Collection of ganglionic tissue within the Temporal Bone in close relation with the jugular bulb.

It is a Non Chromaffin paraganglioma

The slow growth of this tumor means that the diagnosis is often missed until the tumor is very massive.

The commonest presentation is 

Pulsatile tinnitus

Hearing loss

A red mass behind the tympanic membrane (the rising sun sign) is not uncommon.

Quite a high number of patients present with cranial nerve palsies. Otalgia and Aural bleed are other fairly common symptoms.

Classification

Type A - Tumor localised to the middle ear cleft glomus tympanicus tumor.

Type B - Tympano mastoid tumors with no destruction of bone in the infralabyrinthine.

               Compartment of the temporal bone.

Type C -  Tumor invading the infralabyrinthine region and extending towards the petrous epex with desctruction of the infralabyrinthine portion of the temporal bone.

Type D -  Tumors with intracranial extension.

Investiogation

Radiological assessment of rising sun behind the drum 

                                                                                    Axial CT Scan

                            Jugular Fossa Enlarged                                                                Normal Jugular Fossa

     Cortex  eroded                        Cortex Normal                                                         Coronal CT Scan

     Glomus Jugulare                      High Jugular Bulb             Normal Carotid canal                      Laterally Placed carotid canal

                                                                                                      Glomus tymapnicus                          Aberrant Carotid artery

Prior to surgery it is very important to exclude the secretion  of vaso active hormones by the tumor particularly with patients of high blood pressure.

A 24 hr urine collection will demonstrate any increased Vanillyl Mandelic Acid level Direct biopsy should not be necessary if the mentioned radiological test are done.

Direct biopsy of tumor in EAC is done to exclude malignancy. In these cases bleeding will occur but not severe & it will always stop with a soframycine soaked gauze piece (Aural Pack)

Treatment

The current treatment options for glomus jugulare can be summarized as below

  1. No treatment
  2. Radiotherapy
  3. Surgical resection
  4. Surgical resection with planned adjunctive radiotherapy.