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Pathology of Paget's Disease of the Temporal Bone

 Dr Sampurna Roy MD

 

                                                                                                                      

 

 

Syn: Osteitis deformans

The Paget disease is a chronic osteodystrophy of unknown etiology, and is characterized by abnormal bony resorption and deposition.

It affects 3% of the population over forty years of age and males more frequently than females. 

This anatomo-radiologic syndrome is sometimes accompanied by clinical symptoms. 

Paget disease may be monostotic or polyostotic but never generalized.

The disease principally affects the axial skeleton.  

The skull and temporal bones become involved in about two-thirds of the patients.

Progressive involvement of the temporal bones may lead to alteration of position, increase in size and change of architecture of the petrous pyramid, external canal, middle ear and inner ear capsule.

These changes in turn may produce impairment of hearing (about 30-50% of cases) and vestibular function (20-25%).

Cases of deafness due to Paget's disease can be separated into two types :

(1) deafness of a mainly mixed type in which progressive aggravation occurs particularly in the inner ear, and

(2) perceptional deafness which progresses without involvement of the transmission apparatus.

Temporal bone involvement in Paget disease can result in hearing loss by a variety of mechanisms. One such mechanism is compression of neural structures within the internal auditory canal.

The histopathology reveals Pagetic bone invading the internal auditory canal and compressing the cochlear division of the VIIIth cranial nerve resulting in severe neural degeneration.

There are no other histopathologic abnormalities present in the cochlea to explain the hearing loss.

Differential Diagnosis: Otosclerosis 

 

Further reading

Paget disease and sensorineural hearing loss associated with spiral ligament degeneration.

The mechanism of hearing loss in Paget's disease of bone.

Paget's disease and fibrous dysplasia.

A case of Paget's disease with progressive sensorineural hearing loss involved in the bilateral bony labyrinths

Hearing loss in Paget's disease of bone: the relationship between pure-tone thresholds and mineral density of the cochlear capsule.

In search of pathologic correlates for hearing loss and vertigo in Paget's disease. A clinical and histopathologic study of 26 temporal bones.

Pathology of the otic capsule.

Stabilization of hearing loss in Paget's disease with calcitonin and etidronate.

Hearing disorders in Paget's disease

Paget's disease and the temporal bone--a clinical and histopathological review of six temporal bones.

Temporal bone histopathology of Paget's disease with sensorineural hearing loss and narrowing of the internal auditory canal.

Tomography of petrous bone in deafness associated with Paget's disease.

Paget's disease and sensori-neural deafness: temporal bone histopathology of Paget's disease.

Deafness and Paget's disease

Paget's disease with temporal bone involvement, hypoacusis and vertigo. Apropos a case.

Paget's disease of the temporal bone.

Neurosensory complications of Paget's disease

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

 

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