Hearing Loss, Tinnitus and Pineal Germinoma: Parinaud Dorsal Midbrain Syndrome Revisited

Abstract

Purpose: To highlight the association of deafness and tinnitus with pineal region tumors as first reported by Parinaud in 1886. Case Report: A 15 years old Indonesian boy developed increasing morning headaches over a several months period, ultimately associated with emesis and the development of blurred vision, bilateral tinnitus and decreased hearing acuity. He demonstrated failure of upward gaze and bilateral papilledema on initial examination. Brain and total spine MRI revealed a large posterior third ventricular mass without intracranial or spinal leptomeningeal spread. He underwent placement of a ventriculoperitoneal shunt in Singapore, where both serum and ventricular CSF AFP and HCGb were reportedly normal. A presumptive diagnosis of pineoblastoma was made. The family traveled to Los Angeles where follow- up MRI demonstrated a distinct spectroscopic profile with markedly elevated lipid peak, typical of germinoma. Sensori-neural hearing loss was confirmed bilaterally. He underwent a gross total resection of the tumor, histopathologically a pure germinoma. Serum and subsequent lumbar CSF tumor markers and CSF cytology were normal. His tinnitus disappeared completely post-operatively, and his hearing loss was documented to have returned to normal within one week. He was subsequently enrolled on the North American COG trial for localized CNS germinoma, receiving 4 cycles of carboplatin and etoposide chemotherapy followed by ventricular field (18Gy) irradiation with primary site boost to 30Gy total. He has since returned to Indonesia with continued follow-up and is thriving without recurrence now 12+ months from diagnosis. Conclusion(s): Hearing loss, a component of Parinaud's syndrome of central paralysis of eye muscles, has since been intermittently reported in conjunction with pineal region tumors, usually as a late onset manifestation. Due to downward displacement of the inferior colliculi through which most acoustic pathways traverse, this finding should alert clinicians to tumors in this location.