Question

Consider the client's case history: 30 year-old female with gradual hearing loss over the last 3 years. Her older sister reports problems hearing since about the age of 18. She reports a light roaring sound in her left ear, no problem with dizziness, otalgia, pressure or otorrhea. The audiologist is suspicious of otosclerosis but needs an ENT to make the official diagnosis.

Answer 1

Pateint is suffering from Otosleosis as she is female with affected sister age around 30 years with gradual hearing loss over 3 years with roaring sound in noisy enviroment .Other causes are ruled out as there is no otorrhea ,otalgia or dizziness.

Otosclerosis-Disease of bony labyrinth .It is also known as Otospongiosis which is active stage of otosclerosis .

Autosomal Dominant heritance

Common in female age group 20-30 yrs

It may be worsened by Pregnancy,menopause or after major surgery

Van der Hoeve syndrome - Triad of Otosclerosis + Blue sclera + Osteogenesis imperfecta

3 Types of Otosclerosis are there in which m/C type is Stapedial Otosclerosis in which stapes fixation occur and conductive hearing loss present .

M/C site of Otosclerosis - Anterior edge of oval window ( Fistula ante fenestrum / Cozzilina zone )

Clinical features

1. Female presenting with gradually progressive B/L conductive hearing loss .

2. Paracusis willisii - Hearing is better in noisy surrounding

ENT examination on Otoscopy -- Tympanic membrane is normal and mobile .

Schwartz sign --Reddish hue or flemmingo pink reflex seen through intact Tympanic membrane .It is present in early stages of disease and is a contraindication for surgery .

Blue Mantle of Manasse is microscopy sign .

Tunning fork test -- Rinne test negative as there is conductive hearing loss so bone conduction is better than aur conduction .

On PTA pure rone audiometry loss of air conduction is more for lower frequencies .Carharts notch is dip in bone conduction curve in both ears appear at 2000 Hz ,which disappears after Succesful Stapedectomy .

Treatment

Surgical --- Stapedotomy (includes Rosen incision) is Treatment of choice

Stapedectomy with placement of Teflon prosthesis

Medical -- NaF sodium fluoride is used when Schwartz sign is positive,radiologically active Otosclerosis focus is present .It arrests further cochlear loss by decreasing the osteoclastic activity and increasing osteoblastic activity .