Introduction:
Cholesteatoma is the most common space occupying lesion of the middle ear. However,
the present case shows that the clear assessment of a neoplasm preoperatively can
be difficult, as the symptoms and the ENT examination can often be nonspecific.
Methods:
Representation of a case study
Results:
We report on a 38-year-old woman who presented with pressure in the ear and normal
hearing to our outpatient department. A spherical shaped mass was found behind the
intact eardrum of the right ear. The primary suspicion of a cholesteatoma could not
be confirmed intraoperatively. There was a smoothly bordered, yellowish mass reminding
morphologically of a facial nerve neuroma with directly derived EMG potentials of
the VII cranial nerve after stimulation. Therefore, only a tiny sample was taken for
histological examination, which resulted in the diagnosis of an adenoma. Following
in a second session, the tumor was removed, which seemed to derive cranially from
the oval window of the exposed facial nerve. Postoperatively, the facial nerve function
was intact. A conductive hearing loss resulted due to the necessary incus removal.
Histologically, a very low proliferative, highly differentiated neuroendocrine neoplasia
was detected. In future a second look surgical procedure with hearing restoration
and tumor control is planned.
Conclusions:
The adenoma of the middle ear, also referred to as middle ear carcinoma or neuroendocrine
adenoma of the middle ear, is a rare form of tumor that is predominantly classified
as benign, but can also metastasize and cause recurrences. The middle ear adenoma
must be differentiated from adenocarcinoma regarding its morphological, immunohistochemical
and neuroendocrine properties.
