Behçet disease as a cause of hearing loss: A prospective, placebo-controlled study of 29 patients.

We conducted a prospective, placebo-controlled study to determine the incidence and severity of inner ear involvement and hearing loss in patients with Behçet disease. Our study population was made up of 29 patients with Behçet disease and 28 healthy controls. Audiometric pure-tone thresholds and transient evoked otoacoustic emission (TEOAE) levels were determined in both groups. The main outcome measures were pure-tone audiometry (PTA) levels and TEOAE levels in the two groups. PTA detected a sensorineural hearing loss in 10 of the 29 patients (34.5%). The difference in audiometric findings between the two groups was statistically significant at 1, 2, 4, and 8 kHz (p ≤ 0.0498). A comparison of TEOAE levels revealed that the difference in sound-to-noise ratio between the two groups was not significant at 1, 1.5, 2, and 3 kHz, but it was significant in 4 kHz (p = 0.02), and the difference in reproducibility between the two groups was significant at 2 and 4 kHz (p ≤ 0.03). We conclude that all patients with Behçet disease should be screened for hearing impairment and subsequently treated if an impairment is discovered.

Unilateral hearing loss as a presenting manifestation of granulocytic sarcoma (chloroma).

OBJECTIVE: To present a case of acute granulocytic sarcoma of the cerebellopontine angle whose presenting symptom was sudden onset unilateral sensorineural hearing loss. STUDY DESIGN: Case report and review of the literature (MEDLINE, 1962-2005). METHODS: A 34-year-old female patient with acute myeloid leukemia on remission admitted because of sudden hearing loss in her right ear for 10 days. She had experienced occasional tinnitus, ear fullness, and dizziness for a couple of months. After confirmation of her audiometric findings with auditory brainstem responses, the patient was put on a treatment regimen for sudden hearing loss. RESULTS: On the second day of treatment, she developed ipsilateral facial paralysis, hoarseness caused by ipsilateral vocal fold paralysis, and nystagmus. Magnetic resonance imaging of the cranium revealed findings consistent with granulocytic sarcoma at the cerebellopontine angle, infiltrating the internal acoustic canal. As increased intracranial pressure symptoms developed subsequently, subtotal tumor resection was performed. However, the patient was lost, with Cushing's triad at the second postoperative month during postoperative chemotherapy. CONCLUSION: Although up to 40% of leukemic patients may have otologic symptoms, sudden onset of sensorineural hearing loss is very rare. The patient presented in this report is the first reported case with a granulocytic sarcoma of the cerebellopontine angle who presented with acute sensorineural hearing loss. Despite the rarity of such a case, we would like to emphasize that leukemia must be kept in mind as an etiologic factor in sensorineural hearing loss and suggest that complete blood count and temporal bone imaging be routinely obtained.