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congenital anomaly

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congenital anomaly

Post by drspawar on Fri Nov 30, 2007 6:34 pm

commonest congenital anomaly of the inner ear producing sensorineural hearing loss is-

1) Michael type
2) Mondini type
3) Scheibe type
4) Alexander typ
e
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Re: congenital anomaly

Post by behappy on Fri Nov 30, 2007 6:54 pm

Cochleosaccular dysplasia was first described by Schiebe in 1892. It is characterized by a collapse of the cochlear duct and saccule. It is probably the most common form of inner ear pathology in patients with congenital deafness
The Alexander's ear deformity, or the cochlear base turn dysplasia, is related to familial high frequency sensorineural hearing loss.

The complete labyrinthine aplasia, known as Michel's deformity, represents an early failure in development correlating to the third week of gestation. It is extremely rare.

Incomplete partition deformity, also well known as Mondini's deformity, represents a small cochlea with incomplete or no intrascalar septa. The cochlea is usually flat and has one and one-half turns instead of the normal two and one-half turns. Arrest of maturation at the gestational seventh week may result in the Mondini deformity.

Answer is mostly Option 3
'Scheibe" type
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Re: congenital anomaly

Post by drspawar on Sun Dec 02, 2007 8:50 pm

few extra points-

Michael's type is the most severe form of congenital anomaly of inner ear.

Mondini type is the component of Pendred syndrome( b/l cong SNHL+ diffuse nodular goiter)
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Re: congenital anomaly

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