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Physiology of hearing and hearing loss

Question
38 out of 39
 

Sensorineural deafness is seen in: [PGI May 2014]



A Alport's syndrome

B Pend red's syndrome

C Treacher-Collins syndrome

D Crouzon's disease

E Michel's aplasia

Ans. A B, C and E. Aport's syndrome; Pendred's syndrome; Treacher-Collinssyndrome and Michel's aplasiA.

Causes of Congenital Deafness

Conductive

Meatal atresia

Fixation of stapes footplate

Fixation of malleus head

Congential cholesteatoma

·0 Ossicular discontinuity

Crouzons syndrome

Aperts syndrome

Mnemonic

Sensorineural deafness

Assistant

Branch

Manager

W

A

R

K

U

T

Just

Loves

To

Have

Pineapple

And

Orange

Raita

Aplasia

Bartter's syndrome

MELAS

Waardenburg syndrome/ wildervanck syndrome

Alport syndrome (SNHL develops by the age of 30 yrs)

Refsum syndrome

Klippel feil syndrome

Ushers syndrome

Treacher Collins syndrome'>

Jervell and Lange neilson syndrome

Leopard syndrome

Trisomy 13, 15,21

Hyper pigmentation

Pend red syndrome

Albinism

Onychodystrophy

Renal tubular acidosis (DistallType I)

Note

Stickler syndrome Treacher collins syndrome (current otolaryngology 2/e pg-700), vander hoeve syndrome, Pierre Robin syndrome can lead to both SNHL or conductive hearing loss.

Aplasia - Michels aplasia: characterised by lack of development of inner ear. External ear and middle ear may be normally functioning.

Other aplasias: Mondini aplasia/scheibe aplasia / Alexandar aplasiA.

Nail Patella Syndrome

An autosomal dominant trait'l.

Iliac horns develop on the pelvis

Characterised by: multiple osseus abnormalities primarily affecting the elbows knees and nail.

50% patients have clinically evident nephropathy.

It is associated with neural- sensory hearing impairment and GlaucomA. . .. Harrison 17~h/ed p 1794

Physiology of hearing and hearing loss Flashcard List

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