The Relationship Between Sickle Cell Disease and Sudden Onset Sensorineural Deafness

Abstract

Sickle cell anemia (SCA) is a hereditary hemoglobin (Hb) disorder associated with a very specific molecular lesion, which is the exchange of glutamic acid for valine in the sixth residue of the Hb beta chain, originating the S Hb. It is characterized by intermittent episodes of vascular occlusion and end-organ damage. Progressive organ damage may affect any organ with brain, eyes, pulmonary, hepatobiliary, spleen, genitourinary, and the musculoskeletal systems being the most commonly involved and reported. Other complications of the disease that have not been well described or studied include cranio-orbital syndromes, oropharyngeal syndromes, periodontal disease, and otologic syndromes. Considering the vaso-occlusive nature of sickle cell disease (SCD), the potential for auditory damage is not unexpected. However, the incidence of subjective hearing impairment among SCA is very low and and little is known about the relationship between SCA and hearing loss. Here we report a 43-year-old female with SCA who presented with sudden bilateral hearing loss and generalized body ache and admitted as a case of sensorineural deafness with vascular crisis; she received IV fluid and analgesia and improved after five days from the therapy.

Puretone Hearing Threshold Of Patients With Sickle Cell Disease In Kaduna, Nigeria

“…14 Studies have documented that the younger age is at a higher risk of developing hearing loss because the microvasculature of the cochlea in young infants may be more susceptible to damage during a sickle cell crisis. 9,10 Our study found hearing loss in the older age group, in contrast to the higher risk of developing SNHL in children with SCD. 18 The average hearing threshold of patients with SCD in this study falls within the normal range but was consistently higher than that of the controls, similar to findings from other studies.…”

“…Much need to be known about the relationship between SCD and hearing impairment. 9,10 Histopathological degenerative changes in the cochlear are consistent with ischaemic injuries observed in SCD. 11 Several studies have documented the varying prevalence of hearing loss in patients with SCD ranging from 0.2%-66%.…”

The role of red blood cell exchange in sickle cell disease in patient with COVID‐19 infection and pulmonary infiltrates

“…Acute complications include acute chest syndrome (ACS), vaso‐occlusive crisis (VOC), hepatobiliary complications, stroke, splenic sequestration, priapism, acute anemia, and fever 8 . Chronic complications are pulmonary hypertension, hepatic iron overload, kidney disease, avascular necrosis, retinopathy, and legs ulcer 7,9‐12 . Among the acute complications, the most common are VOC and ACS.…”

“…8 Chronic complications are pulmonary hypertension, hepatic iron overload, kidney disease, avascular necrosis, retinopathy, and legs ulcer. 7,[9][10][11][12] Among the acute complications, the most common are VOC and ACS. VOCs are episodes of severe pain due to microvascular occlusion with erythrocytes and leukocytes, thus preventing blood flow and causing organ ischemia.…”

The role of red blood cells exchange in Sickle cell disease in a patient with COVID-19 infection and pulmonary infiltrates. (Case report and mini-review)

“…Acute complications include: Acute chest syndrome (ACS), Vaso-occlusive crisis (VOC), hepatobiliary complications, stroke, splenic sequestration, priapism, acute anemia, and fever [8]. Chronic complications are pulmonary hypertension, hepatic iron overload, kidney disease, avascular necrosis, retinopathy, and legs ulcer [7,[9][10][11][12]. Among the acute complications, the most common are VOC and ACS.…”