SENSORINEURAL HEARING LOSS

AMONG SICKLE CELL PATIENTS

Dr. Priyanka Agrawal

INTRODUCTION:
• Sickle cell disease (SCD) is an autosomal recessive genetic disorder that results from replacement of glutamic acid by
valine at position number 6 in the beta-globin chain of hemoglobin[1]
• There are several types of tests which are used to diagnose and monitor a sickle cell anemia (SCA) patient. Some of
these tests are sickle cell test, complete blood count, hemoglobin (Hb) electrophoresis.
• Normal red blood cells (RBCs) are pliable and oval in shape, which contributes to their smooth flow through vessels.
• In contrast, when red blood cells in patients with SCD are exposed to any stress like dehydration, deoxygenation, cold
temperature or blood infections, they become hard and sickle in shape. [2]
• This change significantly hinders their smooth flow through small vessels, which may lead to obstruction, ischemia
and end-organ hypoxia.
• This is manifested clinically in what is called vaso-occlusive crisis, which may affect different systems of the body.
One of the most vulnerable systems in the body to hypoxia is the nervous system, as it has poor tolerance to low
oxygen levels.
• SCD has diverse complications. One of these, which is related to the nervous system, is sensorineural hearing loss
(SNHL)[3]
• Sensorineural deafness can be due to cochlear ischemia and anoxia that happened due to vessel occlusion secondary to
the sickle RBC. The cochlea is highly sensitive to ischemia because of its anatomical position which is fed by a single
artery called the labyrinthine artery that can be terminal [4]
• Individuals suffering from SCD should be encouraged to have regular assessment of hearing function because of the
high sensitivity of cochlea to develop reversible ischemia, especially during a vaso-occlusive crisis. Early detection of
cochlear ischemia and immediate intervention will prevent permanent hearing disability. [5]
• Pure tone audiometry (PTA) is used to identify SNHL . It is a psychoacoustic test to determine the subject’s minimum
AIM:
• To estimate the prevelance of SNHL in patients with sickle cell disease
MATERIALS AND METHODOLOGY:

• PLACE OF STUDY- DEPT OF ENT AND HNS AND SICKLING INSTITUTE OF OUR COLLEGE

• PERIOD OF STUDY- JANUARY 2022 to JUNE 2022

• STUDY DESIGN- PROSPRECTIVE CASE CONTROL STUDY

• STUDY POPULATION- DIAGNOSED CASES OF SICKLE CELL ANEMIA AND MATCHED CONTROLS.

• STUDY TOOLS: PURE TONE AUDIOMETRY.

INCLUSION CRITERIA EXCLUSION CRITERIA

• Age 10-45 years • Congenital Hearing Loss
• Diagnosed case of SCD • Associated other comorbidities
• History of ototoxic medications use
• History of ear surgery
• History of meningitis
• History of noise exposure
• Positive history of ear discharge or
tympanic membrane perforation
• Sample size- 100
• Pure tone audiometry was done with the standard settings for all the subjects (50) and
controls[50 ]for the frequencies of 250Hz, 500Hz, 1000Hz, 2000Hz, 4000Hz and 8000Hz.
• The pure tone audiograms were categorized into three categories: normal, positive for
conductive hearing loss (CHL) or positive for SNHL.
• The degree of hearing loss was classified according to the pure tone average into mild (26-
40 decibels), moderate (41-55 decibels), severe (56-90 decibels) or profound (more than 90
decibels) hearing loss.
• A subject was considered to have hearing loss when the pure tone average (the average of
the thresholds on 500Hz, 1000Hz, 2000Hz) was more than 25 decibels for either the right or
the left ear.
OBSERVATION AND DISCUSSION
• 50 patients and 50 matching controls were enrolled in the study.
• The controls were matched for age, sex, free from sickle cell disease and other factors causing hearing
loss.
• The pure tone audiogram results from the patient group showed that 12 patients (24%) had hearing loss,
with a pure tone average between 25 and 40 decibels.
• Eight (16%) had bilateral hearing loss, and four patients (8%) had unilateral hearing loss (Table 1).
• The type of hearing loss was SNHL in all patients.
• Only 5 patients (10%) in the patient group had a positive complaint of hearing loss that was
described by the patient as being mild and nondisabling.
• In the control group, results were normal in all subjects (i.e. all subjects were free of hearing loss).
• There was no significant sex or side preponderance among patients with hearing loss.
Table 1. Results of pure tone audiograms in patients and controls

Group Number of Subjects with Bilateral hearing Unilateral Mean of pure Mean of pure
subjects hearing loss* loss hearing loss tone averages** tone averages**
(right side) (left side)

SCD Group 50 12 8 4 21.4 21.1

Control Group 50 0 0 0 15.4 15.2

SCD WITH H/L

33%

67%

B/L HL U/L HL
• The mean of pure tone averages for all subjects in the patient group was 21.4 on the right side and 21.1on the
left side.
• For the control group, it was 15.4 on the right side and 15.2 on the left side.
• The difference between controls and patients was statistically significant for both sides.

MEAN OF PTA
25

20

15

10

0
CASE CONTROL

RIGHT LEFT
• A comparison of means of the hearing thresholds on each side in each group showed statistically significant
differences in the means for all frequencies (Table 2).
Table 2. Comparison of the means of hearing thresholds.
control (n=50) patients (n=50) P value

Freq. (Hz) Right Left Right Left Right Left

250Hz 13.6 12.1 19.8 18.9 <.001 <.001

500Hz 14 13.8 21.3 20 <.001 <.001

1 K Hz 16 15.8 21.1 21.8 .005 .012

2 K Hz 16.4 16.2 22.1 21.6 .011 .007

4 K Hz 17.5 18.4 24.4 23.3 .006 .005

8 K Hz 18 18.5 24.3 22.9 .026 .013

HOSPITAL ADMISSION IN
Patients with hearing loss had a higher average number of hospital admissions/year in the 10 YEARS
last 10 years before the study
30%

70%

HEARING LOSS
WITHOUT HEARING LOSS
• Sickle cell disease is a significant clinical, social and economic burden in Western odisha.
• SCD has several complications that may affect different body systems.
• One of the underreported complications is SNHL.
• It is more likely to occur in older patients with SCD who have suffered more vaso-occlusive crises.[6,7]
• The hypothesized mechanisms of the SNHL in patients with SCD include impairment of oxygen supply
(secondary to deformed RBCs), which results in hypoxia to the cochlea, peripheral and central auditory
pathways, sluggish blood flow to the cochlea due to sickling of the red blood cells, and compression of the
auditory canal and auditory nerve by the hyperactive bone marrow in the petrous part of the temporal bone.
• Another possible mechanism of hearing loss in SCD patient is increased susceptibility to bacterial meningitis,
as the immune function of these patients is affected.[8]
• In this study, although only 5 patients (10%) complained clinically of hearing loss among adults with SCD,12
patients (24%) were diagnosed with hearing loss.
• Furthermore, the difference in the means of the hearing thresholds between the patients and the controls on
all frequencies was statistically significant.
• The average number of hospital admissions per year for the last 10 years in the patients was significantly
greater than the controls.
 CONCLUSION:
• Good control of the disease will reduce the incidence of this complication.
• We recommend that all patients with SCD undergo hearing tests annually, starting at an very early age.
• This will be very helpful as baseline data to prevent the progression of the hearing loss (e.g. to avoid other risk factors for hearing
loss, control of the general status of the disease) and to provide early management (e.g. hearing aids or cochlear implant).
• It is also important to avoid other risk factors for SNHL in SCD patients (e.g. noise exposure and the use of ototoxic medications).

REFERENCES:
• 1. Embury SH, Hebbel RP, Mohandas N, Steinberg MH (ed). Sickle cell disease: basic principles and clinical practice. New York: Raven Press; 1994. p.599-
621.
• 2. Weatherall DJ, Dc W, Weatherall J. The inherited diseases of hemoglobin are an emerging global health burden. Blood 2012: 115: 4331–4336.
• 3. Donegan JO, Lobel JS, Gluckman JL. Otolaryngologic manifestations of sickle cell disease. Am J Otolaryngol 1982: 3: 141–144.
• 4. Powars DR, Chan LS, Hiti A, Ramicone E, Johnson C. Outcome of sickle cell anemia: a 4-decade observational study of 1056 patients. Medicine
(Baltimore) 2005: 84: 363–376.
• 5. Saito N, Nadgir RN, Flower EN, Sakai O. Clinical and radiologic manifestations of sickle cell disease in the head and neck. Radiographics 2010: 30:
1021–1034.
• 6. Ajulo SO, Osiname AI, Myatt HM. Sensorineural hearing loss in sickle cell anaemia a United Kingdom study. J Laryngol Otol The Journal of Laryngology
& Otology. 1993;107(09).
• 7. Okeowo P, Akinsete I. Sensorineural hearing loss in homozygous sickle cell patients: The Nigerian experience. Ghana Med J. 1980;19:109-13.
• 8. Goel A, Sharma S, Aneja S. Sudden onset permanent deafness as an early complication of bacterial meningitis. Indian journal of pediatrics. 2014;81(6).
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