Otosclerosis

Dr. Vishal Sharma

Definition
Hereditary disease of bony labyrinth showing

replacement of lamellar enchondral bone by

irregularly laid new bone. New bone is spongy + more vascular in active Otospongiosis but thicker & more cellular in inactive Otosclerosis.

Antonio Valsalva
First described

ankylosis of stapes

in 1741

Adam Politzer
Coined the term

Otosclerosis

in 1893

Friedrich Siebenmann
Coined the term

Otospongiosis

in 1912

Epidemiology
Exact etiology is unknown (? Viral)

Autosomal dominant: variable penetrance

Race: common in white races & Indians

Female : Male = 2 : 1
Age: Common in 20 - 40 years

Hormonal influence: es in pregnancy,

menopause, stress (trauma, surgery)

Van der Hoeve syndrome

Otosclerosis + osteogenesis imperfecta + blue sclera

Types of Otosclerosis
A. Stapedial

B. Cochlear: otosclerotic focus is seen over

Round window Promontory C. Stapedial + cochlear: mixed type D. Malignant: rapidly progressing cochlear lesion with severe sensori-neural deafness.

Types of Stapedial Otosclerosis

1. Anterior focus (commonest): 2 mm anterior to oval window. 2. Posterior focus: 2 mm behind oval window. 3. Circumferential: involves footplate margin only.

Types of Stapedial Otosclerosis

4. Biscuit type: footplate involved, margin is free.

5. Obliterative: obliterates oval window completely.

Symptoms of Otosclerosis
1. Deafness: Bilateral, slowly progressive

Conductive: stapedial otosclerosis

Sensori-neural: cochlear otosclerosis Mixed: stapedial + cochlear otosclerosis 2. Soft, modulated, monotonous voice 3. Tinnitus & vertigo: in cochlear lesion

Symptoms of Otosclerosis
4. Paracusis Willisii: Pt has better hearing in noisy surroundings (people increase their voice intensity & pts speech discrimination becomes better).

Thomas Willis (1621-1675)

Otoscopy
Normal T.M. is seen in

most cases. Pinkish

colour over promontory

seen in otospongiosis
(2 - 10 % cases)

Schwartze sign /
Flamingo pink blush.

Tuning Fork Tests

Rinne
Stapedial Negative (BC > AC) Positive (AC > BC) Negative (BC > AC)

Weber
Lateralizes to Deaf ear Lateralizes to Better ear Lateralizes to Better ear

A.B.C.
Normal

Cochlear

Decreased

Mixed

Decreased

Gelle & Bing Tests

Vibrating tuning fork placed over mastoid & then: External auditory canal is blocked in Bing test or E.A.C. pressure ed by Siegalization in Gelle test Bing Gelle

Otosclerosis
Normal / SNHL

No change
Intensity es

No change
Intensity es

Pure Tone Audiometry

Low frequency conductive deafness Carharts notch in bone conduction at 2 KHz

Carharts notch
Proposed theories

1. Stapes fixation disrupts normal ossicular

resonance (2000 Hz) 2. Normal compression mode of bone conduction is disturbed because of relative perilymph immobility 3. Mechanical artefact Carharts notch reverses with stapes surgery

Speech Audiometry
Speech Discrimination Score (maximum score achieved) is almost 100 %.

Speech Reception Threshold (intensity at which

50 % words are heard) is increased by the amount of conductive hearing loss.

Speech Audiometry

Impedance Audiometry
As curve seen in 40 %
cases of otosclerosis. Normal middle ear pressure + decreased middle ear compliance. Others have A curve.

Stapedial reflex present

Stapedial reflex absent

C.T. scan temporal bone

200 coronal oblique cuts are taken

Stapedial otosclerosis (coronal)

Cochlear otospongiosis (axial)

Differential Diagnosis
Otitis Media with Effusion: type B tympanogram

Adhesive Otitis Media: absence of T.M. mobility

Tympanosclerosis: white patch on T.M. Ossicular discontinuity: type Ad tympanogram Congenital ossicular chain fixation: tympanotomy Malleus head fixation: tympanotomy

History of development of stapes surgery

Stapes mobilization: Kessel (1880), Rosen (1952) Lateral semicircular canal fenestration: Holmgren

(1923), Sourdille (1932), Lempert (1938)

Complete Stapedectomy: Jack (1893), Shea (1956) Partial Stapedectomy (posterior 1/3): Plester (1960) Stapedotomy: Shea (1962), Marquet (1965) Laser Stapedotomy: Perkins & Di Bartolomeo (1980)

Johannes Kessel

Samuel Rosen

Gunnar Holmgren

Maurice Sourdille

Julius Lempert

John J. Shea Jr.

Inclusion criteria for surgery

Pure Tone Average between 30 - 60 dB
Air bone gap > 15 dB Speech discrimination score > 60 %

Absence of sensorineural deafness

Contraindications for surgery

Only hearing ear Otitis media Menieres disease Otitis externa

Extremes of age

Pregnancy

Professions: divers, high construction workers, frequent travelers, noisy surroundings

Surgical steps for Stapedotomy

Right T.M. (upright)

Right T.M. (supine)

Per-meatal Incision

Tympanomeatal flap raised

Bony overhang curetted

Bony overhang curetted

Chorda tympani preserved

Chorda tympani separated

Confirm footplate fixation

Checking for absence of round window reflex

Depth measurement prosthesis

Incudo-stapedial joint broken

Footplate perforation made

Fenestration with burr

Posterior crus fractured

cm

Teflon piston
Length of piston = medial surface of incus to stapes footplate + 0.25 mm Range = 3.75 4.25 mm

Piston placed in perforation

Stapedius tendon cut

Stapedius tendon cut

Piston crimped around incus

Anterior crus fractured

Stapes superstructure removed

Footplate perforation sealed

Tympanomeatal flap put back

Laser Stapedotomy

Stapedius vaporization

Footplate fenestration

Rossette formation

Stapedotomy Piston

Stapedectomy

Footplate Fenestration

Stapes superstructure removed

Footplate removal

Prosthesis placed over vein graft

Complications of stapes surgery

Intra-operative Floating footplate Submerged footplate Dislocated incus Perforated TM Damage to facial nerve or chorda tympani

Post-operative Otitis media Oval window granuloma Perilymph fistula Sensori-neural HL Persistent air-bone gap Vestibular dysfunction

Persistent stapedial artery Delayed facial palsy in

Perilymph flooding laser surgery

Sodium Fluoride
Given in cochlear otosclerosis & active focus of otospongiosis (Schwartze sign). Acts by: a. Decreases bone resorption b. Increases bone formation

c. Prevents enzymatic damage to cochlea

20 mg orally, thrice daily for 3 - 6 months

Hearing Aid
For patients who:

Are unfit for surgery

Refuse surgery

Thank You