding or arresting active expansion of a focus and resulting in stabilizing or arresting the progression of sensorineural hearing loss that is associated with otospongiosis.

Otolaryngol Clin North Am. 1978 Feb;11(1):125-34.

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Sensorineural hearing loss due to cochlear otospongiosis: etiology.

Causse JR, Chevance LG.

This presentation discusses the most valuable way to correlate specific morphologic changes in cochlear otospongiosis with sensorineural hearing loss. Both biochemical and vascular factors may be responsible for the association of far advanced otospongiosis and histopathologic changes. An enzymatic concept of the disease is proposed on the basis of experimental findings and cytoclinical correlations, and the spread of proteolytic enzymes from the bursting lysosomes in histiocytes of the otospongiotic microfoci of the lateral wall. In addition, according to Ruedi's vascular concept, abnormal vascular connections called "shunts", provoked by active foci breakiny oxygen. Such extensive otospongiotic bone transformation breaking the endosteum of the cochlear is much less frequent than the progressive cochlear component encountered by otologic surgeons in far advanced otospongiosis. It is for this reason that the authors believe that their enzymatic concept of otospongiosis may explain the most important part of the sensorineural impairment in cochlear otospongiosis.

Acta Otolaryngol. 2001 Jan;121(2):200-4.

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Sensorineural hearing loss and otosclerosis: a clinical and radiologic survey of 437 cases.

Shin YJ, Fraysse B, Deguine O, Cognard C, Charlet JP, Sevely A.

Department of Otolaryngology, Purpan Hospital, Toulouse, France. shin.y@chu-toulouse.fr

The aim of this study was to determine if a relationship exists between bone level thresholds and the extension of otosclerotic foci within the otic capsule. The study consisted of a retrospective case review in a university hospital. We included patients who underwent surgery for otosclerosis in our department and who had a CT scan prior to surgery. We analyzed the data charts and CT scans of 437 cases (386 patients). On CT scan, we distinguished patients with fenestral otosclerosis and/or with a pericochlear focus. A pericochlear focus could be extended (Group 2) or not (Group 1) to the cochlear endosteum. Data for Groups 1 and 2 were compared with those for the control group of all patients for whom CT scan showed no cochlear focus (Group 3). Of the 437 CT scans, 399 were positive (91.3%). An anterior focus was reported in 305 cases (69.8%), a footplate thickening in 21 cases (4.8%) and both anomalies were encountered in 60 cases (13.7%). A pericochlear focus was reported in 53 examinations. This focus was extended to the endosteum in 14 cases (26.4% of the pericochlear foci). In Group 1, preoperative air conduction (AC) thresholds were significantly lower than in the control group (p < 0.05). The air--bone gap was also significantly larger in Group 1 (p < 0.05). Bone conduction (BC) thresholds were lower in Group 1 than in the control group but the difference was not significant. In Group 2, preoperative AC thresholds were significantly lower than in the control group (p < 0.05). BC thresholds were also lower in Group 2 than in the control group and the difference was significant (p < 0.05). As a result of this study, we assume that there may be a relationship between bone level thresholds and the radiological extension of otosclerosis within the otic capsule.

Ann Otolaryngol Chir Cervicofac. 1981;98(6):269-97.

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[Enzymatic mechanism of otosclerosis. Action of NaF]

[Article in French]

Causse JR, Uriel J, Berges J, Bretlau P, Causse JB.

The authors present the conclusions they reached after more than four years of multiple correlations made from february 1976 to september 1980. 648 perilymph samples were selected from a total number of 811 samples taken during stapedectomies on otosclerotic patients. These multiple correlations were based on micro-dosages of three selected enzymes (trypsin-alpha 1 antitrypsin and alpha 2 macroglobulin, the fourth cathepsin B having not been found, even in perilymph pools) in each of the selected samples, and on their relationship with the cochlear deterioration expressed in dBs of B.C. decrease and in audiometric stages corrected with reference to the patients' age. This study leads to an enzymatic mechanism, based on the previously reported trypsin-alpha 1 antitrypsin balance, but in which alpha 2 macroglobulin appears to play as essential a role as that of alpha 1 A. This enzymatic mechanism explains NaF efficiency, due in fact to a double action, not only to direct trypsin inhibition, but also to an overall reduction in enzymatic levels in the perilymph of otosclerotic patients. The authors conclude by suggesting the possibility of future NaF replacement by proteinase inhibitors, either of microbial origin currently under study by Japanese researchers, or even of synthetic origin.

Rev Laryngol Otol Rhinol (Bord). 2000;121(1):45-7.

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Pure sensorineural hearing loss and otosclerosis. An imaging case report.

Shin YJ, Deguine O, Sevely A, Manelfe C, Fraysse B.

CHU Purpan, Department of Otolaryngology, Toulouse, France. shin.y@chu-toulouse.fr

Pure sensorineural hearing loss is not a rare finding in otological practice. Numerous aetiologies could be at the origin of such a deficit. However, otosclerosis is very rarely cited as a cause of pure sensorineural hearing loss. We present one such case of pure sensorineural hearing loss linked to otosclerosis in a 30-year old caucasian male and underline the high contribution of computed tomography to confirm such a diagnosis. Pure sensorineural hearing loss due to otosclerosis is a rare event and can be misdiagnosed. The clinical diagnosis of such a disease may be difficult. In these cases, CT-Scan is the exam of choice to confirm the diagnosis.

Am J Otol. 1994 Jul;15(4):545-8.

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Effect of drinking water fluoridation on hearing of patients with otosclerosis in a low fluoride area: a follow-up study.

Vartiainen E, Karjalainen S, Nuutinen J, Suntioinen S, Pellinen P.

Department of Otolaryngology, University of Kuopio, Finland.

Fluorine content in bone samples taken from the middle ears of otosclerotic patients was determined. Otosclerotic stapes footplate was found to have a significantly higher content of fluorine than skeletal bone from the meatus. Fluorine contents in footplate and meatal wall samples of otosclerotic patients drinking fluoridated water were slightly higher than those of patients drinking low-fluoride water. In the clinical part of the study, hearing levels of 280 patients with otosclerosis living in an area with low-fluoride water were assessed. In 344 operated ears, the preoperative and long-term postoperative air conduction and bone conduction thresholds of patients drinking fluoridated water did not differ significantly from those of patients drinking low-fluoride water. After a mean follow-up period of 9.6 years, air conduction thresholds of non-operated ears in patients drinking fluoride-poor water were found to be significantly worse than those of patients drinking fluoridated tap water, likewise there were significant differences in bone conduction thresholds at 2 and 4 kHz. Thus, fluoridation of drinking water has a beneficial effect on non-operated otosclerotic ears but has no significant effect on hearing levels of operated ears.

Am J Otol. 1994 May;15(3):348-57.

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Endolymphatic hydrops associated with otosclerosis.

Shea JJ Jr, Ge X, Orchik DJ.

Shea Clinic, Shea Clinic Foundation, Memphis, TN 38119, USA.

Endolymphatic hydrops (EH) associated with otosclerosis has been noted for many years. However, the causal relationship of these two entities remains controversial. Having reviewed the records of patients with otosclerosis describing fluctuant hearing loss and vertiginous symptoms, the authors found the EH may coexist with otosclerosis preoperatively; they may be two separate diseases that exist coincidentally; or EH may be caused by the otosclerotic process. Secondly, EH may occur with a fistula after surgery. Occurring after stapedectomy, EH may be caused by fistulization of the bony labyrinth, which is effectively treated by surgery to seal the fistula, which may cause EH to subside and hearing to improve. Thirdly, delayed EH may occur months or years after stapedectomy, possibly as a result of otosclerotic foci or surgical insult to the labyrinth. Dexamethasone, diuretics, and a room air rebreather can be used in the treatment of delayed EH. Hearing may be maintained or may deteriorate, but there usually is no dizziness. The clinical manifestations of EH associated with otosclerosis include a conductive or mixed type of hearing loss; the presence of fullness, tinnitus, fluctuation of hearing, episodic vertigo, an elevated negative summating potential (SP), and an increased summating potential:action potential (SP:AP) ratio shown by ECoG. This report presents five cases of EH associated with otosclerosis.

Laryngorhinootologie. 1999 Jan;78(1):20-3.

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[Clinical aspects of the osteolytic (inflammatory) phase of cochlear otosclerosis]

[Article in German]

Arnold W, Kau R, Schwaiger M.

Klinik fur Hals-Nasen-Ohrenkrankheiten, Kopf- und Halschirurgie, Klinikum rechts der Isar, Technische Universitat Munchen.

During the last years it has been demonstrated that the active stage of otosclerosis (French: otospongieuse) is caused by an osteolytic inflammation associated with a measles-virus infection. Under influences that are not yet well understood (e.g. estrogens), the osteolytic process is arrested and changed to osteoblast activity accompanied by new bone formation. This latter process can be understood as a scar formation. If the process takes place within the area of the oval window, it results in fixation of the stapes and conductive hearing loss. When the otosclerotic process is restricted to the cochlea, clinical signs are not well defined. We report on exemplary cases of progressive bilateral sensorineural hearing loss caused by otosclerosis, restricted to the cochlea. The characteristics of the clinical course include: 1. Bilateral asymmetric sensorineural hearing loss. 2. Roaring tinnitus. 3. Episodes of sudden hearing loss. 4. Excellent response to prednisolone therapy. 5. In the late stage of the disease a small but transitory conductive hearing loss. High resolution computed tomography in such cases reveals diffuse osteolytic foci within the cochlear walls. In severe cases of such progressive deafness we discuss an antiproliferative (immunosuppressive) therapy with cyclosporine and/or a radionuclide therapy with Sn-117 m, an isotope with a very small irradiation radius.

Br J Radiol. 2002 Jun;75(894):502-5.

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MRI of cochlear otosclerosis.

Goh JP, Chan LL, Tan TY.

Department of Diagnostic Radiology, Tan Tock Seng Hospital, Department of Diagnostic Radiology, Singapore General Hospital and Department of Radiology, Changi General Hospital, Singapore.

Cochlear otosclerosis is an uncommon cause of mixed and sensorineural hearing loss. This has a characteristic appearance on CT, producing a distinctive pericochlear hypodense double ring. However, its appearance on MRI is not as readily appreciated, producing a ring of intermediate signal in the pericochlear and perilabyrinthine regions on T(1) weighted images, demonstrating mild to moderate enhancement after gadolinium administration. Increased signal on T(2) weighted images may also be seen. Recognition of these MRI features is important as MRI may be the first modality of investigation, especially when patients present with symptoms indicative of sensorineural hearing loss. We review four patients who presented with sensoineural hearing loss, and who were imaged with MRI as the first line of investigation.

Otol Neurotol. 2002 Jul;23(4):431-8.

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Single photon emission computed tomography in otosclerosis: diagnostic accuracy and correlation with age, sex, and sensorineural involvement.

Berrettini S, Ravecca F, Volterrani D, Forli F, Boni G, Neri E, Franceschini SS.

Ear Nose and Throat (ENT) Unit, Neuroscience Department, and Oncology Department, University of Pisa, Via Savi 10, 56100 Pisa, Italy. s.berettini@ent.med.unipi.it

OBJECTIVE: To evaluate the accuracy of single photon emission computed tomography (SPECT) in detecting otospongiotic foci of the labyrinthine capsule in otosclerotic patients and to correlate the metabolic patterns detected by SPECT with age, sex, and sensorineural hearing loss. PATIENTS AND METHODS: Thirty-six patients with surgically confirmed otosclerosis and a control group of 12 subjects with normal hearing; each subject underwent SPECT study of the skull with (99m)technetium-diphosphonate ((99m)Tc-medronate). Statistical analysis of the results was performed by use of the Mann-Whitney U test. RESULTS: SPECT seemed to be very sensitive in differentiating otospongiotic bone from normal bone; in the affected group, only 2 of 72 ears yielded false-negative results (sensitivity 97.2%). Moreover, in the otosclerotic patients, the mean petrosa uptake value was higher than in the control group, a difference that was statistically significant (p < 0.0001). MAIN OUTCOME MEASURES: Regarding uptake in relation to patient age, an inverse relationship was observed between increased metabolic activity and age: the mean uptake index in the younger patients was significantly higher than that in the older patients (p < 0.0001). By contrast, no statistically significant differences were found between men and women (p = 0.1519). Comparison of SPECT alterations with bone conduction thresholds revealed a statistically significant correlation (p = 0.0002 and p = 0.0010) between increased metabolic bone activity and sensorineural involvement only in the younger group of otosclerotic patients. CONCLUSIONS: Diphosphonate bone SPECT is the only functional method that allows in vivo evaluation of disease activity, and it was highly sensitive in detecting otosclerosis. SPECT can be considered useful in the diagnosis of otosclerosis that is difficult to recognize, such as cochlear otosclerosis and far-advanced otosclerosis, and it could also find a place in evaluating the efficacy of medical therapy for otosclerosis.

Acta Otorhinolaryngol Belg. 1981;35(5-6):419-30.

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Otosclerosis. Histopathology, light and electron microscopy.

Friedmann I.

Cochlear otosclerosis, in the histopathological sense, is not uncommon but remains a clinical enigma. Similarly the aetiology of otospongiosis has remained obscure. Nevertheless the enzymatic concept of the initiation and mechanism of the underlying process has been recognized and confirmed together with the essential function of the matrix vesicles forming the site of primary enzymatic calcification. It may be concluded that otospongiosis is a primary lytic process initiated by lysosomal enzymes of matrix vesicles activated in the cartilaginous cell rests of the area. The cause of the process is still unknown.

Ear Nose Throat J. 1989 Jun;68(6):426, 428-9.

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Fluoride therapy for otosclerosis.

Kerr GS, Hoffman GS.

From the extensive morphological studies performed, otosclerosis appears to be ubiquitous, and in many instances subclinical. Despite reports of the efficacy of fluoride for this condition, at present its use should be regarded as experimental.

Otol Neurotol. 2004 Jul;25(4):457-64.

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Spiral ligament and stria vascularis changes in cochlear otosclerosis: effect on hearing level.

Doherty JK, Linthicum FH Jr.

Department of Otolaryngology-Head and Neck Surgery, University of Southern California, Los Angeles, USA. jdoherty@usc.edu

OBJECTIVE: To investigate the effect of changes within the spiral ligament and stria vascularis on hearing in cochlear otosclerosis, we examined spiral ligament hyalinization, stria vascularis atrophy, and sensory hearing loss in cochlear otosclerosis and described changes in ion transport molecule expression. STUDY DESIGN: Retrospective. SETTING: Tertiary referral center. PATIENTS: Thirty-two cochleae from 24 temporal bone donors with histologic evidence of cochlear otosclerosis, including spiral ligament hyalinization. INTERVENTION: Audiography. MAIN OUTCOME MEASURES: Measurements of spiral ligament width, stria vascularis, and bone-conduction thresholds were compared by the amount of hyalinization. Expression of the ion transport molecules Na,K-ATPase, connexin 26, and carbonic anhydrase II were assessed by immunohistochemical techniques. RESULTS: Hyalinization most often involved the posterior basal turn (88%) and the posterior middle turn (27%). Spiral ligament hyalinization correlated significantly with stria vascularis atrophy in the posterior middle turn of the cochlea (rho = -0.63, p < 0.01). There was a trend toward a significant association in the posterior basal turn (rho = -0.31, p < 0.08). Bone-conduction thresholds at 2,000 and 4,000 Hz were significantly associated with the amount of stria vascularis atrophy (rho = -0.44, -0.40, p < 0.05). In addition, we observed decreased immunostaining for both carbonic anhydrase II with Type I fibrocytes and Na,K-ATPase with stria vascularis and Type II and Type IV fibrocytes of the spiral ligament in cochlear otosclerosis sections compared with normal cochlea. Na,K-ATPase staining within the stria vascularis was further decreased in the presence of spiral ligament hyalinization. No significant differences were seen with connexin 26 immunostaining. However, immunostaining results were somewhat inconsistent. CONCLUSION: These data suggest that spiral ligament structure and function are essential for stria vascularis survival. In addition, dampened expression of ion transport molecules within the spiral ligament and stria vascularis may disrupt potassium ion recycling, resulting in loss of endocochlear potential and sensory hearing loss.

Eur Rev Med Pharmacol Sci. 1999 Jul-Aug;3(4):179-82.

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Otosclerosis and cochlear otosclerosis: a post mortem study on temporal bones.

Salvinelli F, Trivelli M, Greco F, Linthicum FH Jr.

Institute of Otolaryngology, Campus Bio-Medico University, Rome, Italy.

We have chosen among many temporal bones of donors deceased individuals with concomitant otosclerosis, three particular cases, one with classic otosclerosis, another with cochlear otosclerosis with concomitant oval window ankylosis and another with cochlear otosclerosis without stapes fixation. The different histopathologic features are discussed and clinical and therapeutical guidelines are proposed.

Am J Otol. 1994 Jul;15(4):536-9.

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Mixed hearing loss in otosclerosis: indication for long-term follow-up.

Ramsay HA, Linthicum FH Jr.

House Ear Institute, Los Angeles, CA 90057, USA.

This retrospective study of 146 ears with long-term follow-up after otosclerosis surgery evaluated the stability of hearing results, the incidence of sensorineural hearing loss, and the effect of fluoride treatment. Follow-up was at least 15 years (mean, 25.2 yr; range, 15-44 yr). There were 97 large fenestra stapedectomy operations, 23 lateral canal fenestrations, 7 mobilizations, and 19 revision stapes operations. The level of air-bone gap achieved at surgery remained stable over time; the mean deterioration rate was only 0.2 dB per year. Profound sensorineural hearing loss ( > or = 65 dB bone conduction average) at the most recent follow-up occurred in 13 ears (8.9%). Such hearing loss occurred in all operative groups. Mean bone conduction average immediately postoperatively was significantly higher in these ears than in others in the study. This finding indicates that a mixed hearing loss at surgery is a factor that increases the risk of later profound cochlear loss. Only 3 percent of ears with pure conductive hearing loss, but 28 percent of patients with mixed hearing loss at surgery eventually suffered profound cochlear loss. Sodium fluoride was used to treat 11 ears with progressive cochlear loss. The rate of bone conduction hearing deterioration decreased in all ears after treatment, and none developed profound hearing loss. Follow-up after the first postoperative year is not necessary if pure conductive hearing loss is present at surgery. Annual follow-up with audiograms is recommended if a mixed hearing loss is present. Fluoride treatment is recommended if inner ear hearing loss progresses.

Otolaryngol Clin North Am. 1993 Jun;26(3):335-52.

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Histopathology of otosclerosis.

Linthicum FH Jr.

House Ear Institute, Los Angeles, California.

This histopathologic review illustrates that otosclerosis is a very pleomorphic bone dyscrasia. It may fix the stapes by a tiny bridge of abnormal bone or may totally obliterate it. It may spread to the cochlea to produce either a sensorineural loss of varying degree or deafness. Lesions may be sclerotic, spongiotic, or fibrous but usually comprise all these histologic types. This diversity of size and anatomic distribution coincides with the variable types of hearing loss, from a mild conductive deficit to a total loss. In spite of more than a century of investigation of otosclerosis, an etiologic agent other than heredity has not been established.

Am J Otol. 1990 May;11(3):196-200.

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Cochlear otosclerosis: does bone formation affect cochlear implant surgery?

Fayad J, Moloy P, Linthicum FH Jr.

House Ear Institute, Los Angeles, California.

This study aimed to demonstrate that new bone formation in the scala tympani of patients deaf from otosclerosis does not preclude cochlear implant surgery. In seven temporal bones from patients with otosclerosis, we measured the extent of new bone from the round window to the distal part of the new growth. We compared results to surgical data on the extent of drilling and depth and ease of placement of the electrode in 20 patients deaf from otosclerosis. We also examined clinical performance and voltage requirements for long-term implant use in patients with and patients without ossification of the scala tympani. Findings in our limited sample of patients and bones show that obstruction of the basal turn, which occurs in some otosclerotic patients, does not preclude implant surgery. The dynamic range in the studied sample was relatively stable long-term and clinical performance did not differ between groups with and without an ossified scala tympani.

Laryngoscope. 1984 Apr;94(4):508-12.

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Otosclerosis and otospongiosis: clinical and histological comparisons.

Parahy C, Linthicum FH Jr.

The simultaneous occurrence of otosclerosis and otospongiosis in the same lesion is well documented. The logical sequence of events would seem to be softening and destruction of the bone by the otospongiotic lesion, which is subsequently converted to otosclerosis. Examination of 46 temporal bones has led us to further classify the lesion as: 1. an active lesion, both otospongiotic and otosclerotic, 2. an inactive lesion, also both otosclerotic and otospongiotic, or 3. a fibrous lesion. Hyalinization of the spiral ligament only occurs adjacent to active otospongiotic or very large otosclerotic lesions. If the lesion adjacent to the spiral ligament is inactive, there is no hyalinization. It seems illogical that a hyalinized spiral ligament will return to normal when the lesion changes from active otospongiosis to inactive otosclerosis. Otospongiotic and otosclerotic lesions are found side by side at the periphery of lesions, and both are adjacent to normal bone. These findings have implications concerning the mode of action of sodium fluoride and other medications that are under evaluation for the control of the sensorineural hearing loss due to otosclerosis.

Ann Otol Rhinol Laryngol. 1984 Mar-Apr;93(2 Pt 1):105-11.

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Histologically proven cochlear otosclerosis with pure sensorineural hearing loss.

Balle V, Linthicum FH Jr.

This paper discusses cochlear otosclerosis without stapedial fixation as a cause of sensorineural hearing loss. Because many cases of cochlear otosclerosis may remain undiagnosed, we believe that patients with a progressive sensorineural hearing loss and a family history of cochlear otosclerosis should be suspected of having cochlear otosclerosis. A positive stapedial reflex on-off effect or polytomographic findings help make the diagnosis. We prescribe sodium fluoride, calcium, and vitamin D for these patients in an effort to prevent progression of the loss.

Ann Otol Rhinol Laryngol Suppl. 1982 Sep-Oct;97:3-15.

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Cochlear and otoconial abnormalities in capsular otosclerosis with hydrops.

Johnsson LG, Hawkins JE Jr, Rouse RC, Linthicum FH Jr.

Temporal bones from four patients with capsular otosclerosis were examined by microdissection. Otoconia and abnormal crystalline deposits were studied by scanning electron microscopy and x-ray analytical methods. One patient showed more or less symmetrical invasion of the basilar membrane and osseous lamina by connective tissue from thickened endosteum adjacent to the larger anterior foci. In one ear sensorineural degeneration was circumscribed; in the other it was extensive and associated with cochleosaccular hydrops. Two other patients were deaf, with severe sensorineural degeneration; one had multiple active foci and evidence of cochleosaccular hydrops. In the fourth patient, who had small anterior foci, no specific inner ear pathology was found related to otosclerosis. In the hydropic labyrinths, apatite was present as abnormal deposits in the cochlear duct and as rigid crusts replacing the otoconia. Apparently these changes had been associated with abnormal labyrinthine fluid dynamics rather than with the otospongiotic process per se.

Ann Otol Rhinol Laryngol Suppl. 1978 Mar-Apr;87(2 Pt 3 Suppl 48):1-40.

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Cochlear and vestibular lesions in capsular otosclerosis as seen in microdissection.

Hawkins JE Jr, Linthicum FH Jr, Johnsson LG.

In 24 temporal bones from patients with otosclerosis prepared by the method of microdissection and surface preparations, otosclerotic foci could be clearly seen during removal of the otic capsule. The state of activity of each focus was estimated on the basis of its consistency and vascularity. Small anterior foci constituted the most common form of involvement of the otic capsule. All were judged to be inactive, and none of them appeared to have caused obvious sensorineural degeneration. No cases of "pure cochlear otosclerosis" were seen. Sensorineural degeneration was associated with large anterior foci which reached the upper basal turn. One specimen displayed a circumscribed sensorineural degeneration in the upper basal turn, with an almost exact correspondence between the location and extent of the cochlear lesion and the site of invasion by the otosclerotic process in the bone and endosteum bordering on scala media and scala tympani. It is postulated that a toxic factor had diffused from the focus and acted directly on the organ of Corti. When multiple foci were present they were usually poorly defined. The otosclerotic process involved the round window, with new lamellar bone formation in the scala tympant of the lower half of the basal turn. The most extensive sensorineural degeneration in the entire material was seen in this group. One specimen also had severe cochlear hydrops. In three specimens large shunts were observed to connect the otosclerotic foci with the cochlear vasculature, which was severely dilated. Where otosclerosis involved the endosteum of the scala tympani, loss of vessels was observed. One specimum with extensive active capsular otosclerosis had severe sensorineural degeneration of the vestibular system. Vestibular pathology in fenestrated ears is also described. In a specimen from a patient with no caloric reaction, numerous hair cells were present in the macular organs.

Ann Otol Rhinol Laryngol. 1978 Jan-Feb;87(1 Pt 1):85-90.

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Otosclerotic inner ear syndrome.

Ghorayeb BY, Linthicum FH Jr.

Twenty-seven patients with clinical otosclerosis and vertigo underwent stapedectomies. Vertigo improved or completely disappeared in 23 patients, indicating that stapedectomy is probably helpful. Three case reports are also presented as well as photomicrographs of temporal bones showing the focus in contact with the pars superior.

Laryngoscope. 1977 Oct;87(10 Pt 1):1746-52.

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Unrelated sensorineural hearing loss in patients with otosclerosis. A report of three cases.

Linthicum FH Jr, Neely JG.

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.

Ann Otol Rhinol Laryngol. 1975 Jul-Aug;84(4 Pt 1):544-51.

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Sensorineural hearing loss due to cochlear otospongiosis: theoretical considerations of etiology.

Linthicum FH Jr, Filipo R, Brody S.

Several theories have been advanced to explain the sensorineural hearing loss that occurs in patients with otospongiosis: toxic substances produced by the otospongiotic focus; vascular shunts betwen the inner ear vessels and the otospongiotic focus; and atrophy of the organ of Corti and stria vascularis due to unknown causes. Presented here is yet another theory: impingement upon the cochlear walls by the otospongiotic focus, causing a narrowing of the lumen of the cochlea and distortion of the basilar membrane.

Ann Otol Rhinol Laryngol. 1975 Jan-Feb;84(1 Pt 1):11-5.

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Sensorineural impairment in unilateral otosclerosis.

Linthicum FH Jr, Lalani AS.

Audiometric findings were evaluated in a group of patients with clinical unilateral otosclerosis. A comparison was made between bone conduction threshold in the better hearing ear and the ear with the mixed hearing loss. Over 50% of the patients had a significant elevation in the bone conduction thresholds in the ear with clinical otosclerosis as compared to the uninvolved ear. A pair of temporal bones from a patient with unilateral otosclerosis and unilateral mixed hearing loss is presented.

 

 

 
 
 

 

 

 

 

 

 

 

 

 

 

 

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Τελευταία Ενημέρωση (Δευτέρα, 08 Ιανουάριος 2018 10:00)

 
Το παρόν άρθρο προστατεύεται από το Νόμο 2121/1993 και 4481/2017 για την πνευματική ιδιοκτησία. Η ολική ή μερική αντιγραφή του παρόντος επιστημονικού άρθρου χωρίς τη γραπτή έγκριση του Δρ Δημητρίου Ν. Γκέλη θεωρείται κλοπή πνευματικής ιδιοκτησίας και διώκεται βάσει της νομοθεσίας.
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