Hearing loss and blindness are seldom encountered as primary manifestations in patients with chronic myeloid leukemia. Chronic myeloid leukemia (CML) commonly presents with weakness, dysphagia, abdominal distension and pain and rarely constitutional symptoms. Chronic myeloid leukemia rarely presents with auditory or visual manifestations. Hyperleukocytosis at presentation can lead to obstruction of the small vessels of mainly brain, lungs and kidneys leading to organ dysfunctions. Headache, convulsions, stroke, papilledema, hearing loss, tinnitus and vertigo constitute the rare neurological spectrum of CML.
We present a case series of four patients who presented with bilateral sudden onset of hearing and visual loss. This report highlights the unusual presentation of chronic phase CML with visual and sensorineural hearing loss.
A detailed evauation of hisory, clinical examination, ENT & Eye examination was done in each patients. Related radiological evaluation was done as needed.
Chronic myeloid leukemia rarely presents with visual or hearing loss and till date, only 29 cases of CML with deafness have been reported. Neurological involvement including deafness, blindness and tinnitus was reported as 15.5% in a series of 33 adults with CML. Otological findings, for instance, sudden hearing loss, vertigo, tinnitus, facial weakness, and infection, were found in 16–40% of patients. However, deafness as an initial sign of this disease is very rare. Bilateral sudden sensorineural hearing loss, vertigo, and nausea were the presenting manifestations of CML in our patients. CML associated deafness presents itself as sensorineural, unilateral, bilateral or starting unilaterally and developing into bilateral. The pathogenesis of this clinical symptom in leukemia is very complex, and may include multiple mechanisms such as leukemic infiltration, hyperviscosity syndrome, inner ear hemorrhage, and infection. A relationship between leukemic infiltration of the cochlea and impairment of hearing in CML has also been mentioned in literature. Cochlear and retinal blastic infiltration may also cause sensorioneural deficit other than leukostasis in CML. Inner ear hemorrhage has been shown as a cause of sudden hearing loss in leukemia by some authors. Hyperleukocytosis is considered to be the foremost pathogenesis of CML with hearing loss, as suggested by recovery of hearing upon leukapheresis in some cases.
CML should be considered as one of the rare diagnostic possibilities in patients presenting with sudden onset of hearing and visual impairment and managed on an emergent basis as delay can result in permanent impairment.