Case report: A rare cause of recurrent hearing loss as differential diagnosis

Introduction Case report of an 77 yrs. old male patient with recurrent unilateral hearing loss of the right ear throughout the years. Immediate treatment with intravenous steroid therapy did not lead to hearing improvement.

Methods Ear microscopy without pathological findings. Pure tone audiometry with moderate, combined hearing loss primarily in high frequencies. Radiological evaluation with MRI scan showing unclear hyperintense tumor mass and CT scan showing bone destruction of the posterior aspect of the right petrous ridge. The inner ear was not affected. Biopsy was obtained via explorative mastoidectomy. Findings: Characteristcal histologial features showed a low-grade adenocarcinoma with petrous bone infiltration as seen in endolymphatic sac tumors (ELST). Radiological staging scans did not show any metastases. After complete tumor resection (R0) and periodical follow ups including clinical examination and MRI scan the patient has been remaining disease-free for 6 yrs. at present. Association with von-Hippel-Lindau (VHL) disease was genetically excluded.

Conclusion ELST is a rare, slow growing and locally destructive disease. Association with von-Hippel-Lindau (VHL) disease may occure. Complete operative excision is the therapy of choice. Periodical follow ups including clinical examination and MRI scan are required due to potential risks of tumor recurrence. ELST should be taken into account as a a differential diagnosis in case of vestibulo-cochlear disorders.

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Publication History

Article published online:
07 August 2020