PP304 - Sudden Sensorineural Hearing Loss as Initial Sign of Acute Myeloid Leukemia

Abstract:
Acute Myeloid Leukemia (AML) is a rare type of cancer in which too many myeloblasts are found in the bone marrow and blood. This case study aims to outline a rare manifestation and initial symptom of Acute Myeloid Leukemia in a young adult who presented to our clinic with a recent history of bilateral sudden sensorineural hearing loss. The presenting history, audiologic test results, imaging results, and the pathophysiological mechanism of the hearing loss will be discussed.

Summary:
Acute Myeloid Leukemia (AML) is a rare cancer in which too many myeloblasts (a type of immature white blood cells) are found in the bone marrow and blood. AML often presents with non-specific symptoms such as weight loss, fatigue, fever, night sweats, headache, and loss of appetite. Mechanisms of hearing loss in patients with leukemia are likely leukemic infiltration, infection, and hemorrhage (Paparella et al., 1973). Leukemic infiltration within the temporal bones can cause bone deterioration resulting in tegmen defect and cerebrospinal fluid leak (Naraev, et al., 2006; Braca et al., 2013).

This case report describes a rare presentation of a patient with AML and severe to profound sudden hearing loss as a presenting symptom. A 25-year-old male presented to our facility’s Acute Cancer Care Center (ACCC) with a diagnosis of AML from a local ER two days prior. In the week leading up to his diagnosis, he noted progressively worsening bilateral aural fullness. In the 48 hours preceding his diagnosis, the patient experienced sudden hearing loss bilaterally along with headache, vertigo, nausea, and vomiting. At the ACCC, he also had fever and bilateral clear otorrhea. Air and bone conduction testing revealed severe to profound sensorineural hearing loss bilaterally. Additional testing could not be completed due to the patient’s condition.

He was examined by a neurotologist who noted persistent clear fluid draining from the ears, which continued to flow while suctioning out the external ear canal. Due to suspicions that this was probable cerebrospinal fluid otorrhea, a CT of the temporal bones was ordered. Imaging revealed bilateral tegmen defects with no labyrinthine hemorrhages and no internal auditory canal tumor. It was determined that the thinning or dehiscence in the tegmen mastoideum was a manifestation of his leukemia. This is likely a result of leukemic infiltration and leptomeningeal disease, a rare but serious complication of leukemia that occurs when the disease invades the central nervous system.

The patient began chemotherapy to treat his AML and was discharged from the hospital nearly two months after his admission. He returned to our clinic for repeat audiologic testing approximately one month later. Otoscopy at this time was unremarkable and tympanometry revealed normal middle ear function bilaterally. His audiogram showed moderate to moderately-severe sensorineural hearing loss in the right ear, and moderate to severe sensorineural hearing loss in the left ear. Word recognition was very poor bilaterally.

Treatment of hearing loss was through the use of hearing aids though a cochlear implant evaluation was recommended. He ultimately decided to defer the cochlear implant evaluation as he was in the process of receiving a stem cell transplant. The patient was fit with hearing aids and subjectively reported good benefit. He will continue to be monitored as there are several reports of hearing loss following stem cell transplants (Getson et al., 2020).

This case study aims to draw attention to the possible connection between sudden sensorineural hearing loss with CSF leak to acute hematological disease. Additionally, consideration should be made for audiologic monitoring in individuals with leukemia.