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OTOSCLEROSIS DR.P.KARTHIKEYAN PROF&HOD DEPT.OF ENT 07.03.2016

Otosclerosis dr.p.kartyhikeyan,07.03.2016

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Page 1: Otosclerosis dr.p.kartyhikeyan,07.03.2016

OTOSCLEROSIS DR.P.KARTHIKEYA

NPROF&HOD

DEPT.OF ENT07.03.2016

Page 2: Otosclerosis dr.p.kartyhikeyan,07.03.2016

OBJECTIVESDefinition of otosclerosisEtiology of otosclerosisTypes of otosclerosisClinical features of otosclerosisDiagnosis of otosclerosisMedical management of

otosclerosisSurgical management of

otosclerosis

Page 3: Otosclerosis dr.p.kartyhikeyan,07.03.2016

A primary disease of otic capsule in which irregular spongy bone replaces the dense endochondral layer of bony otic capsule, thereby fixing the footplate of the stapes and causing conductive hearing loss.

OTOSCLEROSIS

Page 4: Otosclerosis dr.p.kartyhikeyan,07.03.2016

HISTORY OF OTOSCLEROSIS1704 – Valsalva first described stapes

fixation1857 – Toynbee linked stapes fixation

to hearing loss1890 – Katz was first to find

microscopic evidence of otosclerosis1893 – Politzer described the clinical

entity of “otosclerosis”

Page 5: Otosclerosis dr.p.kartyhikeyan,07.03.2016

ETIOLOGYExact etiology – not known1) Idiopathic – Remnants of

embryonic cartilage resting in the otic capsule may be the etiological factor.

2) Heredity3) Hormonal – Symptoms

increase during pregnancy and menopause.

4) Van der Hoeve syndrome – Osteogenesis imperfecta,

otosclerosis, Blue sclera

Page 6: Otosclerosis dr.p.kartyhikeyan,07.03.2016

5) Associated with Paget’s disease.6) Enzymatic theory – (Latest)

Imbalance in trypsin / antitrypsin in the inner ear fluid initiates otosclerosis.

7) Metabolic and immune disorders.8) Anatomical and Histological

anomalies of temporal bone.9) Recent – Relationship between prior

infection with measles virus and later development of otosclerosis.

Page 7: Otosclerosis dr.p.kartyhikeyan,07.03.2016

INCIDENCE0.5-1% of total population Female: Male (2:1) 20 – 30 years of age Common in white races, but

common in Indians and low in chinese and Japanese.

Usually bilateral (85%)

Page 8: Otosclerosis dr.p.kartyhikeyan,07.03.2016

TYPES1) Stapedial otosclerosis : (common)

Sites – 1) Fissula ante fenestrum

2) Fissula post fenestrum

3) Circumferential 4) Biscuit type or rice

grain type with delineated margins

5) Obliterative type

Page 9: Otosclerosis dr.p.kartyhikeyan,07.03.2016
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2) Cochlear otosclerosis:

Involves the region of round window and labyrinth in the absence of stapes

fixation

Sensorineural hearing loss due to liberation of toxic materials from abnormal bone into inner ear.

Page 11: Otosclerosis dr.p.kartyhikeyan,07.03.2016

3) Malignant otosclerosis Severe type of cochlear

otosclerosis Starts early in life and

progresses rapidly.4) Combined otosclerosis –

mixed hearing loss.5) Histological otosclerosis:

9 – 12% cases, No clinical features but

histologically the focus is present.

Page 12: Otosclerosis dr.p.kartyhikeyan,07.03.2016

PATHOLOGYGrossly – appears as chalky white or

yellow focus (inactive) or red in colour due to increased vascularity (active)

Microscopically – 1) In immature (active) foci, there are

numerous marrow and vascular spaces with plenty of osteoblasts and osteoclasts which stains blue on H&E stain. (Blue mantle)

2) In mature (inactive) foci, there is less vascular spaces with lot of fibrous tissue which stains red on H&E stain

Page 13: Otosclerosis dr.p.kartyhikeyan,07.03.2016

CLINICAL FEATURESSymptoms

1) Hearing loss – conductive

2) Tinnitus

3) Paracusis willisi

4) Monotonous, well modulated soft speech.

5) Vertigo

Page 14: Otosclerosis dr.p.kartyhikeyan,07.03.2016

SIGNS1) TM : normal and mobile

2) Schwartze sign or Flamingo pink appearance

3) TFT : Conductive hearing loss

4) PTA : Carhart’s notch

5) Impedance Audiometry – Type As curve

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TYMPANOMETRY

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DIFFERENTIAL DIAGNOSISOssicular discontinuityCongenital stapes fixationMalleus head fixationPaget’s diseaseOsteogenesis imperfecta

Page 18: Otosclerosis dr.p.kartyhikeyan,07.03.2016

Natural History of Otosclerosis90% of all cases are never clinically

apparentFoci begins in childhood

Most commonly becomes symptomatic in the 3rd and 4th decades

After clinical presentation◦ Conductive hearing loss progressive◦ Periods of quiescence and deterioration◦ Worsening tinnitus◦ Associated SNHL (rarely purely SN)

Page 19: Otosclerosis dr.p.kartyhikeyan,07.03.2016

Tab. Sodium fluoride Dose : 50 – 75 mg/day, Duration : 3 months – 2 years

Function 1) helps to hasten the maturity of active

focus and arrest further progression of cochlear loss

2) It has antienzymatic action on proteolytic enzymes which are cytotoxic to cochlea.

MEDICAL TREATMENT

Page 20: Otosclerosis dr.p.kartyhikeyan,07.03.2016

Indications: Cochlear otosclerosis Active stapedial otosclerosis

Side effects : Fracture of long bones and spine due to

fluorosis.Nephritis. Gastritis

Contraindications: Pregnancy & lactation Patient with kidney stones / nephritis Patient with RA

Page 21: Otosclerosis dr.p.kartyhikeyan,07.03.2016

History of Stapes SurgerySamuel Rosen

◦1953 – first suggested mobilization of the stapes Immediate

improved hearing

Re-fixation

Page 22: Otosclerosis dr.p.kartyhikeyan,07.03.2016

History of Stapes SurgeryJulius Lempert

◦ Popularized the single staged fenestration

in the horizontal canal with a tissue graft covering

◦ >2% profound SNHL◦ Rarely complete closure of

the ABGJohn House

◦ Further refined the procedure Popularized blue lining the

horizontal canal

Page 23: Otosclerosis dr.p.kartyhikeyan,07.03.2016

History of Stapes SurgeryJohn Shea

◦1956 – first to perform stapedectomy Oval window

vein graft Nylon prosthesis

from incus to oval window

Page 24: Otosclerosis dr.p.kartyhikeyan,07.03.2016

SURGICAL TREATMENT1) Lempert’s fenestration operation

(1938)2) Rosen’s stapes mobilisation

(1953)3) Shea’s stapedectomy (1958)4) Stapedotomy – small fenestra

stapedectomy. (Laser can be used)

Hearing Aids

Page 25: Otosclerosis dr.p.kartyhikeyan,07.03.2016
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STAPEDECTOMY

Indication: Stapedial otosclerosis (inactive)

Selection of patient: Rinne negative Audiogram : AB gap at least 20dB Should have good speech

discrimination

Page 27: Otosclerosis dr.p.kartyhikeyan,07.03.2016

Contraindications:1. Only hearing ear2. Otitis externa, CSOM3. Cochlear otosclerosis4. Young children / old age5. Athletes, drivers, frequent

air travelers, those who works in noisy environment.

6. General medical illness7. Pregnancy.

Page 28: Otosclerosis dr.p.kartyhikeyan,07.03.2016

Complications

1. Perforation of ear drum2. Total SN loss3. Chronic vertigo4. Facial nerve paralysis5. Perilymph fistula6. Granuloma.

Page 29: Otosclerosis dr.p.kartyhikeyan,07.03.2016

THANK YOU